Pulmonary Haemodynamics at Rest and during Exercise in Patients with Sarcoidosis

Głuskowski, J; Hawryłkiewicz, I; Zych, D; Wojtczak, Andrzej; Zieliński, J

doi:10.1159/000194667

Cited by 54 publications

(46 citation statements)

References 6 publications

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“…As with other forms of interstitial lung disease, Ppa may rise as a result of progressive fibrosis and destruction of alveolar capillary membrane units [11]. Additionally, the present findings, regarding the incidence of PH, are consistent with the observations by GLUSKOWSKI et al [12] that 50% of those with stage III sarcoidosis have increased Ppa.…”

Section: Discussionsupporting

confidence: 93%

Pulmonary hypertension in advanced sarcoidosis: epidemiology and clinical characteristics: Table. 1—

Shorr

Helman²,

Davies³

et al. 2005

Eur Respir J

260

208

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Pulmonary hypertension (PH) is a predictor of poor outcome in sarcoidosis. Little is known about the epidemiology of PH in sarcoidosis.The current authors reviewed the records of patients with sarcoidosis listed for lung transplantation in the USA between January 1995 and December 2002. PH was defined as a mean pulmonary artery pressure of .25 mmHg and severe PH as a mean pulmonary artery pressure of o40 mmHg.The cohort included 363 patients of whom 73.8% had PH. Neither spirometric testing nor the need for corticosteroids was associated with PH. Subjects with PH required more supplemental oxygen (2.7¡1.8 L?min -1 versus 1.6¡1.4 L?min -1 ). The cardiac index was lower in individuals with PH, whereas the pulmonary capillary wedge pressure was higher. In multivariate analysis, supplemental oxygen remained an independent predictor of PH, whereas the relationship between cardiac index and PH was no longer significant. As a screening test, the need for oxygen had a sensitivity and specificity of 91.8% and 32.6%, respectively. Pulmonary hypertension is common in advanced sarcoidosis. The need for oxygen correlates with pulmonary hypertension. Since pulmonary hypertension is associated with poor outcomes and because simple clinical criteria fail to identify patients with sarcoidosis and pulmonary hypertension, more aggressive screening for this should be considered.

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Section: Discussionsupporting

confidence: 93%

Pulmonary hypertension in advanced sarcoidosis: epidemiology and clinical characteristics: Table. 1—

Shorr

Helman²,

Davies³

et al. 2005

Eur Respir J

260

208

View full text Add to dashboard Cite

show abstract

“…PH is a rare complication of another lung granulomatous disease, sarcoidosis [75][76][77]. Although this complication is rare in absolute terms, it is not uncommon in patients with advanced disease, particularly in patients with stage III and IV disease [4].…”

Section: Other Ilds Associated With Phmentioning

confidence: 99%

Pulmonary hypertension in chronic interstitial lung diseases

Caminati¹,

Cassandro²,

Harari³

2013

Eur Respir Rev

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Pulmonary hypertension (PH) is a common complication of interstitial lung diseases (ILDs), particularly in idiopathic pulmonary fibrosis and ILD associated with connective tissue disease. However, other lung diseases, such as combined pulmonary fibrosis and emphysema syndrome, pulmonary Langerhans cell histiocytosis, and lymphangioleiomyomatosis, may also include PH in their clinical manifestations. In all of these diseases, PH is associated with reduced exercise capacity and poor prognosis. The degree of PH in ILDs is typically mild-to-moderate. However, some of these patients may develop a disproportionate increase in PH that cannot be justified solely by hypoxia and parenchymal injury: this condition has been termed “out-of-proportion” PH. The pathogenesis of PH in these diseases is various, incompletely understood and may be multifactorial. The clinical suspicion (i.e. increased dyspnoea, low diffusion capacity) and echocardiographic assessment are the first steps towards proper diagnosis of PH; however, right heart catheterisation remains the current gold standard for diagnosis of PH. At present, no specific therapies have been approved for the treatment of PH in patients with ILDs

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“…Patients who progress to develop stage IV fibrocystic disease commonly manifest physiologically with varying degrees of restrictive and obstructive disease and a decreased diffusing capacity of the lung for carbon monoxide (DL,CO) [1]. Pulmonary hypertension (PH) is an increasingly recognised complication of sarcoidosis with a reported prevalence between 1 and 28% of all patients at rest and up to 43% with exercise [4][5][6]. PH most commonly occurs in association with advanced stage IV disease, but can also occur in the context of relatively normal lung function and preserved parenchymal architecture.…”

Section: Pulmonary Hypertension Overviewmentioning

confidence: 99%

Management of end-stage sarcoidosis: pulmonary hypertension and lung transplantation

Shlobin¹,

Nathan²

2012

Eur Respir J

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Sarcoidosis is not only a multisystem, but also a multinational disease that is prevalent throughout the world, including Europe, the USA and Japan. Lung involvement in sarcoidosis is seemingly invariable, with up to 95% of patients manifesting some form of pulmonary disease during the course of their lifetime. The natural history of sarcoidosis in the lung is quite variable and spans the spectrum from spontaneous resolution to advanced fibrocystic disease in ,5% of cases. Advanced sarcoidosis will be the subject of this review with a special focus on pulmonary hypertension and lung transplantation as a last-resort treatment option for some patients with end-stage disease.

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Pulmonary Haemodynamics at Rest and during Exercise in Patients with Sarcoidosis

Cited by 54 publications

References 6 publications

Pulmonary hypertension in advanced sarcoidosis: epidemiology and clinical characteristics: Table. 1—

Pulmonary hypertension in advanced sarcoidosis: epidemiology and clinical characteristics: Table. 1—

Pulmonary hypertension in chronic interstitial lung diseases

Management of end-stage sarcoidosis: pulmonary hypertension and lung transplantation

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