Search

Objectives: We applied the 2022 American College of Rheumatology/ European Alliance of Association for Rheumatology (ACR/EULAR) criteria for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) to patients histologically diagnosed with lupus nephritis (LN) to investigate the overall rate of and initial contributing factors to the reclassification of overlap syndrome of LN with AAV (OS-LN-AAV). Methods: We retrospectively reviewed the medical records of 1292 patients with systemic lupus erythematosus (SLE) and included 164 patients with LN in this study. Patient demographics, SLE manifestations, LN classes, and laboratory data, including ANCA levels, were recorded. All-cause mortality and end-stage kidney disease (ESKD) were evaluated as poor outcomes. Results: The median age of the 164 patients was 37.0 years, and 12.2% were men. The overall reclassification rate was 37.8%, of which 34.1% and 3.7% of the patients were reclassified as having OS-LN-microscopic polyangiitis and OS-LN-granulomatosis with polyangiitis (GPA), respectively, but none as having eosinophilic GPA. ANCA positivity and AAV-suggesting lung lesions were major contributors to OS-LN-AAV reclassification. When patients were compared based on OS-LN AAV reclassification, ANCA positivity and myeloperoxidase-ANCA (or P-ANCA) positivity favoured for OS-LN-AAV reclassification, whereas oral ulcers did not. However, OS-LN-AAV reclassification did not affect all-cause mortality or ESKD. Conclusions: This is the first study demonstrating a 37.8% reclassification rate in patients histologically diagnosed with LN using the 2022 ACR/EULAR criteria for AAV. Furthermore, it was also the first to reveal ANCA positivity and AAV-suggesting lung lesions as major contributors to OS-LN-AAV reclassification. [ABSTRACT FROM AUTHOR]

Background: There are established associations between viral and autoimmune encephalitis as well as between autoimmune encephalitis and demyelinating central nervous system (CNS) diseases. Here, we report the evolution from varicella zoster virus (VZV) encephalitis to limbic autoimmune encephalitis (AIE) to multiple sclerosis (MS) in one patient. Case report: A woman in her mid-thirties presented with headache, aphasia, and a generalized tonic–clonic seizure. Cerebrospinal fluid (CSF) VZV polymerase chain reaction was positive and treatment with acyclovir was administered for VZV encephalitis. Five months later, the patient presented with cognitive deficits and MRI showed new bilateral hippocampal T2-hyperintensities. CSF analyses revealed pleocytosis and neuropil antibodies in tissue-staining. A diagnosis of limbic AIE was established and treatment with IV steroids and IV immunoglobulins initiated. One year later, the patient developed paresthesia of both legs and magnetic resonance imaging studies now showed new supratentorial and spinal demyelinating lesions. The patient was diagnosed with MS and treatment was changed to rituximab. Conclusions: This unique case report links three important neuroimmunological entities in characterizing the evolution from infectious to autoimmune encephalitis to multiple sclerosis in one patient. Identification of such rare clinical constellations is critical for correct treatment choice and provides important novel insights into the pathophysiology of neuroimmunological disorders including viral triggers and overlap manifestations of autoimmune CNS diseases. [ABSTRACT FROM AUTHOR]

This case report describes an uncommon overlap syndrome between ankylosing spondylitis (AS) and amyotrophic lateral sclerosis (ALS). Initially, the patient was diagnosed with AS, for which he received various specific treatments, including TNF-α inhibitors. After five years of treatment with TNF-α inhibitor etanercept, the patient was referred for a full neurological assessment after he reported balance disturbances, postural instability, muscle weakness, and other neurological symptoms that indicated the presence of a neurological disorder. After a thorough investigation, the patient was diagnosed with ALS. This case report aims to contribute to the limited literature by providing a detailed case study regarding the crosstalk between AS and ALS while also exploring the potential underlying mechanisms and the possible link between TNF-α inhibitors therapy and ALS. [ABSTRACT FROM AUTHOR]

Copyright of Spektrum der Augenheilkunde is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

OBJECTIVE: The aim of the study was to analyze the clinical data of patients with chronic obstructive pulmonary disease and obstructive sleep apnea overlap syndrome (OS) during hospitalization and to evaluate the risk factors of patients treated with Non-Invasive Ventilation (NIV). METHODS: Demographic and clinical data of patients with confirmed OS during hospitalization were retrospectively collected. The patients were divided into two groups according to whether noninvasive ventilator was used during hospitalization, including OS treated with NIV (244 cases) and OS without NIV (239 cases). The t-test, χ2 test, and Kaplan–Meier curve were used to compare the two groups, and multiple logistic regression was used to analyze the risk factors of NIV in patients with OS. RESULTS: Compared with the OS group without NIV, the pulmonary hypertension, lymphocyte count, and left ventricular ejection fraction% of OS patients with NIV were lower, whereas PCO2, uric acid, C-reactive protein, procalcitonin, and N-terminal pro-B-type natriuretic peptide were higher, with statistical differences (P < 0.05). During hospitalization and follow-up, OS patients with NIV had a longer hospital stay (P < 0.001), and there was no significant difference in the rate of readmission within 28 days. The logistic regression analysis showed that the history of diuretic use, previous history of noninvasive ventilator use, and ischemic heart disease were independent risk factors for NIV treatment in OS patients during hospitalization. CONCLUSION: Patients with OS undergoing NIV during hospitalization exhibited more severe overall illness and had prolonged hospital stays compared to OS patients not receiving NIV. History of diuretic use, history of NIV use, and ischemic heart disease are independent risk factors for NIV treatment in OS patients during hospitalization.

Introduction Sjögren’s syndrome (SS) and rheumatoid arthritis (RA) are two chronic autoimmune diseases. To date, there have been few reports on the overlap between SS and RA in China, especially regarding correlated acute renal failure cases. Material and methods To provide a reference for our clinical peers, this article presents the case report of an elderly female patient who was diagnosed with acute renal failure caused by SS and RA overlap syndrome. Results We also provide a relevant analysis of SS and RA overlap syndrome treatment. Conclusions We also provide a relevant analysis of SS and RA overlap syndrome treatment.

This article presents two patients who were diagnosed with autoimmune liver disease (autoimmune hepatitis and primary biliary cholangitis overlap syndrome) during anti-tuberculosis therapy, which is a rarely reported occurrence. It highlights the challenges in distinguishing drug-induced liver injury from authentic autoimmune liver disease. It also points out the importance of considering autoimmune liver disease as a potential diagnosis revealed by the setting of drug-induced liver injury.

Introduction: Overlap syndrome is the association of obstructive sleep apnea (OSA) with chronic obstructive pulmonary disorder and with other chronic respiratory diseases. Patients with overlap syndrome have a worse prognosis compared with chronic obstructive pulmonary disorder or OSA alone. Additionally, patients with combined chronic obstructive pulmonary disorder and OSA are more likely to develop pulmonary hypertension and right heart failure much earlier than those without overlap. Aim: To assess the occurrence of pulmonary hypertension (PH) in newly diagnosed OSA-chronic obstructive pulmonary disorder overlap syndrome patients attending a tertiary care centre. Materials and methods: This cross-sectional study was conducted at Department of Pulmonary Medicine, Government Medical College, Kozhikode, Kerala, South India, among patients with OSA above 40 years of age who were proactively evaluated to pick out those with undiagnosed overlap. A period of 6 months after getting ethical clearance from June 2018 was selected as the study period. Among patients with symptoms suggestive of OSA above 40 years of age who gave the informed consent were enrolled after screening with Standard Sleep questionnaires (Berlin questionnaire, STOP BANG and Epworth Sleepiness Scale). Enrolled patients underwent routine spirometry and sleep study using the standard Level 1 overnight polysomnography (Level1 OPSG). Patients were classified in to two groups as OSA patients (group I), having an apnea hypopnea index (AHI) > 5/hr alone and the second group as those OSA patients (group II), with an obstructive spirometry pattern who were the overlap group. Arterial blood gas analysis (a sample of radial arterial blood was drawn with the patient awake and supine, and was analyzed for pH, PaCO2 and PaO2) and echocardiography (ECHO) of the two groups were compared as a non-invasive method to assess pulmonary artery hypertension and results were analyzed in a systematic manner. Results: Among the 84 patients enrolled, 16.7% had overlap syndrome and the rest had OSA alone. Statistically significant higher mean weight and BMI for those with overlap syndrome compared to the OSA group were observed. Mean FVC (forced vital capacity), FEV1 (forced expiratory volume 1 s), and FEV1/FVC were lower in those with overlap syndrome compared to OSA group. The mean values of ABG parameters revealed higher PaCO2 and lower PaO2 among the group with overlap syndrome which were statistically significant. However, there was no significant difference in resting room air SaO2 value between the two groups. The mean values of sleep duration and efficiency were significantly lower in those with overlap syndrome with a p value < 0.001. The mean value of arousal and REM (rapid eye movement) sleep percentage were significantly higher among those with overlap syndrome (p < 0.001). Mean value of NREM (non-rapid eye movement) sleep percentage was lower among the group with overlap syndrome compared with the OSA group, and this difference was statistically significant. The mean AHI value of the overlap syndrome group was 39.79 ± 7.54 and this was significantly higher than the OSA group (p < 0.004). Among the 14 patients who had ECHO evidence of pulmonary hypertension, 9 (64.3%) belonged to the overlap group which shows that they are a highly vulnerable group for developing pulmonary hypertension (PH) and requires early detection and more rigorous treatment. Conclusion: This study confirms that OSA patients with modest daytime level of hypoxemia and mild-moderate chronic airflow limitation have a high prevalence of PH. Chronic airway obstruction may be asymptomatic in some subjects and this stresses the necessity of pulmonary function test in OSA. [ABSTRACT FROM AUTHOR]

