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pulmonary capillary hemangiomatosis

Epidemiology: rare Pathology: - thin-walled microvessels infiltrating the peribronchial & perivascular interstitium, the lung parenchyma, & the pleura - proliferating microvessels are prone to hemorrhage, resulting in accumulation of hemosiderin-laden macrophages in alveolar spaces Genetics: - associated with EIF2AK4 variants [3] Clinical manifestations: - dyspnea - right heart failure - less common - hemoptysis - pleural effusion Laboratory: - see ARUP consult [3] Special laboratory: - pulmonary artery catheter - normal wedge pressure - lung biopsy required for diagnosis [2] Radiology: - chest computed tomography - interstitial infiltrates - pulmonary nodules - pleural effusion Differential diagnosis: - pulmonary hypertension - pulmonary venoocclusive disease Complications: - pulmonary hypertension Management: - prognosis is poor, median survival 3 years - interferon alfa 2A - epoprostenol used to treat pulmonary hypertension may cause pulmonary edema - lung transplantation

General

capillary disease lung disease

References

  1. Almagro P et al Pulmonary capillary hemangiomatosis associated with primary pulmonary hypertension: report of 2 new cases and review of 35 cases from the literature. Medicine (Baltimore). 2002 Nov;81(6):417-24. PMID: 12441898
  2. Kothari SS et al Images in Cardiovascular Medicine. Pulmonary Capillary Hemangiomatosis. Circulation. 2009; 120: 352-354 PMID: 19635980 http://circ.ahajournals.org/content/120/4/352.full
  3. ARUP Consult: EIF2AK4-Associated Disorders https://arupconsult.com/ati/eif2ak4-associated-disorders