Antineutrophil cytoplasmic antibodies (ANCA) in idiopathic pulmonary hemosiderosis

Abstract

Four children were diagnosed with idiopathic pulmonary hemosiderosis (IPH), over a period of 4 years. Retrospectively, antineurtrophil cytoplasmic antibodies (ANCA) were studied by indirect immunofluorescence (IIF) and ELISA in 18 sera from these patients, stored at -20 degrees 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-proteinase-3 were negative or at border line levels. Rheumatoid factor, antinuclear (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 appearances. The presence of serum ANCA may help to classify children with pulmonary haemorrhage and may have a prognostic value.

Maintenance therapy with dose‐adjusted 6‐mercaptopurine in idiopathic pulmonary hemosiderosis

“…Although its etiology remains unclear, IPH is considered to be an immune-mediated disease. 1,2 The incidence of IPH is very low and was estimated at 0.24 and 1.23 cases per million in some selected populations. 3,4 The clinical course of IPH is greatly variable, and death may occur from acute pulmonary hemorrhage or after progressive pulmonary insufficiency resulting in chronic respiratory failure.…”

Idiopathic pulmonary haemosiderosis: An Oriental experience

“…Pulmonary haemosiderosis (PH) is an abnormal accumulation of hemosiderin in the lungs, which results from diffuse alveolar haemorrhage, 1,2 and may occur as a primary process in the lung or secondary to cardiac diseases, bleeding disorders, collagen‐vascular diseases, or systemic vasculitis 2 . If other disorders associated with intrapulmonary bleeding can be excluded, PH is considered to be idiopathic.…”

Recurrence of Idiopathic Pulmonary Hemosiderosis Associated with Rising Plasma IgE Levels and Antibody Titers to Specific Inhalants during a 10-Year Follow-Up

“…2,3 In addition, pulmonary hemorrhage has been associated with microscopic polyarteritis and segmental, necrotizing crescent glomerulonephritis, 4,5 and with positive antineutrophil cytoplasmic antibodies directed against myeloperoxidase. 4,6 These previous reports strongly suggest that pul-SHIBATA ET AL. monary alveolar hemorrhage results from alveolar or alveolocapillary membrane destruction due to alveolar membrane-antibody reaction or autoimmune microvasculitis.…”