Myositis with anti-Ku-autoantibodies is a rare inflammatory myopathy associated with various connective tissue diseases. Histopathological studies have identified inflammatory and necrotizing aspects, but a precise morphological analysis and pathomechanistic disease model are lacking. We therefore aimed to carry out an in-depth morpho-molecular analysis to uncover possible pathomechanisms. Muscle biopsy specimens from 26 patients with anti-Ku-antibodies and unequivocal myositis were analyzed by immunohistochemistry, immunofluorescence, transcriptomics, and proteomics and compared to biopsy specimens of non-disease controls, immune-mediated necrotizing myopathy (IMNM), and inclusion body myositis (IBM). Clinical findings and laboratory parameters were evaluated retrospectively and correlated with morphological and molecular features. Patients were mainly female (92%) with a median age of 56.5 years. Isolated myositis and overlap with systemic sclerosis were reported in 31%, respectively. Isolated myositis presented with higher creatine kinase levels and cardiac involvement (83%), whereas systemic sclerosis-overlap patients often had interstitial lung disease (57%). Histopathology showed a wide spectrum from mild to pronounced myositis with diffuse sarcolemmal MHC-class I (100%) and -II (69%) immunoreactivity, myofiber necrosis (88%), endomysial inflammation (85%), thickened capillaries (84%), and vacuoles (60%). Conspicuous sarcoplasmic protein aggregates were p62, BAG3, myotilin, or immunoproteasomal beta5i-positive. Proteomic and transcriptomic analysis identified prominent up-regulation of autophagy, proteasome, and hnRNP-related cell stress. To conclude, Ku + myositis is morphologically characterized by myofiber necrosis, MHC-class I and II positivity, variable endomysial inflammation, and distinct protein aggregation varying from IBM and IMNM, and it can be placed in the spectrum of scleromyositis and overlap myositis. It features characteristic sarcoplasmic protein aggregation on an acquired basis being functionally associated with altered chaperone, proteasome, and autophagy function indicating that Ku + myositis exhibit aspects of an acquired inflammatory protein-aggregate myopathy. [ABSTRACT FROM AUTHOR]

Connective tissue represents the support matrix and the connection between tissues and organs. In its composition, collagen, the major structural protein, is the main component of the skin, bones, tendons and ligaments. Especially at the pediatric age, its damage in the context of pathologies such as systemic lupus erythematosus, scleroderma or dermatomyositis can have a significant negative impact on the development and optimal functioning of the body. The consequences can extend to various structures (e.g., joints, skin, eyes, lungs, heart, kidneys). Of these, we retain and reveal later in our manuscript, mainly the respiratory involvement. Manifested in various forms that can damage the chest wall, pleura, interstitium or vascularization, lung damage in pediatric systemic inflammatory diseases is underdeveloped in the literature compared to that described in adults. Under the threat of severe evolution, sometimes rapidly progressive and leading to death, it is necessary to increase the popularization of information aimed at physiopathological triggering and maintenance mechanisms, diagnostic means, and therapeutic directions among medical specialists. In addition, we emphasize the need for interdisciplinary collaboration, especially between pediatricians, rheumatologists, infectious disease specialists, pulmonologists, and immunologists. Through our narrative review we aimed to bring up to date, in a concise and easy to assimilate, general principles regarding the pulmonary impact of collagenoses using the most recent articles published in international libraries, duplicated by previous articles, of reference for the targeted pathologies. [ABSTRACT FROM AUTHOR]

Chronic obstructive pulmonary disease (COPD), obstructive sleep apnea (OSA) and their comorbid association called Overlap Syndrome (OS) are frequent chronic diseases with high individual and societal burdens. Precise descriptions of the respective symptoms, comorbidities, and medications associated with these three conditions are lacking. We used a multidimensional phenotyping approach to identify relevant phenotypes characterizing these 3 disorders. 308 patients with OSA, COPD and OS were prospectively assessed using a combination of body shape measurements and multidimensional questionnaires evaluating sleep, fatigue, depression and respiratory symptoms. Comorbidities and medications were confirmed by physicians. Patients made home blood pressure self-measurements using a connected wearable device to identify undiagnosed or uncontrolled hypertension. Three distinct relevant phenotypes were identified. OSA patients were round in shape with a balanced waist-to-hip ratio, frequent witnessed apneas, nocturia, daytime sleepiness, depression, and high diastolic blood pressure. COPD patients had a thinner body shape with a high waist-to-hip ratio, complained mainly of fatigue, and exhibited a higher resting heart rate. OS patients were round in shape with a balanced waist-to-hip ratio, reported little sleepiness and depression, but had impaired sleep and the highest rate of cardio-metabolic comorbidities. Diminished fitness-to-drive was most apparent in patients with OSA and OS. Home blood pressure measurements identified undiagnosed hypertension in 80 % of patients and in nearly 80 % of those with hypertension it was uncontrolled by their current medications. Our systematic multidimensional phenotyping approach identified distinct body shapes, symptoms, and comorbidity profiles among patients with OSA, COPD, and OS. • Multidimensional distinct and clinically relevant profiles of OSA, COPD and OS. • Objective and subjective assessment of symptoms, comorbidities and treatments. • Blood pressure measurements using a connected wearable device. • Wearable device identified a high rate of undiagnosed and uncontrolled hypertension. [ABSTRACT FROM AUTHOR]

Purpose: Obstructive sleep apnea syndrome (OSAS) and chronic obstructive pulmonary disease (COPD) are prevalent disorders, and the concurrence so-called overlap syndrome (OVS) is not rare either. Early recognition of OVS is essential because this group is more prone to cardiovascular morbidities and requires effective multidisciplinary follow-up. This study aimed to evaluate RDW in patients with severe OSAS and investigate whether it can predict OVS. Patients and methods: 96 patients were retrospectively analyzed, of whom 66 were found to have severe OSAS alone and 30 OVS during diagnostic workups. Demographic, polysomnographic, and laboratory results, including RDW, were compared between groups. Multivariate logistic regression was used to determine independent associates of OVS. Results: Gender and body mass index (BMI) were similar, however, the mean age and RDW were higher in the OVS group (p:0.008, p:0.002). The increase in RDW remained significant after adjustment for age, BMI, and cardiovascular risk factors. An RDW value of >13.65% was shown to have a 78.3% sensitivity and 60% specificity for predicting OVS in severe OSAS (p:0.004). Conclusion: The results suggest that RDW can be a reliable indicator for diagnosing OVS in OSAS. It can help in identifying the subset of patients who would benefit from proper consultations and multidisciplinary follow-up, leading to appropriate treatment of each disease component and effective monitoring to prevent adverse cardiovascular outcomes. [ABSTRACT FROM AUTHOR]

