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Fahidah AlEnzi,1 Bashaer Alqahtani,2 Esam H Alhamad,3 Maha Daghestani,2 Yusra Tashkandy,4 Nashwa Othman,5 Khalid Alshahrani,6 Muthurajan P Paramasivam,3 Rabih Halwani,7 Mohammed A Omair8 1Department of Clinical Science, College of Medicine, Princess Nourah Bint Abdulrahman University, Riyadh, Saudi Arabia; 2Department of Zoology, College of Science, King Saud University, Riyadh, Saudi Arabia; 3Pulmonary Division, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia; 4Department of Statistics and Operations Research, College of Sciences, King Saud University, Riyadh, Saudi Arabia; 5Central Laboratory, King Saud University, Riyadh, Saudi Arabia; 6Rheumatology Division, Department of Medicine, Ad Diriyah Hospital, Ministry of Health, Riyadh, Saudi Arabia; 7Department of Clinical Sciences, Sharjah Institute for Medical Research (SIMR), College of Medicine, University of Sharjah, Sharjah, United Arab Emirates; 8Rheumatology Division, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi ArabiaCorrespondence: Fahidah AlEnzi Department of Clinical ScienceCollege of Medicine, Princess Nourah Bint Abdulrahman University, Riyadh, Saudi ArabiaEmail fmalenzi@pnu.edu.saBackground: Fatigue is a prevalent symptom affecting primary Sjögren’s syndrome (pSS) patients. The purpose of this study is to determine the prevalence of fatigue in Saudi pSS patients and its correlation with disease features and outcome measures using a validated tool.Methods: This is a cross-sectional study evaluating fatigue in pSS using the Arabic version of the fatigue severity scale (FSS). The EULAR Sjögren’s syndrome disease activity index (ESSDAI) and EULAR Sjögren’s syndrome patient reported index (ESSPRI) were calculated.Results: Forty-one patients met the sample criteria and were involved in the final report. There were predominantly females (78%) with a mean (±SD) age and disease duration of 58.76± 12.7 and 4.6± 2.28 years, respectively. Based on the FSS, 18 (43.9%) patients had a positive test with a mean score of 5.43± 0.76. The mean ESSDAI was 9.95± 7.73, while the mean EESPRI was 5.17± 2.4 with individual component scores were dryness (5.23± 2.62), fatigue (5.4± 2.88), and pain (4.88± 3.31). The FSS had a significant correlation with PGA (r=0.559; p< 0.001), PhGA (r=0.671; p< 0.001), ESSDAI (r=0.402; p=0.01), ESSPRI fatigue component (r=0.0.621; p< 0.001), ESSPRI pain component (r=0.558; p< 0.001), and missed significance for the ESSPRI dryness component (r=0.289; p=0.071). There was no correlation between the total ESSPRI score and presence of fatigue (r=− 0.261; p=0.104) nor the FSS score (r=− 0.136; p=0.409).Conclusion: Fatigue is prevalent in Saudi pSS patients. FSS correlated with ESSDAI and ESSPRI components but not its total score signaling other unmeasured factors contributing to fatigue development.Keywords: fatigue, Sjögren’s syndrome, FSS-Ar, ESSPRI

Feisal A Al-Kassimi, Esam H AlhamadDivision of Pulmonology, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi ArabiaAbstract: This review proposes a critical reassessment (based entirely on published evidence) of the following seven common beliefs about chronic obstructive pulmonary disease (COPD): (1) COPD is one disease. (2) There is a valid definition for COPD. (The current definition includes cases of irreversible asthma and bronchiectasis, and occasionally, other obstructive lung conditions). (3) Irreversible asthma in smokers and COPD cannot be differentiated. (4) A “chronic bronchitis” form of COPD exists and is characterized by blue bloater status and normal carbon monoxide diffusion studies. (5) Phenotyping has no bearing on medication choice in COPD. (6) Computerized scoring of lung attenuation on CT scans can diagnose emphysema. (Emphysema scores overlap in irreversible asthma and COPD); however, qualitative visual changes may be useful for differentiation. (7) A definable entity called the overlap (of COPD and asthma) syndrome exists. Conflict over the abovementioned points denies patients proper phenotype-guided therapy and encourages a multidrug approach to COPD management. The recently coined term, overlap syndrome, invites a double-barreled therapy aimed at asthma and COPD, despite the absence of any agreement about how to define the syndrome and the lack of any related drug trials (in the area of inhaled corticosteroids). A diagnosis of COPD is associated with high morbidity and escalating costs, suggesting the need for a thorough new examination of the evidence.Keywords: asthma, computerized tomography, COPD, global initiative for chronic obstructive lung disease, overlap syndrome

Background: Interstitial lung disease (ILD) is an increasingly recognized complication of rheumatoid arthritis (RA) and is associated with significant morbidity and mortality. Many risk factors for RA-related ILD were reported. The current study aims to explore the features and risk factors of Saudi patients with RA-ILD., Methods: This is a multicenter, retrospective, observational study of patients with RA-ILD. Clinical and radiological data from patients with RA-ILD were obtained from electronic medical records, including demographics, clinical characteristics, laboratory tests, pulmonary function tests, ECHO, and HRCT images., Result: Out of 732 patients, 57 had RA-ILD. The mean age at the time of ILD diagnosis was 61.9 (± 12.2) years. RA-ILD diagnosis was significantly less among females (p = 0.008). Patients who ever smoked had significantly more RA-ILD (p < 0.001). Patients with RA-ILD were more likely to present with medical comorbidities, namely diabetes (p < 0.001), hypertension (p < 0.001), ischemic heart disease (p < 0.001), and osteoarthritis (p = 0.030). The multivariate analysis revealed that the age (OR: 1.035, 95% CI: 48.45-52.86, p = 0.0001); gender (OR: 2.581, CI: 1.77-1.86, p = 0.001), DM (OR: 2.498, 95% Cl: 1.65-1.76, P = 0.0001), HTN (OR: 1.975, 95% Cl: 1.61-1.74, P = 0.019), IHD (OR: 6.043, 95% Cl: 1.89-1.93, P = 0.0001) have a significant positive association with RA-ILD. No significant differences were observed between seropositive parameters with or without RA-ILD (p > 0.05). The most common symptoms of RA-ILD were cough (55.6%) and dyspnea (30.2%), and the most common ILD pattern was Non-specific Interstitial Pneumonia (NSIP) (55.6%) followed by Usual Interstitial Pneumonia (UIP) (38.9%). Traction bronchiectasis (75.5%) and glass ground opacities (73.6%) were also observed. The mean FVC and DLCO at baseline were 64.6% and 53.3%, respectively., Conclusion: In this cohort of patients, Saudi RA-ILD patients had a predominant NSIP pattern conversely to what is seen globally. These findings could be explained by the lower rates of smoking in our patient population. Future prospective national studies are needed to confirm the current findings and better evaluate RA-ILD epidemiology and risk factors., (© 2024. The Author(s).)

Objectives: To determine the allelic frequencies and effects of genotypic variations in cytokine gene polymorphisms in a Saudi Arabian population., Methods: This cross-sectional study involved 41 patients with Primary Sjögren's syndrome (pSS) and 71 healthy controls between October 2018 and May 2019. Single nucleotide polymorphisms genotyping was performed using the SEQUENOM MassARRAY ® System, targeting nine polymorphisms in different cytokine genes. Chi-square tests were used to compare the patients and controls., Results: The interleukin-1 beta (IL-1β) rs1143627 CT (control, 52.7%; patients, 21.2%) and TT + CT ( p = 0.003; p =0.033) genotypes were less frequent in patients with pSS than in healthy controls. The C allele in rs10488631 in the interferon regulatory factor 5 (IRF5) gene and the A allele in rs12583006 in the B-cell activating factor (BAFF) gene were associated with an increased risk of pSS development in the patient group., Conclusion: The CT genotype at -31 (rs1143627) in the IL-1β gene was not associated with a high risk of pSS development in the Saudi population, in contrast to what has been verified in other ethnicities. However, the C allele in rs10488631 in IRF-5 and the A allele in rs12583006 in BAFF were associated., (Copyright: © Saudi Medical Journal.)

Pulmonary hypertension (PH) is a risk factor for mortality in patients with sarcoidosis. Severe PH in chronic lung disease has previously been defined as mean pulmonary arterial pressure (mPAP) ≥ 35 mmHg or mPAP 25 ≥ mmHg with cardiac index (CI) ≤ 2 L/min/m 2 . However, there is no clear definition denoting severity of sarcoidosis-associated PH (SAPH). We aimed to determine pulmonary hemodynamic cut-off values where transplant-free survival was worse among patients with SAPH. This was a retrospective cohort analysis of the Registry of SAPH database focusing on pulmonary hemodynamic predictors of transplant-free survival among patients with precapillary SAPH. Cox regression was performed to determine which pulmonary hemodynamic values predicted death or lung transplantation. Kaplan-Meier survival analysis was performed on statistically significant predictors to determine pulmonary hemodynamic cut-off values where transplant-free survival was decreased. Decreased transplant-free survival occurred among SAPH patients with mPAP ≥ 40 mmHg and SAPH patients with pulmonary vascular resistance (PVR) ≥ 5 Woods units (WU). Transplant-free survival was not decreased in patients who fulfilled prior criteria of severe PH in chronic lung disease. We identified new cut-offs with decreased transplant-free survival in the SAPH population. Neither cut-off of mPAP ≥ 40 mmHg nor PVR ≥ 5 WU has previously been shown to be associated with decreased transplant-free survival in SAPH. These values could suggest a new definition of severe SAPH. Our PVR findings are in line with the most recent European Society of Cardiology/European Respiratory Society guideline definition of severe PH in chronic lung disease., Competing Interests: The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: S. J. W. has received honoraria for lectures and advisory boards from Janssen (previously known as Actelion), Bayer, MSD and GSK. He has received travel grants from Janssen and GSK. He has received research grants from Bayer and Janssen. RPB has grant support from Gilead, Bayer, Actelion, Genentech, aTyr, Novartis, and Bellephron for studies in sarcoidosis. O. A. S. consults for and is a speaker for Bayer, United Therapeutics, and Janssen & Janssen. She also consults for Altavant. L. C. P. declares personal fees from Janssen Pharmaceuticals, outside the submitted work. SB has received consultancy fees from aTy and Kinevant. S. D. N. is a consultant for Boehringer‐Ingelheim, Roche, United Therapeutics, Bellerophon, Third Pole, and Merck. He is on the speakers bureau for Boehringer‐Ingelheim and United Therapeutics. R. G. has received consultancy fees from Mallinckrodt. This research did not receive any specific grant from funding agencies in the public, commercial, or not‐for‐profit sectors. The remaining author declare no conflict of interest., (© 2023 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.)

