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The development of clinically progressive fibrosis complicates a wide array of interstitial lung diseases (ILDs). However, there are limited data regarding its prevalence and prognosis.We analysed consecutive patients seen for initial evaluation of a fibrosing form of ILD (FILD). Patients were evaluated for evidence of progressive fibrosis over the first 24 months of follow-up. We defined a progressive phenotype as the presence of at least one of the following: a relative decline in forced vital capacity (FVC) of ≥10%; a relative decline in FVC of ≥5%-10% with a relative decline in diffusing capacity of the lung for carbon monoxide of ≥15%, increased fibrosis on HRCT or progressive symptoms.Eight hundred and forty-four patients (397 with idiopathic pulmonary fibrosis [IPF] and 447 non-IPF FILD) made up the final analysis cohort. Three hundred and fifty-five patients (42.1%) met the progressive phenotype criteria (59.4% of IPF patients and 26.6% of non-IPF FILD patients, p 0.01). In both IPF and non-IPF FILD, transplantation-free survival differed between patients with a progressive phenotype and those without (p 0.01). Multivariable analysis showed that a progressive phenotype was an independent predictor of transplantation-free survival (hazard ratio [HR]: 3.36, 95% CI: 2.68-4.23, p 0.01). Transplantation-free survival did not differ between non-IPF FILD with a progressive phenotype and IPF (HR: 1.12, 95% CI: 0.85-1.48, p = 0.42).Over one-fourth of non-IPF FILD patients develop a progressive phenotype compared to approximately 60% of IPF patients. The survival of non-IPF FILD patients with a progressive phenotype is similar to IPF.

Introduction Monoclonal antibody therapy has been reported to be highly effective for preventing hospitalisation and severe cases in patients with Coronavirus Disease 2019 (COVID-19). However, since the drug is not readily available, it is important to rapidly and appropriately identify high-risk patients who can benefit most from therapy. Therefore, we designed a risk scoring system to identify at-risk COVID-19 patients in our region during the largest surge of COVID-19, from July to September 2021. Methods According to the risk scores, confirmed COVID-19 patients were introduced to receive REGN-CoV-2 to our hospital by regional health centre from 18th August (Term 3). The primary outcome was the comparison of the number of hospitalisation and severe condition with other periods, the 4th wave (Term 1) and the early part of the 5th wave (Term 2) in Japan. Results During Term 3, 115 patients were stratified with the scoring system and administered REGN-COV-2. The number of hospitalisation vs severe cases were 60 (5.2%) vs 14 (1.2%), 8 (1.5%) vs 3 (0.6%) and 21 (1.2%) vs 2 (0.1%), in term 1, 2 and 3, respectively. Among those aged

The prognosis of patients with connective tissue disease (CTD) has improved significantly in recent years, but interstitial lung disease (ILD) associated with connective tissue disease (CTD-ILD) remains a refractory condition, which is a leading cause of mortality. Because it is an important prognostic factor, many observational and interventional studies have been conducted to date. However, CTD is a heterogeneous group of conditions, which makes the clinical course, treatment responses, and prognosis of CTD-ILD extremely diverse. To summarize the current understanding and unsolved questions, the Japanese Respiratory Society and the Japan College of Rheumatology collaborated to publish the world's first guide focusing on CTD-ILD, based on the evidence and expert consensus of pulmonologists and rheumatologists, along with radiologists, pathologists, and dermatologists. The task force members proposed a total of 27 items, including 7 for general topics, 9 for disease-specific topics, 3 for complications, 4 for pharmacologic treatments, and 4 for non-pharmacologic therapies, with teams of 2-4 authors and reviewers for each item to prepare a consensus statement based on a systematic literature review. Subsequently, public opinions were collected from members of both societies, and a critical review was conducted by external reviewers. Finally, the task force finalized the guide upon discussion and consensus generation. This guide is expected to contribute to the standardization of CTD-ILD medical care and is also useful as a tool for promoting future research by clarifying unresolved issues.

