Thrombotic thrombocytopenic purpura associated with mixed connective tissue disease.
We report a male case of thrombotic microangiopathy with mixed connective tissue disease (MCTD). Thrombocytopenia and hemolytic anemia developed despite steroid treatment for acute interstitial pneumonitis. The patient became confused and eventually developed coma. Diffusion-weighted MRI revealed high intensity at the brainstem, frontal lobes, basal nuclei, and insula, findings compatible with multiple brain edema due to microthrombosis. Despite the treatment of successive plasma exchange and steroid pulse therapy, he eventually died by multiple organ failure. This rare case suggested that diffusion-weighted MRI is very sensitive at detecting early alterations of thrombotic thrombocytopenic purpura (TTP)-induced ischemic lesions in the brain. Neuropsychiatric symptoms due to thrombotic microangiopathy could be some of the fatal complications in MCTD patients.
Kato
Akihiko
A
First Department of Medicine, Hamamatsu University School of Medicine, Shizuoka, Japan. a.kato@scchr.jp
Suzuki
Yohji
Y
Fujigaki
Yoshihide
Y
Yamamoto
Tatsuo
T
Yonemura
Katsuhiko
K
Miyajima
Hiroaki
H
Hishida
Akira
A
D001921:Brain / Q000098:blood supply / Q000473:pathology; D003128:Coma / Q000209:etiology / Q000473:pathology; D017809:Fatal Outcome; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000188:drug therapy / Q000209:etiology / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D008947:Mixed Connective Tissue Disease / Q000150:complications* / Q000473:pathology / Q000628:therapy; D011239:Prednisolone / Q000627:therapeutic use; D020551:Pulse Therapy, Drug; D011697:Purpura, Thrombotic Thrombocytopenic / Q000209:etiology* / Q000473:pathology / Q000628:therapy
D011239:Prednisolone; D008775:Methylprednisolone