Background: Primary Sjögren's syndrome (pSS) is an autoimmune disease that can cause fatigue and extraglandular manifestations (EGMs). pSS is associated with cytokine network dysregulation, which may be related to the immune-mediated destruction of exocrine glands., Objective: We determined cytokine levels and their relationship to EGMs, the European League Against Rheumatism (EULAR) Sjögren's syndrome disease activity index (ESSDAI), and fatigue in Saudi patients with pSS., Methods: This study was a cross-sectional, single-center study. We included forty-one patients and 71 controls. Serum samples were collected from random healthy people and pSS patients who were followed in the rheumatology and pulmonary clinics of King Saud University Medical City in Riyadh, Saudi Arabia. Levels of the frequently studied cytokines were measured using Luminex xMAP technology. Each ESSDAI score and EGM were recorded, and the Arabic version of the fatigue severity scale (FSS) was applied to assess fatigue. The main outcome measures were cytokine levels in pSS Saudi patients using/not using immune-suppressive medications (ISMs)., Results: Thirty-six (87.8%) patients had one or more EGMs, and the mean ESSDAI score was 9.95 ± 7.73. There was a significant decrease in TNFα and IL-21 levels in the pSS group compared to those in the control group ( p = 0.034 and p < 0.001, respectively), whereas IL-12 levels were significantly elevated in the pSS group ( p = 0.002). Cytokine levels in patients who used ISMs were the same as those in patients who did not use medications. Decreased IL-1β ( p = 0.014), IL-2 ( p = 0.035), IL-6 ( p = 0.014), and IL-35 ( p = 0.010) levels were observed in patients who had EGMs. Patients who had low disease activity exhibited low IL-10 ( p = 0.018) and high IFN-α ( p = 0.049), IFN-β ( p = 0.049), IL-1β ( p = 0.006), and IL-35 ( p = 0.032) levels compared to patients with high disease activity. A negative association between a positive fatigue score and IL-1β ( p = 0.010), IL-2 ( p = 0.037), IFN-α ( p = 0.025), TNFα ( p = 0.030), IL-17 ( p = 0.029), IL-12 ( p = 0.046), and IL-21 ( p = 0.005) levels was found., Conclusions: Cytokine profiles correlate with EGMs, ESSDAI, and fatigue. Patients with controlled disease activity have a normal cytokine profile that is similar to that of controls.

Introduction: Sarcoidosis associated pulmonary hypertension (SAPH) is a leading contributor to sarcoidosis-related mortality. The 6-min walk test (6MWT) is widely used in assessment of cardiorespiratory conditions. A reduced 6-min walk distance (6MWD) has been associated with increased mortality in SAPH. We examined patients from the Registry of Sarcoidosis Associated Pulmonary Hypertension (ReSAPH) who had performed 6MWT at enrollment to identify variables that affect 6MWD, and the prognostic value of 6MWT variables regarding death or lung transplantation., Material and Methods: ReSAPH patients with available 6MWT were included. Variables analyzed using pre-defined cutoffs included 6MWD, initial and end of test Borg dyspnea score, oxygen saturation, and heart rate at beginning, end, and after 1-min recovery, absolute change in oxygen saturation, modified distance-saturation product (mDSP), and the heart rate recovery at 1-min (HRR)., Findings: 174 patients met inclusion criteria; 48 patients died and 8 underwent lung transplantation. Patients with 6MWD<300 m had a higher chance of dying or undergoing transplantation compared to those with 6MWD>300 m (p = 0.012). No associations with outcome were observed with mDSP cutoff 200 m%, desaturation≥5% and oxygen saturation<88% at end of 6MWT, or multiple HRR cutoffs (13,14,16). 6MWD correlated with initial Borg score, (p = 0.001), DLCO% (p = 0.0001) and sPAP (p = 0.031) on multivariate analysis. These variables were significant for both pre- and post-capillary PH subgroups. 6MWD also correlated with fatigue assessment scale (FAS) (p = 0.015)., Conclusion: Of the parameters evaluated, 6MWD had the greatest prognostic value in SAPH which correlated with other physiologic and hemodynamic variables. 6MWT captures the multidimensional effects of sarcoidosis., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Introduction: Echocardiographic measurement of the right ventricular systolic pressure (RVSP) is commonly used for estimating systolic pulmonary artery pressure (PASP) measured during right heart catheterization (RHC) in patients suspected for pulmonary hypertension (PH). Generally, there seems to be a strong correlation. However, this has been reported as less robust in sarcoidosis. We aim to investigate the correlation between RVSP and RHC measurements using real world data and analyzed factors influencing the relationship between RVSP and PASP in sarcoidosis., Methods & Results: Data of patients with and without sarcoidosis associated PH who had both a measurable echocardiographic RVSP and invasive PASP were collected from the RESAPH registry, PULSAR study and Cincinnati Sarcoidosis Clinic database (n=173, 60.1% female, mean age 56.0±9.5 years). Among them, 124 had PH confirmed by RHC. There was a strong correlation between RVSP and PASP (r=0.640). This correlation was significant in both male and female, white or non-white, forced vital capacity (FVC) >60%, and presence of fibrosis (p<0.001). However, it was less robust in patients with FVC of 50% or less. RVSP was considered inaccurate if the difference with PASP was > 10mmHg. Inaccurate echocardiographic estimation of the invasive PASP occurred in 50.8%, with overestimation mostly in patients without PH, and underestimation in patients with severe PH. An RVSP>50mmHg was associated with worse survival., Conclusions: In this real world multicenter cohort of sarcoidosis patients, we found a significant correlation between RVSP as determined by echocardiography and invasive PASP. Over- or underestimation of PASP occurred frequently. Therefore, echocardiographic RVSP measurement alone to screen for PH in sarcoidosis should be used with caution., (Copyright: © 2021 SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES.)

Background: Information regarding acute exacerbation (AE) in patients with interstitial lung disease (ILD) is limited., Objectives: The objective of the study was to elucidate the clinical features and outcome of AE among ILD patients., Methods: We retrospectively analyzed the data of 667 consecutive ILD (nonidiopathic pulmonary fibrosis [IPF] ILD, n = 463; IPF, n = 204) patients. ILD patients meeting the 2016 definition of AE-IPF were identified. Information analyzed included pulmonary function tests, 6-min walk tests, and right heart catheterization data, among others. Cox regression models were used to identify independent predictors of survival., Results: AE was identified in non-IPF ILD ( n = 113) and IPF ( n = 74). Compared with AE-IPF patients, non-IPF ILD patients with AE were of younger age, predominantly women, and primarily nonsmokers (all, P < 0.0001). The estimated survival probabilities at 1, 3, and 5 years were 88%, 75%, and 70%, respectively, in the ILD without AE group; 80%, 57%, and 50%, respectively, in the non-IPF ILD with AE group; and 53%, 38%, and 28%, respectively, in the AE-IPF group ( P < 0.0001 by log-rank analysis). Age, body mass index, IPF diagnosis, AE, diffusion capacity of the lung for carbon monoxide <35% predicted, 6-min walk distance <300 meters, and cardiac index were independent predictors of survival in the ILD cohort., Conclusions: Non-IPF ILD patients with AE have distinct clinical features compared to AE-IPF patients. Importantly, AE is one of many independent risk factors associated with worsened outcomes regardless of the underlying ILD type., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Annals of Thoracic Medicine.)

Background: Diagnosing primary Sjogren's syndrome (pSS)-associated interstitial lung disease (ILD) is complex and can be very challenging. In addition, information about the prognostic factors is limited., Aims: We aimed to determine the clinical characteristics and prognostic factors that impact pSS-ILD survival., Methods: This retrospective review included 84 consecutive patients diagnosed with pSS-ILD. The information analyzed included the clinical characteristics, laboratory findings, and physiological and hemodynamic data. Prognostic factors were identified using a Cox proportional hazards regression model., Results: The mean age was 60.5 years, and 61.9% were females. The mean time between the onset of symptoms and diagnosis was 21 months (range, 1-98 months). Minor salivary gland biopsy (MSGB) was positive for pSS in 92.3% of the cohort. Fifty percent of the patients had negative autoimmune serology related to pSS. Based on the available hemodynamic data, 40% had pulmonary hypertension (PH), and 20% had severe PH. During follow-up, acute exacerbation was noted in 38% of the cohort. The 5-year survival rate for all patients was 56%. Male sex, usual interstitial pneumonia pattern, and a reduced forced vital capacity were independent predictors of mortality in the pSS-ILD patients., Conclusions: A significant delay between the onset of symptoms and diagnosis was noted in our cohort. Importantly, our study highlights the importance of MSGB and emphasizes that clinicians should not rely solely on serological tests to diagnose pSS in ILD patients. The overall survival was poor, and more efforts are needed to diagnose pSS-ILD at an early stage and refer patients to experienced centers., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Annals of Thoracic Medicine.)

