Scleroderma Renal Crisis-Associated Thrombotic Microangiopathy Preceding Skin Sclerosis in Diffuse Cutaneous Systemic...

A prompt and accurate diagnosis is essential because the management of thrombotic microangiopathy (TMA) depends on its underlying etiology. We report a case of diffuse cutaneous systemic sclerosis (dcSSc) in which scleroderma renal crisis-associated TMA preceded the development of overt skin sclerosis. A 53-year-old woman presented with TMA and severe renal dysfunction. She tested positive for anti-RNA polymerase III antibodies and subsequently developed skin sclerosis, leading to a diagnosis of dcSSc. Angiotensin-converting enzyme inhibitor therapy resulted in a clinical improvement in the patient. Therefore, systemic sclerosis should be considered in the differential diagnosis of TMA, even in the absence of skin manifestations at presentation.