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Thrombotic thrombocytopenic purpura associated with mixed connective tissue disease
Akihiko Kato1, Yohji Suzuki, Yoshihide Fujigaki
1First Department of Medicine, Hamamatsu University School of Medicine, Shizuoka, Japan. a.kato@scchr.jp
Abstract:
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.
Insights
This case highlights thrombotic microangiopathy in mixed connective tissue disease (MCTD). Diffusion-weighted MRI detected brain lesions, suggesting it
Area of Science:
- Neurology
- Rheumatology
- Hematology
Background:
- Mixed connective tissue disease (MCTD) can present with diverse systemic manifestations.
- Thrombotic microangiopathy is a rare but serious complication.
- Acute interstitial pneumonitis is a known pulmonary manifestation of MCTD.
Observation:
- A male patient with MCTD developed thrombocytopenia and hemolytic anemia despite steroid treatment for pneumonitis.
- Neurological decline, including confusion and coma, was observed.
- Diffusion-weighted MRI revealed brainstem, frontal lobe, basal nuclei, and insular lesions consistent with microthrombosis-induced edema.
Findings:
- The case demonstrates a severe thrombotic microangiopathy presentation in MCTD.
- Diffusion-weighted MRI showed high sensitivity in detecting early ischemic brain lesions.
- Despite aggressive treatment including plasma exchange and pulse steroids, the patient experienced multiple organ failure.
Implications:
- This case underscores the potential for thrombotic microangiopathy and associated neuropsychiatric symptoms as fatal complications in MCTD.
- Diffusion-weighted MRI is a valuable tool for early diagnosis of thrombotic thrombocytopenic purpura (TTP)-related brain injury.
- Early recognition and intervention for thrombotic microangiopathy are critical in managing MCTD patients.