Pulmonary capillary hemangiomatosis (PCH) is a rare disease usually associated with pulmonary hypertension which is classified in group I as per the World Health Organization (WHO) classification of pulmonary arterial hypertension (PAH). It is commonly presented as effort intolerance and hypoxia. Pulmonary capillary hemangiomatosis can present with ground-glass opacity on chest radiograph which can mimic interstitial lung disease. We need to consider PCH also in the differential diagnosis of interstitial shadows with these clinical presentations and should consider diagnostic evaluation for the same in case of unsatisfactory clinic-radiological response to treatment. We describe a case presented and treated as interstitial lung disease, which turned out to be PCH on lung biopsy. Interestingly, the case was not associated with any evidence of PAH.
O'Keefe MC, Post MD. Pulmonary capillary hemangiomatosis: A rare cause of pulmonary hypertension. Arch Pathol Lab Med 2015;139(2):274–277. DOI: 10.5858/arpa.2013-0500-RS.
Eltorky MA, Headley AS, Winer-Muram H, et al. Pulmonary capillary hemangiomatosis: A clinicopathologic review. Ann Thorac Surg 1994;57(3):772–776. DOI: 10.1016/0003-4975(94)90595-9.
DuBrock HM, Kradin RL, Rodriguez-Lopez JM, et al. Pulmonary capillary hemangiomatosis: The role of invasive cardiopulmonary exercise testing. Pulm Circ 2015;5(3):580–586. DOI: 10.1086/682227.
Assad AM, Kawut SM, Arcasoy SM, et al. Platelet derived growth factor is increased in pulmonary capillary hemangiomatosis. Chest 2007;131(3):850–855. DOI: 10.1378/chest.06-1680.
Best DH, Sumner KL, Austin ED, et al. EIF2AK4 mutations in pulmonary capillary hemangiomatosis. Chest 2014;145(2):231–236. DOI: 10.1378/chest.13-2366.
Faria IM, Carneiro LH, Tiradentes TA, et al. Pulmonary capillary hemangiomatosis: An uncommon cause of pulmonary hypertension. J Bras Pneumol 2013;39:390–392. https://doi.org/10.1590/S1806-37132013000300019.
Frazier AA, Franks TJ, Mohammed TL, et al. From the archives of AFIP: Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis. Radiographics 2007;27(3):867–882. DOI: 10.1148/rg.273065194.
Kadowaki T, Yano S, Kobayashi K, et al. Pulmonary capillary hemangiomatosis-like foci detected by high resolution computed tomography. Intern Med 2010;49(2):175–178. DOI: 10.2169/internal medicine.49.2739.
Takiguchi Y, Uruma T, Hiroshima K, et al. Stable pulmonary capillary haemangiomatosis without symptomatic pulmonary hypertension. Thorax 2001;56(10):815–817. DOI: 10.1136/thorax. 56.10.815.
Wang W-J, Hong C, Han Q, et al. Pulmonary capillary hemangiomatosis without pulmonary hypertension: An early stage of disease? Chin Med J 2018;131(2):245. DOI: 10.4103/0366-6999.222326.
Nayyar D, Muthiah K, Kumarasinghe G, et al. Imatinib for the treatment of pulmonary arterial hypertension and pulmonary capillary hemangiomatosis. Pulm Circ 2014;4(2):342–345. DOI: 10.1086/675996.
Langleben D. Pulmonary capillary hemangiomatosis: The puzzle takes shape. Chest 2014;145(2):197–199. DOI: 10.1378/chest. 13-2513.