Vasculitis ANCA and Systemic Scleroderma
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Keywords

Anti-neutrophil cytoplasmic antibody-associated vasculitis kidney diseases
scleroderma
systemic
rituximab
cyclophosphamide
kidney diseases.

How to Cite

1.
Hurtado M, Contreras K, González CA, Bustamante A. Vasculitis ANCA and Systemic Scleroderma. Rev. Colomb. Nefrol. [Internet]. 2016 Nov. 18 [cited 2023 Jan. 27];3(2):137-42. Available from: https://revistanefrologia.org/index.php/rcn/article/view/220

Abstract

Systemic sclerosis (SSc) is an autoimmune disorder with microvascular endothelial cell apoptosis, excessive extracellular matrix protein and perivascular infiltration of mononuclear cells in the skin, producing damage and progressive fibrosis of the skin and visceral organs (lungs, heart and kidney). The most common renal diseases linked with SSc is renal scleroderma crisis, which is found in 5 to10% of the cases. ANCA associated vasculitis also has been described but less frequent. It can affect multiple organs and become life-threatening condition. The association between paucity-immune vasculitis and systemic sclerosis is uncommon 1.6% in a cohort of 35 patients. It could lead to severe renal dysfunction including renal replacement therapy and plasma exchange as part of the treatment. Early and timely treatment it is essential for the prognosis of the diseases. We present this rare association and our experiences highlighting the successful evolution due to early diagnosis and treatment. A 59 years old woman with systemic sclerosis who showed a rapidly progressive glomerulonephritis caused by to paucity-immune vasculitis associated with p-ANCA. We dismissed scleroderma crisis and renal biopsy confirmed the diagnosis. We start prednisolone and cyclophosphamide, with a favorable initial response, however, during the follow-up the patient had severe relapse, and therefore we decided to switch to Rituximab induction. After we performed the treatment the patient evolved favorably, included complete remission of the disease. We show you the rare association between paucity-immune vasculitis and systemic sclerosis, and the excellent response taking account an early diagnosis and therapy.
https://doi.org/10.22265/acnef.3.2.220
PDF (Español (España))

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