Centrilobular Nodules in High Resolution Computed Tomography of the Lung in IPAH Patients—Preliminary Data Concerning Clinico-Radiological Correlates

Szturmowicz, Monika; Kacprzak, Aneta; Burakowska, Barbara; Skoczylas, Agnieszka; Bestry, Iwona; Kuś, Jan; Fijałkowska, Anna; Torbicki, Adam; Kurzyna, Marcin

doi:10.5603/piap.2016.0033

Cited by 6 publications

(9 citation statements)

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“…38 Centrilobular ground-glass opacities are frequently seen in PAH and their presence on a CT performed for unexplained breathlessness should raise the possibility of this diagnosis. 38,107,108 Features of cardiac decompensation, pleural effusion/ septal lines, and inferior vena cava size predict outcome. 38 The presence of emphysema or interstitial lung disease or bronchiectasis makes the diagnosis of PH in association with lung disease likely.…”

Section: Ct Aids the Classification Of Phmentioning

confidence: 99%

Statement on imaging and pulmonary hypertension from the Pulmonary Vascular Research Institute (PVRI)

Kiely¹,

Levin²,

et al. 2019

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Pulmonary hypertension (PH) is highly heterogeneous and despite treatment advances it remains a life-shortening condition. There have been significant advances in imaging technologies, but despite evidence of their potential clinical utility, practice remains variable, dependent in part on imaging availability and expertise. This statement summarizes current and emerging imaging modalities and their potential role in the diagnosis and assessment of suspected PH. It also includes a review of commonly encountered clinical and radiological scenarios, and imaging and modeling-based biomarkers. An expert panel was formed including clinicians, radiologists, imaging scientists, and computational modelers. Section editors generated a series of summary statements based on a review of the literature and professional experience and, following consensus review, a diagnostic algorithm and 55 statements were agreed. The diagnostic algorithm and summary statements emphasize the key role and added value of imaging in the diagnosis and assessment of PH and highlight areas requiring further research.

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Section: Ct Aids the Classification Of Phmentioning

confidence: 99%

Statement on imaging and pulmonary hypertension from the Pulmonary Vascular Research Institute (PVRI)

Kiely¹,

Levin²,

et al. 2019

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“…In our own group of IPAH patients, centrilobular nodules as the only parenchymal lung abnormality on HRCT were found in 19% of the cases [27]. Their presence was related to the lower mean age, lack of the persistent foramen ovale, and the higher mean right atrial pressure compared to IPAH patients with no centrilobular nodules [27]. There is no consensus on the pathogenesis of centrilobular ground glass opacifications.…”

Section: Radiological Features Of Pvod/pchmentioning

confidence: 83%

Pulmonary Veno-Occlusive Disease: Pathogenesis, Risk Factors, Clinical Features and Diagnostic Algorithm—State of the Art

Szturmowicz¹,

Kacprzak²,

Szołkowska³

et al. 2018

Advances in Respiratory Medicine

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Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary haemangiomatosis (PCH) are rare disorders, with the estimated prevalence of less than 1 case per million inhabitants. The vascular pathology in PVOD/PCH involves pre-septal and septal veins, alveolar capillaries and small pulmonary arteries. According to the ERS/ESC classification of pulmonary hypertension (PH) from 2015, PVOD/PCH have been included in the subgroup 1' of pulmonary arterial hypertension (PAH). Recent data indicate, however, the possibility of PVOD/PCH pathology in the patients diagnosed in the group 1. The problem may concern PAH associated with scleroderma, drug-induced PAH, PAH due to HIV infection and up to 10% of patients with idiopathic PAH (IPAH). Recently, bi-allelic EIF2AK4 mutations were found in the cases with heritable form of PVOD/PCH and in about 9% of sporadic cases. Moreover, an association between occupational exposure to organic solvents and PVOD/PCH was proved. The present review is an attempt to summarise the current data on pathogenesis, risk factors, clinical features and diagnostic algorithm for PVOD/PCH.

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“…Chest HRCT plays an essential role in the workup of PH [ 1 ], and it is also common that the study is repeated during the disease course for various clinical reasons. The number of previous reports on lung HRCT appearance in IPAH is very limited [ 3 , 6 , 11 – 17 ]. We present the largest and most thorough analysis of the lung parenchyma attenuation inhomogeneity on HRCT scans of IPAH patients so far.…”

Section: Discussionmentioning

confidence: 99%

“…In IPAH, lung scans are expected to display no significant abnormalities that would suggest underling respiratory disease [ 1 ]. On the other hand, it is now well recognised that up to 50% of PAH patients may present GGO on HRCT scans despite not having any airway or lung parenchymal disease [ 1 , 3 , 6 , 10 – 17 ]. CGGO were seen in 23–28% of patients with I/H/anorexigen-induced PAH, with no signs of PVOD/PCH on lung pathologic evaluation [ 3 , 6 , 15 ].…”

Section: Introductionmentioning

confidence: 99%

Predictive value of chest HRCT for survival in idiopathic pulmonary arterial hypertension

Kacprzak¹,

Burakowska²,

Kurzyna³

et al. 2021

Respir Res

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Background Little attention has been paid to chest high resolution computed tomography (HRCT) findings in idiopathic pulmonary arterial hypertension (IPAH) patients so far, while a couple of small studies suggested that presence of centrilobular ground-glass opacifications (GGO) on lung scans could have a significant negative prognostic value. Therefore, the aims of the present study were: to assess frequency and clinical significance of GGO in IPAH, and to verify if it carries an add-on prognostic value in reference to multidimensional risk assessment tool recommended by the 2015 European pulmonary hypertension guidelines. Methods Chest HRCT scans of 110 IPAH patients were retrospectively analysed. Patients were divided into three groups: with panlobular (p)GGO, centrilobular (c)GGO, and normal lung pattern. Association of different GGO patterns with demographic, functional, haemodynamic, and biochemical parameters was tested. Survival analysis was also performed. Results GGO were found in 46% of the IPAH patients: pGGO in 24% and cGGO in 22%. Independent predictors of pGGO were: positive history of haemoptysis, higher number of low-risk factors, and lower cardiac output. Independent predictors of cGGO were: positive history of haemoptysis, younger age, higher right atrial pressure, and higher mixed venous blood oxygen saturation. CGGO had a negative prognostic value for outcome in a 2-year perspective. This effect was not seen in the longer term, probably due to short survival of cGGO patients. Conclusions Lung HRCT carries a significant independent prognostic information in IPAH, and in patients with cGGO present on the scans an early referral to lung transplantation centres should be considered.

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Centrilobular Nodules in High Resolution Computed Tomography of the Lung in IPAH Patients—Preliminary Data Concerning Clinico-Radiological Correlates

Abstract: Centrilobular nodules in IPAH were combined with lack of PFO, higher mRAP and younger age of patients.

Cited by 6 publications

References 0 publications

Statement on imaging and pulmonary hypertension from the Pulmonary Vascular Research Institute (PVRI)

Statement on imaging and pulmonary hypertension from the Pulmonary Vascular Research Institute (PVRI)

Pulmonary Veno-Occlusive Disease: Pathogenesis, Risk Factors, Clinical Features and Diagnostic Algorithm—State of the Art

Predictive value of chest HRCT for survival in idiopathic pulmonary arterial hypertension

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