Introduction: Systemic lupus erythematosus (SLE) is a multidimensional disease; however, the association of another systemic autoimmune disease further complicates its clinical presentation. Aim: We decided to investigate whether the association of overlap syndromes is linked with a different clinical picture compared to pure lupus and whether this association changes the sensitivity of the following commonly used criteria: the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR), the ACR-1997 and the 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria. Method: We performed a retrospective observational study among 382 patients afflicted with lupus: we measured as much of the full clinical and laboratory picture as possible in an unselected cohort. The diagnosis of SLE and other systemic autoimmune diseases was established by the rheumatologist in routine care and then the authors compared the characteristics of patients with pure lupus and those with overlapping pathologies. The diagnosis rates were compared to those that were determined based on the three classification criteria in order to identify various sensitivities and whether the existence of an overlap affects their rates. The fulfillment of each set of criteria was calculated using an Excel-based automatic calculation. Results: Among the patients, the ACR 1997′s sensitivity was 81.2% (310 patients), and the SLICC 2012 criteria achieved 94.5% sensitivity (361 patients). The 2019 EULAR/ACR classification criteria resulted in a slightly lower sensitivity (90.3%—345 patients) when compared to the original publication (96%) due to the lower sensitivity of our anti-nuclear antibody (ANA) test (measured via enzyme-linked immunosorbent assay (ELISA)). Nearly all ANA-negative (21/22—95%) patients showed a positive lupus-associated antibody test. The proportion of ANA-negative cases showed no significant difference among pure and overlap patients. No significant difference was found between patients with overlap (138 patients—36%) and pure SLE (244 patients—64%) through the use of these criteria, with the exception of the SLICC criteria (ACR: 80.4% vs. 81.6%; SLICC: 97.4% vs. 92.6%, p = 0.035; EULAR/ACR 2019: 91.4% vs. 89.6%). Patients with an overlap syndrome were significantly older (55 vs. 50 years, p = 0.001), more likely to suffer from interstitial lung disease (ILD: 20% vs. 11%, p = 0.0343) and less frequently showed class III/IV lupus nephritis (7% vs. 14%, p = 0.029) when compared with their pure lupus counterparts. Conclusion: All investigated criteria regarding sensitivity were similar to the original publication's findings. The sensitivity of the EULAR/ACR 2019 classification criterion in cases with overlap syndrome proved excellent, with results very similar to patients afflicted with pure SLE. In the presence of an overlap syndrome, we found significantly fewer patients with lupus nephritis III/IV but no differences in other typical lupus organ manifestation beyond the kidney, whereas we found a higher proportion of ILD in patients with an overlap, indicating that the presence of an overlap syndrome significantly influences the observed clinical picture in real-world conditions. [ABSTRACT FROM AUTHOR]

Background: Overlap syndrome (OS), the coexistence of chronic obstructive pulmonary disease and obstructive sleep apnea, is frequently characterized by the presence of daytime hypercapnia (pCO2 ≥ 45 mmHg). The aim of this study was to investigate potential differences in anthropometric, sleep and respiratory characteristics between hypercapnic and normocapnic patients with OS. Methods: Consecutive patients who underwent polysomnography, pulmonary function testing and arterial blood gases and had been diagnosed with OS were enrolled in the study. Results: According to pCO2 levels in wakefulness, the patients were divided into group A, consisting of OS patients without hypercapnia (n = 108) or group B, consisting of OS patients with hypercapnia (n = 55). The majority of included patients in both groups were males (n = 92 in group A vs. n = 50 in group B). Group B had increased BMI (p = 0.001), neck (p = 0.017) and waist circumference (p = 0.013), higher scores in Epworth sleepiness scale (ESS) (p = 0.008), increased sleep efficiency (p = 0.033), oxygen desaturation index (p = 0.004) and time with oxyhemoglobin saturation <90% (p = 0.006) than group A. Also, Group B had decreased average and minimum oxyhemoglobin saturation during sleep (p < 0.001). Hypercapnic patients had lower FEV1% (p = 0.003), FVC% (p = 0.004), pO2 and pCO2 (p < 0.001 for both) values compared with normocapnic patients. In binary regression analysis, which assessed various predictors on the likelihood of having hypercapnia, it was found that BMI (OR: 1.313, 95% CI: 1.048–1.646, p = 0.018) and FVC (OR: 0.913, 95% CI: 0.845–0.986, p = 0.020) were the major determinants of hypercapnia in OS patients. Conclusions: Hypercapnic OS patients were more obese and sleepy and presented worse respiratory function in wakefulness and sleep hypoxia characteristics compared with normocapnic OS patients. [ABSTRACT FROM AUTHOR]

Introduction: Mixed connective tissue disease (MCTD) is a rare entity in children. There is a paucity of studies on juvenile‐onset MCTD (jMCTD) worldwide especially from Southeast Asia. Objectives: To describe clinical and laboratory features of jMCTD diagnosed at pediatric rheumatology centers across India. Methods: A predesigned detailed case proforma in an excel format was prepared and was sent to all the Pediatric Rheumatology centers in India. Eleven centers provided the clinical and laboratory data of their jMCTD patients, which was then compiled and analyzed in detail. Results: Thirty‐one jMCTD patients from 11 centers were included in the study. Our cohort had 27 females and four male patients over 12 months (August 2021 to July 2022). The median age at presentation was 12 years (range 5–18 years) and the median duration of symptoms was 24 months at diagnosis (range 2–96 months). The common features included arthritis (90%), malar rash (70.9%), and Raynaud's phenomenon (70.9%). At a mean follow‐up of 43 months (range 1–168 months), 45% of them were in remission. There were two deaths reported, due to macrophage activation syndrome and sepsis respectively. Conclusion: We present the largest multicenter experience on jMCTD from the Indian subcontinent. The study's findings serve as a crucial stepping stone toward unraveling the complexities of jMCTD and improving patient care and management strategies. [ABSTRACT FROM AUTHOR]

Purpose: The aim of this study is to investigate the role of the neutrophil to lymphocyte ratio (NLR) in patients diagnosed with the chronic obstructive pulmonary disease-obstructive sleep apnea (COPD-OSA) overlap syndrome and comorbid pulmonary hypertension (PH). Patients and methods: We enrolled a consecutive of stable COPD patients and conducted spirometry measurements, nocturnal polysomnography (PSG), and echocardiography for all participants. Clinical laboratory data were collected. Results: A total of 178 patients with stable COPD were enrolled among whom 33.14% (59/178) were diagnosed with OSA. Of the patients with overlap syndrome, 35.59% (21/59) showed comorbid PH, compared to 34.45% (41/119) in COPD patients without OSA. There was no significant difference in the occurrence of PH between COPD with and without OSA. NLR was significantly higher in patients with overlap syndrome compared to those with either disease alone. The difference in NLR between COPD-OSA patients with and without PH was not significant. Correlation analysis revealed that NLR was associated with age, total sleep time spent with oxygen saturation below 90% (T90), CRP, pulmonary artery systolic pressure (PASP), and minimum peripheral capillary oxygen saturation (SpO2min) in all COPD patients. NLR was identified as an independent factor contributing to OSA in COPD. The median cut-off value for detecting OSA in stable COPD was 2.49. However, NLR was not found to be a predictor for PH in COPD-OSA overlap syndrome. Conclusions: NLR can serve as a predictive marker for comorbid OSA in patients with COPD. NLR is expected to increase its clinical application as a convenient and cost-effective biomarker for COPD-OSA overlap syndrome. [ABSTRACT FROM AUTHOR]

