Pulmonary capillary hemangiomatosis is a locally aggressive benign vascular neoplasm of lung characterized by cytologically benign thin walled capillary–sized blood vessels proliferating diffusely through the pulmonary interstitium in and around airways and pulmonary vessels.It is a rare disease characterized by pulmonary hypertension and a slowly progressive clinical course.This case reports describe the clinical presentation,diagnostic approach and problems encountered in this rare but clinically important disease.CASE REPORT: Patient is a 51 year old male who presented with dyspnea,functional Class III of the NYHA.He was diagnosed with congestive heart failure of unknown aetilogy seven to eight yrs ago and did reasonably well until recently when his dyspnea worsened and he had two episodes of pre syncope.He also carried the diagnosis of obstructive sleep apnea,diabetes mellitus and hypertension.Physical examination revealed room air saturation of 77% and increasing to 94% with 2 litres of oxygen.He had a loud P2,fixed split,right sided S3 and right ventricular heave.As part of his work up he had an of ANA 1:160 in a speckled pattern.Echo showed dilation of RA/RV,moderately-severe TR with elevated RVSP and EF of 55 %.CXR showed prominent hilar region.High resolution CT showed diffuse ground opacities.Spirometry showed mild obstructive and restrictive pattern.Right Heart catheterization showed right atrial pressure of 9,right ventricular pressure of 79/10, pulmonary artery pressure of 80/28 with mean of 49,wedge pressure was 12.There was no significant response to intravenous prostacyclin.Because of concern for pulmonary veno-occlusive disease he was not started on flolan and underwent a surgical biopsy.Biopsy showed hemosiderin laden alveolar macrophages,vascular changes consistent with pulmonary hypertension and foci resembling pulmonary capillary hemangiomatosis.DISCUSSION: Pulmonary capillary hemangiomatosis is a rare disease with proliferation of thin walled alveolar capillaries associated with infiltration of lung parenchyma and invasion of blood vessels and bronchial tree. We are aware of only twenty nine cases that have been reported.It usually presents as pulmonary hypertension and is confused clinically with primary pulmonary hypertension and pulmonary veno-occlusive disease.It usually presents between ages of 20-40 years with dyspnea,hemoptysis and abnormal radiographic findings.The typical clinical course is that of rapid deterioration. Early diagnosis and bilateral lung transplantation is the only cure. One case of successful treatment has been reported with interferon alpha-2a.There have been case reports of PCH- like foci that were incidental findings at autopsy in which there was no clinical evidence of pulmonary hypertension.PCH-like foci are more likely to be seen in autopsies if multiple lung section are reviewed.It is a clinically significant finding only if the patient has signs and symptoms of pulmonary hypertension.However,the patient should be followed closely.The case report in this article describes all the difficulties encounter in diagnosis and management,late diagnosis,confusion clinically with primary hypertension, veno-occlusive disease and no good, definite treatment so far besides transplantation.Our patient was recently started on Bosentan and we will follow him up clinically and with some objective measurement to see whether his dyspnea and pulmonary hemodynamics has improved or not.There has been no reported cases of therapy with this medication to date that we are aware of.
The cause of PCH is unknown and it is a rare disorder,most cases diagnosed postmortem since diagnosis is delayed. Successful therapy to date has been described with interferon (1 case report) and bilateral lung transplant that offers long term survival.Further studies are needed to settle clear understanding of pathogenesis that will lead to new therapeutic strategies.
N. Salamat, None.