Secondary immune-mediated thrombotic thrombocytopenic purpura in idiopathic inflammatory myopathy: a case-based review

• Published in: Rheumatology international Vol. 43; no. 3; pp. 551 - 557
• Main Authors: Ruffer, Nikolas, Holzer, Marie-Therese, Bal, Lukas Can, Melderis, Simon, Krusche, Martin, Huber, Tobias B., Kötter, Ina
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.03.2023
• Subjects: Adult; Autoimmune Diseases - complications; Case Based Review; Female; Humans; Medicine; Medicine & Public Health; Myositis - complications; Purpura, Thrombotic Thrombocytopenic - complications; Purpura, Thrombotic Thrombocytopenic - therapy; Rheumatology; Rituximab; Thrombocytopenia; Thrombotic microangiopathy; Myositis; Antisynthetase syndrome; protein; ADAMTS13 protein
• ISSN: 1437-160X; 0172-8172; 1437-160X
• DOI: 10.1007/s00296-022-05260-8

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a potentially fatal acquired thrombotic microangiopathy syndrome that frequently develops in the context of infectious diseases or systemic autoimmune conditions including connective tissue diseases. 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 ADAMTS13 activity. The concomitant manifestation of these autoimmune conditions may require intensive immunosuppressive therapy.