Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019079 (hemoptysis)
 6,129 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Idiopathic pulmonary hemosiderosis (IPH) is a rare disorder characterised by intermittent, diffuse alveolar hemorrhage (DAH). Although an inflammatory pulmonary capillaritis can be evidenced in most patients with DAH, IPH is a distinct entity in which pulmonary inflammatory alterations are lacking. Most cases occur in children, although the disease has been exceptionally reported in adults too. Here, we, describe a case of IPH in a 30-year-old woman who was admitted to our hospital because of recurrent episodes of hemoptysis since the age of 21. IPH was diagnosed on the basis of: 1) an open lung biopsy showing focal alveolar edema and hemorrhage without parenchymal inflammatory alterations, 2) a bronchoalveolar lavage showing hemosiderin-laden macrophages, and 3) exclusion of infectious or immunologic causes of hemoptysis. Prednisone administration could control the disease, but every attempt to lower the dose to less than 25 mg per day was followed by recurrence of hemoptysis. Then, azathioprine was started, and after three months prednisone was gradually tapered to the dose of 10 mg per day, without any relapse of the disease. These findings indicate that azathioprine, in combination with prednisone, may be an effective therapy for IPH and suggest that an immunologic mechanism could be involved in the pulmonary capillary damage underlying alveolar bleeding.
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PMID:Idiopathic pulmonary hemosiderosis in an adult. Favourable response to azathioprine. 1166

A 61-year-old white man was admitted to our department because of severe back and upper abdominal pain of 1 month's duration. The patient was diagnosed with Wegener granulomatosis 10 months before the presentation based on chronic otitis media, hoarseness, and hemoptysis; positive c-ANCA; and laryngeal and lung biopsies showing multinucleated giant cells. The patient was treated with monthly injections of cyclophosphamide (1-1.5 g per month) and 80 mg prednisone daily with rapid improvement. Prednisone dose was tapered off and 1 month before the present admission, the patient developed severe low back pain. Extensive workup, including abdominal computed axial tomography scan, computed tomography angiography, magnetic resonance image of the spinal cord, and fluorodeoxyglucose-positron emission tomography (FDG-PET) scan, revealed 2 periaortic soft tissue structures seen at the level of L3 and at the level of the celiac trunk and linear meningeal thickening of the spinal cord at the level of D4-8. All these structures showed strong signal on FDG-PET scan. Treatment with methylprednisolone (1000 mg/d) for 3 consecutive days followed by 80 mg prednisone per day and 100 mg cyclophosphamide per day was started with rapid attenuation of the patient's symptoms. This case describes the clinical course of the rare complication of Wegener granulomatosis, periaortitis, and dural inflammation despite monthly cyclophosphamide and demonstrates the role of magnetic resonance imaging and FDG-PET in their diagnosis.
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PMID:Wegener granulomatosis with back pain, periaortitis, and dural inflammation developing while receiving monthly cyclophosphamide. 1714 61