Scleroderma renal crisis as the sole presenting feature of systemic sclerosis in a postpartum woman

Abstract

Systemic sclerosis is a chronic autoimmune disorder characterized by multiorgan involvement, most notably of the skin through fibrosis and vasculopathy. One of its most feared complications requiring rapid intervention is scleroderma renal crisis, as it can be fatal in the absence of prompt treatment. A 34-year old woman presents with a history of acute renal failure and malignant hypertension occurring one month postpartum and no other scleroderma feature in the following 5 years. Eventually, skin, heart and lung involvement is observed, positive anti-ARN III polymerase antibodies and suggestive capillaroscopic findings. Immunosuppressive therapy with mycophenolate mofetil is initiated and later switched to off-label Rituximab, with significant improvement of disease manifestations. Regular patient monitoring for novel symptom occurrence and appropriate treatment adjustment is essential for optimal management of scleroderma.

Keywords

• systemic sclerosis
• scleroderma renal crisis
• arn-polymerase iii antibodies
• postpartum
• myocarditis
• rituximab