Objectives: To describe primary Sjögren's syndrome (pSS) cohort in Saudi Arabiain view in of clinical/serological/histopathological phentotype, and, diagnostic delay., Methods: A cross-sectional study conducted between October 2018 and May 2019. Diagnostic delay was calculated from symptoms onset to clinical diagnosis. The European League Against Rheumatism (EULAR) Sjögren's Syndrome Disease Activity Index (ESSDAI) and EULAR Sjogren's Syndrome Patient Reported Index (ESSPRI) were calculated., Results: Forty-one patients were included in the study. There were predominantly females (78%) with a mean (±SD) age of 58.76±12.7 and disease duration of 4.6±2.28 years. The mean diagnostic delay was 2.2±2.4 (range 1-11) years. Minor salivary gland biopsy was performed on 38 (92.7%) patients with a mean focus score of 2.3± 1.2 points. Interstitial lung disease and arthritis were the most common extra-glandular manifestations (EGM) affecting 27 (65.9%) patients for both. The mean ESSDAI was 9.95±7.73 and ESSPRI was 5.17±2.4., Conclusion: Saudi primary Sjogren's syndrome patients have a high prevalence of EGM predominantly arthritis and ILD. The diagnostic delay is variable in our cohort., (Copyright: © Saudi Medical Journal.)

Background: Pulmonary hypertension (PH) is a well-established complication in interstitial lung disease (ILD) patients. The aim of this study is to investigate the physiological and hemodynamic parameters that predict mortality in patients with ILD-PH., Methods: Consecutive ILD patients who underwent right heart catheterization ( n = 340) were included. The information analyzed included demographics and physiological and hemodynamic parameters. Cox regression models were used to identify independent predictors of survival., Results: In total, 96 patients had PH and an additional 56 patients had severe PH. The overall survival of idiopathic pulmonary fibrosis (IPF) patients with PH was significantly worse than the survival of patients with other types of ILD with PH ( p < 0.0001 by log-rank analysis). Patients with a reduced diffusing capacity of the lung for carbon monoxide (DLco) (<35% predicted), six-minute walk test final oxygen saturation by pulse oximetry (SpO 2 ) <88% and pulmonary vascular resistance ≥4.5 Wood units in the ILD-PH cohort had significantly worse survival. IPF diagnosis, forced vital capacity, DLco, systolic pulmonary artery pressure and cardiac index were identified as independent predictors of survival among the ILD-PH cohort., Conclusions: Patients with ILD-PH have poor prognosis. Physiological and hemodynamic parameters were important factors independently associated with outcome.

Background: Significant overlap may occur between idiopathic interstitial pneumonia (IIP) and connective tissue diseases (CTDs) that do not meet the established classification criteria for any known CTDs (i.e., occult CTD). Performing minor salivary gland biopsy (MSGB) to detect occult primary Sjogren's syndrome (pSS) in IIP patients is not well studied., Methods: Consecutive IIP patients underwent MSGB to determine the prevalence of positive MSGB findings. Furthermore, we characterised the clinical, physiological and serological profiles of the MSGB-positive patients. Cox regression models were used to identify independent predictors of survival., Results: The data of 155 patients with IIP were available for analysis. Sixty patients (38.7%) had positive MSGB findings. Of them, the mean age was 63.3 years, 51.6% were women, usual interstitial pneumonia (UIP) was the predominant pattern (63.3%), and seronegative antibodies (61.6%) were likely. Patients with positive MSGB findings had significantly greater survival than those with negative MSGB findings (p = 0.041). After stratifying the MSGB cohort based on the presence of a UIP pattern, no significant difference in survival was noted between those with positive MSGB-UIP pattern and those with a negative MSGB-UIP pattern (p = 0.231). Multivariate analysis on all UIP patients showed that higher forced vital capacity (p = 0.010) and smoking status (p = 0.035) were independently associated with survival., Conclusions: A substantial number of IIP patients had underlying occult CTD, highlighting the importance of performing MSGB to identify the salivary component of pSS when evaluating patients with interstitial lung disease of undetermined aetiology., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Background: There is a paucity of information regarding prognostic factors associated with reduced survival in interstitial lung disease (ILD) patients without pulmonary hypertension (PH)., Aims: The aim of this study was to determine physiological and hemodynamic parameters that impact survival among ILD patients without PH based on right heart catheterization (RHC)., Methods: Consecutive ILD patients who underwent RHC ( n = 169) at one center were included. The information analyzed included demographics and physiological and hemodynamic parameters. Cox regression models were used to identify independent predictors of survival., Results: The mean age was 55.0 years, and 49.7% of the patients were females. Thirty-three patients died, and two underwent transplantation. Patients with predicted diffusion capacity of the lung for carbon monoxide <35%, walking distance <300 m, and 6-min walk test (6MWT) final oxygen saturation measured by pulse oximetry (SpO 2 ) <85% were significantly associated with an increased mortality risk ( P = 0.022, P < 0.0001, and P = 0.049, respectively; all by log-rank analysis). Advanced age, idiopathic pulmonary fibrosis diagnosis, reduced forced vital capacity, and low cardiac index were independent predictors of increased mortality in the ILD cohort., Conclusions: Our study demonstrates that parameters obtained from baseline pulmonary function tests and 6MWTs are important determinants of survival in ILD patients without PH. Importantly, cardiac index was the only hemodynamic variable independently associated with survival. Thus, in the absence of PH, when ILD patients perform poorly during the 6MWT manifested as reduced walking distance and desaturation at the end of the test, cardiovascular impairment must be ruled out., Competing Interests: There are no conflicts of interest., (Copyright: © 2020 Annals of Thoracic Medicine.)

Background: Idiopathic pulmonary fibrosis (IPF) is a common subtype of interstitial lung disease (ILD). Information about the associated comorbidities and predictors of survival among Saudi patients with IPF is limited., Aims: The aim of the study was to determine the clinical characteristics, associated comorbidities, and prognostic factors that impact IPF survival., Methods: Consecutive IPF patients diagnosed in our ILD center were included. The information analyzed included demographics, physiological parameters, and associated comorbidities, among others. Cox regression models were used to identify independent predictors of survival., Results: The data of 212 patients with IPF were available for the analysis. The mean age was 66.4 years, and 70.8% were male. The mean time between the onset of symptoms and diagnosis was 11.6 months (range: 1-48 months). Common comorbid conditions noted in the IPF cohort included pulmonary hypertension (49.6%), diabetes mellitus (43.2%), hypertension (42.2%), osteoporosis (40.4%), and gastroesophageal reflux disease (32.1%). Acute exacerbation (AE) was noted in 21.2% of the IPF patients. AE, final saturation <85%, walking distance <300 m, and antifibrotic therapy were independent predictors of survival., Conclusions: In our IPF cohort, we found that there was a significant delay between the onset of symptoms and diagnosis. Moreover, we identified multiple comorbidities associated with IPF, which increases the burden on both IPF patients and clinicians. Importantly, AE and the use of antifibrotic therapy were independent predictors of survival. It is of paramount importance for clinicians to diagnose IPF at an early stage, refer patients to experienced centers, recognize comorbidities, and initiate antifibrotic therapy regardless of the underlying disease severity., Competing Interests: There are no conflicts of interest., (Copyright: © 2020 Annals of Thoracic Medicine.)