Study objectivesThe coexistence of obstructive sleep apnea (OSA) and chronic obstructive pulmonary disease (COPD) in a single individual, also known as overlap syndrome (OVS), is associated with higher cardiovascular risk and mortality than either OSA or COPD alone. However, the underlying mechanisms remain unclear. We hypothesized that patients with OVS have elevated systemic inflammatory biomarkers relative to patients with either disease alone, which could explain greater cardiovascular risk observed in OVS.MethodsWe included 255 participants in the study, 55 with COPD alone, 100 with OSA alone, 50 with OVS, and 50 healthy controls. All participants underwent a home sleep study, spirometry, and a blood draw for high-sensitivity C-reactive protein and total blood count analysis. In a randomly selected subset of 186 participants, inflammatory protein profiling was performed using Bio-Rad Bio-Plex Pro Human Cytokine 27-Plex Assays. Biomarker level differences across groups were identified using a mixed linear model.ResultsLevels of interleukin 6 (IL-6), high-sensitivity C-reactive protein (hs-CRP), and granulocyte colony stimulating factor (G-CSF) were higher in participants with OVS and COPD compared with healthy controls and participants with OSA. Furthermore, participants with OVS had higher circulating levels of leukocytes and neutrophils than those with COPD, OSA, and controls.ConclusionsCOPD and OVS are associated with higher systemic inflammation relative to OSA and healthy controls. This work proposes the potential utilization of interleukin 6, granulocyte colony stimulating factor, and high-sensitivity C-reactive protein as screening biomarkers for COPD in patients with OSA. Inflammatory pathways may not fully explain the higher cardiovascular risk observed in OVS, indicating the need for further investigation.CitationSanchez-Azofra A, Gu W, Masso-Silva JA, et al. Inflammation biomarkers in OSA, chronic obstructive pulmonary disease, and chronic obstructive pulmonary disease/OSA overlap syndrome. J Clin Sleep Med. 2023;19(8):1447-1456.

Anti-Ku antibodies are a special type of myositis-associated antibodies that are characteristic of patients with the overlap syndrome of systemic scleroderma and immune-inflammatory myopathy, as well as occurring in other autoimmune diseases. Patients with these antibodies have their own clinical and laboratory features of the disease and can be identified as a separate anti-Ku syndrome.For the first time in the domestic literature, descriptions of two clinical cases of the development of a cross syndrome associated with anti-Ku antibodies are presented. The features of the course of the disease and the response to various options for immunosuppressive therapy were analyzed.

Introduction. The combination of atopic dermatitis (AD) with allergic contact dermatitis (ACD) or the occurrence of ACD on the background of atopic dermatitis is called the overlap syndrome. Studies have demonstrated several reasons why patients with AD have a similar or even increased risk of developing ACD compared to those without AD. Allergens and haptens are trigger factors in a group of patients with AD and ACD overlap syndrome. The aim of the study. To confirm the diagnosis of ACD in a group of patients with AD – diagnose the overlap syndrome and analyze which allergens and haptens were the trigger factors in this group. Materials and methods. To confirm IgE-dependent sensitization in atopic dermatitis, skin prick tests or determination of specific IgE in blood serum were performed. Skin patch tests (European series S-1000) were performed to determine the mechanisms of delayed-type hypersensitivity. Results. It was found that the highest specific weight of positive allergic reactions has been recorded in response to the following allergens: ticks, ticks/ambrosia, birch and mold. The absolute majority of patients demonstrated positive specific IgE-dependent sensitization to Dermatophagoides pteronyssinus and Dermatophagoides farinae – 24 (50%), in turn, on Ambrósia – 14 (29.2%), and on Alternaria alternata – 8 (16.7%). Also, the reaction was most often recorded to haptens: cobalt, nickel, formaldehyde, PPD, textile dyes. Deterioration of the clinical course and shortening of AD remission periods were observed due to the formation of ACD against the background of impaired skin barrier function and the presence of chronic immune inflammation. Conclusions. Patients with AD are more often diagnosed with ACD, which predictably worsens the course of AD. Patients with confirmed overlap syndrome "AD + ACD" most often show reactions to haptens: Cobalt, Nikel, Formaldehyde, PPD, Textile dye mix – and in the vast majority to 2 haptens or more in one patient.

Introduction: Hepatobiliary overlap syndromes describe the coinciding presentation of more than one immune-mediated biliary and liver disease in a single patient and present complex challenges in diagnosis and treatment. We report a case of ulcerative colitis with primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome responsive to vancomycin. Case Presentation: The patient is a 30-year-old female with known ulcerative pancolitis and autoimmune hepatitis. She presented to the emergency department with a constellation of gastrointestinal symptoms, including diffuse lower abdominal pain, bloody diarrhea, and nausea with bilious vomiting. Subsequent imaging revealed the additional diagnosis of primary sclerosing cholangitis, and she was diagnosed with overlap syndrome. Multiple treatment regimens were trialed with minimal improvement. She eventually achieved normalization of both clinical status and biochemical markers after the addition of vancomycin. Conclusion: Vancomycin is an underutilized therapy; its potential role in primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome has not been previously reported.

Introduction: The presence of three different entities in a single patient is usually of clinical interest and mostly anecdotal. The overlap of systemic sclerosis (SSc), Sjögren syndrome (SS), and ANCA-associated renal-limited vasculitis has been reported only once previously. Case Presentation: A 61-year-old female was evaluated at consultation with 2 years of symptomatology, presenting cardboard-like skin, sclerodactyly, limited oral opening, and dry skin and eyes. She was admitted for progressive renal failure (serum creatinine, 5.5 mg/dL). Her serology work-up showed positive anti-SCL-70, anti-Ro, anti-La, anti-MPO, and antinuclear antibodies. Renal biopsy was performed and confirmed histological findings for SSc, SS, and ANCA-associated vasculitis with active extracapillary glomerulonephritis with fibrous predominance (EUVAS-Berden sclerotic class), active tubulointerstitial nephritis, focal tubular injury, and moderate chronic arteriolopathy. Treatment with 6 monthly doses of methylprednisolone and cyclophosphamide was established. At the last follow-up, the patient maintained a stable serum creatinine level of 2.6 mg/dL and had decreased proteinuria, no erythrocyturia, and no requirement for renal replacement therapy. Conclusion: Systemic sclerosis is a rare autoimmune disease; nevertheless, overlap with Sjögren syndrome is relatively common, although its association with ANCA vasculitis is anecdotal. Diagnostic integration presents a challenge for nephrologists to define the prognosis and a specific treatment.

Aim: to present the difficulties of long-term management of a patient with liver cirrhosis in the outcome of overlap syndrome (autoimmune hepatitis and primary biliary cholangitis) who suffered from severe COVID-19 infection.Key points. The diagnosis of liver cirrhosis as an outcome of overlap syndrome (autoimmune hepatitis and primary biliary cholangitis) was established at the patient’s age of 33 years. At the age of 40, the patient became pregnant for the first time, the pregnancy proceeded well, and a cesarean section was performed at 36 weeks. At the age of 45, the patient suffered a severe new coronavirus infection, followed by decompensation of liver cirrhosis, which required liver transplantation 4 months after COVID-19, followed by a favorable postoperative course.Conclusion. This clinical case demonstrates the successful onset and outcome of pregnancy in a patient with liver cirrhosis in the outcome of overlap syndrome (autoimmune hepatitis and primary biliary cholangitis). The pronounced activity of the disease after severe new coronavirus infection required liver transplantation with successful outcome.

Background Systemic sclerosis (SSc) often overlaps with other autoimmune diseases. More complex autoantibody profiles may be observed in SSc overlap syndrome (SSc OS). To determine the clinical significance of autoantibodies in SSc OS and classify the patients more accurately for better disease assessments, we analysed the correlation between serological profiles, organ involvements and outcomes.Methods A retrospective cohort study was conducted in Peking University People’s Hospital. Chi-square tests and analysis of variance were used to analyse univariate comparisons of clinical symptoms, organ involvement and laboratory indicators. Survival was evaluated using Cox proportional hazards model.Results Among 141 cases, anti-Ro-52 was the most common antibody, followed by anti-centromere antibody and anti-Scl-70 antibody. We analysed the correlation between autoantibodies and vital organ damage in SSc OS patients, and compared the differences across four SSc OS subgroups (SSc SLE, SSc RA, SSc PM/DM and SSc SS) to demonstrate the correlation between autoantibodies and clinical characteristics and organ damage. Cox regression analysis showed that scleroderma renal crisis (SRC) (p = .004) and pulmonary arterial hypertension (PH) (p = .010) were independent risk factors for survival.Conclusions Autoantibodies are associated with clinical features, organ involvement and prognosis in SSc OS patients. Anti-Scl-70 antibody is associated with interstitial lung disease (ILD) and SRC, while ACA is a protective factor of ILD. SRC and PH are risk factors associated with death.

