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Thrombotic thrombocytopenic purpura associated with mixed connective tissue disease.
Rheumatol Int. 2002 Jul; 22(3):122-5.RI

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.

Authors+Show Affiliations

First Department of Medicine, Hamamatsu University School of Medicine, Shizuoka, Japan. a.kato@scchr.jpNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Review

Language

eng

PubMed ID

12111089

Citation

Kato, Akihiko, et al. "Thrombotic Thrombocytopenic Purpura Associated With Mixed Connective Tissue Disease." Rheumatology International, vol. 22, no. 3, 2002, pp. 122-5.
Kato A, Suzuki Y, Fujigaki Y, et al. Thrombotic thrombocytopenic purpura associated with mixed connective tissue disease. Rheumatol Int. 2002;22(3):122-5.
Kato, A., Suzuki, Y., Fujigaki, Y., Yamamoto, T., Yonemura, K., Miyajima, H., & Hishida, A. (2002). Thrombotic thrombocytopenic purpura associated with mixed connective tissue disease. Rheumatology International, 22(3), 122-5.
Kato A, et al. Thrombotic Thrombocytopenic Purpura Associated With Mixed Connective Tissue Disease. Rheumatol Int. 2002;22(3):122-5. PubMed PMID: 12111089.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Thrombotic thrombocytopenic purpura associated with mixed connective tissue disease. AU - Kato,Akihiko, AU - Suzuki,Yohji, AU - Fujigaki,Yoshihide, AU - Yamamoto,Tatsuo, AU - Yonemura,Katsuhiko, AU - Miyajima,Hiroaki, AU - Hishida,Akira, Y1 - 2002/06/06/ PY - 2001/12/17/received PY - 2002/03/18/accepted PY - 2002/7/12/pubmed PY - 2003/1/17/medline PY - 2002/7/12/entrez SP - 122 EP - 5 JF - Rheumatology international JO - Rheumatol. Int. VL - 22 IS - 3 N2 - 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. SN - 0172-8172 UR - https://www.unboundmedicine.com/medline/citation/12111089/Thrombotic_thrombocytopenic_purpura_associated_with_mixed_connective_tissue_disease_ L2 - https://dx.doi.org/10.1007/s00296-002-0202-2 DB - PRIME DP - Unbound Medicine ER -