Abstract
Four children were diagnosed of IPH, during a period of 4 years. Retrospectively, ANCA were studied by indirect immunofluorescence (IIF) and ELISA in 18 sera of these patients, stored at -20°C. ANCA positive sera, from 1/20 to 1/1,200 dilution, were found in sera of 3/4 of the patients, by IIF. The patient with the highest titre of ANCA died 3 months later during an acute crisis. The only case, in permanent remission without treatment, has no ANCA. The other two patients need a minimal dose of steroids. The antibodies anti-myeloperoxidase and anti-proteinase-3 were negative or in questionable levels. Rheumatoid factor, anti-nuclear (Hep-2), anti-endomysial, anti-reticulin and anti-basement membrane antibodies were negative in all sera. The surviving patients were followed-up for more than 10 years with no systemic or renal disease appearences. Serum ANCA must be studied in patients diagnosed of IPH. The presence of these antibodies may have a prognostic value although their lessive role is still discussed.
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