{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,12]],"date-time":"2026-04-12T19:46:15Z","timestamp":1776023175731,"version":"3.50.1"},"reference-count":20,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2026,3,24]],"date-time":"2026-03-24T00:00:00Z","timestamp":1774310400000},"content-version":"vor","delay-in-days":82,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,3,18]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>To describe a novel radiographic phenotype of systemic autoimmune rheumatic disease\u2013associated interstitial lung disease (SARD-ILD) observed within a regional specialist multidisciplinary (MDT) service.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This was a retrospective case series of patients with SARD-ILD complicated by radiographic pulmonary cystic destruction. Cases were identified through review of MDT records from the North Bristol SARD-ILD service. Cases with MDT reported \u2018cystic changes\u2019 were identified and clinico-radio-pathological features reviewed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Five cases of SSc-associated ILD (SSc-ILD) and two cases of antisynthetase syndrome\u2013associated ILD (ASyS-ILD) with cystic changes were identified among a total of 108 SARD-ILD patients. All seven cases presented with radiological patterns of cellular non-specific interstitial pneumonia (NSIP). The median age at diagnosis with SARD was 33\u2009years and six cases had ILD identified within 6\u2009months of SARD diagnosis. The cohort was ethnically diverse, with one ex-smoker. Microcystic destructive changes appeared and progressed within areas of ground glass despite standard-of-care immunomodulation. Changes are radiographically and histologically distinct from traction bronchiolectasis, honeycombing and smoking-related lung disease. Histological specimens were available for two cases confirming fibrotic NSIP, with one including affected tissue demonstrating intimal thickening of the pulmonary vasculature. Over a median follow-up of 49\u2009months, all cases remain alive and transplant free; five fulfilled criteria for progressive disease and six required ambulatory oxygen.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>This represents the first western cohort describing microcystic destructive pulmonary changes in SSc-ILD and the first report in ASyS-ILD. Systematic case identification is required to determine demographic associations and prognostic implications.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/rap\/rkag030","type":"journal-article","created":{"date-parts":[[2026,3,21]],"date-time":"2026-03-21T12:40:21Z","timestamp":1774096821000},"source":"Crossref","is-referenced-by-count":0,"title":["Pulmonary cystic destruction in systemic sclerosis and antisynthetase syndrome associated non-specific interstitial pneumonitis: a case series of a novel radiological phenotype"],"prefix":"10.1093","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8683-3358","authenticated-orcid":false,"given":"Matthew","family":"Wells","sequence":"first","affiliation":[{"name":"Bristol Interstitial Lung Disease Service, North Bristol NHS Trust , Bristol,","place":["UK"]},{"name":"Department of Clinical Specialties and Related Sciences, University of Bristol , Bristol,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giles","family":"Dixon","sequence":"additional","affiliation":[{"name":"Bristol Interstitial Lung Disease Service, North Bristol NHS Trust , Bristol,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nidhi","family":"Bhatt","sequence":"additional","affiliation":[{"name":"Bristol Interstitial Lung Disease Service, North Bristol NHS Trust , Bristol,","place":["UK"]},{"name":"Department of Pathology, North Bristol NHS Trust , Bristol,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jo","family":"Hardy","sequence":"additional","affiliation":[{"name":"Bristol Interstitial Lung Disease Service, North Bristol NHS Trust , Bristol,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8230-175X","authenticated-orcid":false,"given":"Huzaifa I","family":"Adamali","sequence":"additional","affiliation":[{"name":"Bristol Interstitial Lung Disease Service, North Bristol NHS Trust , Bristol,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Harsha","family":"Gunawardena","sequence":"additional","affiliation":[{"name":"Bristol Interstitial Lung Disease Service, North Bristol NHS Trust , Bristol,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2793-2364","authenticated-orcid":false,"given":"John D","family":"Pauling","sequence":"additional","affiliation":[{"name":"Bristol Interstitial Lung Disease Service, North Bristol NHS Trust , Bristol,","place":["UK"]},{"name":"Translational Health Sciences, Bristol Medical School, University of Bristol , Bristol,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3067-7349","authenticated-orcid":false,"given":"Shaney L","family":"Barratt","sequence":"additional","affiliation":[{"name":"Bristol Interstitial Lung Disease Service, North Bristol NHS Trust , Bristol,","place":["UK"]},{"name":"Translational Health Sciences, Bristol Medical School, University of Bristol , Bristol,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anthony","family":"Edey","sequence":"additional","affiliation":[{"name":"Bristol Interstitial Lung Disease Service, North Bristol NHS Trust , Bristol,","place":["UK"]},{"name":"Department of Radiology, North Bristol NHS Trust , Bristol,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2026,3,24]]},"reference":[{"key":"2026041215335914700_rkag030-B1","doi-asserted-by":"publisher","first-page":"2688","DOI":"10.1093\/rheumatology\/keaa680","article-title":"Epidemiology of systemic sclerosis in the UK: an analysis of the Clinical Practice Research Datalink","volume":"60","author":"Pauling","year":"2021","journal-title":"Rheumatology (Oxford)"},{"key":"2026041215335914700_rkag030-B2","doi-asserted-by":"publisher","first-page":"rky035","DOI":"10.1093\/rap\/rky035","article-title":"Increasing incidence of adult idiopathic inflammatory myopathies in the City of Salford, UK: a 10-year epidemiological study","volume":"2","author":"Parker","year":"2018","journal-title":"Rheumatol Adv Pract"},{"key":"2026041215335914700_rkag030-B3","doi-asserted-by":"publisher","first-page":"959653","DOI":"10.3389\/fmed.2022.959653","article-title":"A multidisciplinary approach to the diagnosis of antisynthetase syndrome","volume":"9","author":"Wells","year":"2022","journal-title":"Front Med (Lausanne)"},{"key":"2026041215335914700_rkag030-B4","doi-asserted-by":"publisher","first-page":"754","DOI":"10.1136\/ard.2006.062901","article-title":"Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group database","volume":"66","author":"Walker","year":"2007","journal-title":"Ann Rheum Dis"},{"key":"2026041215335914700_rkag030-B5","doi-asserted-by":"publisher","first-page":"30","DOI":"10.1136\/annrheumdis-2017-211868","article-title":"The EuroMyositis registry: an international collaborative tool to facilitate myositis research","volume":"77","author":"Lilleker","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"2026041215335914700_rkag030-B6","doi-asserted-by":"publisher","first-page":"2734","DOI":"10.1093\/rheumatology\/keae076","article-title":"Lung imaging patterns in connective tissue disease\u2013associated interstitial lung disease impact prognosis and immunosuppression response","volume":"63","author":"Zheng","year":"2024","journal-title":"Rheumatology (Oxford)"},{"key":"2026041215335914700_rkag030-B7","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1186\/s12931-019-0980-7","article-title":"Interstitial lung disease associated with systemic sclerosis (SSc-ILD)","volume":"20","author":"Cottin","year":"2019","journal-title":"Respir Res"},{"key":"2026041215335914700_rkag030-B8","doi-asserted-by":"publisher","first-page":"e0162231","DOI":"10.1371\/journal.pone.0162231","article-title":"Differentiating smoking-related interstitial fibrosis (SRIF) from usual interstitial pneumonia (UIP) with emphysema using CT features based on pathologically proven cases","volume":"11","author":"Chae","year":"2016","journal-title":"PLoS One"},{"key":"2026041215335914700_rkag030-B9","doi-asserted-by":"publisher","first-page":"206","DOI":"10.1111\/pin.12054","article-title":"Airspace enlargement with fibrosis shows characteristic histology and immunohistology different from usual interstitial pneumonia, nonspecific interstitial pneumonia and centrilobular emphysema","volume":"63","author":"Yamada","year":"2013","journal-title":"Pathol Int"},{"key":"2026041215335914700_rkag030-B10","doi-asserted-by":"publisher","first-page":"98","DOI":"10.1016\/j.semarthrit.2018.10.011","article-title":"Combined pulmonary fibrosis and emphysema in systemic sclerosis: a syndrome associated with heavy morbidity and mortality","volume":"49","author":"Champtiaux","year":"2019","journal-title":"Semin