Introduction: Sarcoidosis-associated pulmonary hypertension (SAPH) is associated with reduced survival in single-centre studies. The international Registry for SAPH (ReSAPH) with long-term follow-up was established to enrich our knowledge of this complication of sarcoidosis. This analysis aims to elucidate factors associated with reduced transplant-free survival in SAPH patients., Methods: ReSAPH contains prospectively collected outcomes of SAPH patients since the time of registry enrolment. Information analysed includes right heart catheterisation data, pulmonary function testing, chest radiography, Scadding stage and 6-min walk distance (6MWD), among others. Cox regression models were used to identify independent predictors of transplant-free survival., Results: Data from 215 patients followed for a mean±sd 2.5±1.9 years were available for analysis. In the 159 precapillary patients, the Kaplan-Meier-adjusted 1-, 3- and 5-year transplant-free survival was 89.2%, 71.7% and 62.0%, respectively. Kaplan-Meier-adjusted 1-, 3- and 5-year transplant-free survival in the incident group was 83.5%, 70.3% and 58.3%, respectively, and in the prevalent group was 94.7%, 72.2% and 66.3%, respectively. Patients with reduced diffusing capacity of the lung for carbon monoxide ( D LCO ) (<35% predicted) and 6MWD <300 m in the precapillary cohort had significantly worse transplant-free survival. Reduced 6MWD and preserved forced expiratory volume (FEV 1 )/forced vital capacity (FVC) ratio were identified as independent risk factors for reduced transplant-free survival in the precapillary cohort., Conclusion: Reduced D LCO (<35% pred) and 6MWD (<300 m) at the time of registry enrolment were associated with reduced transplant-free survival in the overall precapillary cohort. Preserved FEV 1 /FVC ratio was identified as an independent risk factor for worsened outcomes., Competing Interests: Conflict of interest: O.A. Shlobin reports personal fees for consultancy from United Therapeutics, Bayer and Johnson & Johnson, outside the submitted work. Conflict of interest: V. Kouranos has nothing to disclose. Conflict of interest: S.D. Barnett has nothing to disclose. Conflict of interest: E.H. Alhamad has nothing to disclose. Conflict of interest: D.A. Culver reports institutional funding for the registry from Gilead, during the conduct of the study; personal fees for advisory board work from Johnson and Johnson, outside the submitted work. Conflict of interest: J. Barney has nothing to disclose. Conflict of interest: F.C. Cordova has nothing to disclose. Conflict of interest: E.M. Carmona reports financial support for the clinical trial from Gilead, during the conduct of the study; personal fees for consultancy and developing education materials from CHEST, personal fees for editorial work from Elsevier, outside the submitted work. Conflict of interest: M.B. Scholand has performed advisory board work and been investigator in clinical trials for Boehringer Ingelheim and Genentech, and been investigator in clinical trials for Fibrogen and Global Blood Therapeutics, outside the submitted work; and has a patent Apparatus, Compositions and Methods for Assessment of Chronic Obstructive Pulmonary Disease Progression among Rapid and Slow Decline Conditions issued. Conflict of interest: M. Wijsenbeek has nothing to disclose. Conflict of interest: S. Ganesh has nothing to disclose. Conflict of interest: E.E. Lower reports grants from Gilead, Bayer and Genentech, during the conduct of the study. Conflict of interest: P.J. Engel has nothing to disclose. Conflict of interest: J. Wort reports grants and personal fees from Actelion Pharmaceuticals and Bayer Pharmaceuticals, personal fees from MSD, outside the submitted work. Conflict of interest: L. Price reports personal fees and education support from Actelion and Johnson & Johnson, during the conduct of the study. Conflict of interest: A.U. Wells reports personal fees from Boehringer Ingelheim, Roche, Intermune and Bayer, outside the submitted work. Conflict of interest: S.D. Nathan reports personal fees for consultancy from United Therapeutics, Bayer Pharmaceuticals, Bellerophon and Actelion, during the conduct of the study. Conflict of interest: R.P. Baughman reports grants from Gilead, Bayer, Genentech, Foundation for Sarcoidosis Research and National Institutes of Health, grants and personal fees for consultancy from Actelion, grants and personal fees for consultancy and lectures from Mallinckrodt, during the conduct of the study., (Copyright ©ERS 2020.)

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNEC) is an abnormal proliferation of pulmonary neuroendocrine cells that occur without underlying etiology. Here, we report a unique case of 55-year-old female with unusual presentation of DIPNECH and thymoma and on the background history of Crohn's disease that might point toward an autoimmune phenomenon. To the best of our knowledge, there were no previous reports of DIPNECH with either thymoma or Crohn's disease. DIPNECH has premalignant potential and reported in association with carcinoid and non-small cell lung cancer; however, its autoimmune association never reported. The presence of multiple lung nodules along with evidence of small airway disease should alert the physician to include DIPNECH as part of the differential diagnosis given its malignant potential. The prognosis is variable and depends on the presence or absence of underlying malignancy as well as the severity of airflow obstruction., Competing Interests: There are no conflicts of interest.

Background: Pulmonary hypertension (PH) is a significant cause of morbidity and mortality in sarcoidosis. We established a multi-national registry of sarcoidosis associated PH (SAPH) patients., Methods: Sarcoidosis patients with PH confirmed by right heart catheterization (RHC) were studied. Patients with pulmonary artery wedge pressure (PAWP) of 15 mmHg or less and a mean pulmonary artery pressure (mPAP) ≥ 25 Hg were subsequently analyzed. Data collected included hemodynamics, forced vital capacity (FVC), diffusion capacity of carbon monoxide (DL CO ), chest x-ray, and 6-min walk distance (6MWD)., Results: A total of 176 patients were analyzed. This included 84 (48%) cases identified within a year of entry into the registry and 94 (53%) with moderate to severe PH. There was a significant correlation between DL CO percent predicted (% pred) andmPAP (Rho = -0.228, p = 0.0068) and pulmonary vascular resistance (PVR) (Rho = -0.362, p < 0.0001). PVR was significantly higher in stage 4 disease than in stage 0 or 1 disease (p < 0.05 for both comparisons). About two-thirds of the SAPH patients came from the United States (US). There was a significant difference in the rate of treatment between US (67.5%) versus non-US (86%) (Chi Square 11.26, p = 0.0008) sites., Conclusions: The clinical features of SAPH were similar across multiple centers in the US, Europe, and the Middle East. The severity of SAPH was related to reduced DLCO. There were treatment differences between the US and non-US centers., (Copyright © 2018. Published by Elsevier Ltd.)

Background: Asthma and chronic obstructive pulmonary disease (COPD) are chronic conditions with an increasing prevalence in developing countries. The evaluation of endobronchial biopsies has emerged as a tool to differentiate between both conditions via the measurement of the reticular basement membrane (RBM) thickness with various conclusions drawn from different studies., Objectives: Compare the thickness of the RBM between asthma and COPD and evaluate other histomorphological features in both groups., Design: Prospective, descriptive and analytical., Setting: University teaching hospital., Patients and Methods: The study included patients with COPD and irreversible and reversible asthma with diagnosis based on clinical assessment, pulmonary function tests and high-resolution computed tomography scans. Endobronchial biopsies were obtained from all patients and, using a light microscope and a computerized image analyzer, the thickness of the reticular basement membrane was calculated in all patients. We also made a qualitative assessment of other histo-morphological features., Main Outcome Measures: Mean RBM thickness., Sample Size: Thirty male patients., Results: The mean RBM thickness in asthmatic patients was 8.9 (2.4) micro m. The mean RBM thickness in COPD patients was 5.3 (1.1) micro m. However, there was no thickening of the RBM in patients with reversible asthma. The RBM was significantly thicker in patients with irreversible asthma than in patients with COPD or reversible asthma. There were no significant differences in epithelial desquamation or metaplasia, mucosal or submucosal inflammation, the presence of eosinophils, submucosal glandular hyperplasia or submucosal smooth muscle hyperplasia between groups., Conclusions: The thickness of the RBM is the only reproducible histopathological feature to differentiate COPD from irreversible asthma., Limitations: The study included a limited number of patients. A qualitative approach was used to compare epithelial cell injury, inflammation, submucosal glandular and muscular hyperplasia., Conflict of Interest: None.

Background: Osteoporosis, the most common extra-pulmonary complication of chronic obstructive pulmonary disease (COPD), may be related to general causes or COPD-specific causes such as low forced expiratory volume in 1 s (FEV1) and hypoxia. A few studies reported that emphysema is an independent risk factor for osteoporosis. However, other workers considered the association to be confounded by low FEV1 and low body mass index (BMI) which cluster with emphysema., Aims: To study the association between osteoporosis and emphysema in a model that includes these potentially confounding factors., Methods: We studied prospectively 52 COPD patients with both high resolution computed tomography and carbon monoxide diffusion coefficient as diagnostic markers of emphysema. Dual-energy X-ray absorptiometry was used to measure the bone mass density (BMD) of lumbar vertebrae and neck of the femur. Vertebral fractures were evaluated using the Genant semiquantitative score. Multiple linear regression analysis was used to identify the following independent variables: age, BMI, FEV1% predicted, PaO 2 , emphysema score, C-reactive protein (CRP), and dyspnea score as related to BMD. P ≤ 0.05 was considered statistically significant., Results: There was no significant difference in the serum Vitamin D levels, vertebral fracture score, or BMD between the emphysematous and nonemphysematous patients. Multivariate analysis showed that (in a model including age, BMI, FEV1, PaO 2 , emphysema score, CRP, and dyspnea score) only reduced BMI, FEV1, and PaO 2 were independent risk factors for low BMD., Conclusions: The emphysematous phenotype is not a risk factor for osteoporosis independently of BMI, FEV1, and PaO 2 ., Competing Interests: There are no conflicts of interest.

Background and Objective: Post-mortem and computed tomography (CT) studies indicated that emphysema is a feature of COPD even in the 'blue bloater/chronic bronchitis' type. We aim to test the hypothesis that the non-emphysematous patients are distinct from the main body of COPD and are more akin to asthmatic patients., Methods: We studied 54 patients with COPD. Emphysema was measured by Goddard's visual scoring of CT scan and the carbon monoxide transfer coefficient (KCO). Bronchial biopsy was offered for thickness of basement membrane (BM) (≥7 µm) as a marker of remodelling in irreversible asthma. Spirometry was repeated after therapy with Budesonide/Formoterol for 1 year., Results: The non-emphysematous phenotype were 24 of 54 patients (44%) by CT scan and 23 of 54 patients (43%) by KCO, showing agreement in 53 out of 54 patients. The non-emphysematous patients were younger, had higher forced expiratory volume in 1 s (FEV 1 ) (median 61% vs 49.7%), greater prevalence of hypertrophy of nasal turbinates and higher serum IgE. The emphysematous phenotype had lower BMI and greater dyspnoea score. The BM was thickened in 11 of 14 and 0 of 10 patients in the non-emphysematous and emphysematous groups, respectively. Three patients without emphysema and a normal BM normalized their FEV 1 upon receiving inhaled corticosteroid (ICS)/long-acting β2 agonist (LABA). All the non-emphysematous improved their FEV 1 after ICS/LABA (median = 215 mL). The median decline in the emphysematous was -65 mL., Conclusion: The non-emphysematous phenotype of COPD displays important features of asthma: clinical picture, histology and response to ICS. CT and KCO can predict spirometric response to ICS/LABA., (© 2016 Asian Pacific Society of Respirology.)