The clinical course of idiopathic pulmonary fibrosis (IPF) is characterized by a progressive decline in lung function; however, predicting changes in lung function is difficult. We sought to determine whether the prior 6-month trend in forced vital capacity (FVC) could predict mortality and the subsequent 6-month trend in FVC.We retrospectively analyzed consecutive patients with newly diagnosed IPF who underwent serial pulmonary function tests. The immediate two years after the initial evaluation were divided into four terms of six months each and stratified on the basis of presence or absence of a ≥10% relative decline in FVC at six months (declined and stable groups, respectively).We included 107 patients with %predicted FVC of 80.8% and %predicted diffusing capacity of the lung for carbon monoxide of 58.9%. In multivariate analysis, a decline in %predicted FVC in the initial six months was found to be an independent prognostic factor (hazard ratio 4.45, 95% confidence interval 2.62-7.56, p 0.01). Among the 46 terms in which the FVC declined during the initial 1.5-year study period, a decline in FVC was exhibited in 23 (50.0%) of the subsequent terms. Among 231 terms in which FVC remained stable, a decline was observed in 32 (13.9%) of the subsequent terms (relative risk 3.61, p 0.01). The frequency of FVC decline in each term was 16-27%. FVC was stable or declined in all four terms in 50.5% and 15.9% of cases, respectively.Six-month decline in FVC predicts subsequent FVC change and mortality in IPF patients in the era of antifibrotic agents.

Background In Japan, the severity staging system for idiopathic pulmonary fibrosis (IPF) has been used to determine medical care subsidies. However, this system requires invasive procedures to measure arterial oxygen tension. Recently, noninvasive and simple measurements of oxygen saturation by pulse oximetry (SpO2) have been used for severity assessments. We propose a pulse oximetry saturation (POS) staging system consisting of SpO2 parameters to predict prognosis. Methods We developed four prototype staging systems based on SpO2 at rest and desaturation, and adopted the system with the highest C-statistic as the POS staging system. The cutoff SpO2 values at rest were 96% and 90%, and desaturation was defined as SpO2 Results Two-hundred and nineteen IPF patients were studied and the C-statistic values of models 1, 2, 3, and 4 were 0.633, 0.643, 0.630, and 0.673, respectively. We judged model 4 to be a superior POS staging system; it defined SpO2 ≥ 96% at rest without desaturation as stage Ⅰ; SpO2 ≥ 96% at rest with desaturation or SpO2 90%–95% at rest without desaturation as stage Ⅱ; and SpO2 90%–95% at rest with desaturation or SpO2 Conclusion A noninvasive and simple POS staging system defined by SpO2 can easily predict prognosis.

Coronavirus disease 2019 (COVID-19) is a recently-emerged infectious disease that has caused millions of deaths, where comprehensive understanding of disease mechanisms is still unestablished. In particular, studies of gene expression dynamics and regulation landscape in COVID-19 infected individuals are limited. Here, we report on a thorough analysis of whole blood RNA-seq data from 465 genotyped samples from the Japan COVID-19 Task Force, including 359 severe and 106 non-severe COVID-19 cases. We discover 1169 putative causal expression quantitative trait loci (eQTLs) including 34 possible colocalizations with biobank fine-mapping results of hematopoietic traits in a Japanese population, 1549 putative causal splice QTLs (sQTLs; e.g. two independent sQTLs at TOR1AIP1), as well as biologically interpretable trans-eQTL examples (e.g., REST and STING1), all fine-mapped at single variant resolution. We perform differential gene expression analysis to elucidate 198 genes with increased expression in severe COVID-19 cases and enriched for innate immune-related functions. Finally, we evaluate the limited but non-zero effect of COVID-19 phenotype on eQTL discovery, and highlight the presence of COVID-19 severity-interaction eQTLs (ieQTLs; e.g., CLEC4C and MYBL2). Our study provides a comprehensive catalog of whole blood regulatory variants in Japanese, as well as a reference for transcriptional landscapes in response to COVID-19 infection., 「コロナ制圧タスクフォース」COVID-19患者由来の血液細胞における遺伝子発現の網羅的解析 --重症度に応じた遺伝子発現の変化には、ヒトゲノム配列の個人差が影響する--. 京都大学プレスリリース. 2022-08-23.

Diffuse alveolar damage (DAD) is known to be a pathological hallmark of acute respiratory distress syndrome or acute interstitial pneumonia, and to have a poor prognosis. We report a case of anti‐melanoma differentiation‐associated gene 5 (MDA5) antibody‐positive clinically amyopathic dermatomyositis (CADM) with rapidly progressive interstitial lung disease (RP‐ILD), in which DAD was confirmed by transbronchial lung cryobiopsy at an early stage without respiratory failure. Although this patient initially did not show respiratory failure, his respiratory condition gradually worsened despite intensive immunosuppression therapy and he died 3 months later. Therefore, the early pathological findings of DAD did not match the clinical picture, which showed no respiratory failure. However, these findings were consistent with the subsequent course and poor outcome. Histological DAD, even in the absence of respiratory failure, may indicate a subsequent poor prognosis and explain the refractory course of RP‐ILD with anti‐MDA5 antibody‐positive CADM., We report a case showing diffuse alveolar damage in a patient with rapidly progressive interstitial lung disease (RP‐ILD) with anti‐melanoma differentiation‐associated gene 5 antibody‐positive clinically amyopathic dermatomyositis, diagnosed by transbronchial lung cryobiopsy (TBLC) at an early stage without respiratory failure. Although this patient initially did not show respiratory failure, his respiratory condition gradually worsened despite intensive immunosuppression therapy and he died 3 months later. In patients with RP‐ILD, TBLC has the potential to predict prognosis and treatment response by evaluating the pathological pattern.

