{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:17:45Z","timestamp":1784557065720,"version":"3.55.0"},"reference-count":21,"publisher":"BMJ","issue":"7","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2026,7,7]]},"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:p>A man in his 80s with chronic obstructive pulmonary disease presented with fever, arthralgia and periorbital erythema resembling a heliotrope rash, accompanied by progressive interstitial lung disease (ILD). Laboratory investigations revealed neutrophilic leucocytosis and markedly elevated C-reactive protein, ferritin and Krebs von den Lungen-6. Dermatomyositis, particularly clinically amyopathic dermatomyositis, was considered in the differential diagnosis; however, myositis-specific autoantibodies were negative and histopathological findings supported a diagnosis of adult-onset Still\u2019s disease (AOSD). Despite the high-dose corticosteroids and ciclosporin, lung disease progressed to respiratory failure. Treatment with tocilizumab led to clinical and radiological remission with normalisation of inflammatory markers.<\/jats:p>\n                  <jats:p>This case highlights that AOSD can present with rapidly progressive ILD and closely mimic dermatomyositis, underscoring the importance of careful differential diagnosis and timely cytokine-targeted therapy.<\/jats:p>","DOI":"10.1136\/bcr-2026-273147","type":"journal-article","created":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T13:50:11Z","timestamp":1784555411000},"page":"e273147","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Adult-onset Still\u2019s disease presenting with periorbital erythematous rash and progressive interstitial lung disease"],"prefix":"10.1136","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-8343-3432","authenticated-orcid":false,"given":"Tomoko","family":"Oshima","sequence":"first","affiliation":[{"name":"Respiratory Medicine and Allergology, NHO Tenryu Hospital, Hamamatsu, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-0192-8547","authenticated-orcid":false,"given":"Seiich","family":"Miwa","sequence":"additional","affiliation":[{"name":"Respiratory Medicine and Allergology, NHO Tenryu Hospital, Hamamatsu, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-9707-2973","authenticated-orcid":false,"given":"Yasuhiro","family":"Ito","sequence":"additional","affiliation":[{"name":"Respiratory Medicine and Allergology, NHO Tenryu Hospital, Hamamatsu, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9043-881X","authenticated-orcid":false,"given":"Masahiro","family":"Shirai","sequence":"additional","affiliation":[{"name":"Respiratory Medicine and Allergology, NHO Tenryu Hospital, Hamamatsu, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2026,7,20]]},"reference":[{"key":"2026072006500549000_19.7.e273147.1","first-page":"424","article-title":"Preliminary criteria for classification of adult Still\u2019s disease","volume":"19","author":"Yamaguchi","year":"1992","journal-title":"J Rheumatol"},{"key":"2026072006500549000_19.7.e273147.2","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-200205000-00003"},{"key":"2026072006500549000_19.7.e273147.3","doi-asserted-by":"crossref","first-page":"e136","DOI":"10.1097\/RHU.0000000000000787","article-title":"Adult-Onset Still Disease Presenting With Insterstitial Lung Disease and Dermatomyositis-Like Atypical Persistent Eruption Successfully Treated With Mycophenolate Mofetil","volume":"25","author":"Mart\u00edn-Nares","year":"2019","journal-title":"J Clin Rheumatol"},{"key":"2026072006500549000_19.7.e273147.4","article-title":"Adult-Onset Still\u2019s Disease with Dermatomyositis-Like Lesions","volume":"12","author":"Segovia Rodr\u00edguez","year":"2025","journal-title":"Eur J Case Rep Intern Med"},{"key":"2026072006500549000_19.7.e273147.5","doi-asserted-by":"crossref","first-page":"969","DOI":"10.1016\/j.jaad.2018.05.020","article-title":"Increased severity and epidermal alterations in persistent versus evanescent skin lesions in adult-onset Still disease","volume":"79","author":"Zuelgaray","year":"2018","journal-title":"J Am Acad Dermatol"},{"key":"2026072006500549000_19.7.e273147.6","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1111\/1346-8138.16373","article-title":"Heliotrope-like manifestation of adult-onset Still disease with macrophage activation syndrome: A case-based