This case report of a 70-year-old Thai female with type 2 diabetes and essential hypertension who presented with dyspnea, rapidly declining of kidney function, malignant hypertension, and thrombotic microangiopathy after a two-week course of high-dose corticosteroid administration for interstitial lung disease (ILD). A physical examination revealed characteristics compatible with limited cutaneous systemic sclerosis (lcSSc). However, SSc was not diagnosed at the time the patient’s ILD discovered. Laboratory investigations showed proteinuria with microscopic hematuria, the presence of anti-topoisomerase I antibodies, high titer of antinuclear antibodies, and low serum of complement C3 and C4. The diagnosis required differentiation between severe proliferative type of lupus nephritis and scleroderma renal crisis, as treatment for these two diseases differs greatly. Thus, the patient underwent percutaneous kidney biopsy, and the renal pathology revealed an onion-skin appearance with fibrinoid necrosis at the arterioles and arteries. The final diagnosis was lcSSc with scleroderma renal crisis.

Keywords: Scleroderma renal crisis, Limited cutaneous scleroderma, Systemic sclerosis

Received 19 Jul 2018 | Revised 25 Mar 2019 | Accepted 29 Mar 2019