Scleroderma Renal Crisis in Mixed Connective Tissue Disease in a Patient: A Challenging Disease

Abstract

A 52-year-old woman with Raynaud syndrome diagnosed less than 1 year ago, consulted the emergency room due to 4 months of progressive symmetrical edema in the lower limbs, muscle weakness, dyspnea, hypertension crisis, and dark urine. Investigations revealed left ventricular dysfunction (ejection fraction 14%) without coronary artery disease and acute renal injury. Mixed Connective Tissue Disease was diagnosed and because of the renal and systemic findings in the patient a renal biopsy was performed confirming scleroderma renal crisis. To the best of our knowledge, this is the tenth patient who reported scleroderma renal crisis as a complication in patients with Mixed Connective Tissue Disease, from these 4 patients who became hemodialysis dependent, 1 died, and 4 responded to therapy. The treatment chosen was Angiotensin-converting-enzyme inhibitors and steroids based on what is known in renal scleroderma crisis with a substantial recovery of the left ventricular function and stabilization of the glomerular filtration rate followed by discharge from hospitalization.