Is Presence of ANCA in Crescentic IgA Nephropathy a Coincidence or Novel Clinical Entity? A Case Series

Bantis, Christos; Stangou, Maria; Schlaugat, Christoph; Alexopoulos, Efstathios; Pantzaki, Aphroditi; Memmos, Dimitrios; Ivens, Katrin; Heering, Peter J.

doi:10.1053/j.ajkd.2009.09.031

« Previous Next »American Journal of Kidney Diseases
Volume 55, Issue 2 , Pages 259-268, February 2010

Is Presence of ANCA in Crescentic IgA Nephropathy a Coincidence or Novel Clinical Entity? A Case Series

Christos Bantis, MD
- Department of Nephrology, Heinrich-Heine University of Düsseldorf, Germany
- Department of Nephrology, Aristotle University of Thessaloniki, Greece
Maria Stangou, MD
- Department of Nephrology, Aristotle University of Thessaloniki, Greece
Christoph Schlaugat, MD
- Department of Nephrology, Städisches Klinikum Solingen, University of Cologne, Germany
Efstathios Alexopoulos, MD, PhD
- Deceased.
- Department of Nephrology, Aristotle University of Thessaloniki, Greece
Aphroditi Pantzaki, MD
- Department of Nephrology, Aristotle University of Thessaloniki, Greece
Dimitrios Memmos, MD, PhD
- Department of Nephrology, Aristotle University of Thessaloniki, Greece
Katrin Ivens, MD, PhD
- Department of Nephrology, Heinrich-Heine University of Düsseldorf, Germany
Peter J. Heering, MD, PhD
- Department of Nephrology, Heinrich-Heine University of Düsseldorf, Germany
- Department of Nephrology, Städisches Klinikum Solingen, University of Cologne, Germany
- Address correspondence to Peter J. Heering, MD, PhD, Department of Nephrology, Staedtisches Klinikum Solingen, Gotenstrasse 1, 42653 Solingen, Germany

Received 31 March 2009; accepted 28 September 2009. published online 31 December 2009.

Background

There are few anecdotal reports of circulating antineutrophil cytoplasmic autoantibodies (ANCAs) in patients with immunoglobulin A (IgA) nephropathy.

Study Design

Retrospective case series.

Setting & Participants

We studied 8 patients with crescentic IgA nephropathy associated with ANCAs against myeloperoxidase (n = 5) and proteinase 3 (n = 3) followed up for 2.4 ± 1.7 years. They were compared with 26 patients with IgA nephropathy with > 10% crescentic glomeruli, but negative for ANCAs.

Outcomes

We analyzed clinical and histologic features of patients and their response to treatment.

Measurements

Screening for ANCAs was performed using indirect immunofluorescence, and positive results were verified using enzyme-linked immunosorbent assay.

Results

All patients with crescentic IgA nephropathy and positive for ANCAs, compared with only one-third of ANCA-negative patients, presented with the clinical syndrome of rapid progressive glomerulonephritis. ANCA-positive patients reached a higher peak serum creatinine level within the first 3 months (4.2 ± 2.2 vs 2.5 ± 1.9 mg/dL; estimated glomerular filtration rate, 19.3 ± 10.2 vs 45.9 ± 30.1 mL/min/1.73 m²). ANCA-positive patients with IgA nephropathy had a higher percentage of crescentic glomeruli (54.3% ± 18%) compared with ANCA-negative patients with crescentic IgA nephropathy (34.5% ± 26%). ANCA-positive patients were treated using cyclophosphamide and corticosteroids. Kidney function improved in all these patients: serum creatinine level decreased from the peak of 4.2 ± 2.2 to 1.7 ± 0.7 mg/dL at the end of follow up (estimated glomerular filtration rate, 19.3 ± 10.2 to 44.6 ± 11.1 mL/min/1.73 m²). In contrast, no significant improvement was achieved in ANCA-negative patients.

Conclusion

Patients with IgA nephropathy, crescents, and positive for ANCAs represent a clinical entity with a diverse more exaggerated clinical and histologic picture. However, disease in these patients responded well to aggressive immunosuppressive therapy.

Index Words: IgA nephropathy, antineutrophil cytoplasmic autoantibodies (ANCAs), rapidly progressive glomerulonephritis, crescents