Assessment of activity and damage in ANCA-associated vasculitis in India

Background

Wegener's Granulomatosis, once thought to be uncommon in India, is being recognized with increasing frequency in Indians. In the present study, the assessment of the primary systemic necrotizing Vasculitis was done using the Birmingham Vasculitis Activity Score (BVAS) and Vasculitis Damage Index (VDI).

Method

Seventy-six patients with ANCA-associated vasculitis were evaluated using the BVAS and VDI, between January 1990 and June 2001. The diagnosis of Wegener's granulomatosis and Churg Strauss disease were made by 1990 ACR criteria and that of Microscopic Polyangiitis by Chapel Hill Consensus. ANCA, ANA and anti-DNA were estimated by indirect immunofluorescence and antibodies to PR3 and MPO by ELISA. All other causes for secondary vasculitis and infections were excluded.

Results

There were 40 males and 36 females. The mean age at diagnosis was 43.4. The mean disease duration prior to diagnosis was 3.4 months. The distribution of vasculitis were: Wegener's granulomatosis, 48, microscopic polyangiitis, 10, Churg Strauss, 6 and crescentic glomerulonephritis, 12. cANCA was positive in 48 (63.15%) and pANCA in 21 (27.63%). ANA and anti-DNA were negative in all the patients. The mean BVAS score at baseline was 16.4. The mean VDI system score was 3 and the mean total VDI score was 4.6. Using the VDI, the following items of damage were seen: musculoskeletal damage, 12 (15.8%); skin damage, 16 (21%); ENT damage, 28 (36.8%); pulmonary damage, 42 (55.3%); cardiovascular damage, 34 (44.7%); renal damage, 51 (67.1%); peripheral vascular damage, 26 (34.2%); ocular damage, 21 (27.6%); neuropsychiatric damage, 48 (63.2%); and other damage & drug toxicity, 14 (18.4%).

Conclusions

1) ANCA-associated vasculitis were rare in India, present in only 0.001% of Hospital admissions. 2) Neuropsychiatric manifestations were common (63%). 3) The BVAS and VDI offer a comprehensive and cumulative measure of disease activity and damage in the serial assessment of vasculitis patients.