review","volume":"49","author":"Ikeda","year":"2022","journal-title":"J Dermatol"},{"key":"2026072006500549000_19.7.e273147.7","doi-asserted-by":"publisher","DOI":"10.1111\/ced.15294"},{"key":"2026072006500549000_19.7.e273147.8","doi-asserted-by":"crossref","DOI":"10.1136\/bcr-2019-229332","article-title":"Fever with persistent flagellate erythema in a primigravida: a rare presentation of adult-onset Still\u2019s disease","volume":"12","author":"Bhatia","year":"2019","journal-title":"BMJ Case Rep"},{"key":"2026072006500549000_19.7.e273147.9","doi-asserted-by":"crossref","first-page":"851","DOI":"10.1097\/DAD.0000000000001447","article-title":"Adult-Onset Still Disease Presenting With Dermatomyositis-Like Persistent Pruritic Lesions","volume":"41","author":"Qiao","year":"2019","journal-title":"Am J Dermatopathol"},{"key":"2026072006500549000_19.7.e273147.10","doi-asserted-by":"crossref","first-page":"947","DOI":"10.1001\/archderm.148.8.947-b","article-title":"A 38-year-old woman with eyelid discoloration. Heliotrope-like manifestation of adult-onset Still disease (AOSD)","volume":"148","author":"Moraites","year":"2012","journal-title":"Arch Dermatol"},{"key":"2026072006500549000_19.7.e273147.11","doi-asserted-by":"crossref","first-page":"958","DOI":"10.1111\/j.1346-8138.2012.01549.x","article-title":"Adult-onset Still\u2019s disease with dermatomyositis-like eruption","volume":"39","author":"Ohashi","year":"2012","journal-title":"J Dermatol"},{"key":"2026072006500549000_19.7.e273147.12","doi-asserted-by":"crossref","DOI":"10.3389\/fmed.2022.989777","article-title":"Rare, rarer, lung involvement in adult-onset Still\u2019s disease: A mini-review","volume":"9","author":"Nies","year":"2022","journal-title":"Front Med (Lausanne)"},{"key":"2026072006500549000_19.7.e273147.13","doi-asserted-by":"publisher","DOI":"10.1186\/s13075-020-02245-5"},{"key":"2026072006500549000_19.7.e273147.14","first-page":"23","article-title":"Adult-onset Still\u2019s disease-associated interstitial lung disease represents severe phenotype of the disease with higher rate of haemophagocytic syndrome and relapse","volume":"37 Suppl 121","author":"Takakuwa","year":"2019","journal-title":"Clin Exp Rheumatol"},{"key":"2026072006500549000_19.7.e273147.15","doi-asserted-by":"crossref","first-page":"2271","DOI":"10.1002\/art.40320","article-title":"2017 European League Against Rheumatism\/American College of Rheumatology Classification Criteria for Adult and Juvenile Idiopathic Inflammatory Myopathies and Their Major Subgroups","volume":"69","author":"Lundberg","year":"2017","journal-title":"Arthritis Rheumatol"},{"key":"2026072006500549000_19.7.e273147.16","doi-asserted-by":"publisher","DOI":"10.1067\/mjd.2002.120621"},{"key":"2026072006500549000_19.7.e273147.17","first-page":"343","article-title":"Interstitial pneumonia in adult-onset Still\u2019s disease","volume":"25","author":"Nanke","year":"2007","journal-title":"Clin Exp Rheumatol"},{"key":"2026072006500549000_19.7.e273147.18","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2021.06.004"},{"key":"2026072006500549000_19.7.e273147.19","doi-asserted-by":"crossref","DOI":"10.3390\/jcm10040733","article-title":"Adult-Onset Still\u2019s Disease: Clinical Aspects and Therapeutic Approach","volume":"10","author":"Tomaras","year":"2021","journal-title":"J Clin Med"},{"key":"2026072006500549000_19.7.e273147.20","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2018-213920"},{"key":"2026072006500549000_19.7.e273147.21","doi-asserted-by":"crossref","first-page":"849","DOI":"10.1080\/14397595.2017.1416924","article-title":"Efficacy and safety of tocilizumab with inhibition of interleukin-6 in adult-onset Still\u2019s disease: A meta-analysis","volume":"28","author":"Ma","year":"2018","journal-title":"Mod Rheumatol"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2026-273147","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T13:50:14Z","timestamp":1784555414000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2026-273147"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7]]},"references-count":21,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2026,7,20]]},"published-print":{"date-parts":[[2026,7]]}},"alternative-id":["10.1136\/bcr-2026-273147"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2026-273147","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,7]]}}}