Sarcoidosis-Associated Pulmonary Hypertension

Fisher, Kimberly A.; Serlin, David M.; Wilson, Kevin C.; Walter, Robert; Berman, Jeffrey S.; Farber, Harrison W.

doi:10.1378/chest.130.5.1481

Cited by 140 publications

(34 citation statements)

References 29 publications

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“…Similar findings were noted in (group 3) ILD patients, emphasizing that the clinical suspicion of PH should be high particularly in the context of diffuse parenchymal lung disorders. Treatment of sarcoid patients with PH with PH-specific agents has not been clearly established, but retrospective analyses and small case series[47–50] have suggested that there may be potential benefits in some patients. Future studies will be needed to determine that sarcoidosis patients would be most likely to respond to these agents.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary hypertension in Saudi Arabia: A single center experience

et al. 2013

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CONTEXT:Several international studies have described the epidemiology of pulmonary hypertension (PH). However, information about the incidence and prevalence of PH in Saudi Arabia is unknown.AIMS:To report cases of PH and compare the demographic and clinical characteristics of PH due to various causes in a Saudi population.METHODS:Newly diagnosed cases of PH [defined as mean pulmonary artery pressure >25 mmHg at right heart cauterization (RHC)] were prospectively collected at a single tertiary care hospital from January 2009 and June 2012. Detailed demographic and clinical data were collected at the time of diagnosis, along with hemodynamic parameters.RESULTS:Of the total 264 patients who underwent RHC, 112 were identified as having PH. The mean age at diagnosis was 55.8 ± 15.8 years, and there was a female preponderance of 72.3%. About 88 (78.6%) of the PH patients were native Saudis and 24 (21.4%) had other origins. Twelve PH patients (10.7%) were classified in group 1 (pulmonary arterial hypertension), 7 (6.2%) in group 2 (PH due to left heart disease), 73 (65.2%) in group 3 (PH due to lung disease), 4 (3.6%) in group 4 (chronic thromboembolic PH), and 16 (14.3%) in group 5 (PH due to multifactorial mechanisms). PH associated with diastolic dysfunction was noted in 28.6% of group 2 patients, 31.5% of group 3 patients, and 25% of group 5 patients.CONCLUSIONS:These results offer the first report of incident cases of PH across five groups in Saudi Arabia.

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Section: Discussionmentioning

confidence: 99%

Pulmonary hypertension in Saudi Arabia: A single center experience

et al. 2013

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“…However, studies have shown that neither of these factors accurately predicts the incidence or severity of pulmonary hypertension [7,21,22]. This is supported not only by a higher incidence of pulmonary hypertension in sarcoidosis than that seen in other destructive lung diseases [23], but also by the fact that the mean pulmonary artery pressure of patients awaiting transplant is significantly higher for patients with sarcoidosis than those with idiopathic pulmonary fibrosis [24]. Other potential markers, such as spirometry, lung volumes, and diffusing capacity, are equally unreliable [6,7,10,21].…”

Section: Discussion and Literature Reviewmentioning

confidence: 99%

“…Optimal treatment is unknown but is currently the subject of ongoing research. Several small studies and case series, including one published in this journal, have reported variable responses to vasodilator treatments [23,25,26,27]. While these studies suggest that at least a subset of patients may have a clinical response, the question must be addressed more rigorously by large randomized controlled trials.…”

Section: Discussion and Literature Reviewmentioning

confidence: 99%

Sarcoidosis-Associated Fibrosing Mediastinitis with Resultant Pulmonary Hypertension: A Case Report and Review of the Literature

Toonkel

Borczuk²,

Pearson³

et al. 2009

Respiration

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Pulmonary hypertension, a common manifestation of advanced sarcoidosis, is thought to result from fibrosis with chronic hypoxia and destruction of small vessels, extrinsic compression of pulmonary arteries, or granulomatous vasculitis. We report a case of sarcoidosis-associated pulmonary hypertension due to fibrosing mediastinitis. Our patient presented with cough and dyspnea on exertion and was found to have pulmonary artery enlargement, pulmonary venous compression, and mediastinal soft tissue enhancement on magnetic resonance imaging. Pulmonary hypertension was confirmed by right heart catheterization and sarcoidosis was diagnosed by histologic examination of tissue obtained at mediastinoscopy. Treatment with steroids resulted in decreased pulmonary artery pressures as well as symptomatic improvement. While pulmonary hypertension is a common complication of sarcoidosis, fibrosing mediastinitis is an unusual etiology that should be considered by clinicians.

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“…Small studies have addressed the role of specific therapy for patients with PH related to sarcoidosis. One series of eight patients described a favorable response to intravenous epoprostenol in most patients (Fisher et al, 2006). …”

Section: Treatment Algorithmsmentioning

confidence: 99%

Therapeutic Strategies in Pulmonary Hypertension

Fuso¹,

Baldi²,

Perna³

2011

Front. Pharmacol.

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Pulmonary hypertension (PH) is a life-threatening condition characterized by elevated pulmonary arterial pressure. It is clinically classified into five groups: patients in the first group are considered to have pulmonary arterial hypertension (PAH) whereas patients of the other groups have PH that is due to cardiopulmonary or other systemic diseases. The management of patients with PH has advanced rapidly over the last decade and the introduction of specific treatments especially for PAH has lead to an improved outcome. However, despite the progress in the treatment, the functional limitation and the survival of these patients remain unsatisfactory and there is no cure for PAH. Therefore the search for an “ideal” therapy still goes on. At present, two levels of treatment can be identified: primary and specific therapy. Primary therapy is directed at the underlying cause of the PH. It also includes a supportive therapy consisting in oxygen supplementation, diuretics, and anticoagulation which should be considered in all patients with PH. Specific therapy is directed at the PH itself and includes treatment with vasodilatators such as calcium channel blockers and with vasodilatator and pathogenetic drugs such as prostanoids, endothelin receptor antagonists and phosphodiesterase type-5 inhibitors. These drugs act in several pathogenetic mechanisms of the PH and are specific for PAH although they might be used also in the other groups of PH. Finally, atrial septostomy and lung transplantation are reserved for patients refractory to medical therapy. Different therapeutic approaches can be considered in the management of patients with PH. Therapy can be established on the basis of both the clinical classification and the functional class. It is also possible to adopt a goal-oriented therapy in which the timing of treatment escalation is determined by inadequate response to known prognostic indicators.

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Sarcoidosis-Associated Pulmonary Hypertension

Cited by 140 publications

References 29 publications

Pulmonary hypertension in Saudi Arabia: A single center experience

Pulmonary hypertension in Saudi Arabia: A single center experience

Sarcoidosis-Associated Fibrosing Mediastinitis with Resultant Pulmonary Hypertension: A Case Report and Review of the Literature

Therapeutic Strategies in Pulmonary Hypertension

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