Fatal Thrombotic Microangiopathy and Posterior Reversible Encephalopathy Syndrome in a Patient with Anti-melanoma Differentiation-associated Gene 5 Antibody-positive Dermatomyositis

Shotaro Yamamoto; Takao Nagashima; Yoichiro Akiyama; Katsuya Nagatani; Masahiro Iwamoto; Seiji Minota

doi:10.2169/internalmedicine.7309-21

CASE REPORTS

Fatal Thrombotic Microangiopathy and Posterior Reversible Encephalopathy Syndrome in a Patient with Anti-melanoma Differentiation-associated Gene 5 Antibody-positive Dermatomyositis

Shotaro Yamamoto, Takao Nagashima, Yoichiro Akiyama, Katsuya Nagatani, Masahiro Iwamoto, Seiji Minota

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Keywords: hemophagocytic syndrome, hemophagocytic lymphohistiocytosis, interstitial lung disease, thrombotic thrombocytopenic purpura

JOURNAL OPEN ACCESS

2021 Volume 60 Issue 20 Pages 3329-3333

DOI https://doi.org/10.2169/internalmedicine.7309-21

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Abstract

A 56-year-old woman presented with dermatomyositis positive for anti-melanoma differentiation-associated gene 5 antibody. No interstitial lung disease was detected. Despite treatment with methylprednisolone pulse therapy and cyclosporine, dysphagia developed. Furthermore, the presence of thrombocytopenia, elevated lactate dehydrogenase levels, and an undetectable haptoglobin level suggested the possibility of thrombotic microangiopathy (TMA). Disturbed consciousness developed shortly after TMA onset, and brain magnetic resonance imaging revealed hyperintensity lesions in the bilateral basal ganglia, thalami, and brainstem. The patient was diagnosed with atypical posterior leukoencephalopathy syndrome before dying of heart failure later that day. In conclusion, early TMA recognition and prompt intensive treatment are critical in such cases.

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