Successful treatment of scleroderma renal crisis in systemic sclerosis using angiotensin-converting enzyme inhibitor, peritoneal dialysis, and rituximab: a case report and literature review

Abstract A 53-year-old woman developed edema of the extremities and Raynaud’s phenomenon 8 months before hospitalization. She was diagnosed with systemic sclerosis (SSc) and was referred to our hospital after 4 months because of rapidly progressing skin sclerosis. Treatment with prednisolone (15 mg/day) and intermittent intravenous cyclophosphamide was initiated at the outpatient clinic of our hospital, followed by dose tapering. She was admitted to our hospital with worsening cough and dyspnea lasting 7 days. Hypertension, acute kidney injury, and signs of heart failure were observed. In addition, the features of skin sclerosis suggested scleroderma renal crisis (SRC). Blood pressure was managed with an angiotensin-converting enzyme (ACE) inhibitor, which improved the symptoms of heart failure. However, renal function did not recover, necessitating peritoneal dialysis (PD). Continued treatment with an ACE inhibitor and PD successfully controlled the hypertension, heart failure, and renal failure. Despite dialysis, rituximab therapy was administered to address the progression of skin sclerosis without significant adverse effects. Following rituximab administration, skin sclerosis and activities of daily living (ADL) significantly improved. This case highlights the efficacy of PD with an ACE inhibitor in preserving residual renal function and managing SRC. Comprehensive treatment of SSc improved complications and the patient’s ADL.