Analysis of 9 adult patients with idiopathic pulmonary hemosiderosis

Introduction: Alveolar hemorrhage syndrome (AHS) whose cause could not be found is called as idiopathic pulmonary hemosiderosis (IPH). Method: Among the patients followed with a diagnosis of AHS since 2000, files of patients having diagnosis of IPH were investigated retrospectively. Results: 9 patients with IPH were detected. All patients were male. Hemosiderin laden macrophages were seen in biopsy samples or in BAL fluid in all of the patients. Associated Celiac disease was found in 3 patients. Seven patients had hemoptysis, 5 patients had dyspnea and 4 patients had cough. Anemia was detected in 6 patients. DLCO/VA% value was markedly increased (151%) in one of the 6 patients having diffusion test. None of the 8 patients having spirometry test had a FVC% value lower than 80%. Ground glass opacity was the most frequent finding in HRCT. Steroids were used for the treatment in 5 patients. Discussion and Conclusion: According to our results, Celiac disease should be investigated in all patients with IPH. Existence of patients without hemoptysis suggests that absence of hemoptysis does not need to rule out IPH. DLCO/VA was elevated only in one patient. Low or normal DLCO does not rule out IPH. Existence of ground glass opacity in HRCTs of a significant number of patients suggests that IPH should be kept in mind in the differential diagnosis of ground glass opacity.