Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0034063 (pulmonary edema)
 10,665 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Continuous intravenous infusion of prostacyclin is an effective treatment for primary pulmonary hypertension (PPH), and has recently been shown to be of benefit in PH associated with scleroderma (SSc). Pulmonary capillary hemangiomatosis (PCH) is a rare cause of PPH. Prostacyclin therapy has been complicated by pulmonary edema in cases of PCH. We describe a case of PH associated with limited SSc, where treatment with prostacyclin was complicated by pulmonary edema. Autopsy revealed PCH as the pathologic basis for the PH. There were no clinical features of PCH prior to initiation of vasodilator therapy, illustrating the potential difficulty in establishing the diagnosis. This is the first reported case of PCH in SSc.
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PMID:Pulmonary edema complicating prostacyclin therapy in pulmonary hypertension associated with scleroderma: a case of pulmonary capillary hemangiomatosis. 1072 66

Pulmonary capillary hemangiomatosis (PCH) is a rare cause of primary pulmonary hypertension characterized by thin-walled microvessels infiltrating the peribronchial and perivascular interstitium, the lung parenchyma, and the pleura. These proliferating microvessels are prone to bleeding, resulting in accumulation of hemosiderin-laden macrophages in alveolar spaces. Here we report 2 cases of PCH with pulmonary hypertension, 1 of them associated with mechanical intravascular hemolysis, a feature previously reported in other hemangiomatous diseases, but not in PCH. Case 2 was diagnosed by pulmonary biopsy; to our knowledge the patient is the second adult to be treated with interferon alpha-2a. Review of the literature identified 35 patients with PCH and pulmonary hypertension. The prognosis is poor and median survival was 3 years from the first clinical manifestation. Dyspnea and right heart failure are the most common findings of the disease. Hemoptysis, pleural effusion, acropachy, and signs of pulmonary capillary hypertension are less common. Chest X-ray or computed tomography scan usually shows evidence of interstitial infiltrates, pulmonary nodules, or pleural effusion. Hemodynamic features include normal wedge pressures. Radiologic and hemodynamic findings are undifferentiated from those of pulmonary veno-occlusive disease but differ from other causes of primary pulmonary hypertension. Epoprostenol therapy, considered the treatment of choice in patients with primary pulmonary hypertension, may produce pulmonary edema and is contraindicated in patients with PCH. Regression of lesions was reported in 1 patient treated with interferon therapy and 2 other patients stabilized, including our second patient. PCH was treated successfully by lung transplantation in 5 cases. Early recognition of PCH in patients with suspected primary pulmonary hypertension is possible based on clinical and radiologic characteristics. Diagnosis by pulmonary biopsy is essential for allowing appropriate treatment.
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PMID:Pulmonary capillary hemangiomatosis associated with primary pulmonary hypertension: report of 2 new cases and review of 35 cases from the literature. 1244 98

Pulmonary capillary hemangiomatosis (PCH) is a rare and an only recently described vascular disease. As one of the causes of primary pulmonary hypertension, the need for the establishment of helpful diagnostic radiological criteria for this condition has become imperative. However, the unstable hemodynamic condition of patients with primary pulmonary hypertension often denies pathological sampling as a definitive diagnostic tool. This diagnostic dilemma can be fatal to those patients with PCH treated for pulmonary hypertension because a fatal pulmonary edema may occur. We report a case of PCH in a 26-year-old patient who died of severe circulatory compromise while undergoing transplant evaluation. A discussion of the computed tomography findings, a review of the pathological features, and a review of the relatively limited imaging literature available on this subject are conducted.
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PMID:Pulmonary capillary hemangiomatosis imaging findings and literature update. 1788 42