Background and Aims: Information regarding autoimmune symptoms that do not meet the diagnostic criteria for connective tissue disease in patients with idiopathic pulmonary fibrosis (IPF) is limited. The aim of the present study was to investigate differences in the clinical characteristics and prognosis of IPF patients with and without autoimmune symptoms., Methods: Consecutive patients diagnosed with IPF (N = 96) from January 2008 to December 2012 were included. We compared the clinical characteristics of patients with and without autoimmune symptoms. Survival was compared by log-rank and Cox proportional hazard analyses., Results: Thirty-six (38%) patients reported autoimmune symptoms. There were no significant differences in clinical characteristics between those with and without autoimmune symptoms. Patients with autoimmune symptoms had a better survival rate than those without symptoms [hazard ratio (HR) 0.27; 95% confidence interval (CI) 0.09-0.82; P = 0.020]. After adjusting for age, gender and smoking status, the presence of autoimmune symptoms was associated with improved survival (HR 0.29, 95% CI 0.09-0.89; P = 0.032). However, after adjusting for other covariates, including per cent predicted forced vital capacity and high-resolution computed tomography total extent score, the presence of autoimmune symptoms did not influence survival (HR 0.49, 95% CI 0.15-1.61; P = 0.240). The median follow-up period for the studied cohort was 31.5 months., Conclusion: It appears that autoimmune symptoms are associated with better prognosis among IPF patients. However, future studies are needed to validate our findings., (© 2014 John Wiley & Sons Ltd.)

Background: Current protocols for detection of circulating fibrocytes (CFs) in peripheral blood described in various pulmonary and nonpulmonary disorders involve complex and time consuming, non standardized techniques., Objective: Testing a method to rapidly detect and quantify CFs using whole blood lysis flow cytometry-based assay in patients with idiopathic pulmonary fibrosis (IPF) and healthy controls., Methods: One milliliter of venous blood sample in ethylenediaminetetraacetic acid (EDTA) from 33 IPF patients and 35 healthy control subjects was collected. Using whole blood lysis method peripheral blood leukocytes were labeled with monoclonal antibodies for cell surface (CD34 and CD45) and intracellular markers (collagen-1) for flow cytometric analysis. CFs were defined as CD45(+) cells coexpressing collagen-I and CD34 molecules., Results: In 29 (87.8%) IPF patients and 10 (28.5%) control subjects, a well-defined highly granular CD45(+) cell population was detected in dot plots generated by side scatter properties of CD45(+) cells. These CD45(+) cells were identified as CFs on the basis of coexpression of collagen-I and CD34; none of the other cell types in the peripheral blood were labeled with these monoclonal antibodies. In IPF patients the percentage of CFs was significantly higher compared to healthy controls (median (range): 1.37% (0.52-5.65) and 1.04% (0.1-1.84), respectively; P = 0.03)., Conclusions: Whole blood lysis method combined with fluorescence-activated cell sorting (FACS) allows detecting a well-defined homogeneous population of CFs. This method is simple, reproducible, and provides an accurate and rapid estimation of CFs.

Objective: To estimate the prevalence and associated risk factors of chronic obstructive pulmonary disease (COPD) in Saudi adults aged ⩾40 years using standardised post-bronchodilator spirometry according to the Burden of Obstructive Lung Disease (BOLD) protocol., Methods: Saudi men and women aged ⩾40 years were recruited by stratified multistage random sampling in Riyadh, Saudi Arabia. Participants completed questionnaires on respiratory symptoms and exposure to risk factors for COPD. Spirometry was performed according to standard methods. COPD was defined as post-bronchodilator FEV1/FVC (forced expiratory volume in 1 s/forced vital capacity) ratio <70% (Global Initiative for Obstructive Lung Disease [GOLD]) or less than the lower limit of normal (LLN) (95(th) percentile) of the population distribution for FEV1/FVC., Results: Seven hundred subjects underwent acceptable post-bronchodilator spirometry. The overall prevalence of GOLD COPD was 4.2% (men 5.7%, women 2.5%). The overall prevalence of COPD stage 1 or higher using the LLN was lower than estimates using the GOLD criteria (3.2%). The overall prevalence of GOLD stage 2 or higher COPD was 3.7%. Male sex, increasing age and smoking were significantly associated with COPD diagnosis., Conclusion: The overall prevalence of COPD in Saudi Arabia is 4.2%. Male, increasing age and smoking were the main risk factors for COPD.

Objective: To determine the prevalence of low bone mineral density (BMD) and the risk factors for osteoporosis in patients with interstitial lung disease (ILD)., Methods: Consecutive newly diagnosed ILD patients (n=196) were included in the study. Detailed demographic and clinical data were collected at the time of diagnosis, along with BMD measurements. Univariate and multivariate logistic regression analyses were used to determine the risk factors for osteoporosis., Results: Forty-four percent of the patients had osteoporosis, and 36% had osteopenia. The diagnosis of usual interstitial pneumonia (UIP) was more frequently observed in the patients with osteoporosis than in those without osteoporosis (67 vs. 47%, respectively; p=0.005). The univariate analyses identified the following five variables that were associated with an increased risk of osteoporosis at any site: older age (odds ratios [OR], 1.06; 95% confidence intervals [CI], 1.04 - 1.09; p<0.0001), UIP diagnosis (OR, 2.39; 95% CI, 1.33 - 4.30; p=0.003), higher partial pressure of carbon dioxide (PaCO2) (OR, 1.08; 95% CI, 1.01 - 1.14; p=0.01), hypertension (OR, 1.92; 95% CI, 1.05 - 3.49; p=0.033) and diabetes mellitus (OR, 2.38; 95% CI, 1.25 - 4.56; p=0.008). However, older age was the only independent predictor of osteoporosis (adjusted OR, 1.05; 95% CI, 1.02 to 1.08; p=0.001)., Conclusions: We observed a high prevalence of osteoporosis and osteopenia among newly diagnosed ILD patients. Our findings suggest that there is a substantially increased risk of osteoporosis and that early screening and aggressive treatment with various anti-bone resorptive therapies are necessary in ILD patients.

Background: Previous studies have reported conflicting survival rates for connective tissue disease (CTD)-associated usual interstitial pneumonia (UIP) and idiopathic pulmonary fibrosis (IPF/UIP). This study investigated the differences in the clinical characteristics and prognoses of patients diagnosed with CTD-UIP and IPF/UIP., Methods: A retrospective review of patients with IPF (n=88) and CTD-UIP (n=67) from January 2008 to June 2013 was completed. We compared the demographics and clinical characteristics between the two groups. Survival rates were compared using a log-rank and Cox proportional hazard regression analysis., Results: Undifferentiated-CTD (UCTD) accounted for 33% of the established CTD-UIP cases. No significant differences were identified in the demographic characteristics or physiological parameters between the UCTD and CTD patients (all P>0.05). However, the CTD-UIP patients were more likely to be young, female, and nonsmokers compared with the IPF/UIP group (all P<0.01). There was no difference in survival between the IPF/UIP and CTD-UIP patients [hazard ratio (HR), 1.74; 95% confidence interval (CI), 0.86-3.50; P=0.113]. However, the removal of the UCTD patients from the CTD group revealed that IPF/UIP was associated with a 2.47-fold increased risk of mortality compared with CTD-UIP (HR, 2.47; 95% CI, 1.01-6.09; P=0.049)., Conclusions: Our findings indicate that the survival of CTD-UIP patients was similar compared with that of IPF/UIP patients. However, it appears that UCTD influences the survival rate of CTD-UIP patients.

Background: Recent trials involving pirfenidone suggest a beneficial effect in the treatment of idiopathic pulmonary fibrosis (IPF)., Objective: To report on the efficacy and safety of pirfenidone in the treatment of patients with IPF, at a tertiary care hospital in Saudi Arabia., Methods: The study included 58 patients with IPF who were evaluated from March 2012 to March 2013. During the study period, 33 patients received pirfenidone, and the remaining patients (n = 25) served as a control group. Baseline clinical characteristics, physiological parameters and the results of a 36-Item Short Form Health Survey (SF-36) were compared between the groups. Furthermore, we compared changes in forced vital capacity (FVC), diffusion capacity of the lung for carbon monoxide (DLco), six-minute walk distance (6MWD) and SF-36 for both groups during follow-up. The last follow-up period ended in January 2014., Results: There were no significant differences in baseline clinical characteristics between the groups. Furthermore, we found no differences in FVC, DLco and SF-36 during follow-up (median, 12 months). However, patients receiving pirfenidone treatment were less likely to experience reductions in 6MWD compared with the control group (13% vs. 52%, respectively; P = 0.001). Although adverse events were more frequently reported by the pirfenidone group compared with the control group (85 vs. 56%, respectively; P = 0.015), these patients did not require discontinuation of treatment., Conclusion: Pirfenidone treatment preserves functional capacity, as reflected by the 6MWD. Adverse events associated with pirfenidone treatment were generally well tolerated by the patients.

