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

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