ANTINEUTROPHIL CYTOPLASM ANTIBODIES IN SYSTEMIC POLYARTERITIS-NODOSA WITH AND WITHOUT HEPATITIS-B VIRUS-INFECTION AND CHURG-STRAUSS-SYNDROME - 62 PATIENTS

Objective. Antibodies directed against components of neutrophil cytopl asm have been detected in various systemic vasculitides and especially in Wegener's granulomatosis. In polyarteritis nodosa (PAN) and Churg- Strauss syndrome, few data are available and correlation between clini cal manifestations and autineutrophil cytoplasm antibodies (ANCA) has not been established. Therefore, we tested, before treatment of vascul itis, 62 consecutive patients suffering from PAN with hepatitis B viru s (HBV) markers, PAN of unknown etiology or Churg-Strauss syndrome. Me thods. Only patients with PAN and Churg-Strauss syndrome were included in the study. The diseases were histologically and/or angiographicall y proven. Every patient's serum was tested by an indirect immunofluore scence assay (IFA) and, in 37 cases, by an enzyme linked immunosorbent assay (ELISA). Results. ANCA detected by IFA were observed in 10.7% o f the patients with PAN with HBV markers, in 27.3% of the patients wit h PAN without HBV markers and in 66.7% of the patients with Churg-Stra uss syndrome. When ELISA was performed, 11.1% of the patients with PAN associated with HBV infection, 20% of the patients with PAN without H BV markers and 55.6% of the patients with Churg-Strauss syndrome were positive. ANCA were positively correlated with asthma and purpura and negatively correlated with HBV markers. Conclusion. Regardless of the technique used, Churg-Strauss syndrome was associated with ANCA in abo ut 60% of the cases while, in PAN of unknown etiology, ANCA were found in about 25% of cases. In contrast, IFA and ELISA only detected ANCA in a limited number of cases of PAN related to HBV infection. ELISA po sitivity in patients with PAN and Churg-Strauss syndrome was usually a ssociated with antimyeloperoxidase antibodies. In our cases of PAN, AN CA and purpura were significantly correlated, suggesting that, in thes e cases, small vessels are involved and therefore macroscopic and micr oscopic PAN coexist. Thus it seems that ANCA are essentially present i n the cases of small vessel vasculitis, as has been described, and are not a marker of pure macroscopic PAN, at least at our present level o f understanding of these antibodies.