Background: Endoscopic ultrasound-guided transbronchial fine-needle aspiration (EBUS-TFNA) is a minimally invasive technique for diagnosis of mediastinal masses/lesions. Although most studies have reported the utility of EBUS-TFNA in malignancy, its use has been extended to the benign conditions as well., Objective: The present study focused on utility of EBUS in contributing to reach the final diagnosis of sarcoidosis., Design: From May 2010 to December 2013, 19 of 80 patients who underwent EBUS-TFNA at one center for radiologically suspicious lesions for sarcoidosis, and with no definite histological diagnosis, were included in this retrospective study., Results: When the histological diagnosis was taken as the gold standard, the sensitivity of EBUS-TFNA was 84.2% and specificity 100% with the positive predictive value of 100. The combined diagnostic sensitivity of EBUS-TFNA and transbronchial lung biopsy was 100%., Conclusion: EBUS is a valuable, minimally invasive diagnostic modality to support the diagnosis of sarcoidosis in appropriate clinical setting, after conventional work-up - particularly if patients have suspicious radiological findings. This minimally invasive procedure helps in providing a final diagnosis without exposing the patient to the risk of complications from more invasive procedures.

Objective: The objective of this study is to evaluate the cytological accuracy of endobronchial ultrasound-guided transbronchial fine-needle aspiration (EBUS-TFNA) of the mediastinal mass/nodular lesions., Study Design: Over 3½ years from inception at King Khalid University Hospital, a retrospective analysis of the cytological diagnoses of all the EBUS-TFNA procedures performed in 80 patients who had mediastinal mass/nodular enlargement. Cytology results were reviewed and correlated with the histologic follow-up., Results: Of the 80 patients who underwent EBUS-TFNA, 15 cases (18.75%) were positive for malignancy, 48 cases (60%) negative for malignancy and 17 cases (21.25%) unsatisfactory. Of the 48 cases, which were negative for malignancy, 24 (50%) cases were of granulomatous inflammation. The overall diagnostic yield of our EBUS-TFNA specimen was 78.75%. Forty-seven cases (58.75%) of 80 cases had histological follow-up biopsies. Among them, 32 cases (68%) had the same cytological and histological diagnosis and 15 cases (31.09%) had discordance between the cytology and the follow-up histological diagnosis. The sensitivity, specificity, and positive and negative predictive values for diagnosing granulomas by EBUS-TFNA are 77%, 82%, 83%, and 75% and for diagnosing malignancy are 71%, 100%, 100%, and 82%, respectively., Conclusion: Preliminary results show that cytological samples obtained through EBUS-TFNA are accurate and specific in making a diagnosis of the mediastinal mass/nodular lesions. Its optimum use depends on the effective collaboration between the cytotechnologist, pathologist, and the bronchoscopist.

Background: Information regarding lung function parameters and functional capacity in renal failure and post renal transplantation patients with pulmonary hypertension (PH) is limited. The purpose of this study was to examine the clinical characteristics of patients with PH who were receiving hemodialysis (HD) or peritoneal dialysis (PD) or who had undergone renal transplantation., Methods: A prospective study was performed on 116 patients (HD =55, PD =17, and post renal transplantation =44) who underwent Doppler echocardiography. PH was defined as systolic pulmonary artery pressure (SPAP) ≥40 mmHg. Demographic information, clinical characteristics, pulmonary function tests (PFTs) and the six-minute walk test (6MWT) were collected and compared between the patients with and without PH., Results: Twelve (21.8%) patients receiving HD, four (23.5%) patients receiving PD, and eight (18.2%) post renal transplantation patients had PH. In the HD group, the physiological indicators (including pulmonary function test parameters, the final Borg score, and walking distance during the 6MWT) were all significantly lower in the patients with PH compared with those without PH (all P<0.0001). However, in the PD and post renal transplantation groups, no significant differences were noted in the demographic characteristics or in the physiological parameters when the PH patients were compared with those without PH (all P>0.05)., Conclusions: Among HD patients, marked aberrations in PFT results or walking distance may identify a subset of patients suffering from PH.

Objectives: To derive prediction equations of spirometric values of healthy Saudi adults and to compare the derived equations with equations reported in selected population., Methods: Cross-sectional study of healthy nonsmoking men and women Saudi adults. The measured spirometric values were the forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1 ), peak expiratory flow (PEF) and forced mid-expiratory flow (FEF 25%-75%)., Results: A total of 621 spirometric tests were done. The prediction equations were derived using the following formula: Predicted spirometric value = constant + (b1 × age) + (b2 × height (cm)), where b1 and b2 represent the regression coefficients for age and height, respectively. Variable Constant Age (years) Height (cm) Variable Constant Age (years) Height (cm) Males (n = 292) Females (n = 175) FVC -2.933 -0.018 0.046 FVC -3.470 -0.016 0.045 FEV1 -1.886 -0.019 0.036 FEV1 -2.482 -0.018 0.036 FEV1 /FVC (%) 98.41 -0.095 -0.068 FEV1 /FVC (%) 100.67 -0.142 -0.072 PEF 17.274 -1.243 3.471 PEF -226.648 -0.499 4.076 FEF25%-75% 0.100 -0.024 0.027 FEF25%-75% -1.337 -0.021 0.031 The means of the measured FVC and FEV1 were significantly lower than the predicted values derived by the American equations of -7.2% and -4.6% among males, respectively (P value < 0.00001), and -4.7%, and -5.26% among females, respectively (P value < 0.00001)., Conclusion: The reference spirometric values derived in our study were significantly lower than the predicted values derived by the American equations., (© 2013 John Wiley & Sons Ltd.)

Background: Studies have demonstrated associations between cytokine gene polymorphisms and the risk of idiopathic pulmonary fibrosis (IPF). We therefore examined polymorphisms in the genes encoding interleukin (IL)-6, IL-10, interferon gamma (IFN-γ), tumor necrosis factor alpha (TNF-α), and transforming growth factor-beta 1 (TGF-β1), and compared the serum levels of these cytokines in IPF patients and healthy controls. Furthermore, we examined the association of the studied genotypes and serum cytokine levels with physiological parameters and the extent of parenchymal involvement determined by high-resolution computed tomography (HRCT)., Methods: Sixty patients with IPF and 150 healthy controls were included. Cytokine genotyping was performed using the polymerase chain reaction sequence specific primer (PCR-SSP) method. In a subset of patients and controls, serum cytokine levels were determined by enzyme-linked immunosorbent assay., Results: There was no difference between IPF patients and controls in the genotype and allele distributions of polymorphisms in TNF-α, IFN-γ, IL-6, IL-10, and TGF-β1 (all p > 0.05). The TNF-α (-308) GG, IL-6 (-174) GG and CG, and IL-10 (-1082, -819, -592) ACC ATA genotypes were significantly associated with HRCT scores (all p < 0.05). IL-10 (-1082, -819, -592) ACC haplotype was associated with the diffusion capacity of the lung for carbon monoxide, and ATA haplotype was associated with the partial pressure of oxygen (PaO2) (all p < 0.05). The TGF-β1 (codons 10 and 25) TC GG, TC GC, CC GG and CC GC genotypes were significantly associated with the PaO2 and HRCT scores (p < 0.05). The TGF-β1 (codons 10 and 25) CC GG genotype (5 patients) was significantly associated with higher PaO2 value and less parenchymal involvement (i.e., a lower total extent score) compared to the other TGF-β1 genotypes (81.5 ± 11.8 mm Hg vs. 67.4 ± 11.1 mm Hg, p = 0.009 and 5.60 ± 1.3 vs. 8.51 ± 2.9, p = 0.037, respectively). Significant differences were noted between patients (n = 38) and controls (n = 36) in the serum levels of IL-6 and IL-10 (both, p < 0.0001), but not in the levels of TNF-α and TGF-β1 (both, p > 0.05)., Conclusion: The studied genotypes and alleles do not predispose to the development of IPF but appear to play an important role in disease severity. Our results suggest that the TGF-β1 (codons 10 and 25) CC GG genotype could be a useful genetic marker for identifying a subset of IPF patients with a favorable prognosis; however, validation in a larger sample is required.

Context: Several international studies have described the epidemiology of pulmonary hypertension (PH). However, information about the incidence and prevalence of PH in Saudi Arabia is unknown., Aims: To report cases of PH and compare the demographic and clinical characteristics of PH due to various causes in a Saudi population., Methods: Newly diagnosed cases of PH [defined as mean pulmonary artery pressure >25 mmHg at right heart cauterization (RHC)] were prospectively collected at a single tertiary care hospital from January 2009 and June 2012. Detailed demographic and clinical data were collected at the time of diagnosis, along with hemodynamic parameters., Results: Of the total 264 patients who underwent RHC, 112 were identified as having PH. The mean age at diagnosis was 55.8 ± 15.8 years, and there was a female preponderance of 72.3%. About 88 (78.6%) of the PH patients were native Saudis and 24 (21.4%) had other origins. Twelve PH patients (10.7%) were classified in group 1 (pulmonary arterial hypertension), 7 (6.2%) in group 2 (PH due to left heart disease), 73 (65.2%) in group 3 (PH due to lung disease), 4 (3.6%) in group 4 (chronic thromboembolic PH), and 16 (14.3%) in group 5 (PH due to multifactorial mechanisms). PH associated with diastolic dysfunction was noted in 28.6% of group 2 patients, 31.5% of group 3 patients, and 25% of group 5 patients., Conclusions: These results offer the first report of incident cases of PH across five groups in Saudi Arabia.

