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Renal disease associated with circulating antineutrophil cytoplasm activity
P J Garrett1, A G Dewhurst, L S Morgan
1Department of Renal Medicine, Royal South Hants Hospital, UK.
The Quarterly Journal of Medicine
|October 1, 1992
Summary
Antineutrophil cytoplasm activity (ANCA) related kidney disease is not rare, affecting seven in a million annually. Early ANCA testing aids in identifying patients for successful treatment with steroids and cyclophosphamide.
Area of Science:
- Nephrology
- Immunology
- Rheumatology
Background:
- Antineutrophil cytoplasm activity (ANCA) is associated with glomerulitis and necrotizing vasculitis.
- ANCA-related renal disease presents a significant clinical challenge.
Purpose of the Study:
- To detail observations on ANCA-related renal disease in 30 patients.
- To assess the incidence, diagnostic accuracy, treatment efficacy, and prognostic factors of ANCA-related renal disease.
Main Methods:
- Observation of 30 consecutive patients with histologically proven glomerulitis/necrotizing vasculitis and ANCA.
- Analysis of ANCA test sensitivity and specificity for glomerular vasculitis.
- Evaluation of treatment response to cyclophosphamide and steroids, and assessment of survival rates.
Main Results:
- Annual incidence of ANCA-related renal disease is seven cases per million.
- ANCA test sensitivity was 79%, specificity 87% for glomerular vasculitis.
- One-year survival was 60%; hypoxic lung disease was an adverse prognostic factor. Treatment complications occurred in over half of patients.
Conclusions:
- ANCA-related renal disease is common and ANCA serology accurately identifies a homogeneous patient group.
- Distinguishing between Wegener's granulomatosis and microscopic polyarteritis is not clinically useful.
- Early ANCA testing and treatment with immunosuppressants are crucial for managing ANCA-related renal disease, even in elderly patients.