Pulmonary hypertension and granulomatous vasculitis in sarcoidosis

Nagai, Sonoko; Sharma, Om P.

doi:10.1097/mcp.0b013e328273bc5c

Cited by 34 publications

(18 citation statements)

References 23 publications

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“…The exact prevalence is not clear with a reported range of 1 to 28%. 1 Among patients listed for lung transplantation in the United States, 74% had PH documented by right heart catheterization. 2 Sarcoidosis is classified under the miscellaneous category (group 5) and considered separately from other respiratory disorders, such as interstitial and obstructive lung diseases (group 3), in the Venice 2003 clinical classification of PH.…”

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confidence: 99%

Treatment of Sarcoidosis-Associated Pulmonary Hypertension

Barnett

Bonura

Nathan

et al. 2009

Chest

144

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Background: Pulmonary hypertension (PH) is a common complication of sarcoidosis that is associated with increased mortality. The pathogenesis of PH in sarcoidosis is uncertain, and the role of pulmonary arterial hypertension (PAH)-specific therapies remains to be determined. Methods: We conducted a retrospective study of patients with sarcoidosis and PH at two referral centers. New York Heart Association (NYHA) functional class, exercise capacity, hemodynamic data, pulmonary function tests, and survival were collected and analyzed. Results: Twenty-two sarcoidosis patients treated with PAH-specific therapies were identified. After a median of 11 months of follow-up, NYHA class was improved in nine subjects. Mean 6-min walk distance (n ‫؍‬ 18) increased by 59 m (p ‫؍‬ 0.032). Patients with a higher FVC experienced a greater increment in exercise capacity. Among 12 patients with follow-up hemodynamic data, mean pulmonary artery pressure was reduced from 48.5 ؎ 4.3 to 39.4 ؎ 2.8 mm Hg (p ‫؍‬ 0.008). The 1-and 3-year transplant-free survival rates were 90% and 74%, respectively. Conclusions: PAH-specific therapy may improve functional class, exercise capacity, and hemodynamics in PH associated with sarcoidosis. Prospective, controlled trials of PAH therapies for sarcoidosis are warranted to verify this apparent benefit. Mortality among the study population was high, highlighting the need for urgent evaluation at a lung transplant center.

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confidence: 99%

Treatment of Sarcoidosis-Associated Pulmonary Hypertension

Barnett

Bonura

Nathan

et al. 2009

Chest

144

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“…Hierbei war insbesondere die Notwendigkeit einer LTOT mit einer hohen Wahrscheinlichkeit für das Vorliegen einer PH assoziiert [17]. Betrachtet man das Gesamtkollektiv von Sarkoidose-Patienten, liegt die Häufigkeit einer PH zwischen 1 und 28%, wobei insbesondere das fortgeschrittene Fibrosestadium mit einer hohen Prävalenz einer PH vergesellschaftet zu sein scheint [16]. Es muss daraufhin gewiesen werden, dass auch bei fehlenden radiologischen Zeichen einer Fibrose ein Sarkoidose-assoziierter Lungenhochdruck auftreten kann.…”

Section: Sarkoidoseunclassified

“…Mögliche Mechanismen hierfür sind u.a. der direkte Befall der Pulmonalgefäße im Sinne einer granulomatösen Vaskulopathie, eine Kompression der Pulmonalarterien durch vergrößerte mediastinale Lymphknoten, aber auch eine mögliche diastolische Dysfunktion des linken Ventrikels bei myokardialem Befall [16].…”

Section: Sarkoidoseunclassified

“…Ob die Gabe von Kortikosteroiden einen Sarkoidose-assoziierten Lungenhochdruck günstig beeinflussen kann, wird kontrovers diskutiert; die Datenlage hierzu ist nicht eindeutig [16]. Nur wenige Studien haben bislang den Einfluß von Vasodilatatoren auf einen Sarkoidose-assoziierten Lungenhochdruck untersucht.…”

Section: Sarkoidoseunclassified

“…Möglicherweise hat auch die Langzeitgabe von Epoprostenol einen günstigen Effekt. Die Ergebnisse kleinerer retrospektiver Studien legen weiterhin einen günstigen Einfluss von Sildenafil und Bosentan auf die pulmonale Hämodynamik bei dieser Patientengruppe nahe [2,14,16]. Weitere klinische Studien sind notwendig um das mögliche Potenzial dieser Substanzen zur Behandlung eines Sarkoidose-assoziierten Lungenhochdruck genauer zu evaluieren.…”

