대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Pulmonary capillary hemangiomatosis

Radiologic Findings

Fig 1: The chest radiograph shows diffuse reticular opacities with poorly circumscribed nodular opacities.
Fig 2-4: Axial and coronal reformatted CT images show diffuse centrilobular nodules of ground-glass opacity and interlobular septal thickening. A mildly dilated pulmonary artery and a straightened interventricular septum suggest pulmonary hypertension. A small amount of bilateral pleural effusion is also noted.

Brief Review

Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension that results from abnormal capillary proliferation within the alveolar interstitium. These proliferating capillaries infiltrate the interstitium of the lung, the walls of small pulmonary vessels, bronchi, and pleura. Although regarded as different disease entities, PCH and PVOD may in fact represent a continuum of the same disease process. The dilated capillaries secondary to the passive congestion in the setting of distal venous obstruction in PVOD may give rise to PCH.
Progressive dyspnea and fatigue characterize the clinical manifestations of both PVOD and PCH, which are thus typically misdiagnosed as pulmonary arterial hypertension (PAH). The only clinical features that may distinguish PCH from PVOD are the presence of hemoptysis (evident in 30% of patients with PCH but not reported in PVOD) and hemorrhagic pleural effusions (absent in PVOD but reported in up to 25% of patients with PCH).
Chest radiography demonstrates enlarged central pulmonary arteries and right-sided prominence of the heart, accompanied by diffuse or bibasilar reticulonodular or micronodular areas of opacity.
At CT, main pulmonary arterial enlargement and widespread ill-defined centrilobular nodules of ground-glass opacity are consistently described in PCH, often mixed with lobular ground-glass opacities. Sporadically reported findings include septal thickening, lymphadenopathy, pleural effusion, enlargement of the right chambers of the heart, and pericardial effusion.
Establishing the correct diagnosis is critically important, as therapies commonly used for treatment of patients with PAH can be harmful or even fatal in patients with PVOD or PCH. In patients with PVOD and PCH, vasodilator treatment likely results in increased transcapillary hydrostatic pressure and pulmonary edema because of the presence of fixed, elevated pulmonary venous resistance.
Overall, the prognosis of the disease is poor, with a median survival of 3 years from the time of diagnosis. Lung transplantation remains the only effective therapy.

References

1. Frazier AA, Franks TJ, Mohammed TL, Ozbudak IH, Galvin JR.From the Archives of the AFIP: pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis. Radiographics. 2007;27(3):867-82.
2. Webb, W. R., M

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