Scleroderma Renal Crisis after Steroid Therapy in
Interstitial Lung Disease, as Initial Presentation in a
Limited Cutaneous Scleroderma Patient:
Case Report:
Tonsawan P, MD, MSc¹, Chan-on C, MD¹, Puapairoj A, MD², Suwannaroj S, MD³
Affiliation : ¹ Division of Nephrology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand ² Department of Pathology, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand ³ Division of Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand
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
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