To elucidate the host genetic loci affecting severity of SARS-CoV-2 infection, or Coronavirus disease 2019 (COVID-19), is an emerging issue in the face of the current devastating pandemic. Here, we report a genome-wide association study (GWAS) of COVID-19 in a Japanese population led by the Japan COVID-19 Task Force, as one of the initial discovery GWAS studies performed on a non-European population. Enrolling a total of 2,393 cases and 3,289 controls, we not only replicated previously reported COVID-19 risk variants (e.g., LZTFL1, FOXP4, ABO, and IFNAR2), but also found a variant on 5p35 (rs60200309-A at DOCK2) that was significantly associated with severe COVID-19 in younger (-8 (odds ratio = 2.01, 95% confidence interval = 1.58-2.55). This risk allele was prevalent in East Asians, including Japanese (minor allele frequency [MAF] = 0.097), but rarely found in Europeans. Cross-population Mendelian randomization analysis made a causal inference of a number of complex human traits on COVID-19. In particular, obesity had a significant impact on severe COVID-19. The presence of the population-specific risk allele underscores the need of non-European studies of COVID-19 host genetics.

Background The recent recognition of the cicatricial organizing pneumonia (ciOP) indicates that the ciOP may resemble or simulate fibrotic interstitial pneumonia; however, there has been great uncertainty regarding the affected populations, pathogenesis, clinical relevance, and characteristics. In this study, we compared the characteristics of fibrotic interstitial pneumonia with and without ciOP. Method We enrolled 121 patients from the consultation archive whose pathological findings were fibrotic interstitial pneumonia and for whom follow-up clinical data were available. We reviewed these cases histopathologically and classified them according to whether or not they showed ciOP. We compared the clinicopathological features between the two groups. Result CiOP histopathologically characterized by deposition of dense collagenous fibers within the alveolar space without destruction of the lung structure was found in 48 patients (39.7%). None of the cases with ciOP experienced acute exacerbation during 12 months follow-up. The group with ciOP had more severe diffusion impairment but this, along with restrictive ventilatory impairment, improved significantly compared to the group without ciOP. Conclusion CiOP is a histopathological finding commonly found in fibrotic interstitial pneumonia. It does not relate to acute exacerbation or decrease in pulmonary function.

Background: We have sometimes observed interstitial pneumonia which had chronic course and unknown causes but not diagnosed as idiopathic pulmonary fibrosis (IPF). However, the treatment strategy was not established definitely. To clarify the usefulness of cyclosporin A (CsA) in idiopathic chronic fibrosing interstitial pneumonia (iCFIP) without IPF, we examined longitudinal changes in pulmonary physiology. Methods: Japanese patients with iCFIP without IPF treated with CsA were identified retrospectively. Linear mixed-effects models were used to evaluate changes in pulmonary physiology after adjusting for age, sex, and smoking history. Primary outcomes were longitudinal trajectories of the percent predicted forced vital capacity (%FVC), percent predicted diffusing capacity for carbon monoxide (%DLco), and composite physiologic index (CPI) before and after CsA. Results: Thirty-three patients were included. Before CsA initiation, %FVC, %DLco, and CPI declined at rates of 9.1%, 8.6% and −7.1 per 1 year, respectively. After CsA initiation, the gradient of %FVC showed significant improvements in 0–1 years (6.2%±3.0%; P

