Idiopathic pulmonary hemosiderosis

Idiopathic pulmonary hemosiderosis (IPH), also known as idiopathic pulmonary haemosiderosis or Ceelen-Gellerstedt syndrome, is a rare disorder characterized by recurrent episodes of diffuse alveolar hemorrhage (bleeding into the lungs) without an identifiable underlying cause. The disease primarily affects the respiratory system, leading to the accumulation of iron (hemosiderin) in the lung tissue from repeated bleeding episodes. Over time, this can result in progressive pulmonary fibrosis and impaired lung function. The hallmark clinical features of IPH include a triad of iron deficiency anemia, hemoptysis (coughing up blood), and diffuse pulmonary infiltrates seen on chest imaging. Patients frequently present with chronic or recurrent cough, dyspnea (shortness of breath), fatigue, pallor, and failure to thrive in children. The disease most commonly presents in childhood, though it can occur at any age. Episodes of acute pulmonary hemorrhage may alternate with periods of remission. The course of the disease is highly variable, ranging from mild intermittent episodes to severe, life-threatening hemorrhage. Diagnosis is typically made by excluding other causes of pulmonary hemorrhage, such as vasculitis, autoimmune conditions (including Goodpasture syndrome), infections, and cardiac disease. Bronchoalveolar lavage revealing hemosiderin-laden macrophages supports the diagnosis. Treatment is largely supportive and empirical, as no definitive cure exists. Systemic corticosteroids are the mainstay of therapy during acute episodes and are sometimes used as maintenance therapy to reduce recurrence. Immunosuppressive agents such as azathioprine, hydroxychloroquine, or cyclophosphamide may be considered in refractory cases. Iron supplementation is often necessary to manage chronic anemia. Long-term prognosis varies, with some patients achieving prolonged remission while others develop progressive pulmonary fibrosis and respiratory failure.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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