Overlap syndrome (OVS) is a distinct clinical entity that seems to result in potential cardiovascular consequences. We aimed to estimate the prevalence and risk factors for OVS in OSA patients and analyze clinical and PSG characteristics associated with OVS. In this cross-sectional study, 2616 patients evaluated for OSA underwent type-1 polysomnography (PSG). They were grouped as pure OSA (AHI > 15/h) and OVS patients. Demographics, PSG data, pulmonary function tests and arterial blood gases (ABGs) were compared between groups after adjustments for confounders. OSA was diagnosed in 2108 out of 2616 patients. Of those, 398 (19%) had OVS. Independent predictors of OVS were older age [OR: 5.386 (4.153–6.987)], current/former smoking [OR: 11.577 (7.232–18.532)], BMI [OR: 2.901 (2.082–4.044)] and ABG measurements [PaCO2 ≥ 45 OR: 4.648 (3.078–7.019), PO2 [OR: 0.934 (0.920–0.949)], HCO3− [OR: 1.196 (1.133–1.263), all p < 0.001]. OVS was also associated with prevalent hypertension [OR: 1.345 (1.030–1.758), p = 0.03] and cardiovascular disease [OR: 1.617 (1.229–2.126), p < 0.001], depressive symptoms [OR: 1.741 (1.230–2.465), p = 0.002] and nocturia [OR: 1.944 (1.378–2.742), p < 0.001], as well as with indices of OSA severity. Disturbances in sleep architecture were more prominent in OVS expressed by lower %N3 and REM% and higher arousal index. Our data suggest that OVS is prevalent among OSA patients, with distinct clinical and PSG characteristics. These characteristics could be utilized as predictive factors for early identification and further evaluation of these patients towards desirable patient-reported outcomes. [ABSTRACT FROM AUTHOR]

Overlap syndrome, characterized by the coexistence of autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC), presents a complex and challenging clinical scenario. This case report details the diagnostic journey of a 61-year-old female patient with overlapping AIH and PBC, showcasing clinical features, investigations, and therapeutic interventions. The case emphasizes the importance of multidisciplinary collaboration, tailored treatment approaches, and close follow-up for optimal management of this uncommon but relevant overlap syndrome. [ABSTRACT FROM AUTHOR]

Copyright of Practical Pharmacy & Clinical Remedies is the property of Editorial Department of Practical Pharmacy & Clinical Remedies and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background. Overlap syndromes are complex conditions, comprising elements of more than one distinct autoimmune disease, as well as multiorgan involvement. Methods. We present the case of a 48-year-old female patient with systemic lupus erythematosus (SLE)/systemic sclerosis (SSc)/Sjögren syndrome (SS) overlap and a myriad of neurological manifestations, including bilateral trigeminal neuralgia, bilateral facial nerve palsy, mononeuritis multiplex and migraines cephalalgia. Results. Physical examination revealed bilateral involuntary spasms of the lower face, bilateral facial motor deficit, a mild motor deficit of the lower limbs (4/5), tactile hypoesthesia on the left thigh, a diminished right patellar reflex and a positive bilateral Hoffman sign. This neurological symptomatology was present during an apparent inactive state of the rheumatologic condition, with no signs of active arthritis, and the following laboratory results: a mild normocytic normochromic anemia, mild leukopenia, a mildly decreased C3 and a mildly elevated ESR. Cerebral MRI revealed T2/FLAIR-hyperintense subcortical white matter lesions, suggestive of demyelination, without meeting the multiple sclerosis (MS) pattern. Conclusion. The main clinical question arising from this case resides in the nature of the neurological involvement, in relation to the overlap syndrome. [ABSTRACT FROM AUTHOR]

Overlap syndromes are inflammatory rheumatic conditions in which patients show clinical manifestations suggestive of multiple distinct immune diseases. This case report discusses a complex instance involving Sjögren’s disease, rheumatoid arthritis (RA), and systemic lupus erythematosus (SLE) in a woman in her 30s. Lupus nephritis, a severe kidney inflammation associated with SLE, and diffuse alveolar hemorrhage (DAH), a life-threatening lung complication characterized by bleeding into the alveolar spaces, further complicated her clinical course. Initially presenting with acute lower limb pain, swelling, and rash, suggesting systemic involvement beyond RA, laboratory findings of hypocomplementemia and renal involvement raised suspicion of SLE. The subsequent development of polyserositis, mesenteric vasculitis, and DAH underscored the complexity of her condition. This case highlights the importance of a holistic approach that integrates clinical expertise, advanced diagnostics, and tailored therapeutic interventions to optimize patient outcomes. Early recognition and prompt management of complications are crucial in mitigating morbidity and improving prognosis, emphasizing the need for vigilant monitoring and interdisciplinary collaboration in the management of connective tissue diseases. [ABSTRACT FROM AUTHOR]

Both scleroderma and immunoglobulin G4-related disease (IgG4-RD) are systemic fibro-inflammatory diseases characterised by lymphoplasmacytic infiltrates. IgG4-RD and systemic sclerosis (SSc) may share common pathophysiological mechanisms, but no examples of co-occurrence of the diseases have been found. Autologous haematopoietic stem cell transplantation (AHSCT) is implemented in selected rapidly progressive SSc with a high risk of organ failure. However, existing guidelines are based on clinical trials that do not represent the entire patient population and exclude critically ill patients with no therapeutic alternatives. Examples of AHSCT in IgG4-RD are absent. We report the case of a 44-year-old female patient with overlapping progressive diffuse SSc and sinonasal IgG4-RD. After 11 years of ineffective SSc treatment, AHSCT was performed. The 63-month follow-up showed a regression of SSc symptoms. AHSCT was not intended as treatment in the case of IgG4RD, although the first symptoms of the disease developed before transplantation. The sinus lesions progressed after AHSCT and remained indolent only after surgical treatment (bilateral ethmoidectomy, sphenoidotomy, intranasal buccal antrostomy), which allowed histopathological confirmation of IgG4-RD. [ABSTRACT FROM AUTHOR]

Chronic obstructive pulmonary disease (COPD) is a major cause of morbidity and mortality throughout the world. The coexistence of COPD and obstructive sleep apnea (OSA) (i.e., overlap syndrome) has been reported in several studies. Both disorders independently increase the risk of cardiovascular complications. Hence, there is a theoretical possibility that cardiovascular parameters may be worse in patients with overlap syndrome compared to those with only COPD. However, this has been sparsely assessed in the literature. This study aimed to compare the clinical characteristics, echocardiography, and sleep parameters amongst COPD patients with and without sleep-related breathing disorders (SRBD). This observational, cross-sectional study included 30 patients with stable COPD. All participants underwent a detailed clinical evaluation, followed by level 1 polysomnography (PSG). Each participant underwent echocardiographic evaluation to estimate mean pulmonary artery pressure from right ventricular systolic pressure (RVSP). Based on their PSG findings, participants were classified into non-SRBD and SRBD groups. Both groups were further compared with respect to clinical characteristics, echocardiographic, and PSG parameters. We found that most of the participants (93.3%) were male, and the mean age of the study population was 56±8.2 years. The only SRBD identified in this study was OSA, which was observed in 80% of participants. In this group, OSA was not associated with obesity. Systemic hypertension (50%) was the most common comorbidity, followed by diabetes mellitus (26.67%), but both were not significantly different between the groups. The mean RVSP was significantly higher amongst OSA patients than non-OSA patients (41.25±14.98 versus 30.83±5.84, respectively; p=0.01). OSA was seen in 80% of participants with stable COPD, even in the absence of obesity. The presence of OSA was associated with a higher RVSP in this patient group.

