In search of markers of treatment failure and poor prognosis in IPAH – the value of mosaic lung attenuation pattern on thin-section CT scans

Monika Szturmowicz; Aneta Kacprzak; Barbara Burakowska; Marcin Kurzyna; Anna Fijałkowska; Iwona Bestry; Adam Torbicki

doi:10.4081/mrm.2010.562

In search of markers of treatment failure and poor prognosis in IPAH – the value of mosaic lung attenuation pattern on thin-section CT scans

Authors

Monika Szturmowicz Department of Internal Medicine, National Institute of Tuberculosis and Lung Diseases, Warsaw
Aneta Kacprzak Department of Internal Medicine, National Institute of Tuberculosis and Lung Diseases, Warsaw
Barbara Burakowska Department of Radiology, National Institute of Tuberculosis and Lung Diseases, Warsaw
Marcin Kurzyna Department of Internal Medicine, National Institute of Tuberculosis and Lung Diseases, Warsaw
Anna Fijałkowska Department of Internal Medicine, National Institute of Tuberculosis and Lung Diseases, Warsaw
Iwona Bestry Department of Radiology, National Institute of Tuberculosis and Lung Diseases, Warsaw
Adam Torbicki Department of Internal Medicine, National Institute of Tuberculosis and Lung Diseases, Warsaw

DOI: https://doi.org/10.4081/mrm.2010.562

Keywords:

CT scan, idiopathic pulmonary hypertension, mosaic lung attenuation, pulmonary veno-occlusive disease.

Abstract

Despite the development of specific therapies for pulmonary arterial hypertension (PAH) some patients fail to respond to such treatment. One of the potential reasons for the unresponsiveness to targeted therapies may be the presence of fibrous occlusion of small pulmonary veins that accompanies pre-capillary arteriopathy. This type of pathologic change is called pulmonary veno-occlusive disease (PVOD). Underdiagnosed PVOD occurs probably in 5-10% of idiopathic pulmonary hypertension (IPAH) and in a substantial proportion of PAH related to connective tissue diseases (mainly in scleroderma). A definite diagnosis of PVOD requires histologi- cal examination of lung sample, but surgical lung biopsy in pulmonary hypertension is combined with high risk of bleeding. Thus major interest is focused on a non-invasive diagnostic approach enabling early recognition of PVOD and referral for lung transplantation. The present review is focused on the radiological features suggestive of PVOD-like vasculopathy in PAH.