Background The Pfizer-BioNTech COVID-19 vaccine has recently received emergency approval from the US FDA. The mRNA technology was used to manufacture the Pfizer vaccine; however, as a pioneering technology that has never been used in the manufacture of vaccines, many people have concerns about the vaccine’s side effects. Thus, the current study aimed to track the short-term side effects of the vaccine. Methods The information in this study was gathered by a Google Form-questionnaire (online survey). The results included the responses of 455 individuals, all of whom are Saudi Arabia inhabitants. Adverse effects of the vaccine were reported after the first and the second doses. Results The most common symptoms were injection site pain, headaches, flu-like symptoms, fever, and tiredness. Less common side effects were a fast heartbeat, whole body aches, difficulty breathing, joint pain, chills, and drowsiness. Rare side effects include Bell’s palsy and lymph nodes swelling and tenderness. Flu-like symptoms were more common among those under 60 years of age, while injection site pain was more frequent among recipients who were 60 years and older. The study revealed a significant increase in the number of females who suffered from the vaccine side effects compared to males. Difficulty of breathing was more reported among recipients who had been previously infected with the coronavirus compared to those who had not been previously infected. Conclusion Most of the side effects reported in this study were consistent with Pfizer’s fact sheet for recipients and caregivers. Further studies are required to determine the long-term side effects.

Background and objective Interstitial lung disease (ILD) is a common pulmonary manifestation of systemic sclerosis (SSc). It is unknown whether radiographic fibrosis score predicts mortality in SSc-associated ILD (SSc-ILD). We retrospectively analysed patients with SSc-ILD to evaluate whether radiographic fibrosis score was a useful predictor of mortality. Methods We identified SSc-ILD patients evaluated at Kurashiki Central Hospital (Japan) from 2006 to 2016, and radiographic fibrosis scores based on the extent of reticulation and honeycombing on high-resolution computed tomography (HRCT) scanning were calculated by manually tracing around each fibrotic area. Independent predictors of overall survival were determined using the Cox proportional hazards model. Results The study included 48 patients, of whom 19 had usual interstitial pneumonia on HRCT. The median follow-up period was 56.6 months, and over the follow-up period 15 patients died. The 5-year survival was 72.4%. In the multivariate analysis, radiographic fibrosis score, age, being male and forced vital capacity were independently associated with an increased risk of death, while HRCT pattern was not. Conclusion A high radiographic fibrosis score was a poor prognostic factor in SSc-ILD. More widespread fibrosis was associated with an increased risk of death, independent of HRCT pattern.

Background Anti-synthetase syndrome (ASS) is characterized by the presence of anti-aminoacyl-tRNA synthetase antibody and ASS-associated interstitial lung disease (ILD) often recurs. The effectiveness of remission induction therapy with corticosteroids and calcineurin inhibitor (CNI) and the predictive factors for ASS-ILD recurrence were examined. Methods We retrospectively identified consecutive patients with ASS-ILD treated with corticosteroids and CNI during 2006–2017 and evaluated the predictive factors for recurrence using logistic regression analysis. Results Of the 57 patients included in this study, 54 (94.7%) exhibited improved response to remission induction therapy. There were 32 recurrence patients during maintenance therapy. The median period until recurrence was 27 months. There were no significant differences in the baseline characteristics between the recurrence and nonrecurrence groups. In the recurrence group, respiratory function and St. George's Respiratory Questionnaire score deteriorated over the clinical course. The Krebs von den Lungen-6 (KL-6) level changed with disease behavior. The multivariate analysis revealed that KL-6 increase rate from remission (odds ratio: 3.21, 95% CI: 1.17–8.86, p = 0.02) and CNI discontinuation (odds ratio: 8.09, 95% CI: 1.39–47.09, p = 0.02) were independent predictive factors for recurrence. The receiver operating characteristics analysis revealed that the optimal cut-off point of KL-6 increase rate was 2.0. The positive predictive values of the KL-6 increase rate from remission of >2.0 and CNI discontinuation were 90.0 and 88.9%, respectively. The CNI treatment duration and recurrence were not related. Conclusions Recurrence influenced long-term deterioration. KL-6 was a serum biomarker for disease behavior and recurrence prediction. The results suggest the importance of CNI continuation.

