Progressive fibrosing interstitial lung disease associated with systemic autoimmune diseases

AbstractInterstitial lung disease (ILD) is a common manifestation of systemic autoimmune diseases and a leading cause of death in these patients. A proportion of patients with autoimmune ILDs develop a progressive fibrosing form of ILD, characterized by increasing fibrosis on high-resolution computed tomography, worsening of lung function, and early mortality. Autoimmune disease –related ILDs have a variable clinical course and not all patients will require treatment, but all patients should be monitored for signs of progression. Apart from systemic sclerosis–associated ILD, the limited evidence to support the efficacy of immunosuppression as a treatment for ILDs is bas ed mainly on small retrospective series and expert opinion. Non-clinical data suggest that there are commonalities in the mechanisms that drive progressive fibrosis in ILDs with an immunological trigger as in other forms of progressive fibrosing ILD. This suggests that nintedanib and pirfenidone, dr ugs known to slow disease progression in patients with idiopathic pulmonary fibrosis, may also slow the progression of ILD associated with systemic autoimmune diseases. In the SENSCIS® trial, nintedanib reduced the rate of ILD progression in patients with systemic sclerosis–associated ILD. The re sults of other large clinical trials will provide further insights into the role of anti-fibrotic therapies in the treatment of autoimmune disease–related ILDs.
Source: Clinical Rheumatology - Category: Rheumatology Source Type: research

Related Links:

ConclusionAntisynthetase syndrome is a rare clinical entity which apart from clinical presentation requires specific serological investigations for diagnosis. Concomitant association of interstitial lung disease gives it a guarded prognosis.
Source: Journal of Medical Case Reports - Category: General 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
CONCLUSIONS: The current clinical case emphasizes issues that a physician may encounter in the differential diagnostics of amiodarone-induced lung toxicity with other lung diseases. PMID: 30594249 [PubMed - in process]
Source: BMC Pharmacology and Toxicology - Category: Drugs & Pharmacology Tags: BMC Pharmacol Toxicol Source Type: research
Background: Non Specific Interstitial Pneumonia (NSIP) is a complication of many connective tissue diseases (CTD). Patients with interstitial lung disease (ILD) without a definitive diagnosis of CTD were historically described as undifferentiated connective tissue disease (UCTD-ILD). Recently a new classification, interstitial lung disease with autoimmune features (IPAF), has been proposed. Aim of this study was to describe the prevalence, clinical characteristics and prognostic factors of UCTD and IPAF subjects in a cohort of NSIP patients.Methods: This was a retrospective, observational study enrolling 102 adult patients...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: ILD/DPLD of known origin Source Type: research
We report a case of an 18-month-old boy with severe respiratory failure. At 16 months of age he was hospitalised for acute respiratory distress. Chest X-ray showed a large bilateral parenchymal opacities, while the chest CT scan showed a bilateral picture of ground-glass opacification. Due the difficulty of oxygen weaning and considering the poor nutritional condition, the baby was transferred to our hospital. The chest CT scan images and the clinical course were suggestive interstitial lung disease1. The cytological analysis performed on bronchoalveolar lavage showed abundant periodic acid-Schiff staining material. Repeat...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Paediatric bronchology Source Type: research
Authors: Biffi A, Dei G, De Giacomi F, Stainer A, Parma LO, Pozzi MR, Faverio P, Pesci A Abstract Patients with Interstitial Lung Disease (ILD) without a definitive diagnosis of connective tissue diseases (CTD) were historically described as Undifferentiated Connective Tissue Disease (UCTD-ILD). Recently a new classification, Interstitial Pneumonia with Autoimmune Features (IPAF), has been proposed. Aim of this study was to describe the prevalence, clinical characteristics and prognostic factors of UCTD and IPAF subjects in a cohort of Non-Specific Interstitial Pneumonia (NSIP) patients. This retrospective, observa...
Source: Monaldi Archives for Chest Disease - Category: Respiratory Medicine Tags: Monaldi Arch Chest Dis Source Type: research
AbstractTo investigate the clinical features, risk factors and outcomes of patients with interstitial pneumonia with autoimmune features (IPAF). A total of 1429 patients with idiopathic interstitial pneumonia (IIP) and undifferentiated connective tissue disease-associated interstitial lung disease (UCTD-ILD) were screened to identify patients who met IPAF criteria. Clinical, serological, and morphological features of patients with IPAF were characterized. Outcomes between patients with IPAF, UCTD-ILD, and IIP who were divided into idiopathic pulmonary fibrosis (IPF) and non-IPF groups were compared using survival as an end...
Source: Clinical Rheumatology - Category: Rheumatology Source Type: research
This study impacts the RA and ILD fields by helping to identify a therapeutic treatment that may improve both diseases. As this model replicates the characteristics of RA-ILD, the results may be translatable to the human disease. PMID: 29543042 [PubMed - as supplied by publisher]
Source: American Journal of Physiology. Lung Cellular and Molecular Physiology - Category: Cytology Authors: Tags: Am J Physiol Lung Cell Mol Physiol Source Type: research
This article is protected by copyright. All rights reserved.
Source: Arthritis Care and Research - Category: Rheumatology Authors: Tags: Clinicopathologic Conference Source Type: research
CONCLUSIONS: This review, which is based on studies of varying methodological quality, demonstrates that overall, in this population, small benefit may be derived from the use of cyclophosphamide in terms of mean difference in % FVC when compared with placebo, but not of the difference in % DLCO, or when compared with mycophenolate. Modest clinical improvement in dyspnoea may be noted with the use of cyclophosphamide. Clinical practice guidelines should advise clinicians to consider individual patient characteristics and to expect only modest benefit at best in preserving FVC. Clinicians should carefully monitor for advers...
Source: Cochrane Database of Systematic Reviews - Category: General Medicine Authors: Tags: Cochrane Database Syst Rev Source Type: research
More News: Allergy & Immunology | Autoimmune Disease | Clinical Trials | CT Scan | Interstitial Lung Disease | Rheumatology