PMID- 34471427 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240403 IS - 1759-720X (Print) IS - 1759-7218 (Electronic) IS - 1759-720X (Linking) VI - 13 DP - 2021 TI - Natural history and screening of interstitial lung disease in systemic autoimmune rheumatic disorders. PG - 1759720X211037519 LID - 10.1177/1759720X211037519 [doi] LID - 1759720X211037519 AB - Interstitial lung disease (ILD) is a relatively frequent manifestation of systemic autoimmune rheumatic disorders (SARDs), including systemic sclerosis (SSc), rheumatoid arthritis (RA), idiopathic inflammatory myopathies (IIM), systemic lupus erythematosus (SLE), primary Sjogren's syndrome (pSS), and anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis. Interstitial pneumonia with autoimmune features (IPAF) has been proposed to describe patients with ILD who have clinical or serological findings compatible with SARDs but they are not sufficient for a definite diagnosis. ILD may present with different patterns among patients with SARDs, but most commonly as nonspecific interstitial pneumonia (NSIP), with the exception of RA and ANCA vasculitis that more often present with usual interstitial pneumonia (UIP). The natural history of ILD is quite variable, even among patients with the same SARD. It may present with subclinical features following a slow progressively course or with acute manifestations and clinically significant rapid progression leading to severe deterioration of pulmonary function and respiratory failure. The radiographic pattern of ILD, the extent of the disease, the baseline pulmonary function, the pulmonary function deterioration rate over time and clinical variables related to the primary SARD, such as age, sex and the clinical phenotype, are considered prognostic factors for SARDs-ILD associated with adverse outcomes and increased mortality. Different modalities can be employed for ILD detection including clinical evaluation, pulmonary function tests, high resolution computed tomography and novel techniques such as lung ultrasound and serum biomarkers. ILD may determine the clinical outcome of SARDs, since it is associated with significant morbidity and mortality and therefore screening of patients with SARDs for ILD is of great clinical importance. CI - (c) The Author(s), 2021. FAU - Panagopoulos, Panagiotis AU - Panagopoulos P AUID- ORCID: 0000-0002-1630-7045 AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. FAU - Goules, Andreas AU - Goules A AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece. FAU - Hoffmann-Vold, Anna Maria AU - Hoffmann-Vold AM AUID- ORCID: 0000-0001-6467-7422 AD - Department of Rheumatology, Oslo University Hospital, Oslo, Norway. FAU - Matteson, Eric L AU - Matteson EL AD - Division of Rheumatology, Mayo Clinic College of Medicine and Science, Rochester, MN, USA. FAU - Tzioufas, Athanasios AU - Tzioufas A AD - Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Mikras Asias 75, Athens 11527, Greece. LA - eng PT - Journal Article PT - Review DEP - 20210828 PL - England TA - Ther Adv Musculoskelet Dis JT - Therapeutic advances in musculoskeletal disease JID - 101517322 PMC - PMC8404673 OTO - NOTNLM OT - Sjogren's syndrome OT - anti-synthetase syndrome OT - dermatomyositis OT - interstitial lung disease OT - interstitial pneumonia with autoimmune features OT - natural history OT - polymyositis OT - rheumatoid arthritis OT - screening OT - systemic lupus erythematosus OT - systemic sclerosis OT - vasculitis COIS- Conflict of interest statement: The authors declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: E.M.: Consultant/advisory board, speaker (Boehringer-Ingelheim); speaker: (Novartis); editor, writer (UpToDate). A.M.H.V. has received research funding and/or consulting fees and/or other remuneration from Actelion, Boehringer Ingelheim, Roche, Bayer, Merck Sharp & Dohme, ARXX, Lilly and Medscape. P.P., A.G. and A.T. declare no conflict of interest. EDAT- 2021/09/03 06:00 MHDA- 2021/09/03 06:01 PMCR- 2021/08/28 CRDT- 2021/09/02 06:55 PHST- 2021/04/10 00:00 [received] PHST- 2021/07/19 00:00 [accepted] PHST- 2021/09/02 06:55 [entrez] PHST- 2021/09/03 06:00 [pubmed] PHST- 2021/09/03 06:01 [medline] PHST- 2021/08/28 00:00 [pmc-release] AID - 10.1177_1759720X211037519 [pii] AID - 10.1177/1759720X211037519 [doi] PST - epublish SO - Ther Adv Musculoskelet Dis. 2021 Aug 28;13:1759720X211037519. doi: 10.1177/1759720X211037519. eCollection 2021.