Background Chronic hypersensitivity pneumonitis (CHP) is a fibrotic interstitial lung disease (ILD) caused by repeated exposure to a variety of organic particles. In November 2017, new criteria for CHP diagnosis were proposed by Morisset et al. based on a modified Delphi survey of ILD experts. However, it remains unclear whether these criteria are useful to accurately diagnose CHP. We aimed to evaluate the newly proposed CHP diagnostic criteria. Methods We retrospectively applied Morisset's CHP diagnostic criteria to consecutive Japanese patients who underwent surgical lung biopsy for diagnosis of ILD from 2008 to 2015. All patients underwent bronchoalveolar lavage and pulmonary function testing. Patients who had connective tissue disease complications or showed an acute or subacute disease onset were excluded. Results A total of 251 patients were included. The diagnoses based on multidisciplinary discussion (MDD) were CHP (n = 27), idiopathic pulmonary fibrosis (n = 117), unclassifiable interstitial pneumonia (IP) (n = 65), and other diagnoses (n = 42). Of the 27 MDD-CHP patients, 14 were classified as a CHP group with diagnostic confidence >50% and 13 were not categorized (sensitivity, 51.9%; specificity, 77.7%). Morisset's CHP diagnostic criteria could help avoid SLB for the diagnosis of CHP in seven patients. Of the 13 MDD-CHP patients who were not categorized in the CHP group with diagnostic confidence >50%, the reason for the exclusion was an inconsistent with UIP pattern without CHP features. Conclusions Half of the MDD-CHP patients were diagnosed with CHP using Morisset's CHP diagnostic criteria. Further investigation will be important for developing improved diagnostic criteria for CHP.

Hypersensitivity pneumonitis (HP) is an interstitial lung disease (ILD) caused by exposure to a wide variety of organic particles. In this article, we report a rare case of a 44-year-old male patient with cough and shortness of breath for more than 4 years and exacerbation for 1 month. The findings of lung computed tomography (CT) and pathology supported a diagnosis of chronic HP. The patient had a history of ulcerative colitis for 5 years, and used to take salazosulfapyridine more than 4 years since 2013. We organized domestic and foreign experts to discuss the diagnosis and treatment of this case. The final clinical diagnosis was chronic interstitial pneumonia [related to sulfasalazine or inflammatory bowel disease (IBD) itself], pulmonary infections. It is recommended to discontinuation of sulfasalazine and oral administration of glucocorticoids and required long-term follow-up.

Background Most patients with acute eosinophilic pneumonia (AEP) show rapid improvement. However, some cases of AEP prove fatal. The aims of this study were to determine the clinical, radiographic, and pathologic characteristics of patients in whom AEP has a fatal outcome and to identify predictors of a poor prognosis. Methods We retrospectively identified the medical records of all patients diagnosed with AEP at our institution in Japan from July 2005 to July 2013. Results There were four deaths among 41 patients diagnosed to have AEP during the study period. All the patients who died were male; three cases were idiopathic and one was medication-related. The median bronchoalveolar lavage eosinophil differential count was 59%. An autopsy was performed on the patient with medication-related AEP who died and the pathologic finding was diffuse alveolar damage with eosinophilic infiltration. Diffuse ground-glass attenuation and traction bronchiectasis (TBE) were identified on high-resolution computed tomography in the four patients with fatal AEP. TBE was observed in six patients (five with idiopathic AEP, one with medication-related AEP), and 67% of these patients died. None of the patients with smoking-related AEP had TBE; all these patients had better responses to treatment and survived. Conclusions We observed the characteristics of patients with fatal AEP who did not respond to treatment. TBE was observed in all fatal cases and may be associated with a poor prognosis.

High-resolution computed tomography (HRCT) parenchymal patterns have been used to predict prognosis in patients with interstitial lung disease (ILD). In idiopathic pulmonary fibrosis, the fibrosis score (i.e. the combined extent of reticulation and honeycombing) has been associated with worse survival. This study aimed to identify HRCT patterns and patient characteristics that can predict poor prognosis in rheumatoid arthritis-related ILD (RA-ILD).We retrospectively analysed 65 patients with newly diagnosed RA-ILD from 2007 to 2016 at Kurashiki Central hospital. Using univariate and bivariate Cox regression analysis, associations with mortality, were identified.During a median follow-up of 56.5 months, 16/65 (24.6%) patients died. Univariate analysis identified six significant poor prognostic factors: lower baseline % predicted forced vital capacity, total interstitial disease score, reticulation score, traction bronchiectasis score, fibrosis score, and definite UIP pattern. Fibrosis score remained to be an independently significant poor prognostic factor of survival on bivariate analysis. Patients with a fibrosis score20% had higher mortality (HR, 9.019; 95% CI, 2.87-28.35; p .05).This study showed that fibrosis score is strongly associated with worse survival in RA-ILD, and patients with fibrosis score20% had a 9.019-fold increased risk of mortality.

