Thrombotic Thrombocytopenic Purpura Associated with Mixed Connective Tissue Disease: A Case Report

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a multisystemic disorder characterized by microangiopathic hemolytic anemia and thrombocytopenia, which may be accompanied by fever, renal, or neurologic abnormalities. Cases are divided into acute idiopathic TTP and secondary TTP. Autoimmune diseases, especially systemic lupus erythematosus, in association with TTP have been described so far in many patients. In contrast, TTP occurring in a patient with mixed connected tissue disease (MCTD) is extremely rare and has only been described in nine patients. We describe the case of a 42-year-old female with MCTD who developed thrombocytopenia, microangiopathic hemolytic anemia, fever, and neurological symptoms. The patient had a good clinical evolution with infusion of high volume of fresh frozen plasma, steroid therapy, and support in an intensive care unit. Although the occurrence of TTP is rare in MCTD patients, it is important to recognize TTP as a cause of thrombocytopenia and hemolytic anemia in any patient with autoimmune diseases. Prompt institution of treatment remains the cornerstone of treatment of TTP even if plasma exchange is not available like what frequently happens in developing countries.

Thrombotic thrombocytopenic purpura in the presence of connective tissue disease and HIV infection: A diagnostic and therapeutic challenge in a resource- constrained setting

“…In addition, however, early plasmapheresis is essential to reduce mortality. [6,11,12] Where plasmapheresis is not available, plasma infusion is a recommended albeit inferior alternative.…”

Mixed Connective Tissue Disease Associated with Other Autoimmune Diseases as Giant Cell Myocarditis, Vitiligo and Autoimmune Hemolytic Anemia, A Multiple Autoimmune Syndrome Impossible to Type, Revised Multiple Autoimmune Syndrome Classification Extended to Type 4 Needed. About Two Cases

“…However, there are some difficulties with this current multiple autoimmune syndrome classification [1] that was based on the prevalence of autoimmune disease associations. For example, the mixed connective tissue disease (MCTD) associated with other autoimmune diseases that is more and more reported [4][5][6][7][8][9][10][11][12]. MCTD is a distinct disease entity with mixed features of systemic lupus erythematosus, systemic sclerosis, myositis, and rheumatoid arthritis with high titers of antibodies to U1 ribonucleoprotein (U1RNP) [13].…”

An Autopsy Case of Mixed Connective Tissue Disease Complicated by Thrombotic Thrombocytopenic Purpura

“…Mixed connective tissue disease (MCTD) is a systemic autoimmune disease that has at least two features of three other CTDs [SLE, SSc, and polymyositis (PM)], along with the presence of anti-U1 ribonucleoprotein (RNP) antibodies (18). While a large number of patients with SLE or SSc have been reported (19, 20), MCTD-associated TTP is relatively rare, and only 15 cases have been described to date (21)(22)(23)(24)(25)(26)(27)(28)(29)(30)(31)(32)(33)(34).…”