Clinical Characteristics, Haemodynamics and Treatment of Pulmonary Hypertension in Sarcoidosis in a Single Centre, and Meta-Analysis of the Published Data

Dobarro, David; Schreiber, Benjamin; Handler, Clive; Beynon, Huw; Denton, Christopher P.; Coghlan, J G

doi:10.1016/j.amjcard.2012.09.031

Cited by 58 publications

(45 citation statements)

References 23 publications

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“…[27,32] Mortality or transplantation rate is reported as 41% with a median survival of 5.3 years in a meta-analysis of sarcoidosis patients with PH. [33*] Treatment with pulmonary vasodilators may improve functional parameters and hemodynamics, but it is not clear if it affects survival. [33*] The pathophysiology of PH in sarcoidosis is varied and can be due to granulomatous involvement of the heart, lungs, or vasculature, abnormal vaso-reactivity, porto-pulmonary hypertension, or compression by lymph nodes.…”

Section: Morbiditymentioning

confidence: 99%

“…[33*] Treatment with pulmonary vasodilators may improve functional parameters and hemodynamics, but it is not clear if it affects survival. [33*] The pathophysiology of PH in sarcoidosis is varied and can be due to granulomatous involvement of the heart, lungs, or vasculature, abnormal vaso-reactivity, porto-pulmonary hypertension, or compression by lymph nodes. It is more common in patients with pulmonary fibrosis or advanced lung disease, but can be present and severe in the absence of lung disease.…”

Section: Morbiditymentioning

confidence: 99%

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Morbidity and mortality in sarcoidosis

Gerke

2014

Current Opinion in Pulmonary Medicine

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Purpose of Review Chronic sarcoidosis is a complex disease with numerous comorbid conditions and can be fatal in some cases. Recognizing causes of morbidity and mortality is important to effectively select treatments, manage symptoms, and improve outcomes. The purpose of this review is to examine emerging knowledge on morbidity and mortality in sarcoidosis. Recent Findings Approximately one to five percent of patients with sarcoidosis die from complications of sarcoidosis. Recent population studies indicate that mortality may be increasing over the past decade. The reasons behind these trends are unclear, but could include increasing incidence, detection rates, severity of disease, or age of the population. Morbidity of sarcoidosis is reflected by a trend of increased hospitalizations over recent years and increased use of healthcare resources. Morbidity can be caused by organ damage from granulomatous inflammation, treatment complications, and psychosocial effects of the disease. Recent studies are focused on morbidity related to cardiopulmonary complications, bone health, and aging within the sarcoidosis population. Last, sarcoidosis is associated with autoimmune diseases, pulmonary embolism, and malignancy; however, the underlying mechanisms linking diseases continue to be debated. Summary Morbidity in sarcoidosis is significant and multifactorial. Mortality is infrequent, but may be increasing over the years.

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Section: Morbiditymentioning

confidence: 99%

Section: Morbiditymentioning

confidence: 99%

Morbidity and mortality in sarcoidosis

Gerke

2014

Current Opinion in Pulmonary Medicine

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“…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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“…Our patient survived 6 years from diagnosis of SAPH. In the literature, median survivals in SAPH patients were 4.2 and 5.3 years [7,19], and factors associated with worse prognosis were WHO FC IV and moderate/severe pulmonary fibrosis [5,6,19] -both present in our patient.…”

mentioning

confidence: 97%

“…Improvement of exercise capacity was less common. In 2013, Dobarro et al [19] performed a meta-analysis of the published reports indexed in MEDLINE. Overall, the improvement in haemodynamics was substantial, with mean reduction in mPAP of 8.03 mm Hg, increase in cardiac output of 0.97 L/min, and a decrease in PVR of 4.24 Wood units.…”

mentioning

confidence: 99%

Pulmonary Function Tests Leading to the Diagnosis of Vascular Malformations in School-Aged Children

Kusak

Cichocka‐Jarosz

Jedynak-Wąsowicz

et al. 2017

Advances in Respiratory Medicine

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Development of sarcoidosis-associated pulmonary hypertension (SAPH) significantly worsens prognosis in sarcoidosis patients. Unfortunately, there is no treatment of proven benefit for this condition. Medications used for treatment of pulmonary arterial hypertension are of great interest in this respect. Here, we report a case of a patient with severe SAPH treated with sildenafil. A significant, but only temporary improvement in functional status was observed, and the patient died of gradually progressing heart and respiratory failure while awaiting for lung transplantation.

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Clinical Characteristics, Haemodynamics and Treatment of Pulmonary Hypertension in Sarcoidosis in a Single Centre, and Meta-Analysis of the Published Data

Cited by 58 publications

References 23 publications

Morbidity and mortality in sarcoidosis

Morbidity and mortality in sarcoidosis

Pulmonary hypertension in chronic interstitial lung diseases

Pulmonary Function Tests Leading to the Diagnosis of Vascular Malformations in School-Aged Children

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