Section: Sarkoidoseunclassified

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Pulmonale Hypertonie bei COPD und interstitiellen Lungenerkrankungen

Markart

Ghofrani

Grimminger

et al. 2009

Dtsch med Wochenschr

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Pulmonary hypertension (PH) is a common complication of chronic obstructive pulmonary disease (COPD) and of interstitial lung diseases (ILD) such as idiopathic pulmonary fibrosis and sarcoidosis. When present in these patients, PH is usually mild to moderate. When severe PH is diagnosed in COPD and ILD patients, other potentially better treatable underlying causes should be ruled out. In COPD patients, PH is associated with an increased risk of severe exacerbations and a reduced life expectancy. Similarly, in patients with ILD, the presence of PH correlates with a poor prognosis. Doppler echocardiography is the best non-invasive method for the diagnosis of PH, but is frequently inaccurate in patients with advanced lung diseases. Thus, when clinical suspicion remains high, right heart catheterization in a reference center is required to ultimately confirm the presence of PH. Treatment of PH in COPD and ILD is primarily based on long term oxygen therapy. Drugs approved for pulmonary arterial hypertension, such as prostanoids, phosphodiesterase inhibitors, and endothelin receptor antagonists, may represent promising options for COPD and ILD patients, however, their use may be hampered by potentially deleterious effects on gas exchange and their efficacy yet remains to be proven in appropriately designed and controlled clinical trials. Lung transplantation may be considered in all patients with an advanced disease.

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Cardiac Tissue Characterization and the Diagnostic Value of Cardiovascular Magnetic Resonance in Systemic Connective Tissue Diseases

Mavrogeni

Sfikakis²,

Gialafos

et al. 2013

Arthritis Care & Research

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Objective. Accurate diagnosis of cardiovascular involvement in connective tissue diseases (CTDs) remains challenging. We hypothesized that cardiovascular magnetic resonance (CMR) demonstrates cardiac lesions in symptomatic CTD patients with normal echocardiography. Methods. CMR from 246 CTD patients with typical cardiac symptoms (TCS; n ‫؍‬ 146, group A) or atypical cardiac symptoms (ATCS; n ‫؍‬ 100, group B) was retrospectively evaluated. Group A included 9 patients with inflammatory myopathy (IM), 35 with sarcoidosis, 30 with systemic sclerosis (SSc), 14 with systemic lupus erythematosus (SLE), 10 with rheumatoid arthritis (RA), and 48 with small vessel vasculitis. Group B included 25 patients with RA, 20 with SLE, 20 with sarcoidosis, 15 with SSc, 10 with IM, and 10 with small vessel vasculitis. CMR was performed by 1.5T; left ventricular ejection fraction, T2 ratio (edema imaging), and late gadolinium enhancement (LGE; fibrosis imaging) were evaluated. Acute and chronic lesions were characterized as LGE positive plus T2 ratio >2 and T2 ratio <2, respectively. According to LGE, lesions were characterized as diffuse subendocardial, subepicardial, and subendocardial/transmural due to vasculitis, myocarditis, and myocardial infarction, respectively. A stress study by dobutamine echocardiography or stress, nuclear, or adenosine CMR was performed in CTD patients with negative rest CMR. Results. Abnormal CMR was identified in 32% (27% chronic) and 15% (12% chronic) of patients with TCS and ATCS, respectively. Lesions due to vasculitis, myocarditis, and myocardial infarction were evident in 27.4%, 62.6%, and 9.6% of CTD patients, respectively. Stress studies in CTD patients with negative CMR revealed coronary artery disease in 20%. Conclusion. CMR in symptomatic CTD patients with normal echocardiography can assess disease acuity and identify vasculitis, myocarditis, and myocardial infarction.

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Pulmonary hypertension and granulomatous vasculitis in sarcoidosis

Abstract: Pulmonary hypertension is not infrequently observed in sarcoidosis. Further studies are needed to elucidate the epidemiology, mechanisms, treatment, and significance of sarcoidosis-associated pulmonary hypertension.

Cited by 34 publications

References 23 publications

Treatment of Sarcoidosis-Associated Pulmonary Hypertension

Treatment of Sarcoidosis-Associated Pulmonary Hypertension

Pulmonale Hypertonie bei COPD und interstitiellen Lungenerkrankungen

Cardiac Tissue Characterization and the Diagnostic Value of Cardiovascular Magnetic Resonance in Systemic Connective Tissue Diseases

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