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

Author:

Ruffer NikolasORCID,Holzer Marie-ThereseORCID,Bal Lukas CanORCID,Melderis SimonORCID,Krusche MartinORCID,Huber Tobias B.ORCID,Kötter InaORCID

Abstract

AbstractImmune-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.

Funder

Universitätsklinikum Hamburg-Eppendorf (UKE)

Publisher

Springer Science and Business Media LLC

Subject

Immunology,Immunology and Allergy,Rheumatology

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