Scleroderma with crescentic glomerulonephritis: a case report

Abstract

IntroductionSystemic sclerosis or scleroderma is an autoimmune rheumatic disease characterized by organ-based fibrosis. Renal involvement in scleroderma occurs mainly in the form of scleroderma renal crisis, affecting 5 to 10% of patients. It remains one of the most important and immediately life-threatening complications of scleroderma, but the prognosis improves considerably after treatment with angiotensin-converting enzyme inhibitors. Other renal pathologies can occur in scleroderma. These include scleroderma overlap syndromes with associated features of lupus nephritis, myeloperoxidase anti-neutrophil cytoplasmic antibodies (ANCA) or proteinase 3 ANCA-associated glomerulonephritis, or crescentic glomerulonephritis. These alternative pathologies should be suspected in any individual patient with a differing clinical picture and the patient should be appropriately investigated. Crescentic glomerulonephritis occurs very rarely in scleroderma. This report describes a patient with scleroderma and crescentic glomerulonephritis.Case presentationA 52-year-old woman with a known history of scleroderma and hypertension on angiotensin-converting enzyme inhibitors was referred to the nephrologist because of a rapid decline in renal function. Kidney biopsy was performed which revealed immune complex type crescentic glomrulonephritis. Cytoplasmic-staining ANCA was negative. Despite immunosuppressive treatment the patient rapidly went into end-stage renal failure and is still on hemodialysis.ConclusionScleroderma is a complex disease, and the best characterized renal involvement in scleroderma is scleroderma renal crisis. However, other renal pathologies can occur in scleroderma. These alternative pathologies should be suspected in any patient with a differing clinical picture and the patient should be appropriately investigated, as the clinical course and treatment are different from the more common scleroderma renal crisis.

Significance of anti-neutrophil cytoplasmic antibodies in systemic sclerosis

“…Systemic sclerosis (SSc) is a multisystem autoimmune disease characterised by a triad of progressive skin and internal organ fibrosis, autoantibody production and small vessel vasculopathy [1, 2]. Clinically, SSc patients are classified as having limited (lcSSc) or diffuse (dcSSc) SSc, defined by the extent of skin thickening [3].…”

Antineutrophil Cytoplasmic Antibody Pauci-Immune Crescentic Glomerulonephritis and Interstitial Lung Disease in Scleroderma. Case Report and Literature Review

“…We included 8 cases with a history of D-penicillamine use. Six cases were excluded: 1 case complicated by renal cell cancer [33], a case of arthralgia and GERD but without Raynaud's or skin changes to suggest scleroderma [24], a report of vasculitis of the foot without renal involvement [16], and 3 cases where the renal biopsy findings were not typical of PICGN (extensive deposition of IgG, IgA, IgM and C3 on immunofluorescence [34], renal biopsy describing glomerular sclerosis rather than Glomerulonephritis (GN) [35], and a renal biopsy with the overall impression of crescentic GN with granular immune deposits [36]).…”

Scleroderma Renal Crisis: A Pathology Perspective

“…As the name implies, the presence of crescents is the hallmark of crescentic GN. In typical SRC cases, crescents are extremely rare and, when detected, are very small [ 39 ]. In SSc patients, most of the encountered crescentic GN are ANCA-associated.…”