Pulmonary veno-occlusive disease: The bête noire of pulmonary hypertension in connective tissue diseases?

O’Callaghan, Dermot S.; Dorfmüller, Peter; Jaïs, Xavier; Mouthon, Luc; Sitbon, Olivier; Simonneau, Gérald; Humbert, Marc; Montani, David

doi:10.1016/j.lpm.2010.10.017

Cited by 26 publications

(21 citation statements)

References 91 publications

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“…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].…”

Section: Diagnosismentioning

confidence: 99%

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

et al. 2015

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Guidelines summarize and evaluate all available evidence on a particular issue at the time of the writing process, with the aim of assisting health professionals in selecting the best management strategies for an individual patient with a given condition, taking into account the impact on outcome, as well as the risk–benefit ratio of particular diagnostic or therapeutic means. Guidelines and recommendations should help health professionals to make decisions in their daily practice. However, the final decisions concerning an individual patient must be made by the responsible health professional(s) in consultation with the patient and caregiver as appropriate.

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Section: Diagnosismentioning

confidence: 99%

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

et al. 2015

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“…Obraz kliniczny jest bardzo podobny do IPAH i u niektórych chorych z rozpoznaniem IPAH można ostatecznie rozpoznać współistniejącą CTD, wykonując przesiewowe testy immunologiczne. Współistnienie śródmiąższowej choroby płuc i/lub PVOD można zdiagnozować za pomocą CT o wysokiej rozdzielczości [326,332,337]. Izolowane zmniejszenie DLCO jest częstą nieprawidłowością w PAH w przebiegu SSc [327,338].…”

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

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“…Documentation of PH with radiographic evidence consistent with pulmonary edema in the face of normal left atrial and pulmonary arterial wedge pressures-both ruling out left-sided heart failure as a causeis highly suggestive of a diagnosis of PVOD. 20,24 PVOD tends to be much more clinically severe than most other forms of PAH, with an average survival time of 2 years following diagnosis. 19,28 The severity of the clinical disease reflects the vascular remodeling characteristic of PVOD and its predicted impact on pulmonary blood flow.…”

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Pulmonary Veno-Occlusive Disease

et al. 2016

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Pulmonary hypertension is a well-known though poorly characterized disease in veterinary medicine. In humans, pulmonary veno-occlusive disease (PVOD) is a rare cause of severe pulmonary hypertension with a mean survival time of 2 years without lung transplantation. Eleven adult dogs (5 males, 6 females; median age 10.5 years, representing various breeds) were examined following the development of severe respiratory signs. Lungs of affected animals were evaluated morphologically and with immunohistochemistry for alpha smooth muscle actin, desmin, CD31, CD3, CD20, and CD204. All dogs had pulmonary lesions consistent with PVOD, consisting of occlusive remodeling of small-to medium-sized pulmonary veins, foci of pulmonary capillary hemangiomatosis (PCH), and accumulation of hemosiderophages; 6 of 11 dogs had substantial pulmonary arterial medial and intimal thickening. Ultrastructural examination and immunohistochemistry showed that smooth muscle cells contributed to the venous occlusion. Increased expression of CD31 was evident in regions of PCH indicating increased numbers of endothelial cells in these foci. Spindle cells strongly expressing alpha smooth muscle actin and desmin co-localized with foci of PCH; similar cells were present but less intensely labeled elsewhere in non-PCH alveoli. B cells and macrophages, detected by immunohistochemistry, were not co-localized with the venous lesions of canine PVOD; small numbers of CD3-positive T cells were occasionally in and around the wall of remodeled veins. These findings indicate a condition in dogs with clinically severe respiratory disease and pathologic features resembling human PVOD, including foci of pulmonary venous remodeling and PCH.

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Pulmonary veno-occlusive disease: The bête noire of pulmonary hypertension in connective tissue diseases?

Cited by 26 publications

References 91 publications

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

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

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

Pulmonary Veno-Occlusive Disease

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