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FRI0162 Clinical features of a set of patients with anti-neutrophil cytoplasmic antibodies
  1. EG Tomero Muriel,
  2. G García-Melcón,
  3. R Velayos,
  4. AM Ortiz,
  5. I González-Alvaro
  1. Rheumatology, Hospital de La Princesa, Madrid, Spain

Abstract

Objectives To analyse characteristics and clinical features of patients with positive antineutrophil cytoplasmic antibodies (ANCA).

Methods Retrospectively we reviewed 71 patients tested for the presence of ANCA using indirect inmunofluorescence (IFF; ptterns pANCA and cANCA) and/or antimieloperoxidase ELISA (aMPO) from 1993 to 1999. The variables observed were age, sex, origin department, clinical manifestations, final diagnosis, disease evolution and treatment.

Results Mean age was 60.2 years (16–97) being women 63.3%. cANCA was positively tested in 42% of patients and pANCA in 58%(59% aMPO). The distribution among departments was: Internal Medicine 22.5%, Rheumatology 22.5%, Nephrology 16.5%, Pneumology 14.9%, Gastroenterology 13.5% and Dermatology 12%. 28 patients suffered from lung affections, 27 had kidney affections (30% rapidly progressing glomerulonephritis), 18 skin involvement, 19 musculoesqueletal manifestations, 5 hearts affections, 3 neuropathies and 2 eye involvement. Diagnosis were: 5 Wegeners Granulomatosis, 5 Microscopic polyangiitis, 8 pANCA associated vasculities, 4 leucocytoclastic vasculitis, 7 rheumatoid arthritis, 7 inflammatory bowel diseases, 6 systemic inmunolopathologycal diseases, 21 not autoimmune disorders and 8 without final diagnosis. Progress: 42% suffered from periodical relapses, 28% without clinical manifestations, 21% died. 53% of death were directly caused by ANCA associated vasculitis, 26% were due to concomitant infections and the rest were caused by non related complications.

Conclusion 1. Despitethe high percentage of detected cANCA by IIF only Wegeners granulomatosis were diagnosed, so the use of ELISA antiproteinase-3 must be used. 2. It was observed a high mortality rate in patients in patients with positive ANCA.

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