Anti-neutrophil cytoplasmic antibody negative crescentic paucimmune glomerulonephritis in a case of scleroderma with systemic lupus erythematosus overlap

• Published in: Saudi journal of kidney diseases and transplantation 2016-05, Vol.27 (3), p.602-605
• Main Authors: Nijhawan, V. S., Subramaniam, Shankar, Kumar, Arun, Badwal, Sonia, Tewari, Rohit, Srinivas, V.
• Format: Article
• Language: English
• Subjects: Adult; Antibodies, Antineutrophil Cytoplasmic; Care and treatment; Case studies; Complications and side effects; Development and progression; Female; Glomerulonephritis; Humans; Kidney Glomerulus - pathology; Lupus Erythematosus, Systemic; Scleroderma (Disease); Scleroderma, Systemic; Systemic lupus erythematosus; الاستجابة المناعية; التهاب كبيبات الكلى; الذئبة الحمامية; تصلب الجلد
• ISSN: 1319-2442; 2320-3838
• DOI: 10.4103/1319-2442.182439

Renal Involvement in scleroderma is a known problem and the manifestations are well described. Renal involvement in systemic lupus erythematosus (SLE) is also well known. However, in scleroderma and SLE overlap syndrome, the renal findings may vary being a combination of features of immune complex mediated glomerulonephritis as well as thrombotic microangiopathy. We report a case in which the renal manifestation in such a situation was of a focal necrotising pauci-immune glomerulonephritis with crescents, anti-neutrophil cytoplasmic antibody negative. To the best of our knowledge, such manifestations have not been described before. Renal dysfunction in a normotensive setting in such a case should direct one towards evaluation for other causes and should prompt a kidney biopsy. This would be valuable in delineating the pathological process in the kidney and would help in guiding therapy.