Scleroderma Renal Crisis in Mixed Connective Tissue Disease With Full Renal Recovery Within 3 Months: A Case Report With Expanding Treatment Modalities to Treat Each Clinical Sign as an Independent Entity

Abstract

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.

Scleroderma Renal Crisis in a Case of Mixed Connective Tissue Disease Treated Successfully with Angiotensin-Converting Enzyme Inhibitors

“…Renal involvement in MCTD is uncommon (10%–26% of patients) and is often asymptomatic [ 5 ]. In a study looking at the renal involvement in MCTD, it was found that the only early indicator of renal disease was an abnormal urinalysis with no overt clinical features.…”

“…MCTD can potentially affect various organ systems resulting in pulmonary, renal, cardiovascular, gastrointestinal, and central nervous system manifestations [ 3 , 4 ]. Scleroderma renal crisis (SRC) is an extremely rare complication in MCTD [ 5 , 6 ]. It typically presents as an accelerated hypertension of sudden onset and acute renal injury, with or without microangiopathic hemolytic anemia or thrombocytopenia [ 5 , 7 , 8 ].…”

“…Scleroderma renal crisis (SRC) is an extremely rare complication in MCTD [ 5 , 6 ]. It typically presents as an accelerated hypertension of sudden onset and acute renal injury, with or without microangiopathic hemolytic anemia or thrombocytopenia [ 5 , 7 , 8 ]. Several reports have emphasized the use of angiotensin-converting enzyme inhibitors (ACEi) and their dramatic improvement on the outcomes and survival of scleroderma patients experiencing SRC [ 8 – 11 ].…”

“…Several reports have emphasized the use of angiotensin-converting enzyme inhibitors (ACEi) and their dramatic improvement on the outcomes and survival of scleroderma patients experiencing SRC [ 8 – 11 ]. However, only a few reports on the treatment of SRC in MCTD exist [ 5 , 6 , 12 ]. In this article, we report a rare case of MCTD complicated by SRC which was treated successfully with ACEi.…”

ANCA-Associated Vasculitis Co-Occurrence With Systemic Sclerosis: A Case Report of a Rare Diagnostic Dilemma

“… 4 A worse prognosis for SRC exists in patients with dcSSc, cardiac involvement, renal crisis with normotensive blood pressures, and skin scores ≥20. 15 …”