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Case Reports
. 2018 May-Jun;28(3):244-246.
doi: 10.4103/ijn.IJN_292_16.

Perinuclear Antineutrophil Cytoplasmic Antibody Positive Glomerulonephritis in a Case of Limited Cutaneous Scleroderma

H Vora  1 , B Kulkarni  2 , S Singh  3 , N Kulkarni  1
Affiliations
Case Reports

Perinuclear Antineutrophil Cytoplasmic Antibody Positive Glomerulonephritis in a Case of Limited Cutaneous Scleroderma

H Vora et al. Indian J Nephrol. 2018 May-Jun.

Abstract

Antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis in a patient of scleroderma is very rare. Very few cases have been reported in English literature. We report a case of a 58-year-old male with long-standing limited cutaneous scleroderma (Scl-70 positive) presenting with normotensive scleroderma renal crisis. Perinuclear ANCA with antimyeloperoxidase antibody was found to be strongly positive. Renal biopsy showed pauci immune-necrotizing crescentic glomerulonephritis. We believe that this case report will be helpful in understanding clinical features of normotensive ANCA-associated glomerulonephritis in scleroderma patients.

Keywords: Antineutrophil cytoplasmic antibody; glomerulonephritis; scleroderma.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
×40 glomerulus showing a partial fibrocellular crescent. A segmental area of necrosis is noted (arrow). The underlying glomerulus is compressed. The interstitium shows mild fibrosis and a chronic inflammatory infiltrate
Figure 2
Figure 2
×40 glomerulus is almost completely replaced by a fibrocellular crescent showing areas fibrinoid necrosis (arrows). The interstitium shows fibrosis
See this image and copyright information in PMC

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