Studies have demonstrated associations between cytokine gene polymorphisms and ethnicity. In the present work the authors examine polymorphisms in the genes encoding interleukin-6 (IL-6), IL-10, interferon-gamma (IFNgamma), tumour necrosis factor-alpha (TNFalpha) and transforming growth factor-beta (TGFbeta1) using the polymerase chain reaction sequence-specific primer (PCR-SSP) method in 150 healthy unrelated Saudis, and results compared with those from other studied populations. The genotype distributions were consistent with the Hardy-Weinberg equilibrium, and the genotype frequencies observed among Saudis showed both similarity and difference to other populations. The most notable difference was in the distribution of IL-6, where the Saudi population showed a lower CG genotype frequency compared with White American (22% vs. 39.2%, P = 0.004), Italian (22% vs. 50%, P < 0.0001) and Brazilian (22% vs. 40.8%, P < 0.0001) populations. The study population also showed a higher frequency of the IL-6 GG genotype compared with White Americans (72% vs. 45.1%, P < 0.0001), Italians (72% vs. 41%, P < 0.0001) and Brazilians (72% vs. 49.3%, P < 0.0001). These results may have significant clinical relevance to the understanding of prevalent diseases in Saudi Arabia.

A survey of pulmonologists attending a clinical meeting of the Saudi Thoracic Society found that only 55% of responders considered that inhaled corticosteroids (ICS) had a positive effect on quality of life in Chronic Obstructive Pulmonary Disease (COPD). Why the divergence of opinion when all the guidelines have concluded that ICS improve quality of life and produce significant bronchodilation? ICS unequivocally reduce the rate of exacerbations by a modest 20%, but this does not extend to serious exacerbations requiring hospitalization. Bronchodilatation with ICS is now documented to be restricted to some phenotypes of COPD. Withdrawal of ICS trials reported a modest decline of FEV(1) (<5%) in half the studies and no decline in the other half. In spite of the guidelines statements, there is no concurrence on whether ICS improve the quality of life and there is no conclusive evidence that the combination of long-acting ß2 agonists (LABA) with ICS is superior to LABA alone in that regard. The explanation for these inconclusive results may be related to the fact that COPD consists of three different phenotypes with divergent responses to LABA and ICS. Therapy tailored to phenotype is the future for COPD.

Background: There are relatively few epidemiological studies on interstitial lung disease (ILD) worldwide., Objective: To report the incident cases of ILD and compare our data with reports from other populations., Methods: Newly diagnosed ILDs were prospectively collected at a single tertiary care hospital from January 2008 to December 2011. Detailed demographic and clinical data were collected at the time of diagnosis, along with the results from diagnostic procedures, including high-resolution computed tomography (HRCT), serological tests, bronchoalveolar lavage (BAL), transbronchial lung biopsy, endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) and surgical lung biopsy., Results: A total of 330 cases were included. The mean age was 55.4 ± 14.9 years. There was a slight predominance of females (202; 61.2%), and the male-to-female ratio was 1:1.37. The most frequent disease was connective tissue disease (CTD)-associated ILD (34.8%), followed by idiopathic pulmonary fibrosis (IPF) (23.3%), sarcoidosis (20%), and hypersensitivity pneumonitis (6.3%). Non-classifiable ILD was present in 1.8% of the total ILD cases. HRCT was performed in 97.3% of the cases, BAL in 17.5%, transbronchial lung biopsy in 21.8%, EBUS-TBNA in 4.5%, and surgical lung biopsy in 22.7% (38.6% of which were performed among the idiopathic interstitial pneumonia cases)., Conclusions: CTD-ILD and IPF were the most frequently observed ILDs in this Saudi Arabian population. Similarities and differences were found with respect to the previous reports from other countries.

Objective: To determine the clinical characteristics and outcomes of patients with lungdominant connective tissue disease (LD-CTD) with a usual interstitial pneumonia (UIP) who do not meet the criteria for any form of CTD, and to compare these parameters with those of patients with idiopathic pulmonary fibrosis (IPF/UIP) and CTD-associated-UIP., Methods: We conducted a prospective study on 118 patients diagnosed with UIP [LD-CTD, n = 28; CTDUIP, n = 29; and IPF/UIP, n = 61]. We compared the clinical characteristics, physiological findings, serum albumin concentrations, high-resolution computed tomography (HRCT) imaging data, and outcomes among the three groups and used Cox's proportional hazards regression analysis to identify variables associated with an increased risk of death., Results: The LD-CTD and CTD-UIP patients were younger, more often female, and predominantly nonsmokers, compared with the IPF/UIP group. A significant difference in survival was evident between patients in the CTD-UIP and IPF/UIP groups (p = 0.028), but not between LD-CTD and IPF/UIP (p = 0.164) or between LD-CTD and CTD-UIP (p = 0.254). The variables associated with poorer survival in all UIP patients were (reduced) initial SpO2 level (hazard ratio [HR], 2.89; 95% confidence interval [CI] 2.1-3.7; p = 0.009) and lower serum albumin concentration (HR 2.16; 95% CI 1.6-2.7; p = 0.008)., Conclusions: LD-CTD has distinct clinical characteristics that suggest an autoimmune background resembling that of CTD-UIP but differing from that of IPF/UIP. However, LD-CTD with a UIP pattern was not associated with improved survival. The resting oxygen saturation level and serum albumin concentration were independent predictors of mortality in all of the studied UIP patients, regardless of UIP type., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Background and Objective: Some studies show a decline of FEV(1) only one month after withdrawal of inhaled corticosteroids (ICS), while others show no decline. We speculate that the presence of an asthma phenotype in the Chronic Obstructive Pulmonary Disease (COPD) population, and that its exclusion may result in no spirometric deterioration., Methods: We performed a prospective clinical observation study on 32 patients who fulfilled the Global Initiative for Chronic Obstructive lung disease definition of COPD (Grade II-IV). They were divided into two phenotypic groups. 1. Irreversible asthma (A and B) (n = 13): A. Asthma: Bronchial biopsy shows diffuse thickening of basement membrane (≥ 6.6 μm). B. Airflow limitation (AFL) likely to be asthma: KCO > 80% predicted if the patient refused biopsy. 2. COPD (A and B) (n = 19): A. COPD: hypercapneic respiratory failure with raised bicarbonate, panlobular emphysema with multiple bullas, or bronchial biopsy showing squamous metaplasia and epithelial/subepithelial inflammation without thickening of the basement membrane. B. AFL likely to be COPD: KCO < 80% predicted., Results: The asthma phenotype was significantly younger, had a strong association with hypertrophy of nasal turbinates, and registered a significant improvement of FEV(1) (350 ml) vs a decline of - 26.5 ml in the COPD phenotype following therapy with budesonide/formoterol for one year. Withdrawal of budesonide for 4 weeks in the COPD phenotype resulted in FEV(1) + 1.33% (SD ± 5.71) and FVC + 1.24% (SD ± 5.32); a change of <12% in all patients., Conclusions: We recorded no spirometric deterioration after exclusion of the asthma phenotype from a COPD group.

In the last decade, significant progress has been made toward a better understanding of interstitial lung disease (ILD). A valuable tool for the clinician is high-resolution computed tomography, which aids in narrowing the differential diagnosis in patients with ILD and obviates the need for surgical lung biopsy when a usual interstitial pneumonia pattern is present. Clinicians evaluating and caring for patients with ILD need to recognize associated comorbidities. Substantial evidence shows that implementation of a multidisciplinary approach provides a high standard of care for patients, leading to improvements in the accuracy of clinical diagnosis that can significantly affect patient outcome., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Purpose: To study the reliability of pulmonary vascular measurements based on computed tomography (CT) in the prediction of pulmonary hypertension (PH) in patients with advanced interstitial lung disease (ILD) compared with those without ILD., Materials and Methods: The study was approved by the Institutional Review Board. All patients gave written informed consent. A prospective study of 134 patients who underwent right-sided heart catheterization and chest CT scanning within 72 hours of admission was conducted. Patients were divided into two groups-one with ILD (group A, n = 100) and one without ILD (group B, n = 34). CT measurements of the main pulmonary artery diameter (PAD), the ratio of PAD to the ascending aorta diameter (AAD), right pulmonary artery diameter (RPAD), and left pulmonary artery diameter (LPAD) were obtained. Univariate logistic regression analysis was performed, and receiver operating characteristic curves were constructed to assess the predictive ability of vascular measurements obtained by using CT in the identification of PH., Results: Main PAD was significantly greater in patients with PH than in those without PH in both groups (group A, P = .008; group B, P = .02). A PAD greater than 25 mm in patients with ILD was predictive of PH, with a sensitivity of 86.4% (32 of 37), a specificity of 41.2% (26 of 63), a positive predictive value of 46.3% (32 of 69), and a negative predictive value of 83.8% (26 of 31). In patients without ILD, a PAD greater than 31.6 mm and an LPAD greater than 21.4 mm were predictive of PH (sensitivity, 47.3% [nine of 19]; specificity, 93.3% [14 of 15]; positive predictive value, 90.0% [nine of 10]; and negative predictive value, 58.3% [14 of 24])., Conclusion: CT-derived vascular measurements were of limited utility in the prediction of PH in patients with ILD compared with those without ILD.