Connective tissue represents the support matrix and the connection between tissues and organs. In its composition, collagen, the major structural protein, is the main component of the skin, bones, tendons and ligaments. Especially at the pediatric age, its damage in the context of pathologies such as systemic lupus erythematosus, scleroderma or dermatomyositis can have a significant negative impact on the development and optimal functioning of the body. The consequences can extend to various structures (e.g., joints, skin, eyes, lungs, heart, kidneys). Of these, we retain and reveal later in our manuscript, mainly the respiratory involvement. Manifested in various forms that can damage the chest wall, pleura, interstitium or vascularization, lung damage in pediatric systemic inflammatory diseases is underdeveloped in the literature compared to that described in adults. Under the threat of severe evolution, sometimes rapidly progressive and leading to death, it is necessary to increase the popularization of information aimed at physiopathological triggering and maintenance mechanisms, diagnostic means, and therapeutic directions among medical specialists. In addition, we emphasize the need for interdisciplinary collaboration, especially between pediatricians, rheumatologists, infectious disease specialists, pulmonologists, and immunologists. Through our narrative review we aimed to bring up to date, in a concise and easy to assimilate, general principles regarding the pulmonary impact of collagenoses using the most recent articles published in international libraries, duplicated by previous articles, of reference for the targeted pathologies.

Aims: Autoimmune liver diseases (AILD) represent a spectrum of related yet distinct immune-mediated disorders. The literature on the prevalence of these AILDs in Indian population is scarce. This study aims to assess the prevalence and clinicopathological spectrum of various AILDs especially the overlap syndrome. Materials and Methods: A 10-year (2011–2020) cross-sectional, retrospective observational study of histological proven cases of AILD was conducted. Clinical, demographic, and laboratory parameters were retrieved. Two pathologists independently reviewed the liver biopsies and reassessed 18 histopathological parameters. Results: During the study period, 17664 liver biopsies were received, out of which 1060 (6%) biopsies of AILD were identified. After exclusion, we had 721 cases which revealed a distribution of autoimmune hepatitis (AIH)-64.7%, primary biliary cholangitis (PBC)-14.8%, primary sclerosing cholangitis (PSC)-7.6%, overlap AIH-PBC 11%, and overlap AIH-PSC 1.7%. AIH patients had significantly higher prevalence for severe lobular inflammation (27%, P ≤ 0.001), several lobular plasma cells (37%, P ≤ 0.001), central perivenulitis (30%, P ≤ 0.001), hepatic rosettes (51%, P ≤ 0.001), and necrosis (35.5%, P ≤ 0.001), while PBC patients had significantly higher frequency of florid duct lesions (11.2%, P ≤ 0.001), duct loss (83.17%, P ≤ 0.001), bile duct damage (76.6%, P ≤ 0.001), and periportal copper deposits (19.6%, P ≤ 0.001). Overlap AIH-PBC group had the highest proportion of severe portal inflammation (27.5%, P ≤ 0.001), prominent portal plasma cells (75%, P ≤ 0.001), moderate interface activity (53.7%, P ≤ 0.001), Mallory-Denk bodies (27.5%, P ≤ 0.001), and periportal cholate stasis (25%, P ≤ 0.001). Conclusion: Prevalence of biopsy-proven AILDs in our study cohort is 6%. AIH (64.7%) is the most common AILD followed by PBC (14.8%). Overlap syndrome (AIH-PBC) showed prevalence of 11%.

This case report describes an uncommon overlap syndrome between ankylosing spondylitis (AS) and amyotrophic lateral sclerosis (ALS). Initially, the patient was diagnosed with AS, for which he received various specific treatments, including TNF-α inhibitors. After five years of treatment with TNF-α inhibitor etanercept, the patient was referred for a full neurological assessment after he reported balance disturbances, postural instability, muscle weakness, and other neurological symptoms that indicated the presence of a neurological disorder. After a thorough investigation, the patient was diagnosed with ALS. This case report aims to contribute to the limited literature by providing a detailed case study regarding the crosstalk between AS and ALS while also exploring the potential underlying mechanisms and the possible link between TNF-α inhibitors therapy and ALS.

Rationale: The co-occurrence of obstructive sleep apnea and chronic obstructive pulmonary disease, termed overlap syndrome, has a poor prognosis. However, data on positive airway pressure (PAP) treatments and their impact on outcomes and costs are lacking. Objectives: This retrospective observational study investigated the effects of PAP on health outcomes, resource usage, and costs in patients with overlap syndrome. Methods: Deidentified adjudicated claims data for patients with overlap syndrome in the United States were linked to objectively measured PAP user data. Patients were considered adherent to PAP therapy if they met Centers for Medicare and Medicaid Services criteria for eight 90-day timeframes from device setup through 2-year follow-up. Propensity score matching was used to create comparable groups of adherent and nonadherent patients. Healthcare resource usage was based on the number of doctor visits, all-cause emergency room visits, all-cause hospitalizations, and PAP equipment and supplies, and proxy costs were obtained. Measurements and Main Results: A total of 6,810 patients were included (mean age, 60.8 yr; 56% female); 2,328 were nonadherent. Compared with the year before therapy, there were significant reductions in the number of emergency room visits, hospitalizations, and severe acute exacerbations during 2 years of PAP therapy in patients who were versus were not adherent (all P

Study objectivesChronic obstructive pulmonary disease and obstructive sleep apnea overlap syndrome is associated with excess mortality, and outcomes are related to the degree of hypoxemia. People at high altitudes are susceptible to periodic breathing, and hypoxia at altitude is associated with cardio-metabolic dysfunction. Hypoxemia in these scenarios may be described as superimposed sustained hypoxia (SH) plus intermittent hypoxia (IH), or overlap hypoxia (OH), the effects of which have not been investigated. We aimed to characterize the cardio-metabolic consequences of OH in mice.MethodsC57BL/6J mice were subjected to either SH (FiO2 = 0.10), IH (FiO2 = 0.21 for 12 h, and FiO2 oscillating between 0.21 and 0.06, 60 times/hour, for 12 h), OH (FiO2 = 0.13 for 12 h, and FiO2 oscillating between 0.13 and 0.06, 60 times/hour, for 12 h), or room air (RA), n = 8/group. Blood pressure and intraperitoneal glucose tolerance test were measured serially, and right ventricular systolic pressure (RVSP) was assessed.ResultsSystolic blood pressure transiently increased in IH and OH relative to SH and RA. RVSP did not increase in IH, but increased in SH and OH by 52% (p < .001) and 20% (p = .001). Glucose disposal worsened in IH and improved in SH, with no change in OH. Serum low- and very-low-density lipoproteins increased in OH and SH, but not in IH. Hepatic oxidative stress increased in all hypoxic groups, with the highest increase in OH.ConclusionsOH may represent a unique and deleterious cardio-metabolic stimulus, causing systemic and pulmonary hypertension, and without protective metabolic effects characteristic of SH.

BACKGROUND: An increasing number of coronavirus disease 2019 (COVID-19) related autoimmune hepatitis (AIH) and autoimmune liver disease (AILD) has been already described so far in the last three years. This rise has set up some diagnostic and therapeutic concerns, although steroid therapy has mostly been efficient, avoiding main significant side effects. CASE PRESENTATION: We report the case of a 52-year-old subject displaying liver function impairment at the laboratory tests while positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) swab. Needle liver biopsy showed severe portal inflammation, interface hepatitis, lobular inflammation, abundant plasma cells, bridging necrosis, endothelialitis, bile duct vanishing disease, and ductular reaction. The diagnosis of autoimmune liver disease (AILD) was performed. After a month of steroid and ursodeoxycholic acid medications, liver function fully recovered. Azathioprine was introduced, and steroids were gradually reduced. CONCLUSIONS: Probably triggered by the SARS-CoV-2-induced cytokine storm, the association between COVID-19 and autoimmune-related inflammatory injury may display a particular paradigm of AILD pathogenesis. [ABSTRACT FROM AUTHOR]

