Recognition and Management of Myositis-Associated Rapidly Progressive Interstitial Lung Disease.

Recognition and Management of Myositis-Associated Rapidly Progressive Interstitial Lung Disease. Chest. 2020 Feb 11;: Authors: Jablonski R, Bhorade S, Strek ME, Dematte J Abstract Idiopathic inflammatory myopathies are autoimmune processes that are characterized by skeletal muscle inflammation. The lung is the most commonly involved extramuscular organ and, when present, pulmonary disease drives morbidity and mortality. A subset of patients can present with rapidly progressive hypoxemic respiratory failure due to myositis-related interstitial lung disease. Confirmatory autoantibody testing requires sending samples to a reference laboratory, thus diagnosis of rapidly progressive myositis-associated interstitial lung disease relies on a high index of suspicion and careful history and physical examination. Although the cornerstone of therapy for these patients remains multi-modality immunosuppression, emerging data supports a role for advanced therapies including extracorporeal membrane oxygenation and lung transplant in appropriately selected patients. It is hoped that greater awareness of the clinical features of this syndrome will allow for appropriate diagnosis and treatment of these potentially treatable patients as well as raise awareness of the need for multicenter collaboration to prospectively study how to manage this complex disease. PMID: 32059958 [PubMed - as supplied by publisher]
Source: Chest - Category: Respiratory Medicine Authors: Tags: Chest Source Type: research

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Purpose of review This review provides a risk-stratified and evidence-based management for subsets of systemic sclerosis (SSc) patients in the first five years from disease onset. Recent findings Cardiopulmonary disease remains the primary cause of mortality in SSc patients. Morbidity and mortality in SSc-associated pulmonary arterial hypertension have improved with combination treatment, in either an upfront or sequential treatment pattern. Traditional therapies for interstitial lung disease (SSc-ILD) have targeted those with clinically significant and progressive ILD with immunosuppression. New data suggest a possib...
Source: Current Opinion in Rheumatology - Category: Rheumatology Tags: CLINICAL THERAPEUTICS AND HEMATOLOGIC COMPLICATIONS: Edited by W. Joseph McCune Source Type: research
In conclusion, we establish a new mouse model of COPA syndrome to identify a previously unknown function for Copa in thymocyte selection and demonstrate that a defect in central tolerance is a putative mechanism by which COPA mutations lead to autoimmunity in patients. PMID: 32198142 [PubMed - as supplied by publisher]
Source: Journal of Immunology - Category: Allergy & Immunology Authors: Tags: J Immunol Source Type: research
CONCLUSION: Current drug studies may provide innovative therapeutic perspectives for SSc-ILD and could significantly improve the prognosis of affected patients in the future. PMID: 31754786 [PubMed - as supplied by publisher]
Source: Zeitschrift fur Rheumatologie - Category: Rheumatology Authors: Tags: Z Rheumatol Source Type: research
We report on novel therapeutic approaches such as GM-CSF substitution, stem cell transplantation, pioglitazone, statins and immunomodulation. Recent findings The discovery of new genetic mutations underlying this syndrome raises the question whether the classification should be radically revised in the future. Serum GM-CSF autoantibody is the best diagnostic marker for autoimmune PAP, the most common form, but does not correlate with disease severity. Several circulating biomarkers have been investigated to assess disease activity and predict outcome. Imaging techniques have also enormously evolved and offer new tools t...
Source: Current Opinion in Pulmonary Medicine - Category: Respiratory Medicine Tags: INTERSTITIAL LUNG DISEASE: Edited by Francesco Bonella and Paolo Spagnolo Source Type: research
Abstract The association between inflammatory myopathies anti-synthetase syndrome and interstitial lung disease has been recognized since the 1950s. Patients generally present with gradual onset of symptoms and slow progression of fibrosis over months to years. Herein, we describe a previously well 51-year-old man who presented with three months of progressive small joint arthritis, cough, dyspnea, and eventually hypoxemic respiratory failure following a viral prodrome. He continued to decompensate despite high dose corticosteroids and mycophenolate mofetil, ultimately requiring extracorporeal membranous oxygenati...
Source: Respiratory Care - Category: Respiratory Medicine Authors: Tags: Respir Med Case Rep Source Type: research
Publication date: Available online 15 June 2019Source: Respiratory Medicine Case ReportsAuthor(s): Andrew R. Deitchman, Or Kalchiem-Dekel, Nevins Todd, Robert M. ReedAbstractThe association between inflammatory myopathies anti-synthetase syndrome and interstitial lung disease has been recognized since the 1950s. Patients generally present with gradual onset of symptoms and slow progression of fibrosis over months to years. Herein, we describe a previously well 51-year-old man who presented with three months of progressive small joint arthritis, cough, dyspnea, and eventually hypoxemic respiratory failure following a viral ...
Source: Respiratory Medicine Case Reports - Category: Respiratory Medicine Source Type: research
CONCLUSIONS: ANCA-antibody positivity is uncommon in North American patients with IPF and not associated with baseline disease severity or transplant-free survival; however, a significant proportion of MPO-positive IPF patients develop clinical vasculitis.; Abbreviation List: ANCA: Antineutrophil cytoplasmic antibodies; CLIA: Clinical Laboratory Improvement Amendments; CT: Computed tomography; CTD: Connective tissue disease; DLCO: Diffusing capacity of the lung for carbon monoxide; FEV1: Forced expiratory volume in one second; FVC: Forced vital capacity; HR: Hazard ratio; ILD: Interstitial lung disease; IPF: Idiopathic pul...
Source: Chest - Category: Respiratory Medicine Authors: Tags: Chest Source Type: research
Leukocyte telomere length (LTL), MUC5B rs35705950 and TOLLIP rs5743890 have been associated with idiopathic pulmonary fibrosis (IPF). In this observational cohort study, we assessed the associations between these genomic markers and outcomes of survival and rate of disease progression in patients with interstitial pneumonia with autoimmune features (IPAF, n=250) and connective tissue disease-associated interstitial lung disease (CTD-ILD, n=248). IPF (n=499) was used as a comparator. The LTL of IPAF and CTD-ILD patients (mean age-adjusted log-transformed T/S of –0.05±0.29 and –0.04±0.25, ...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Interstitial and orphan lung disease Original Articles: Interstitial lung disease Source Type: research
ConclusionsSTAT3 GOF syndrome is a new clinical entity to consider when confronted with a patient with early-onset poly-autoimmunity, lymphoproliferation, and growth failure. At this time, precise therapeutic guidelines are lacking, but use of anti-IL-6 receptor and JAK inhibitor biologics are attractive possibilities.
Source: The Journal of Allergy and Clinical Immunology: In Practice - Category: Allergy & Immunology Source Type: research
Regulator of telomere length 1 (RTEL1) mutations have been evidenced in 5–9% of familial pulmonary fibrosis; however, the phenotype of patients with interstitial lung disease (ILD) and RTEL1 mutations is poorly understood. Whole exome sequencing was performed in 252 probands with ILD and we included all patients with ILD and RTEL1 mutation. RTEL1 expression was evaluated by immunochemistry in the lungs of controls, as well as in RTEL1 and telomerase reverse transcriptase (TERT) mutation carriers. We identified 35 subjects from 17 families. Median age at diagnosis of ILD was 53.1 years (range 28.0–80.6). Th...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Interstitial and orphan lung disease Original Articles: Interstitial lung diseases Source Type: research
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