TY - JOUR
T1 - Plasma exchange for thrombotic microangiopathy secondary to dermatomyositis associated with acute kidney injury and complement activation
T2 - A case report with literature review
AU - Hayashi, Norifumi
AU - Okada, Keiichirou
AU - Tsuruyama, Yuko
AU - Kagaya, Yu
AU - Kumano, Sho
AU - Ishikura, Yuki
AU - Takeda, Kiminobu
AU - Nanbu, Masayuki
AU - Fujimoto, Keiji
AU - Adachi, Hiroki
AU - Yokoyama, Hitoshi
N1 - Publisher Copyright:
© 2019 The Author(s).
PY - 2019/12/5
Y1 - 2019/12/5
N2 - Background: Thrombotic microangiopathy (TMA) in patients with connective tissue disease is rare but life-Threatening. In particular, the survival rate of patients with dermatomyositis (DM) that develop TMA is low. The effectiveness of plasma exchange (PEX) therapy is unclear for the treatment of TMA secondary to DM. Case presentation: We describe a case of a 28-year-old woman who developed severe DM complicated by aspiration pneumonia from dysphagia and acute kidney injury. The patient was unresponsive to corticosteroids and intravenous immunoglobulin (IVIG) therapy and developed TMA. In this case, immunofluorescence of skin biopsy revealed that complement activation was involved in the pathogenesis of DM. After 6 PEX therapies, thrombocytopenia improved. She was successfully treated by intensive care and PEX therapy. Conclusions: PEX therapy was effective to treat TMA secondary to DM associated with complement activation.
AB - Background: Thrombotic microangiopathy (TMA) in patients with connective tissue disease is rare but life-Threatening. In particular, the survival rate of patients with dermatomyositis (DM) that develop TMA is low. The effectiveness of plasma exchange (PEX) therapy is unclear for the treatment of TMA secondary to DM. Case presentation: We describe a case of a 28-year-old woman who developed severe DM complicated by aspiration pneumonia from dysphagia and acute kidney injury. The patient was unresponsive to corticosteroids and intravenous immunoglobulin (IVIG) therapy and developed TMA. In this case, immunofluorescence of skin biopsy revealed that complement activation was involved in the pathogenesis of DM. After 6 PEX therapies, thrombocytopenia improved. She was successfully treated by intensive care and PEX therapy. Conclusions: PEX therapy was effective to treat TMA secondary to DM associated with complement activation.
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U2 - 10.1186/s41100-019-0244-5
DO - 10.1186/s41100-019-0244-5
M3 - Review article
AN - SCOPUS:85084399572
SN - 2059-1381
VL - 5
JO - Renal Replacement Therapy
JF - Renal Replacement Therapy
IS - 1
M1 - 48
ER -