pulmonary capillary hemangiomatosis
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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
Clinical manifestations
- dyspnea
- right heart failure
- less common
Laboratory
Diagnostic procedures
- pulmonary artery catheter
- normal wedge pressure
- lung biopsy required for diagnosis[2]
Radiology
Complications
Differential diagnosis
Management
- prognosis is poor, median survival 3 years
- interferon alfa 2A
- epoprostenol used to treat pulmonary hypertension may cause pulmonary edema
- lung transplantation
More general terms
References
- ↑ 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: https://www.ncbi.nlm.nih.gov/pubmed/12441898
- ↑ 2.0 2.1 Kothari SS et al Images in Cardiovascular Medicine. Pulmonary Capillary Hemangiomatosis. Circulation. 2009; 120: 352-354 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/19635980 <Internet> http://circ.ahajournals.org/content/120/4/352.full
- ↑ 3.0 3.1 3.2 ARUP Consult: EIF2AK4-Associated Disorders https://arupconsult.com/ati/eif2ak4-associated-disorders