Ceelen-Gellerstedt-Syndrom
Idiopathic pulmonary hemosiderosis (IPH) is an uncommon form of pulmonary hemosiderosis. It is characterized by the triad of
- hemoptysis
- iron deficiency anemia
- diffuse pulmonary infiltrates, usually represented by diffuse pulmonary hemorrhage
The diagnosis is usually made by exclusion .
Epidemiology
It is rare disorder that can occur at any age but typically affects children and young adults. The age of presentation may be bimodal, with frequency peaks in children less than five years of age and in adolescents 11 years or older. There is no definite recognized gender predilection.
Pathology
Hemosiderin-laden macrophages are found in the sputum or at bronchoalveolar lavage. Its exact cause is unknown but may be related to an autoimmune process. Patients have recurrent episodes of diffuse pulmonary hemorrhage without associated glomerulonephritis or serological abnormality.
Radiographic features
HRCT chest
Can be variable dependent on the phase and extent of the disease. Can range from showing diffuse pulmonary hemorrhage to fibrotic changes in the context of repeated events.
Treatment and prognosis
It can have a variable natural history . Some authors suggest corticosteroid (alone or in combination with other immunosuppressive agents) for exacerbations or maintenance therapy.
History and etymology
It was first described by Ceelen in 1921.
Siehe auch:
Assoziationen und Differentialdiagnosen zu Ceelen-Gellerstedt-Syndrom:
