Hemosiderosis pulmonar idiopática: seguimiento de pacientes durante 25 años (1995-2019)

Abstract

Idiopathic pulmonary hemosiderosis (IPH) is a cause of diffuse alveolar hemorrhage. Objective: to describe the evolution of children with IPH in our institution. Retrospective monitoring with a follow-up protocol was carried out. 13 patients, seven males, were recruited. From an agricultural area (6/13). Not all of patients had the complete diagnostic triad: cotton infiltrates (9/13), anemia (11/13), hemop- tysis (9/13). Hemosiderin-laden macrophages counting in the bronchoalveolar lavage fluid was over 30% in all the patients. Computed chest tomography was informed as normal (5/13), interstitial pattern (5/13), ground glass (2/13) and fibrosis (1/13). Spirometry: normal (7/13), restrictive (4/13), obstructive (1/13) and not performed (1/13). Treatment during the acute phase: bolus of methylprednisolone (7/13) or prednisone (6/13) or hydrocortisone (1/13). In the maintenance phase: prednisone (13/13) plus an immunosuppressant, azathioprine (12/13), hydroxychloroquine (1/13), mycophenolate (1/13), plus bu- desonide MDI (13/13). Eight patients stopped the bleeding episodes. Two patients died and there were five physiological pregnancies in 3 adolescents. It was observed:(a) different modes of IPH presentation that delayed its diagnosis; (b) large exposure to pesticides; (c) prognosis improved if diagnosis and treatment were early, also in adolescent girls; (d) most of the patients stopped the bleeding episodes.