Background: Irreversible airways obstruction in smokers is usually attributed to chronic obstructive pulmonary disease (COPD). We speculate that some of these are cases of asthma indistinguishable from COPD., Objectives: To determine the prevalence of asthma in a 'COPD' population and how to differentiate the two conditions., Methods: This was a prospective observational study of smokers fulfilling the Global Initiative for Chronic Obstructive Lung Disease definition of COPD [mean post-salbutamol forced expiratory volume in 1 s (FEV1) 66.9% predicted]. They were classified into 4 groups, as follows: (1) inhaled corticosteroid (ICS)-responsive asthma, defined by normalization of spirometry upon ICS treatment; (2) irreversible asthma, defined as airway obstruction for 1 year and bronchial biopsy indicating asthma; (3) COPD, in the presence of bilateral panlobular emphysema with bullae on high-resolution computed tomography, hypercapneic respiratory failure or bronchial biopsy indicating COPD, and (4) unclassified airflow limitation (AFL)., Results: Eighty patients fulfilled the definition of COPD. The initial diagnosis was COPD in 57.5% and asthma in 42.5%. The final diagnosis was ICS-responsive asthma in 48 patients (60%), irreversible asthma in 8 (10%), COPD in 16 (20%) and unclassified AFL in 8 (10%). A normal transfer coefficient for carbon monoxide (KCO) and an FEV1 fluctuation ≥18% during 1 year of follow-up distinguished irreversible asthma and COPD. Seven of the 8 patients with irreversible asthma had improved FEV1 at the end of 1 year (median 320 ml compared with -29 ml in COPD). Five out of the 8 unclassified AFL cases had normal KCO and a large improvement in FEV(1) suggestive of irreversible asthma., Conclusions: COPD, even in heavy smokers, includes cases of asthma. FEV1 fluctuation during 1 year is a novel concept which may distinguish irreversible asthma and COPD., (Copyright © 2011 S. Karger AG, Basel.)

Background: We assessed the relationship between physiologic parameters, computed tomography patterns, 6 minute walk distance (6MWD) and the distance-saturation product [DSP; defined as the product of the 6MWD and the lowest oxygen saturation during the 6 minute walk test (6MWT)]. In addition, we investigated factors affecting 6MWD in patients with pulmonary sarcoidosis., Methods: We performed a retrospective study of patient demographics, treatment, pulmonary function, 6MWT, echocardiography and computed tomography results., Results: Fifty nine patients were included in this study. Their mean+standard deviation age was 47.5 years + 12.5 years, and 42 (71.2%) were female. Mean pulmonary function parameters for forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1) and total lung capacity (TLC) results, as percentages of predicted values, were 77.6 +/- 22.2, 77.1 +/- 22.8 and 78.7 +/- 16.1, respectively. Comparison of the DSP with distance walked revealed a significant correlation with factors underlying reduced 6MWD, including gender, pulmonary function indices, partial pressure of oxygen (PaO2), and Borg dyspnea score. Other factors were significantly associated with DSP but not distance; these included lung fibrosis (p = 0.02), pulmonary hypertension (p = 0.01) and systemic therapy (p = 0.04). Backward elimination stepwise multiple regression analysis revealed that gender, and FEV1 were independent predictors of 6MWD, but FEV1 was more strongly related when DSP applied [DSP, R2 = 0.53, p = 0.02; distance, R2 = 0.45, p < 0.0001]., Conclusion: Our findings reveal that, compared to 6MWD alone, the DSP is correlated with a greater number of factors associated with reduced 6MWT performance. Therefore, the DSP may be a useful indicator of functional status in patients with sarcoidosis. Additional large-scale studies are warranted to validate our findings.

Background: Pulmonary hypertension (PH) occurs in many patients with interstitial lung disease, including sarcoidosis. We explored the frequency, clinical characteristics and outcomes of PH in Arab patients diagnosed with pulmonary sarcoidosis., Methods: A retrospective study in three tertiary hospitals was performed on 96 patients who underwent Doppler echocardiography. Demographic and clinical characteristics, physiological studies and computed tomography (CT) results were collected, and compared between patients with and without PH., Results: Twenty (20.8%) patients were found to have PH. Patients with PH were more likely to be symptomatic (cough, P = 0.008; dyspnea, P = 0.04), to have an advanced radiographic stage (P = 0.001), and to be receiving systemic therapy (P = 0.001), compared to those without PH. Physiological data including pulmonary function test parameters, arterial blood gas levels and oxygen saturation at rest and after exercise were all significantly lower in patients with PH compared to those without PH. Comparison of CT patterns between patients with and without PH showed significant differences in the frequencies of ground-glass opacity (61.5 vs. 28.8%, P = 0.032) and fibrosis (76.9 vs. 44.2%, P = 0.035). In total, four patients died during the study period, including three with evidence of PH., Conclusions: The frequency of PH in the present study was 20.8%. Clinical, physiologic and radiographic characteristics appeared to differentiate patients with PH from those without PH. The presence of PH contributed to poor outcomes in patients with pulmonary sarcoidosis.

Introduction: Familial interstitial lung disease has been reported worldwide, mostly in Europe and North America; limited information is available on the disease among Arab patients., Case Presentation: A 45-year-old woman presented to our outpatient clinic with a 1-year history of progressive dyspnea. At the age of 37 years, based on clinical and radiological features, our patient was diagnosed with idiopathic pulmonary fibrosis. A family history showed that of five deceased siblings, four had died of disease of undetermined etiology. In addition, we screened other family members, and three were shown to have clinical, radiological, and pathological features consistent with interstitial pneumonia., Conclusion: Our report illustrates that younger age at presentation appears to be a common feature in patients with familial interstitial pneumonia. Otherwise, clinical, radiological, and histological features are indistinguishable from those of sporadic cases. Furthermore, our work highlights the importance of compiling a thorough family history in individuals presenting with cough and dyspnea, particularly in younger patients identified with idiopathic pulmonary fibrosis.

Background and Objective: Sarcoidosis is prevalent worldwide with significant heterogeneity across different ethnic groups. We aimed To describe the clinical characteristics and computed tomography findings among Arab patients with pulmonary sarcoidosis., Methods: A retrospective study of patient demographics, symptoms, co-morbid illness, sarcoidosis stage, treatment, pulmonary function and CT results., Results: Of 104 patients, most (77%) were 40 years of age or older at diagnosis, and females in this category (40 years ) significantly outnumbered male patients (69/104 (66.3%) vs. 35/104 (33.7%), P=.003). The most common complaints were dyspnea (76%), cough (72.1%) and weight loss (32.7%). The majority of patients displayed impairment in lung function parameters at presentation. However, significant impairment in forced vital capacity, percentage predicted (FVC%) (, Conclusion: At presentation, clinical manifestations of sarcoidosis among this sample of Arab patients were similar to reports from other nations. Further studies are needed to explore the effects of race and ethnicity on disease severity in the Middle East.

Background: The 6-min walk test (6MWT) is a useful tool to assess prognosis and functional impairment in various pulmonary diseases., Aims: To evaluate functional capacity during various stages of pulmonary sarcoidosis and develop a scoring system clinical radiological physiological score (CRP) that can potentially be used to assess the functional status among patients with sarcoidosis., Materials and Methods: We performed a retrospective study on 26 patients diagnosed with pulmonary sarcoidosis from 2001 to 2007. All patients completed the 6MWT. The parameters assessed during the test included spirometry, arterial blood gas, 6-min walk distance (6MWD), Borg dyspnea score, and initial and end oxygen saturation., Results: Females covered a significantly shorter distance than males (343 m (223-389) vs. 416.5 m (352-500); P < 0.0001). In addition, females had a significantly lower SpO₂ at the end of the 6MWT than males (90.5 (61-99) vs. 96 (75-98); P < 0.03). The 6MWD was inversely correlated with the final Borg score (ρ = -0.603, P = 0.004) and the CRP score (ρ = -0.364, P = 0.047) and positively correlated with forced expiratory volume in 1 s (FEV₁) % ( ρ = 0.524, P = 0.006) and forced vital capacity (FVC) % (ρ = 0.407, P = 0.039)., Conclusions: Female gender, FEV₁%, final Borg score, FVC%, CRP score, and SpO₂ at the end of the 6MWT are associated with reduced 6MWD. It appears that Saudi patients diagnosed with sarcoidosis have a markedly reduced walking distance compared with other races. The effect of race and ethnicity and the utility of the CRP score as a potential marker to assess functional status require further exploration.

Background: Baseline clinical and physiological variables have been described as relevant predictors of survival among patients with idiopathic pulmonary fibrosis (IPF). However, substantial heterogeneity in both survival time and mortality has been observed with many of these predictive factors. The incidence and mortality rates of IPF vary from country to country, with race potentially contributing to such variations., Objective: We sought to describe baseline clinical features to determine their predictive value among Middle Eastern patients diagnosed with IPF., Methods: We retrospectively examined 61 patients diagnosed with IPF at a university hospital in Riyadh, Saudi Arabia., Results: At presentation, most patients exhibited either dyspnea or cough. The median survival time for all patients was 92 months. Diminished survival was significantly associated with finger clubbing (P = 0.01). Factors not influencing survival were age, gender, percent predicted forced vital capacity, percent predicted forced expiratory volume in 1 s, percent predicted total lung capacity, percent predicted diffusion capacity of the lung for carbon monoxide and resting oxygen saturation., Conclusions: Finger clubbing is a significant predictive variable and was associated with a 5-fold increase in mortality. Other baseline demographic characteristics as well as pulmonary function tests were not predictive of prognosis in Middle Eastern patients with IPF. It appears that IPF patients of Middle Eastern descent have a longer median survival curve compared to other races.

Pulmonary function tests have been widely accepted and utilized in the management of interstitial lung diseases. Although the tests performed have changed little over the past several decades, extensive literature has been published highlighting their clinical role in the diagnosis, staging, prognostication, and follow-up of patients with a wide variety of interstitial lung diseases.