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

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@article{cd3b695de76a4598b62e2edbcc4ec343,
title = "Secondary immune-mediated thrombotic thrombocytopenic purpura in idiopathic inflammatory myopathy: a case-based review",
abstract = "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.",
author = "Nikolas Ruffer and Marie-Therese Holzer and Bal, {Lukas Can} and Simon Melderis and Martin Krusche and Huber, {Tobias B} and Ina K{\"o}tter",
note = "{\textcopyright} 2022. The Author(s).",
year = "2023",
month = mar,
doi = "10.1007/s00296-022-05260-8",
language = "English",
volume = "43",
pages = "551–557",
journal = "RHEUMATOL INT",
issn = "0172-8172",
publisher = "Springer",
number = "3",

}

RIS

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 -