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Four children were diagnosed with idiopathic pulmonary hemosiderosis (IPH), over a period of 4 years. Retrospectively, antineutrophil cytoplasmic antibodies (ANCA) were studied by indirect immunofluorescence (IIF) and ELISA in 18 sera from these patients, stored at ‐20°C. ANCA‐positive sera, from 1/20 to 1/1, 200 dilution, were found in 3/4 of the patients, by IIF. The patient with the highest titre of ANCA died 3 months later during an acute crisis, the other two patients need a minimal dose of steroids. In one case only, a patient who is still without treatment, had no ANCA. The antibodies anti‐myeloperoxidase and anti‐pro‐teinase‐3 were negative or at border line levels. Rheumatoid factor, anti‐nuclear (Hep‐2), anti‐endomysial, anti‐reticulin and antibasement 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. The presence of serum ANCA may help to classify children with pulmonary haemorrhage and may have a prognostic value.
Pediatric Allergy and Immunology – Wiley
Published: Nov 1, 1994
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