Computed tomography findings of pulmonary venoocclusive disease in scleroderma patients presenting with precapillary pulmonary hypertension

Abstract

Objective. Pulmonary venoocclusive disease (PVOD) is an uncommon form of pulmonary hypertension (PH) characterized by obstruction of small pulmonary veins. Pulmonary venous involvement has been reported in pathologic assessment of patients with systemic sclerosis (SSc) presenting with precapillary PH. High-resolution computed tomography (HRCT) of the chest is a noninvasive diagnostic tool used to screen for PVOD. No HRCT data are available on SSc patients with precapillary PH. We undertook this study to evaluate the frequency and effect on prognosis of HRCT signs of PVOD in SSc patients with precapillary PH.Methods. We reviewed chest HRCT data from 26 SSc patients with precapillary PH and 28 SSc patients without pulmonary arterial hypertension (PAH) or interstitial lung disease (ILD).Results. The radiographic triad of HRCT signs of PVOD (lymph node enlargement [57.7% versus 3.6%], centrilobular ground-glass opacities [46.2% versus 10.7%], and septal lines [88.5% versus 7.1%]) was significantly more frequent in SSc patients with precapillary PH than in SSc patients without PAH or ILD (all P < 0.005). Indeed, 61.5% of SSc patients with precapillary PH had >2 of these signs. Cardiomegaly (P < 0.0001), pulmonary artery enlargement (P < 0.0001), and pericardial effusion (P < 0.0005) were also significantly more frequent in SSc patients with precapillary PH. Pulmonary venous involvement was histologically confirmed in 2 patients with radiographic signs of PVOD. The presence of >2 radiographic signs of PVOD was associated with the occurrence of pulmonary edema after initiation of PAH-specific therapy (in 8 of 16 patients) and with more rapid progression from diagnosis of PH to death.Conclusion. HRCT signs of PVOD are frequently observed in SSc patients with precapillary PH, correlated with histologic assessment, and were associated with a high risk of pulmonary edema.Pulmonary arterial hypertension (PAH) is a severe and progressive disease, characterized by elevated pulmonary artery pressure leading to right-sided heart failure and death (1,2). A definite diagnosis of PAH requires right-sided heart catheterization (RHC) showing a mean pulmonary artery pressure (PAP) of Ն25 Drs.

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

“…The prevalence of haemodynamically proven pre-capillary PH in large cohorts of SSc patients ranges from 5 to 12% [46,325,330,331]. In these patients, PH may occur in association with interstitial lung disease or as a result of an isolated pulmonary vascular disease, which may affect pre-capillary arterioles (PAH) and post-capillary venules (PVOD) [326,332]. In addition, group 2 pulmonary venous hypertension from LHD may be present [76,326,333].…”

“…Symptoms and clinical presentation are very similar to IPAH and occasional patients thought to have IPAH can be identified as having an associated CTD by immunological screening tests. High-resolution tomography of the chest is helpful for evaluating the presence of associated interstitial lung disease and/or PVOD [326,332,337]. An isolated reduction of DLCO is a frequent abnormality in SSc associated with PAH [327,338].…”

Pulmonary Arterial Hypertension in Connective Tissue Diseases

“…Снижение ДСЛ у пациентов с ССД в первую очередь связывают с частой ассоциацией ЛАГ с веноокклюзионной болезнью. Если у пациентов при компьютерной томографии (КТ) легких выявлялось более двух признаков веноокклюзионной болезни, значения ДЛС были ниже, чем в группе без определяемых на КТ из-менений [18]. При морфологическом исследовании легоч-ной ткани у пациентов с ЛАГ-ССД признаки поражения венул обнаруживают чаще, чем у пациентов с идиопатиче-ской ЛГ [19].…”

Recent Advances in Systemic Scleroderma in Childhood

“…Pulmonary veno-occlusive disease (PVOD) is a new field of interest, as patients having this disorder appear to exhibit poor survival. PVOD is characterised by the radiological triad of lymph node enlargement, centrilobular ground-glass opacities and septal lines [34]. The gold standard diagnostic tool for PAH is right heart catheterisation (RHC).…”