Introduction:

pulmonary hemosiderosis (PH) is a rare, chronic disease characterized by alveolar hemorrhage and accumulation of hemosiderin in alveolar macrophages. It can occur as a primary lung disease or secondary to vasculitis and heart disease.

Objectives:

to describe the diagnostic and therapeutic approach used on a girl with PH secondary to microscopic polyangiitis.

Clinical case:

2 years of age, female. Down syndrome (DS), corrected atrioventricular septal defect. Severe bronchiolitis at 22 months, later recurrent wheezing. One month prior to admission, severe iron deficiency anemia requiring transfusion. Four days prior, respiratory symptoms and progressive fatigue occur in apyrexia. Physical examination: reactive, fair general condition, non-mucosal skin pallor. Drawings, wheezing and bilateral subcrackles. Systolic murmur 2/6. We carried out oxygen therapy, bronchodilators and corticosteroids. Radiography: bilateral and diffuse interstitial infiltrate, without cardiomegaly. Hemogram: mild, microcytic, hypochromic anemia. Iron metabolism confirms iron deficiency. Urine: transient microhematuria. Normal kidney function. Fiberoptic bronchoscopy with bronchioalveolar lavage (LAB): macrophages with brownish granules suggestive of hemosiderin. Etiological studies: Antibodies (Ab): positive antinuclear (1/80), negative anticardiolipins and lupus inhibitor. Negative anti-glomerular basement membrane (GBM) Ab. Positive anti-neutrophil cytoplasmic Ab (ANCA), P-ANCA pattern, positive anti-myeloperoxidase Ab (MPO), negative anti-proteinase 3 Ab (PR3). After the presentation of microscopic polyangiitis, we started administering methylprednisolone, then oral corticosteroids and immunosuppressants. Clinical improvement, without relapses, oxygen was suspended, microhematuria did not recur, she continued receiving mycophenolate during follow-up.

Discussion:

severe iron deficiency anemia with hemodynamic repercussions in a child with DS led to HP. The LAB confirmed it. The absence of renal involvement with transient microhematuria and negative anti-GBM antibodies ruled out Goodpasture syndrome. Positive ANCA Abs with the detected pattern indicated microscopic polyangiitis. The prognosis is variable, usually worse in cases with relapses, corticosteroid dependence or lack of complete recovery. Individualized, interdisciplinary long-term follow-up is suggested.

        · resumen en Español | Portugués     · texto en Español     · Español ( pdf )