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We report the case of a 42-year-old female suffering from severe iTTP associated with anti-Jo-1 positive antisynthetase syndrome, which was successfully treated with combination therapy of intravenous immune globulin, rituximab and plasma exchange. Based on a systematic review of the literature, two additional cases of idiopathic inflammatory myopathy-associated iTTP (secondary iTTP) were identified. In conclusion, iTTP may be a rare complication of IIM that clinicians should consider in cases of marked thrombocytopenia. Further work-up of this finding should include a peripheral blood smear (schistocyte count) and <jats:italic>ADAMTS13<\/jats:italic> activity. The concomitant manifestation of these autoimmune conditions may require intensive immunosuppressive therapy.<\/jats:p>","DOI":"10.1007\/s00296-022-05260-8","type":"journal-article","created":{"date-parts":[[2022,12,28]],"date-time":"2022-12-28T08:02:54Z","timestamp":1672214574000},"page":"551-557","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Secondary immune-mediated thrombotic thrombocytopenic purpura in idiopathic inflammatory myopathy: a case-based review"],"prefix":"10.1007","volume":"43","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8394-969X","authenticated-orcid":false,"given":"Nikolas","family":"Ruffer","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2064-6728","authenticated-orcid":false,"given":"Marie-Therese","family":"Holzer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4731-2311","authenticated-orcid":false,"given":"Lukas Can","family":"Bal","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3035-999X","authenticated-orcid":false,"given":"Simon","family":"Melderis","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0582-7790","authenticated-orcid":false,"given":"Martin","family":"Krusche","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7175-5062","authenticated-orcid":false,"given":"Tobias B.","family":"Huber","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9262-005X","authenticated-orcid":false,"given":"Ina","family":"K\u00f6tter","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2022,12,28]]},"reference":[{"issue":"10","key":"5260_CR1","doi-asserted-by":"publisher","first-page":"1181","DOI":"10.1182\/blood-2017-04-636431","volume":"130","author":"JE Sadler","year":"2017","unstructured":"Sadler JE (2017) Pathophysiology of thrombotic thrombocytopenic purpura. 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