Mixed Airway and Pulmonary Parenchymal Disease in Patients With Primary Sjögren Syndrome: A 6-year Follow-up

2020 ◽  
pp. jrheum.200247
Author(s):  
Anna Matilda Nilsson ◽  
H. Laura Aaltonen ◽  
Peter Olsson ◽  
Hans Lennart Persson ◽  
Roger Hesselstrand ◽  
...  
Keyword(s):  
Pulmonary Function ◽  
Respiratory Symptoms ◽  
Pulmonary Involvement ◽  
Sjögren Syndrome ◽  
Cigarette Consumption ◽  
Sjogren Syndrome ◽  
Primary Sjögren Syndrome ◽  
Parenchymal Disease ◽  
Over Time

Objective To assess pulmonary function and chronic obstructive pulmonary disease (COPD) development over time in patients with primary Sjögren syndrome (pSS), as well as the association between pulmonary function, radiographic findings, respiratory symptoms, and clinical features of pSS, taking cigarette consumption into account. Methods Forty patients with pSS (mean age 66 yrs; range 42–81 yrs; 39 women), previously participating in a cross-sectional study on pulmonary involvement in pSS, were reassessed by pulmonary function tests after a mean follow-up time of 6 years. At follow-up, patients were also assessed by high-resolution computed tomography of the chest, as well as for pSS disease activity, respiratory symptoms, and cigarette consumption. Results Patients with pSS showed significantly decreased percentages of predicted total lung capacity (TLC), residual volume (RV), RV/TLC ratio, and diffusing capacity of the lungs for carbon monoxide, as well as an increase in predicted forced expiratory volume in 1 second/vital capacity (FEV1/VC) ratio from baseline to follow-up. The proportion of COPD in patients with pSS did not change significantly from baseline to follow-up (38% vs 40%, respectively). Radiographic signs of bronchial involvement and interstitial lung disease were each found in 38% of the patients. Conclusion Both airway and pulmonary parenchymal disease were commonly found in patients with pSS, with a coexistence of both an obstructive and restrictive pulmonary function pattern, where the latter tended to deteriorate over time. COPD was a common finding. Airway and pulmonary involvement may be underdiagnosed in pSS, which is why special attention to clinical assessment of pulmonary involvement in patients with pSS is mandated.

Chronic Obstructive Pulmonary Disease Is Common in Never-smoking Patients with Primary Sjögren Syndrome

2015 ◽  
Vol 42 (3) ◽  
pp. 464-471 ◽  
Author(s):  
Anna Matilda Nilsson ◽  
Sandra Diaz ◽  
Elke Theander ◽  
Roger Hesselstrand ◽  
Eeva Piitulainen ◽  
...  
Keyword(s):  
Chronic Obstructive Pulmonary Disease ◽  
Cigarette Smoking ◽  
Disease Activity ◽  
Lung Disease ◽  
Respiratory Symptoms ◽  
Sjögren Syndrome ◽  
Chronic Obstructive ◽  
Sjogren Syndrome ◽  
Obstructive Pulmonary Disease ◽  
Primary Sjögren Syndrome

Objective.To assess the prevalence of chronic obstructive pulmonary disease (COPD) in patients with primary Sjögren syndrome (pSS) and to study the association of COPD with cigarette smoking, radiographic features, respiratory symptoms, disease activity, and laboratory inflammatory and serological features in patients with pSS.Methods.Fifty-one consecutive patients with pSS (mean age 60 yrs, range 29–82 yrs, 49 women) were assessed by pulmonary function tests (PFT). The PFT results were compared with previously studied population-based controls, standardizing results with regard to sex, age, height, weight, and cigarette smoking. In addition, patients with pSS were assessed by computed tomography of the chest, the European League Against Rheumatism Sjögren Syndrome Disease Activity Index and Patient Reported Index, the St. George’s Respiratory Questionnaire (which evaluates respiratory symptoms), and by laboratory inflammatory and serological tests.Results.Forty-one percent of all patients with pSS and 30% of the never-smoking patients with pSS fulfilled the Global Initiative for Chronic Obstructive Lung Disease criteria for COPD. Vital capacity (VC), forced expiratory volume in 1 s (FEV1), FEV1/VC ratio, and DLCO were significantly decreased while residual volume (RV) and the RV/total lung capacity ratio were significantly increased in patients with pSS. Moderate correlations between PFT results, symptoms, and disease activity were found. However, laboratory inflammatory and serological features were poorly associated with PFT results in patients with pSS.Conclusion.COPD was a common finding in patients with pSS, even among never-smoking patients. An obstructive pattern was the predominant PFT finding in patients with pSS, although a superimposed restrictive lung disease could not be excluded. The results suggest that the disease per se is involved in the development of COPD in pSS.