Several serum biomarkers for fibrosis assessment have been proposed in various liver diseases, but in autoimmune hepatitis (AIH) or overlap with primary biliary cholangitis (PBC; AIH-PBC) patients, the data are scarce. This retrospective cross-sectional study was conducted to validate six non-invasive biomarkers in the diagnosis of cirrhosis (F4 fibrosis) in such patients. We included adult patients diagnosed with AIH or AIH-PBC overlap syndrome who underwent a liver biopsy between 2011 and 2021. Laboratory data were collected to calculate the following scores: red cell distribution width to platelet ratio (RPR), aspartate aminotransferase/platelet ratio index (APRI), Fibrosis-4 index (FIB-4), aspartate aminotransferase (AST) to alanine aminotransferase (ALT) ratio (AAR), neutrophil-to-lymphocyte ratio (NLR), and lymphocyte-to-platelet ratio (LPR). A total of 139 patients were eligible (111 AIH and 28 AIH-PBC). The prevalence of cirrhosis was 35.3% (36% in AIH and 32.1% in AIH-PBC). The AUROCs of the RPR, FIB-4, APRI, AAR, LPR, and NLR in all patients were 0.742, 0.724, 0.650, 0.640, 0.609, and 0.585, respectively. RPR was significantly superior to APRI, NLR, and LPR. Moreover, RPR showed the highest AUROC (0.915) in the overlap AIH-PBC subgroup. In conclusion, RPR yielded the highest diagnostic accuracy to predict cirrhosis in AIH and AIH-PBC overlap syndrome patients, while FIB-4 was considerably optimal. [ABSTRACT FROM AUTHOR]

Guillain-Barré syndrome/Miller-Fisher syndrome (GBS/MFS) overlap syndrome is an extremely rare variant of Guillain-Barré syndrome (GBS) in which Miller-Fisher syndrome (MFS) coexists with other characteristics of GBS, such as limb weakness, paresthesia, and facial paralysis. We report the clinical case of a 12-year-old patient, with no pathological history, who acutely presents with ophthalmoplegia, areflexia, facial diplegia, and swallowing and phonation disorders, followed by progressive, descending, and symmetrical paresis affecting first the upper limbs and then the lower limbs. An albuminocytological dissociation was found in the cerebrospinal fluid study. Magnetic resonance imaging of the spinal cord showed enhancement and thickening of the cauda equina roots. The patient was treated with immunoglobulins with a favorable clinical outcome. [ABSTRACT FROM AUTHOR]

Introduction: The presence of three different entities in a single patient is usually of clinical interest and mostly anecdotal. The overlap of systemic sclerosis (SSc), Sjögren syndrome (SS), and ANCA-associated renal-limited vasculitis has been reported only once previously. Case Presentation: A 61-year-old female was evaluated at consultation with 2 years of symptomatology, presenting cardboard-like skin, sclerodactyly, limited oral opening, and dry skin and eyes. She was admitted for progressive renal failure (serum creatinine, 5.5 mg/dL). Her serology work-up showed positive anti-SCL-70, anti-Ro, anti-La, anti-MPO, and antinuclear antibodies. Renal biopsy was performed and confirmed histological findings for SSc, SS, and ANCA-associated vasculitis with active extracapillary glomerulonephritis with fibrous predominance (EUVAS-Berden sclerotic class), active tubulointerstitial nephritis, focal tubular injury, and moderate chronic arteriolopathy. Treatment with 6 monthly doses of methylprednisolone and cyclophosphamide was established. At the last follow-up, the patient maintained a stable serum creatinine level of 2.6 mg/dL and had decreased proteinuria, no erythrocyturia, and no requirement for renal replacement therapy. Conclusion: Systemic sclerosis is a rare autoimmune disease; nevertheless, overlap with Sjögren syndrome is relatively common, although its association with ANCA vasculitis is anecdotal. Diagnostic integration presents a challenge for nephrologists to define the prognosis and a specific treatment. [ABSTRACT FROM AUTHOR]

Introduction: Hepatobiliary overlap syndromes describe the coinciding presentation of more than one immune-mediated biliary and liver disease in a single patient and present complex challenges in diagnosis and treatment. We report a case of ulcerative colitis with primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome responsive to vancomycin. Case Presentation: The patient is a 30-year-old female with known ulcerative pancolitis and autoimmune hepatitis. She presented to the emergency department with a constellation of gastrointestinal symptoms, including diffuse lower abdominal pain, bloody diarrhea, and nausea with bilious vomiting. Subsequent imaging revealed the additional diagnosis of primary sclerosing cholangitis, and she was diagnosed with overlap syndrome. Multiple treatment regimens were trialed with minimal improvement. She eventually achieved normalization of both clinical status and biochemical markers after the addition of vancomycin. Conclusion: Vancomycin is an underutilized therapy; its potential role in primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome has not been previously reported. [ABSTRACT FROM AUTHOR]

La maladie associée aux IgG4 est caractérisée par une ou plusieurs atteintes d'organes pseudotumorales pouvant atteindre virtuellement tous les organes, et partageant des lésions histologiques communes. Dans cette monographie, nous proposons une revue de la littérature synthétisant les différentes atteintes d'organes possibles, les principales caractéristiques biologiques et histologiques retrouvées chez ces patients, ainsi que les critères diagnostiques et de classification à disposition des cliniciens. Un focus est également réalisé sur les exceptionnelles atteintes articulaires de la maladie ainsi que sur les rares formes secondaires/de chevauchement avec d'autres maladies systémiques. Enfin, les récentes avancées dans la compréhension physiopathologique de la maladie sont présentées, ainsi que les principales recommandations et avis d'experts concernant la prise en charge thérapeutique de ces patients. IgG4-related (IgG4-RD) disease is a rare multi-organ fibro-inflammatory condition characterized by mass lesions and strictures, that can affect virtually all organs with characteristic histological lesions. In this review, we purpose an overview of the different organ involvements, biological and histological characteristics of the patients, as well as a literature review on diagnostic and classification criteria available for physicians. A specific focus on anecdotal IgG4-related arthritis/synovitis and rare "secondary" IgG4-RD associated with other systemic immune-mediated conditions is also purposed for rheumatologists. Finally, recent advances on pathophysiology are presented, as well as main expert consensus and recommendations on IgG4-RD management. [ABSTRACT FROM AUTHOR]

Obstructive sleep apnea syndrome (OSAS) and chronic obstructive pulmonary disease (COPD) are two chronic diseases that afflict many individuals worldwide with negative effects on health that may overlap in Overlap Syndrome (OS). The aim of our study was to investigate the differences in mortality between OSAS alone and OS and the risk factors involved. The study was conducted on patients with OSAS or OS diagnosis that completed 15-year follow-up between 2005 and 2023. Of these, the clinical, functional, sleep and survival data were registered and analysed. Risk factors were found by regression analysis. 501 patients (428 OSAS and 73 OS) were enrolled. Patients with OS had higher mortality than OSAS (p < 0,001). The morality risk factors for the overall population found were age >65 years (odds ratio (OR) = 10.69 (95%CI 3,85–29,69), p < 0,001) and low forced-expiratory volume in 1-s (FEV 1) (OR = 10.18 (95%CI 2,32–44,68), p = 0,002). In patients with OSAS, age and nocturnal hypoxemia (NH) (OR = 2.41 (95%CI 1,07–5,41), p = 0,03) were risk factors, while adherence to nighttime positive airway pressure (PAP) reduced mortality (OR = 0,36 (95%CI 0,15–0,83), p = 0,017). Multivariate analysis confirmed age and FEV 1 as risk factors in OS. Conversely, the risk factors for the overall population under 65 years were NH, which is confirmed in patients with OSAS alone (OR = 4,72 (95%CI 1,07–20,77), p = 0,04) in whom, on the other hand, PAP compliance reduced the mortality risk. The study suggests that NH is a risk factor for all-cause mortality in sleep disorders by excluding the age; conversely, nighttime PAP improves the survival. • OSAS and COPD are two widespread diseases leading to increased morbidity and mortality. • Overlap Syndrome increases the mortality risk compared to OSAS alone. • Age over 65 years is a common mortality risk factor for OSAS and OS. • Impaired lung function and nocturnal hypoxemia are factors that influence survival. • In younger subjects, nocturnal hypoxemia increases the mortality risk, whereas PAP adherence improves survival. [ABSTRACT FROM AUTHOR]

PurposeObstructive sleep apnea syndrome (OSAS) and chronic obstructive pulmonary disease (COPD) are prevalent disorders, and the concurrence so-called overlap syndrome (OVS) is not rare either. Early recognition of OVS is essential because this group is more prone to cardiovascular morbidities and requires effective multidisciplinary follow-up. This study aimed to evaluate RDW in patients with severe OSAS and investigate whether it can predict OVS.Patients and methods96 patients were retrospectively analyzed, of whom 66 were found to have severe OSAS alone and 30 OVS during diagnostic workups. Demographic, polysomnographic, and laboratory results, including RDW, were compared between groups. Multivariate logistic regression was used to determine independent associates of OVS.ResultsGender and body mass index (BMI) were similar, however, the mean age and RDW were higher in the OVS group (p:0.008, p:0.002). The increase in RDW remained significant after adjustment for age, BMI, and cardiovascular risk factors. An RDW value of >13.65% was shown to have a 78.3% sensitivity and 60% specificity for predicting OVS in severe OSAS (p:0.004).ConclusionThe results suggest that RDW can be a reliable indicator for diagnosing OVS in OSAS. It can help in identifying the subset of patients who would benefit from proper consultations and multidisciplinary follow-up, leading to appropriate treatment of each disease component and effective monitoring to prevent adverse cardiovascular outcomes.

