Right Heart Function in Systemic Lupus Erythematosus: Insights from Myocardial Doppler Tissue Imaging

2006 ◽  
Vol 19 (4) ◽  
pp. 441-449 ◽  
Author(s):  
Pei-Lan Gin ◽  
Wen-Chin Wang ◽  
Shu-Hsin Yang ◽  
Shih-Hung Hsiao ◽  
Jui-Cheng Tseng
2006 ◽  
Vol 19 (6) ◽  
pp. 822-828 ◽  
Author(s):  
Shih-Hung Hsiao ◽  
Chiu-Yen Lee ◽  
Shu-Mei Chang ◽  
Shu-Hsin Yang ◽  
Shih-Kai Lin ◽  
...  

2006 ◽  
Vol 19 (5) ◽  
pp. 507-514 ◽  
Author(s):  
Shih-Hung Hsiao ◽  
Chiu-Yen Lee ◽  
Shu-Mei Chang ◽  
Shih-Kai Lin ◽  
Chun-Peng Liu

2012 ◽  
Vol 2012 ◽  
pp. 1-12 ◽  
Author(s):  
Atiya Dhala

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.


2017 ◽  
Vol 5 ◽  
pp. 2050313X1770715
Author(s):  
Cody Lee ◽  
Jean Elwing

Objective: The pulmonary vascular targeted treatment for systemic lupus erythematosus–associated pulmonary arterial hypertension is similar to other connective tissue disease–associated pulmonary arterial hypertension. In addition, there also appears to be a role for immunosuppression in the overall management. However, the optimal immunosuppressive regimen and what patients will respond to treatments are currently not clearly elucidated given the lack of randomized controlled trials on the subject. Our objective is to highlight the importance of early immunosuppression in systemic lupus erythematosus–associated pulmonary arterial hypertension and the role of pulse dose steroids in management. Methods: This case describes a 23-year-old woman who presented with pulmonary arterial hypertension diagnosed by right heart catheterization with mean pulmonary artery pressure of 74 mmHg, pulmonary capillary wedge pressure of 12 mmHg, and a pulmonary vascular resistance of 1908 dyne s cm−5. Due to the aggressive nature of her disease, she declined despite management with epoprostenol and sildenafil. Because of coexisting systemic lupus erythematosus with hemolytic anemia and worsening pulmonary arterial hypertension, intensive immunosuppressive therapy with pulse dose steroids was initiated. Results: Shortly after initiation of pulse dose steroids and maintenance immunosuppression, she had a dramatic symptomatic and hemodynamic response with a decrease in her pulmonary vascular resistance from 1908 to 136 dyne sec cm−5 and improvement in her mean pulmonary artery pressure from 74 to 27 mmHg on repeat right heart catheterization. Conclusion: Early immunosuppression is important to consider in those with systemic lupus erythematosus–associated pulmonary arterial hypertension. Limited studies are available, but most have focused on the use of cyclophosphamide. Pulse dose steroids may be a potentially less toxic but equally effective manner to aid in the treatment of systemic lupus erythematosus–pulmonary arterial hypertension when intensive immunosuppression is being considered.


Author(s):  
Francis R. Comerford ◽  
Alan S. Cohen

Mice of the inbred NZB strain develop a spontaneous disease characterized by autoimmune hemolytic anemia, positive lupus erythematosus cell tests and antinuclear antibodies and nephritis. This disease is analogous to human systemic lupus erythematosus. In ultrastructural studies of the glomerular lesion in NZB mice, intraglomerular dense deposits in mesangial, subepithelial and subendothelial locations were described. In common with the findings in many examples of human and experimental nephritis, including many cases of human lupus nephritis, these deposits were amorphous or slightly granular in appearance with no definable substructure.We have recently observed structured deposits in the glomeruli of NZB mice. They were uncommon and were found in older animals with severe glomerular lesions by morphologic criteria. They were seen most commonly as extracellular elements in subendothelial and mesangial regions. The deposits ranged up to 3 microns in greatest dimension and were often adjacent to deposits of lipid-like round particles of 30 to 250 millimicrons in diameter and with amorphous dense deposits.


2000 ◽  
Vol 6 (7) ◽  
pp. 821-825 ◽  
Author(s):  
ELIZABETH LERITZ ◽  
JASON BRANDT ◽  
MELISSA MINOR ◽  
FRANCES REIS-JENSEN ◽  
MICHELLE PETRI

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