Background To identify the prognostic factors for survival in patients with interstitial pneumonia with autoimmune features (IPAF) who meet the serological domain of the IPAF criteria. Methods We retrospectively analysed 99 IPAF patients who met the serological domain and were hospitalised at the Respiratory Medicine Unit of Kurashiki Central Hospital from 1999 to 2015. The high-resolution computed tomography findings were usual interstitial pneumonia (UIP; n = 1), non-specific interstitial pneumonia (NSIP; n = 63), NSIP with organizing pneumonia (OP) overlap (n = 15), and OP (n = 20). One patient who had radiological UIP pattern, and met the serological and clinical domains was excluded. The clinical characteristics, radiological findings, administered therapy, and prognosis of the remaining 98 IPAF patients who met the serological and morphological domains were analysed. Results The median age of the 98 IPAF patients was 68 years, and 41 (41.8%) of them were men. Twelve (12.2%) of the 98 IPAF patients developed other characteristics and were diagnosed with connective tissue disease (CTD) later during the median follow-up of 4.5 years. Univariate Cox analysis revealed systemic sclerosis (SSc)-specific and SSc-associated antibodies (ANA nucleolar pattern, ANA centromere pattern, anti-ribonucleoprotein and anti-Scl-70) positive IPAF, radiological NSIP pattern, bronchoalveolar lavage fluid lymphocytes >15%, and age as significant prognostic factors for survival. Multivariate Cox analysis revealed radiological NSIP pattern (hazard ratio [HR], 4.48; 95% confidence interval [CI], 1.28–15.77, p = 0.02) and age (HR, 1.07; 95% CI, 1.02–1.11, p = 0.01) were significantly associated with worse survival. Conclusions We confirmed that radiological NSIP pattern and age are poor prognostic factors for the survival of IPAF patients. This study suggested that the autoantibodies that are highly specific for certain connective tissue diseases might be less important for the prognosis of IPAF compared with the radiological-pathological patterns. The relatively high proportion of IPAF patients who developed CTD later suggests the importance of careful observation for evolution to CTD in IPAF.

Interstitial lung disease (ILD) is a common pulmonary manifestation of systemic sclerosis (SSc). It is unknown whether radiographic fibrosis score predicts mortality in SSc-associated ILD (SSc-ILD). We retrospectively analysed patients with SSc-ILD to evaluate whether radiographic fibrosis score was a useful predictor of mortality.We identified SSc-ILD patients evaluated at Kurashiki Central Hospital (Japan) from 2006 to 2016, and radiographic fibrosis scores based on the extent of reticulation and honeycombing on high-resolution computed tomography (HRCT) scanning were calculated by manually tracing around each fibrotic area. Independent predictors of overall survival were determined using the Cox proportional hazards model.The study included 48 patients, of whom 19 had usual interstitial pneumonia on HRCT. The median follow-up period was 56.6 months, and over the follow-up period 15 patients died. The 5-year survival was 72.4%. In the multivariate analysis, radiographic fibrosis score, age, being male and forced vital capacity were independently associated with an increased risk of death, while HRCT pattern was not.A high radiographic fibrosis score was a poor prognostic factor in SSc-ILD. More widespread fibrosis was associated with an increased risk of death, independent of HRCT pattern.

The receiver operating characteristic curve for the neutrophil differential count in BALF for predicting the OS in AE of CFIIP. (PPTX 78Â kb)

Background: Idiopathic chronic fibrosing interstitial pneumonia (CFIP) has been recognized to have a potential risk of acute exacerbation (AE). However, regarding the therapeutic response and prognosis of AE of CFIP, the usefulness of cellular analyses of bronchoalveolar lavage fluid (BALF) has never been analyzed. Objectives: This study aimed to evaluate the impact of lymphocyte differential count in BALF on the mortality of patients with AE of CFIP. Methods: Retrospective analysis of 37 patients who satisfied the criteria for AE of CFIP and underwent BAL on admission. The patients studied were divided into two groups: one group had a lymphocyte differential count >15%, the other had a lymphocyte differential count ≤15%. We compared the 30-day mortality between the two groups as the primary outcome, and the 90-day mortality and overall survival as the secondary outcomes, using two-tailed log-rank test. Results: Median age was 75 years (range, 72 to 78); thirty-one patients were men. Twenty-four patients had a lymphocyte differential count greater than 15% and 13 patients had counts equal or less than 15%. The 30-day mortality was significantly higher in the group with lymphocyte differential count ≤ 15% compared to the group with lymphocyte differential count > 15% (p=0.002). The 90-day mortality and overall survival were also worse in the group with lymphocyte differential count ≤ 15% compared to the group with lymphocyte differential count > 15% (p=0.003 and p Conclusions: The lymphocyte differential count in BALF may be associated with favorable outcomes in patients with AE of CFIP.