scholarly journals A Case of Sjögren’s Syndrome-Related Pulmonary Arterial Hypertension Treated with Iloprost and Bosentan Combination Therapy

2018 ◽  
Vol 7 (2) ◽  
pp. 59-62
Author(s):  
Ayşe Baha ◽  
Berkay Ekici ◽  
Nalan Ogan ◽  
Evrim Eylem Akpınar
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Combination Therapy ◽  
Arterial Hypertension ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Pulmonary Arterial ◽  
Sjögren‘S Syndrome

Primary Sjögren’s Syndrome and Cardiovascular Disease

2020 ◽  
Vol 18 (5) ◽  
pp. 447-454 ◽  
Author(s):  
Konstantinos Melissaropoulos ◽  
Dimitrios Bogdanos ◽  
Theodoros Dimitroulas ◽  
Lazaros I. Sakkas ◽  
George D. Kitas ◽  
...  
Keyword(s):  
Sjögren’S Syndrome ◽  
Lupus Erythematosus ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Current Data ◽  
Pulmonary Arterial ◽  
Cv Disease ◽  
Meta Analyses ◽  
Sjögren‘S Syndrome

Sjögren’s syndrome is a rheumatic autoimmune disease that primarily affects middle-aged women and runs a slowly progressing course with sicca symptoms being the prevalent manifestation. Premature atherosclerosis and increased cardiovascular (CV) morbidity and mortality are frequently encountered in rheumatic diseases characterized by significant systemic inflammation, such as the inflammatory arthritides, systemic vasculitides and systemic lupus erythematosus. In the same context, chronic inflammation and immune aberrations underlying Sjögren’s syndrome are also reported to be associated with augmented risk of atherosclerosis. Increased CV disease (CVD) frequency has been found in recent meta-analyses. The involvement of the CV system is not a common feature of Sjögren’s syndrome; however, specific manifestations, such as autoantibody-mediated heart block, pericarditis, pulmonary arterial hypertension and dysautonomia, have been described. This review focuses on studies addressing CV morbidity in Sjögren’s syndrome and presents current data regarding distinct CV features of the disease.

scholarly journals Successful Treatment of Sjögren’s Syndrome Presenting as a Condition Similar to Chronic Capillary Leak Syndrome Using Combination Therapy with High-Dose Intravenous Immunoglobulin and Glucocorticoid

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Masami Tokura ◽  
Tomoko Niwano ◽  
Kenji Nagasaka
Keyword(s):  
Combination Therapy ◽  
Intravenous Immunoglobulin ◽  
Sjögren’S Syndrome ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Capillary Leak Syndrome ◽  
Capillary Leak ◽  
Prednisolone Dose ◽  
Sjögren‘S Syndrome

A 70-year-old woman with Sjögren’s syndrome (SS) complained of generalized edema. Computed tomography showed thoracoabdominal fluid, suggesting serositis with SS. 35 mg/day of prednisolone as a monotherapy was ineffective. Moreover, hemoconcentration with hypoalbuminemia without inflammatory signs lead us to consider the systemic capillary leak syndrome (SCLS). Additional treatment with intravenous immunoglobulin (IVIG) and prednisolone dramatically decreased the thoracoabdominal fluid. However, when reducing the prednisolone dose, the thoracoabdominal fluid reincreased. Retreatment with IVIG without increasing the prednisolone dose was ineffective. However, additional prednisolone of 35 mg/day was effective, suggesting SCLS with SS might require combination therapy with IVIG and glucocorticoid.

The prognosis of pulmonary arterial hypertension associated with primary Sjögren's syndrome: a cohort study

Lupus ◽  
2018 ◽  
Vol 27 (7) ◽  
pp. 1072-1080 ◽  
Author(s):  
Z Liu ◽  
X Yang ◽  
Z Tian ◽  
J Qian ◽  
Q Wang ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Primary Sjögren’S Syndrome ◽  
Primary Sjogren’S Syndrome ◽  
Primary Sjögren's Syndrome ◽  
Pulmonary Arterial ◽  
Sjögren‘S Syndrome

Pulmonary arterial hypertension (PAH) is a rare and severe complication of primary Sjögren's syndrome (pSS). Cohort studies indicate that the underlying diseases of PAH associated with connective tissue disease (CTD-PAH) in Asian countries are different from that in the United States and in Europe. This study investigated the clinical characteristics, survival, and prognostic factors of pSS-PAH in Chinese patients. We enrolled 29 patients with pSS-PAH who visited our referral center during August 2007 and May 2015. PAH was confirmed by right heart catheterization (RHC). Baseline demographic data, clinical manifestations, laboratory tests, autoantibody results, hemodynamics data, and treatment regimens were analyzed. All patients were followed up at the Department of Rheumatology, Peking Union Medical College Hospital (PUMCH) every three to six months. All patients were female. Mean age of pSS onset was 34.4 ± 11.1 years. Mean age of PAH diagnosed by RHC was 40.6 ± 9.0 years. There was no significant difference in age, disease duration, and hemodynamic findings between the survivors and the non-survivors in this cohort. The overall one-, three-, and five-year survival rates were 80.2%, 74.8%, and 67.4%, respectively. Prognostic factors of mortality were time between pSS onset and PAH onset (HR 1.102, 95% CI 1.017–1.185, p = 0.018) and cardiac index < 2 l/min/m2 (HR 5.497, 95% CI 1.063–28.434, p = 0.042). In contrast, the use of immunosuppressants (HR 0.110, 95% CI 0.024–0.495, p = 0.004) was related to better survival. This study demonstrates that the predictors of mortality in patients with pSS-PAH include delayed onset of PAH in pSS and worse cardiac function. Immunosuppressants can improve the prognosis of pSS-PAH.

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