Frequency of Pleural Effusions in Patients With Pulmonary Arterial Hypertension Associated With Connective Tissue Diseases

Luo, Yi; Robbins, Ivan M.; Karataş, Mevlüt; Brixey, Anupama G.; Rice, Todd W.; Light, Richard W.

doi:10.1378/chest.10-0227

Cited by 36 publications

(28 citation statements)

References 15 publications

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“…Pleural effusion is more frequent in the population of PAH patients described here (68%), which may in part be reflective of the thoroughness of clinical evaluation of pulmonary symptoms in this group. PAH is not a recognized complication of pleural effusion; however, in PAH associated with connective tissue diseases, the two conditions have been reported to occur concomitantly in 39% of patients [31]. Although the higher incidence of pleural effusions in the subset of dasatinib-treated patients with PAH compared with the broader dasatinib-treated population is intriguing, it is not possible to draw any conclusions regarding a potential causal relationship, and in light of the relatively small number of cases reported to date, routine screening for PAH in dasatinib-treated patients who develop pleural effusion appears unwarranted.…”

Section: Discussionmentioning

confidence: 99%

Clinical features of pulmonary arterial hypertension in patients receiving dasatinib

et al. 2015

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The prognosis of most leukemia patients treated with BCR-ABL tyrosine kinase inhibitors (TKIs) is favorable, and a more precise understanding of serious and potentially irreversible treatment-related toxicities is essential to properly inform treatment choice. Few cases of pulmonary arterial hypertension (PAH) have been reported in patients with leukemia treated with dasatinib, a second-generation BCR-ABL TKI. To better understand characteristics and outcomes of dasatinib-treated patients with PAH, all clinical cases of PAH confirmed by right-heart catheterization in the Bristol-Myers Squibb pharmacovigilance database (N 5 41), including 22 previously unpublished cases, were examined for previous treatments for leukemia, patient characteristics, time to PAH onset, and outcomes. Our analysis shows that compared with PAH due to other etiologies, dasatinib-related PAH is atypical, in that it is associated with partial to complete reversibility upon treatment discontinuation. The incidence of dasatinib-related PAH appears to be low. Most PAH cases were observed in patients who had received prior treatments for leukemia. No specific patient attributes appear to be associated with an increased risk of developing PAH while receiving dasatinib. Symptoms of PAH in dasatinib-treated leukemia patients should prompt a thorough workup, including consideration of confirmatory right-heart catheterization. In cases of confirmed PAH, dasatinib should be discontinued.

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Section: Discussionmentioning

confidence: 99%

Clinical features of pulmonary arterial hypertension in patients receiving dasatinib

et al. 2015

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“…In a study of 89 patients with PAH associated with CTD, Luo et al [66], demonstrated that 39.3% of the patients had trace to small and bilateral pleural effusions including 37.5% of the patients (6 out of 16) with SLE-aPAH. When compared with the patients without pleural effusions, the patients with pleural effusions had significantly higher mean right atrial pressures and lower cardiac indices.…”

Section: Clinical Featuresmentioning

confidence: 99%

Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus: Current Status and Future Direction

Dhala

2012

Clinical and Developmental Immunology

101

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Pulmonary arterial hypertension (PAH) is commonly associated with connective tissue diseases (CTDs) including systemic sclerosis and systemic lupus erythematosus (SLE). The prevalence of PAH in SLE is estimated to be 0.5% to 17.5%. The pathophysiology of PAH involves multiple mechanisms from vasculitis andin-situthrombosis to interstitial pulmonary fibrosis which increases pulmonary vascular resistance, potentially leading to right heart failure. Immune and inflammatory mechanisms may play a significant role in the pathogenesis or progression of PAH in patients with CTDs, establishing a role for anti-inflammatory and immunosuppressive therapies. The leading predictors of PAH in SLE are Raynaud phenomenon, anti-U1RNP antibody, and anticardiolipin antibody positivity. The first-line of diagnostic testing for patients with suspected SLE-associated PAH (SLE-aPAH) involves obtaining a Doppler echocardiogram. Once the diagnosis is confirmed by right heart catheterization, SLE-aPAH patients are generally treated with oxygen, anticoagulants, and vasodilators. Although the prognosis and therapeutic responsiveness of these patients have improved with the addition of intensive immunosuppressive therapies, these treatments are still largely unproven. Recent data put the one-year survival rate for SLE-aPAH patients at 94%. Pregnant women are most at risk of dying due to undiagnosed SLE-aPAH, and screening should be considered essential in this population.

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“…In another small prospective study with 34 patients, Raynaud’s phenomenon was strongly related to increased PASP as assessed by TTE 16. Pleural effusions were also demonstrated to occur with higher frequency in CTD-associated PAH; patients with pleural effusions had higher mean right atrial pressure (mRAP) and lower cardiac output 17. However, most patients in that study had overt right heart failure, which may have contributed to pleural effusion.…”

Section: Predictive Factorsmentioning

confidence: 86%

Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies

Tselios¹,

Gladman²,

Urowitz³

2016

OARRR

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Systemic lupus erythematosus (SLE) is characterized by the second highest prevalence of pulmonary arterial hypertension (PAH), after systemic sclerosis, among the connective tissue diseases. SLE-associated PAH is hemodynamically defined by increased mean pulmonary artery pressure at rest (≥25 mmHg) with normal pulmonary capillary wedge pressure (≤15 mmHg) and increased pulmonary vascular resistance. Estimated prevalence ranges from 0.5% to 17.5% depending on the diagnostic method used and the threshold of right ventricular systolic pressure in studies using transthoracic echocardiogram. Its pathogenesis is multifactorial with vasoconstriction, due to imbalance of vasoactive mediators, leading to hypoxia and impaired vascular remodeling, collagen deposition, and thrombosis of the pulmonary circulation. Multiple predictive factors have been recognized, such as Raynaud’s phenomenon, pleuritis, pericarditis, anti-ribonuclear protein, and antiphospholipid antibodies. Secure diagnosis is based on right heart catheterization, although transthoracic echocardiogram has been shown to be reliable for patient screening and follow-up. Data on treatment mostly come from uncontrolled observational studies and consist of immunosuppressive drugs, mainly corticosteroids and cyclophosphamide, as well as PAH-targeted approaches with endothelin receptor antagonists (bosentan), phosphodiesterase type 5 inhibitors (sildenafil), and vasodilators (epoprostenol). Prognosis is significantly affected, with 1- and 5-year survival estimated at 88% and 68%, respectively.

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Frequency of Pleural Effusions in Patients With Pulmonary Arterial Hypertension Associated With Connective Tissue Diseases

Cited by 36 publications

References 15 publications

Clinical features of pulmonary arterial hypertension in patients receiving dasatinib

Clinical features of pulmonary arterial hypertension in patients receiving dasatinib

Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus: Current Status and Future Direction

Systemic lupus erythematosus and pulmonary arterial hypertension: links, risks, and management strategies

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