PANEL 501
ANCA vasculitis/anti-GBM disease (Goodpasture syndrome)
Indication
Diagnosis and follow-up of ANCA-associated systemic vasculitis (such as Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (GPA)).
Clinical background
Patients with GPA (formerly called Wegener’s granulomatosis) have, with few exceptions, ANCA. About 90% have PR3-ANCA, and 5-10% have MPO-ANCA. In patients with a limited disease and in patients in remission ANCA is positive in half the cases.
Patients with microscopic polyangiitis (MPA) are, as a rule, positive for MPO-ANCA, but cases with positive PR3-ANCA occur. Even patients with Churg-Strauss disease and Goodpasture syndrome may be positive for MPO-ANCA. Both in the case of GPA and of MPA an increase in the ANCA level may precede a relapse.
Relevant literature
- Arranz, O et al.Comparison of anti-PR3 capture and anti-PR3 direct ELISA for detection of ANCA in long-term clinical follow-up of PR3-ANCA-associated vasculitis patients. Clinical Nephrology 2001; 56: 295-301.
- Csernok E, Ahlquist D, Ullrich S, Gross W.L. A critical evaluation of commercial immunoassays for antineutrophil cytoplasmic antibodies directed against proteinase 3 and myeloperoxidase in Wegener’s granulomatosis and microscopic polyangiitis. Rheumatology 2002;41:1313-1317.
- Csernok E, Holle J, Hellmich B et al. Evaluation of capture ELISA for detection of antineutrophil cytoplasmic antibodies directed against proteinase 3 in Wegener’s granulomatosis: first results from a multicenter study. Rheumatology 2004; 43: 174-180
- Gisslen, K et al.Relationship between ANCA determined with conventional binding and the capture assay and long-term clinical course in vasculitis.J. Intern Med 2002; 251: 0129-135.
- Sanders et al. Prediction of relapses in PR3-ANCA-associated vasculitis by assessing responses of ANCA titres to treatment. Rheumatology 2006; 45: 724-729.
- Savage COS et al. ABC of arterial and vascular disease - Vasculitis. Br Med J 2000; 320: 1326-1328
- Savige, J et al. ANCA and associated diseases: A review of the clinical and laboratory features. Kidney Int 2000; 57: 846-862.
- Segelmark M, et al. How and why should we detect ANCA? Clin Exp Rheumatol 2000, 18, 629-635.
- Westman, K et al.Relaps rate, renal survival and cancer morbidity in patients with Wegener’s granulomatosis or microscopic polyangiitis with renal involvement.J Am Soc Nephrol 1998; 9: 842-852.
- Won H, et al.Serial ANCA titers. Useful tool for the prevention of relapses in ANCA associated vasculitis. Kidney Int, 2003, 63, 1079-1085.
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PAKET 501
ANCA vaskulit/anti-GBM sjukdom (Goodpastures syndrom)
Indikation
Diagnos och uppföljning av ANCA-associerade systemiska vaskuliter (såsom Granulomatos med polyangiit (GPA) och mikroskopisk polyangit (MPA)).
Klinik
Patienter med GPA (tidigare kallade Wegeners granulomatos) har med få undantag ANCA. Cirka 90% har PR3-ANCA och 5-10% har MPO-ANCA. Hos patienter med begränsad sjukdom och hos patienter i remission är ANCA positiv i hälften av fallen.
Patienter med mikroskopisk polyangit (MPA) är som regel positiva för MPO-ANCA, men fall med positiv PR3-ANCA förekommer. Även patienter med Churg-Strauss sjukdom och Goodpastures syndrom kan vara positiva för MPO-ANCA.
Vid både GPA och MPA kan en ökning av ANCA-nivån förebåda skov.
Relevant litteratur
- Arranz, O et al.Comparison of anti-PR3 capture and anti-PR3 direct ELISA for detection of ANCA in long-term clinical follow-up of PR3-ANCA-associated vasculitis patients. Clinical Nephrology 2001; 56: 295-301.
- Csernok E, Ahlquist D, Ullrich S, Gross W.L. A critical evaluation of commercial immunoassays for antineutrophil cytoplasmic antibodies directed against proteinase 3 and myeloperoxidase in Wegener’s granulomatosis and microscopic polyangiitis. Rheumatology 2002;41:1313-1317.
- Csernok E, Holle J, Hellmich B et al. Evaluation of capture ELISA for detection of antineutrophil cytoplasmic antibodies directed against proteinase 3 in Wegener’s granulomatosis: first results from a multicenter study. Rheumatology 2004; 43: 174-180
- Gisslen, K et al.Relationship between ANCA determined with conventional binding and the capture assay and long-term clinical course in vasculitis.J. Intern Med 2002; 251: 0129-135.
- Sanders et al. Prediction of relapses in PR3-ANCA-associated vasculitis by assessing responses of ANCA titres to treatment. Rheumatology 2006; 45: 724-729.
- Savage COS et al. ABC of arterial and vascular disease - Vasculitis. Br Med J 2000; 320: 1326-1328
- Savige, J et al. ANCA and associated diseases: A review of the clinical and laboratory features. Kidney Int 2000; 57: 846-862.
- Segelmark M, et al. How and why should we detect ANCA? Clin Exp Rheumatol 2000, 18, 629-635.
- Westman, K et al.Relaps rate, renal survival and cancer morbidity in patients with Wegener’s granulomatosis or microscopic polyangiitis with renal involvement.J Am Soc Nephrol 1998; 9: 842-852.
- Won H, et al.Serial ANCA titers. Useful tool for the prevention of relapses in ANCA associated vasculitis. Kidney Int, 2003, 63, 1079-1085.