Kikuchi-Fujimoto disease (KFD) is a benign, self-limiting histiocytic necrotizing lymphadenitis systemic disorder with unknown etiology. KFD has been known for half a century, but difficulties in distinguishing it remain. Its diagnostic significance is related to the increasing prevalence of KFD with autoimmune diseases in various timeframes. Systemic lupus erythematosus (SLE) is the most prevalent autoimmune connective tissue disease (AICTD) appearing alongside KFD. An 18-year-old female presented with acute muscle weakness, shortness of breath, fever, and significant weight loss for 5 months before admission. Pain and morning joint stiffness had been felt for 9 months. One year ago, she lumped her right neck and was diagnosed with KFD from the excision biopsy and immunohistochemical staining (CD68). Creatine-kinase enzymes and C-Reactive protein were elevated with a high anti-Ku and anti-Jo-1 negative level. There was a low level of complements, high anti-nuclear antibody titer, with positive anti-SS-A. Sialometry and Schirmer test showed reduced salivary and lacrimal gland production. We diagnosed this patient as having an overlap syndrome preceded by KFD. The AICTD involved was Sjögren’s syndrome and SLE. Although KFD is considered a self-limiting disease, its occurrence should be noticed regarding the possibility of other autoimmune conditions. KFD usually coincides with AICTD, although it could also precede or occur afterward. This case is reported to raise awareness of the overlap syndrome preceded by KFD.

Abstract Background The co-existence of chronic obstructive pulmonary disease (COPD) and obstructive sleep apnea (OSA) is known as overlap syndrome, and both conditions share common risk factors and are associated with co-morbidities and poor outcomes. Methodology Sixty stable COPD patients were included in the study. We assessed body measurements, pulmonary functions to diagnose and assess COPD severity, arterial blood gases, STOB-BANG questionnaire (SBQ), Epworth sleepiness scale (ESS), and polysomnography (PSG) for diagnosis and classification of OSA severity. The aim of the study is to assess predictors of OSA among COPD patients. Results The prevalence of overlap syndrome was 70% among studied stable COPD patients, with a male-to-female ratio of 2:1, and SBQ and ESS were statistically higher in overlap syndrome with p values

Background. Stress, individual characteristics of each patient, visceral hypersensitivity and intestinal motility have the key importance in the pathogenesis of irritable bowel syndrome (IBS). In recent years, there has been growing interest in the use of selective serotonin and norepinephrine reuptake inhibitors (SNRIs) in the complex therapy of IBS patients with somatoform disorders. Aim. To examine the effectiveness of the SNRIs antidepressant therapy in the treatment of patients with IBS and diarrhea (IBS-D) with extraintestinal manifestations. Materials and methods. 42 patients with severe IBS and diarrhea (IBS-D) were examined, among them 22 female with a median age of 32 years old (22; 38), and 20 male with a median age of 31 years old (25; 35). Treatment with duloxetine 60 mg/day was prescribed. The effectiveness of the therapy was assessed after eight weeks. The IBS clinical symptoms dynamics were assessed by the intensity of pain syndrome and bloating, which were determined using Visual Analogue Pain Scale (VAS), stool frequency and shape based on the Bristol stool scale; Visceral sensitivity threshold was assessed according to the Balloon dilatation test. There was studied the effect of the duloxetine on the extraintestinal manifestations of IBS. The psycho-emotional state was assessed using the Beck scale of anxiety and depression and the SpielbergerKhanin scale by psychiatrist, neurologist-vegetol. Results. All patients showed positive dynamics after eight weeks duloxetine treatment: the decrease of pain syndrome from 9 (9; 10) to 2 (2; 3) points, bloating from 8 (8; 9) points to 2,5 (1; 3) points according to VAS, and defecation frequency from 10 (9; 12) to 2 (1; 2) times a day; the change of stool consistency from 6th (6; 7) to 3rd (3; 4) type. The visceral sensitivity threshold increased: the time of appearance of the first urge to defecate increased from 56 (34; 74) ml to 95 (80; 98) ml. Significantly decreased extraintestinal manifestations of IBS. In reassessing each patients individual characteristics there were the decrease of the depression level according to the Beck scale from 26 (23; 32) to 11.5 (10; 13) points and personal personal anxiety level according to the SpielbergerKhanin scale from 42.5 (35; 53) to 22 (20; 24) points, as well as the decrease of situational anxiety from 40 (37; 49) to 22 (21; 36) points. Conclusion. The severe course of IBS-D is mainly associated with the patients individual characteristics and anxiety or anxiety-depressive syndromes. The positive impact of duloxetine therapy in severe IBS-D with extraintestinal manifestations is associated with the regulation of serotonergic and noradrenergic activity of the central.

The overlap syndrome indicates an association between autoimmune hepatitis (AIH) and sclerosis cholangitis or primary biliary cholangitis (PBC). We report a case of a 32-year-old patient who experienced overlap syndrome ten days after receiving the first dose of the mRNA Coronavirus disease-2019 (COVID-19) vaccine. Liver biopsy showed primarily AIH and “overlap syndrome” for PBC. Serum IgG was elevated, and ANA (1/100) and anti-mitochondrial antibody was positive. The patient’s treatment plan included prednisolone, ursodeoxycholic acid and azathioprine. Overlap syndrome related to the mRNA COVID-19 vaccines is a very rare entity. After the histopathological diagnosis of the patient, remission was achieved following treatment.

A close relationship between inflammatory bowel diseases (IBD) and hepatobiliary disorders, such as autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC), is well established. Hepatobiliary manifestations occur in 30% of patients with IBD, and 5% develop chronic liver disease. The spectrum of symptoms of the overlap syndrome ranges from asymptomatic elevated liver function tests to serious diseases. The pathogenesis of liver complications in IBD is not fully recognized. It may either have an autoimmune background or be caused by intestinal inflammation, metabolic impairment, or drug toxicity. The aim of this review is to summarize AIH and PSC overlap syndrome in IBD.

Jeevagan A. Int J Gen Med. 2010;3:143–146. We, the Editor and Publisher of the journal International Journal of General Medicine, have retracted the following article. Following publication of the article, concerns were raised that images used for Figures 3 and 4 were not derived from the patient described in the case report but were obtained from unrelated sources. Specifically, The image for Figure 3 was previously reported by Radiopaedia.org in a case by Yang N, from 2009 - https://radiopaedia.org/cases/primary-sclerosing-cholangitis-psc-1. The image for Figure 4 was previously reported by Radiology Assistant in a case by Levy AD, from 2009 - https://radiologyassistant.nl/abdomen/biliary-system/biliary-duct-pathology. The author did not respond to our queries and was unable to provide a satisfactory explanation for the duplicated images or provide satisfactory data for the study. As verifying the validity of published work is core to the integrity of the scholarly record, the Publisher and Editor requested to retract the article and the author was notified of this. We have been informed in our decision-making by our editorial policies and COPE guidelines. The retracted article will remain online to maintain the scholarly record, but it will be digitally watermarked on each page as “Retracted”.