Primary Sjögren syndrome in a 2-year-old patient: role of the dentist in diagnosis and dental management with a 6-year follow-up

2011 ◽  
Vol 21 (6) ◽  
pp. 471-475 ◽  
Author(s):  
MARCIO AUGUSTO De OLIVEIRA ◽  
NATHALIE PEPE MEDEIROS De REZENDE ◽  
CÉLIA MÁRCIA FERNANDES MAIA ◽  
MARINA GALLOTTINI
Keyword(s):  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Patient Role ◽  
Primary Sjögren Syndrome ◽  
Dental Management

scholarly journals Lung complications of Sjogren syndrome

2020 ◽  
Vol 29 (157) ◽  
pp. 200021
Author(s):  
Fabrizio Luppi ◽  
Marco Sebastiani ◽  
Nicola Sverzellati ◽  
Alberto Cavazza ◽  
Carlo Salvarani ◽  
...  
Keyword(s):  
Lymphocytic Infiltration ◽  
Airway Disease ◽  
Pulmonary Involvement ◽  
Sjögren Syndrome ◽  
Systemic Autoimmune Disease ◽  
Sjogren Syndrome ◽  
Recent Trial ◽  
Risk Of Death ◽  
Primary Sjögren Syndrome ◽  
Related Quality

Primary Sjogren syndrome (pSS) is a systemic autoimmune disease characterised by lymphocytic infiltration of exocrine glands and by a number of systemic manifestations, including those regarding the lung. Pulmonary involvement in pSS includes interstitial lung disease (ILD) and airway disease, together with lymphoproliferative disorders.Patients with pSS-ILD report impaired health-related quality of life and a higher risk of death, suggesting the importance of early diagnosis and treatment of this type of pulmonary involvement. In contrast, airway disease usually has little effect on respiratory function and is rarely the cause of death in these patients.More rare disorders can be also identified, such as pleural effusion, cysts or bullae.Up to date, available data do not allow us to establish an evidence-based treatment strategy in pSS-ILD. No data are available regarding which patients should be treated, the timing to start therapy and better therapeutic options. The lack of knowledge about the natural history and prognosis of pSS-ILD is the main limitation to the development of clinical trials or shared recommendations on this topic. However, a recent trial showed the efficacy of the antifibrotic drug nintedanib in slowing progression of various ILDs, including those in pSS patients.

scholarly journals Cardiovascular Risk and Endothelial Dysfunction in Primary Sjogren Syndrome Is Related to the Disease Activity

Nutrients ◽  
2021 ◽  
Vol 13 (6) ◽  
pp. 2072
Author(s):  
Anna Łuczak ◽  
Rafał Małecki ◽  
Michał Kulus ◽  
Marta Madej ◽  
Ewa Szahidewicz-Krupska ◽  
...  
Keyword(s):  
Endothelial Dysfunction ◽  
Disease Activity ◽  
Plasma Concentrations ◽  
Pulmonary Involvement ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Primary Sjögren Syndrome ◽  
Disease Characteristics ◽  
Plasma Adma

The aim of our study was to evaluate if endothelial-dysfunction (ED) occurs in patients with primary Sjogren syndrome (pSS) and whether it is associated with the disease characteristics and activity. A total of 46 patients with pSS and 30 controls, without known cardiovascular disease, were enrolled in this study. A flow-mediated-dilation (FMD) of the brachial artery, plasma concentrations of the nitric oxide (NO) metabolic pathway (ADMA, L-arginine, SDMA, cGMP), and markers of endothelial inflammatory function (PAI-1, sE-selectin) and angiogenesis (angiostatin, VEGF) were analyzed. The FMD was significantly lower in pSS patients (7.56 ± 3.08 vs. 10.91 ± 1.02%, p = 0.043) and positively correlated with the Ro/SS-A-antibodies (r = 0.34, p = 0.03), pulmonary involvement (r = 0.52, p = 0.001) and inversely with ADMA (r = −0.35, p = 0.04). Plasma ADMA, L-arginine and angiostatin levels were significantly higher in pSS patients (0.39 ± 0.08 vs. 0.36 ± 0.06 µmol/L, p = 0.05; 29.07 ± 6.7 vs. 25.4 ± 5.23 µmol/L, p = 0.01; 152.25 ± 60.99 vs. 120.07 ± 38.7 pg/mL, p = 0.0, respectively). ADMA was associated with ESSDAI (r = 0.33, p = 0.02), SCORE (r = 0.57, p = 0.00003) and focus score (r = 0.38, p = 0.04). In the multiple regression analysis, the ESSDAI was significantly and independently associated with plasma ADMA levels (β = 0.24, p = 0.04). Moreover, plasma cGMP concentrations were negatively correlated with the disease duration (r = −0.31, p = 0.03). Endothelial function is impaired in patients with pSS and associated with the measures of disease activity, which supports the key-role of inflammation in developing and maintaining accelerated atherosclerosis.

Atypical Autoantibodies in Patients with Primary Sjögren Syndrome: Clinical Characteristics and Follow-Up of 82 Cases

2006 ◽  
Vol 35 (5) ◽  
pp. 312-321 ◽  
Author(s):  
Manuel Ramos-Casals ◽  
Norma Nardi ◽  
Pilar Brito-Zerón ◽  
Sira Aguiló ◽  
Victor Gil ◽  
...  
Keyword(s):  
Clinical Characteristics ◽  
Sjögren Syndrome ◽  
Sjogren Syndrome ◽  
Primary Sjögren Syndrome
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