[Association between pulmonary hemosiderosis and juvenile dermatomyositis].

INTRODUCTION

Pulmonary hemosiderosis (PH) is characterized by recurrent diffuse intra-alveolar hemorrhage associated to the presence of hemosiderin deposits inside macrophages. In the present Pediatric Rheumatology Service, during a 24 years period, 143 patients had JDM diagnosis and only one of them had associated PH (0.7%). There is no previous case report of PH and juvenile dermatomyositis (JDM) associated.

CASE REPORT: A male patient, 13 years old of age had recurrent anemia, cough, hemoptysis for 8 months. He had severe anemia and lung interstitial infiltrate on radiology studies. Bronchoalveolar lavage demonstrated hemosiderin deposits inside macrophage cells and positive Perl's reaction with PH diagnosis. The treatment was initiated with corticosteroids. After one month, he developed muscle weakness, Gottron's papules and elevated serum muscle enzymes. Muscle biopsy disclosed JDM diagnosis. Due to recurrent pulmonary hemorrhage, monthly methylprednisolone and cyclophosphamide pulses and intravenous immunoglobulin (IVIG) were started and clinical remission was achieved. He developed massive pulmonary hemorrhage, death and the necropsy confirmed PH.

DISCUSSION: PH had never been reported in the JDM population. The PH was a severe clinical complication and occurred previously to JDM diagnosis.