Secondary immune-mediated thrombotic thrombocytopenic purpura in idiopathic inflammatory myopathy: a case-based review
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Secondary immune-mediated thrombotic thrombocytopenic purpura in idiopathic inflammatory myopathy: a case-based review. / Ruffer, Nikolas; Holzer, Marie-Therese; Bal, Lukas Can; Melderis, Simon; Krusche, Martin; Huber, Tobias B; Kötter, Ina.
in: RHEUMATOL INT, Jahrgang 43, Nr. 3, 03.2023, S. 551–557.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Review › Forschung
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TY - JOUR
T1 - Secondary immune-mediated thrombotic thrombocytopenic purpura in idiopathic inflammatory myopathy: a case-based review
AU - Ruffer, Nikolas
AU - Holzer, Marie-Therese
AU - Bal, Lukas Can
AU - Melderis, Simon
AU - Krusche, Martin
AU - Huber, Tobias B
AU - Kötter, Ina
N1 - © 2022. The Author(s).
PY - 2023/3
Y1 - 2023/3
N2 - 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.
AB - 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.
U2 - 10.1007/s00296-022-05260-8
DO - 10.1007/s00296-022-05260-8
M3 - SCORING: Review article
C2 - 36576539
VL - 43
SP - 551
EP - 557
JO - RHEUMATOL INT
JF - RHEUMATOL INT
SN - 0172-8172
IS - 3
ER -