{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T20:19:13Z","timestamp":1790194753928,"version":"4.1.0"},"reference-count":13,"publisher":"BMJ","issue":"1","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2025,1,16]]},"published-print":{"date-parts":[[2025,1]]},"abstract":"<jats:p>Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome and haemophagocytic lymphohistiocytosis (HLH) are rare but severe immune-mediated diseases with overlapping clinical manifestations. We present a case of a woman in her late 40s with rheumatoid arthritis who developed DRESS\/HLH overlap syndrome after starting hydroxychloroquine and leflunomide therapy. Despite corticosteroid treatment, her condition worsened, necessitating etoposide therapy. The persistent pancytopenia required supportive measures, including transfusions of blood products and administration of growth factors. Tofacitinib was successfully used as a steroid-sparing agent and resulted in the resolution of symptoms without relapse during a 2\u2009month follow-up. This case emphasises the diagnostic and therapeutic challenges posed by the co-occurrence of DRESS and HLH and highlights the importance of tailoring treatment strategies to achieve good outcomes.<\/jats:p>","DOI":"10.1136\/bcr-2024-262809","type":"journal-article","created":{"date-parts":[[2025,1,29]],"date-time":"2025-01-29T08:40:50Z","timestamp":1738140050000},"page":"e262809","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":3,"title":["Leflunomide-induced drug reaction eosinophilia systemic symptoms and haemophagocytic lymphohistiocytosis overlap syndrome with rheumatoid arthritis"],"prefix":"10.1136","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8022-1086","authenticated-orcid":false,"given":"Anjely Pulparampil","family":"Sebastian","sequence":"first","affiliation":[{"name":"Department of Infectious Diseases, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Praveen Kumar","family":"Tirlangi","sequence":"additional","affiliation":[{"name":"Department of Infectious Diseases, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kavitha","family":"Saravu","sequence":"additional","affiliation":[{"name":"Department of Infectious Diseases, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roopa","family":"Acharya","sequence":"additional","affiliation":[{"name":"Department of Infectious Diseases, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, Karnataka, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2025,1,29]]},"reference":[{"key":"2025082723501528000_18.1.e262809.1","doi-asserted-by":"crossref","unstructured":"Yang JJ , Lei DK , Ravi V , et al . Overlap between hemophagocytic lymphohistiocytosis and drug reaction and eosinophilia with systemic symptoms: a review. Int J Dermatol 2020. doi:10.1111\/ijd.15196","DOI":"10.1111\/ijd.15196"},{"key":"2025082723501528000_18.1.e262809.2","doi-asserted-by":"crossref","first-page":"1323","DOI":"10.1111\/liv.15189","article-title":"Leflunomide-induced liver injury: Differences in characteristics and outcomes in Indian and US registries","volume":"42","author":"Devarbhavi","year":"2022","journal-title":"Liver Int"},{"key":"2025082723501528000_18.1.e262809.3","doi-asserted-by":"crossref","first-page":"40","DOI":"10.4103\/idoj.idoj_196_21","article-title":"RegiSCAR DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) Validation Scoring System and Japanese Consensus Group Criteria for Atypical Drug-Induced Hypersensitivity Syndrome (DiHS): A Comparative Analysis","volume":"13","author":"Sasidharanpillai","year":"2022","journal-title":"Indian Dermatol Online J"},{"key":"2025082723501528000_18.1.e262809.4","doi-asserted-by":"publisher","DOI":"10.1093\/ajcp\/aqw076"},{"key":"2025082723501528000_18.1.e262809.5","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1111\/ejh.14030","article-title":"Anakinra versus etoposide-based therapy added to high-dose steroids for the treatment of secondary hemophagocytic lymphohistiocytosis","volume":"111","author":"Lee","year":"2023","journal-title":"Eur J Haematol"},{"key":"2025082723501528000_18.1.e262809.6","doi-asserted-by":"crossref","first-page":"809","DOI":"10.1093\/rheumatology\/keh150","article-title":"Leflunomide-induced peripheral neuropathy: rapid resolution with cholestyramine wash-out","volume":"43","author":"Hill","year":"2004","journal-title":"Rheumatology (Sunnyvale)"},{"key":"2025082723501528000_18.1.e262809.7","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1080\/03009742.2020.1729405","article-title":"Tofacitinib-induced remission in refractory adult-onset Still\u2019s disease complicated by macrophage activation syndrome","volume":"49","author":"Honda","year":"2020","journal-title":"Scand J Rheumatol"},{"key":"2025082723501528000_18.1.e262809.8","doi-asserted-by":"crossref","DOI":"10.1002\/mco2.538","article-title":"Tofacitinib for elderly onset hemophagocytic lymphohistiocytosis with gene mutations: a case report","volume":"5","author":"Liu","year":"2024","journal-title":"MedComm"},{"key":"2025082723501528000_18.1.e262809.9","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1310476"},{"key":"2025082723501528000_18.1.e262809.10","doi-asserted-by":"crossref","first-page":"60","DOI":"10.4103\/2229-5151.152349","article-title":"Severe hemophagocytic lymphohistiocytosis as a complication of drug-induced hypersensitivity syndrome","volume":"5","author":"Gauchan","year":"2015","journal-title":"Int J Crit Illn Inj Sci"},{"key":"2025082723501528000_18.1.e262809.11","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1177\/14782715231220108","article-title":"Drug-induced secondary haemophagocytic lymphohistiocytosis in hairy cell leukaemia","volume":"54","author":"Stark","year":"2024","journal-title":"J R Coll Physicians Edinb"},{"key":"2025082723501528000_18.1.e262809.12","doi-asserted-by":"crossref","DOI":"10.1186\/s40164-022-00362-2","article-title":"The role of etoposide in the treatment of adult patients with hemophagocytic lymphohistiocytosis","volume":"12","author":"Zondag","year":"2023","journal-title":"Exp Hematol Oncol"},{"key":"2025082723501528000_18.1.e262809.13","doi-asserted-by":"crossref","DOI":"10.1038\/s41598-024-69054-7","article-title":"Viral reactivation and clinical outcomes in Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS)","volume":"14","author":"Chan","year":"2024","journal-title":"Sci Rep"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2024-262809","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,28]],"date-time":"2025-08-28T06:50:24Z","timestamp":1756363824000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2024-262809"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,1]]},"references-count":13,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2025,1,29]]},"published-print":{"date-parts":[[2025,1]]}},"alternative-id":["10.1136\/bcr-2024-262809"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2024-262809","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,1]]}}}