Scleroderma Renal Crisis and Concurrent Isolated Pulmonary Hypertension in Mixed Connective Tissue Disease and Overlap Syndrome: Report of Two Cases

2002 ◽  
Vol 21 (2) ◽  
pp. 164-169 ◽  
Author(s):  
G. N. Andersen ◽  
J. Vasko
2001 ◽  
Vol 40 (12) ◽  
pp. 1250-1253 ◽  
Author(s):  
Toru YAMAGUCHI ◽  
Shiro OHSHIMA ◽  
Toshio TANAKA ◽  
Satoshi TSUKADA ◽  
Masato MATSUSHITA ◽  
...  

2017 ◽  
Vol 5 (4) ◽  
pp. 232470961773401 ◽  
Author(s):  
Jordana Cheta ◽  
Suresh Rijhwani ◽  
Harlan Rust

Mixed connective tissue disease (MCTD) is a rheumatologic overlap syndrome that can present with symptoms of systemic lupus erythematous, scleroderma, and polymyositis. A severe but rare complication that can occur in MCTD is scleroderma renal crisis. With multiple poor prognostic indicators, the renal outcome is usually poor. The clinical and histological picture is one of a thrombotic microangiopathy. Clinical suspicion has to be high for additional thrombotic or autoimmune processes coexisting due to associated morbidity. In this article, we report a rare case of scleroderma renal crisis in a patient with MCTD who we treated with plasma exchange for clinical suspicion for an underlying thrombotic thrombocytopenia and mycophenolate mofetil for MCTD. The patient had multiple poor prognostic indicators yet made a full renal recovery in less than 3 months.


2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Jomana Madieh ◽  
Iman Khamayseh ◽  
Alaa Hrizat ◽  
Abdurrahman Hamadah ◽  
Kamel Gharaibeh

Mixed connective tissue disease (MCTD) is a rheumatic disease syndrome with overlapping features of scleroderma, systemic lupus erythematosus, and polymyositis. An extremely rare but serious complication that can occur in MCTD is scleroderma renal crisis (SRC). There have been different approaches to the treatment of SRC associated with MCTD. We present a case of MCTD with chronic features of Raynaud’s phenomenon, dermatomyositis, and thrombocytopenia complicated with acute SRC which showed a great response to ACE inhibitors. Here, we advise the early and aggressive use of ACE inhibitors as soon as SRC is suspected.


2006 ◽  
Vol 26 (9) ◽  
pp. 1545-1547 ◽  
Author(s):  
Mehmet Celikbilek ◽  
Rengin Elsurer ◽  
Baris Afsar ◽  
Handan B. Ozdemir ◽  
Siren Sezer ◽  
...  

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