Interstitial lung diseases and pulmonary hypertension

Valentini, Adele; Savietto, Giovanni; Meloni, Federica; Dore, Roberto; Borghesi, Andrea; Romei, Chiara; Pulzato, Ilaria; Rampinelli, Cristiano; Preda, Lorenzo; Larici, Anna Rita

doi:10.23736/s2723-9284.21.00123-9

Cited by 2 publications

(4 citation statements)

References 59 publications

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“…Hypoxic vasoconstriction, vascular remodeling, and proliferative vaso-occlusive lesions usually oc-cur at this distal level [16,17]. This is the reason why the earliest appreciable CT sign related to PH is the dilation of the proximal PAs and the sudden caliber decrease in peripheral ones [18].…”

Section: Ct Indirect Signs Of Phmentioning

confidence: 99%

“…Even though the majority of SAPH patients show an advanced parenchymal disease, some subjects with limited parenchymal disease demonstrate disproportional elevated mPAP [78]. This means that apart from granulomata within the PAs and/or pulmonary veins and lung fibrosis, other mechanisms could determine PH in sarcoidosis, such as extrinsic compression of the pulmonary vessels by lymphadenopathies or fibrosing mediastinitis, vasospasm, vasculopathy, CTEPH, myocardial dysfunction, and portopulmonary hypertension [18,79,80]; indeed, pre-and post-capillary mechanisms may be both involved, sometimes in combination. Like other diseases complicated with PH, SAPH is associated with significant morbidity and increased mortality compared with sarcoidosis without PH [79,80].…”

Section: Group 5 Phmentioning

confidence: 99%

“…PH occurs in 92-100% of patients affected by advanced PLCH [86,87]. With respect to COPD and IPF, the incidence and severity of PH in patients with advanced PLCH are higher [18]. This is the reason why it has been postulated that other disease-related mechanisms (other than that of the ILD spectrum) may contribute to the development of PH in PLCH [88].…”

Section: Group 5 Phmentioning

confidence: 99%

“…Advanced PLCH, when PH is usually more frequent, may have HRCT imaging features with large cysts and extensive lung parenchyma disruption, which overlap with emphysema and/or fibrosis as well as with LAM (Figure 6b). Therefore, in advanced cases, differential diagnosis among these entities may be challenging, and the typical radiological presentation of early PLCH (small nodules, sparing of posterior costophrenic recesses) make it difficult to recognize; it could be helpful to compare images with previous exams and to look for small cysts with bizarre morphology, peculiarly present in PLCH [18].…”

Section: Group 5 Phmentioning

confidence: 99%

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Pulmonary Hypertension in Chronic Lung Diseases: What Role Do Radiologists Play?

Valentini

Franchi²,

Cicchetti

et al. 2023

Diagnostics

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Pulmonary hypertension (PH) is a pathophysiological disorder, defined by a mean pulmonary arterial pressure (mPAP) > 20 mmHg at rest, as assessed by right heart catheterization (RHC). PH is not a specific disease, as it may be observed in multiple clinical conditions and may complicate a variety of thoracic diseases. Conditions associated with the risk of developing PH are categorized into five different groups, according to similar clinical presentations, pathological findings, hemodynamic characteristics, and treatment strategy. Most chronic lung diseases that may be complicated by PH belong to group 3 (interstitial lung diseases, chronic obstructive pulmonary disease, combined pulmonary fibrosis, and emphysema) and are associated with the lowest overall survival among all groups. However, some of the chronic pulmonary diseases may develop PH with unclear/multifactorial mechanisms and are included in group 5 PH (sarcoidosis, pulmonary Langerhans’ cell histiocytosis, and neurofibromatosis type 1). This paper focuses on PH associated with chronic lung diseases, in which radiological imaging—particularly computed tomography (CT)—plays a crucial role in diagnosis and classification. Radiologists should become familiar with the hemodynamical, physiological, and radiological aspects of PH and chronic lung diseases in patients at risk of developing PH, whose prognosis and treatment depend on the underlying disease.

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Section: Ct Indirect Signs Of Phmentioning

confidence: 99%