Arthritis Rheum"},{"key":"2026041215335914700_rkag030-B11","doi-asserted-by":"publisher","first-page":"100","DOI":"10.1186\/s13075-021-02494-y","article-title":"Effect of combined pulmonary fibrosis and emphysema on patients with connective tissue diseases and systemic sclerosis: a systematic review and meta-analysis","volume":"23","author":"Koo","year":"2021","journal-title":"Arthritis Res Ther"},{"key":"2026041215335914700_rkag030-B12","doi-asserted-by":"publisher","first-page":"e0161908","DOI":"10.1371\/journal.pone.0161908","article-title":"Clinical features of idiopathic interstitial pneumonia with systemic sclerosis-related autoantibody in comparison with interstitial pneumonia with systemic sclerosis","volume":"11","author":"Yamakawa","year":"2016","journal-title":"PLoS One"},{"key":"2026041215335914700_rkag030-B13","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1186\/s12890-018-0591-y","article-title":"Emphysematous change with scleroderma-associated interstitial lung disease: the potential contribution of vasculopathy?","volume":"18","author":"Yamakawa","year":"2018","journal-title":"BMC Pulm Med"},{"key":"2026041215335914700_rkag030-B14","doi-asserted-by":"publisher","first-page":"1718","DOI":"10.1056\/NEJMoa1908681","article-title":"Nintedanib in progressive fibrosing interstitial lung diseases","volume":"381","author":"Flaherty","year":"2019","journal-title":"N Engl J Med"},{"key":"2026041215335914700_rkag030-B15","doi-asserted-by":"publisher","first-page":"e44","DOI":"10.1164\/rccm.201807-1255ST","article-title":"Diagnosis of idiopathic pulmonary fibrosis. An official ATS\/ERS\/JRS\/ALAT clinical practice guideline","volume":"198","author":"Raghu","year":"2018","journal-title":"Am J Respir Crit Care Med"},{"key":"2026041215335914700_rkag030-B16","doi-asserted-by":"publisher","first-page":"560","DOI":"10.1148\/radiol.2322031223","article-title":"CT features of lung disease in patients with systemic sclerosis: comparison with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia","volume":"232","author":"Desai","year":"2004","journal-title":"Radiology"},{"key":"2026041215335914700_rkag030-B17","doi-asserted-by":"publisher","first-page":"1747","DOI":"10.1136\/annrheumdis-2013-204424","article-title":"2013 classification criteria for systemic sclerosis: an American College of Rheumatology\/European League Against Rheumatism collaborative initiative","volume":"72","author":"van den Hoogen","year":"2013","journal-title":"Ann Rheum Dis"},{"key":"2026041215335914700_rkag030-B18","first-page":"abstract L07","article-title":"The classification criteria for anti-synthetase syndrome (CLASS) project [abstract]","volume":"76(Suppl 9)","author":"Zanframundo","year":"2024","journal-title":"Arthritis Rheumatol"},{"key":"2026041215335914700_rkag030-B19","doi-asserted-by":"publisher","first-page":"211","DOI":"10.1016\/j.rmcr.2018.09.009","article-title":"Emphysema formation in a never-smoker with scleroderma-related interstitial pneumonia","volume":"25","author":"Yamakawa","year":"2018","journal-title":"Respir Med Case Rep"},{"key":"2026041215335914700_rkag030-B20","doi-asserted-by":"publisher","first-page":"371","DOI":"10.1183\/09031936.00106008","article-title":"Pulmonary arterial hypertension in limited cutaneous systemic sclerosis: a distinctive vasculopathy","volume":"34","author":"Overbeek","year":"2009","journal-title":"Eur Respir J"}],"container-title":["Rheumatology Advances in Practice"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/rheumap\/advance-article-pdf\/doi\/10.1093\/rap\/rkag030\/67502286\/rkag030.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/rheumap\/article-pdf\/10\/2\/rkag030\/67502286\/rkag030.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/rheumap\/article-pdf\/10\/2\/rkag030\/67502286\/rkag030.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,12]],"date-time":"2026-04-12T19:34:09Z","timestamp":1776022449000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/rheumap\/article\/doi\/10.1093\/rap\/rkag030\/8539948"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026]]},"references-count":20,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2026,3,18]]}},"URL":"https:\/\/doi.org\/10.1093\/rap\/rkag030","relation":{},"ISSN":["2514-1775"],"issn-type":[{"value":"2514-1775","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2026]]},"published":{"date-parts":[[2026]]},"article-number":"rkag030"}}