[Diagnostics and therapy of idiopathic pulmonary hemosiderosis].
Idiopathic pulmonary hemosiderosis (IPH) is a rare clinical entity characterized by recurrent episodes of diffuse alveolar hemorrhage. The disease--also called Ceelen's syndrome--was subsequently defined as a clinical entity comprising the triade of hemoptysis, opacities in X-ray, and anemia, in which the etiology is still unknown. Intensive search for a specific etiology ends up negative, and there are no features, which are specifically pathognomonic for IPH. Therefore, the diagnosis relies solely on the exclusion of other disorders in which diffuse alveolar hemorrhage is a cardinal sign. Acute episodes may occur frequently, eventually leading to lung fibrosis in the chronic stage. Usually, the therapy consists of high doses of corticosteroids, which can be combined with immunosuppressive drugs. In addition to this review, a case having Ceelen's syndrome is presented. After a complicated clinical course, the patient could finally be stabilized with a combination therapy of prednisolone and azathioprine.
Willms
Hinrich
H
Robert-Koch-Klinik, Thoraxzentrum des Klinikums St. Georg, Leipzig.
Gutjahr
Kathleen
K
Juergens
Uwe R
UR
Hammerschmidt
Stefan
S
Gessner
Christian
C
Hoheisel
Gerhard
G
Wirtz
Hubert
H
Gillissen
Adrian
A
D000328:Adult; D018798:Anemia, Iron-Deficiency / Q000209:etiology / Q000473:pathology; D001379:Azathioprine / Q000008:administration & dosage; D001706:Biopsy; D001999:Bronchoscopy; D003937:Diagnosis, Differential; D006469:Hemoptysis / Q000209:etiology / Q000473:pathology; D006470:Hemorrhage / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D006486:Hemosiderosis / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008168:Lung / Q000473:pathology; D008171:Lung Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D008297:Male; D011239:Prednisolone / Q000008:administration & dosage; D011650:Pulmonary Alveoli / Q000473:pathology; D013577:Syndrome; D014057:Tomography, X-Ray Computed
D007166:Immunosuppressive Agents; D011239:Prednisolone; D001379:Azathioprine