Lung complications of Sjogren syndrome

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.

Download Full-text

FRI0563 It's More than Dryness and Fatigue: The Patient Perspective on Health-Related Quality of Life in Primary Sjögren Syndrome - A Qualitative Study

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2016-eular.3905 ◽

2016 ◽

Vol 75 (Suppl 2) ◽

pp. 644.3-645

Author(s):

A. Lackner ◽

A. Ficjan ◽

J. Unger ◽

W. Graninger ◽

C. Dejaco

Keyword(s):

Quality Of Life ◽

Qualitative Study ◽

Patient Perspective ◽

Sjögren Syndrome ◽

Sjogren Syndrome ◽

Health Related Quality ◽

Primary Sjögren Syndrome ◽

Related Quality ◽

Health Related

Download Full-text

Cutting the “Gordian Knot” — Cardiac Involvement in Primary Sjögren Syndrome

The Journal of Rheumatology ◽

10.3899/jrheum.201171 ◽

2021 ◽

pp. jrheum.201171

Author(s):

George Markousis-Mavrogenis ◽

Sophie I. Mavrogeni

Keyword(s):

Myocardial Infarction ◽

Cardiovascular Disease ◽

Autoimmune Disease ◽

Cardiac Involvement ◽

Sjögren Syndrome ◽

Systemic Autoimmune Disease ◽

Sjogren Syndrome ◽

Exocrine Glands ◽

Primary Sjögren Syndrome ◽

Increased Risk

Sjögren syndrome (SS) is a systemic autoimmune disease typically characterized by inflammatory involvement of the exocrine glands1. The association of SS with an increased risk of cardiovascular disease (CVD) including manifestations such as stroke and myocardial infarction has been demonstrated by numerous previous studies1.

Download Full-text

Cerebral Venous Thrombosis Revealing Primary Sjögren Syndrome: Report of 2 Cases

Case Reports in Medicine ◽

10.1155/2013/747431 ◽

2013 ◽

Vol 2013 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

A. Mercurio ◽

M. Altieri ◽

V. M. Saraceni ◽

T. Paolucci ◽

G. L. Lenzi

Keyword(s):

Nervous System ◽

Venous Thrombosis ◽

Cerebral Venous Thrombosis ◽

Lymphocytic Infiltration ◽

Sjögren Syndrome ◽

Sjogren Syndrome ◽

Exocrine Glands ◽

Neurologic Complications ◽

Primary Sjögren Syndrome ◽

Syndrome Report

Sjögren syndrome (SS) is an autoimmune disease of the exocrine glands, characterized by focal lymphocytic infiltration and destruction of these glands. Neurologic complications are quite common, mainly involving the peripheral nervous system (PNS). The most common central nervous system (CNS) manifestations are myelopathy and microcirculation vasculitis. However, specific diagnostic criteria for CNS SS are still lacking. We report two cases of primary SS in which the revealing symptom was cerebral venous thrombosis (CVT) in the absence of genetic or acquired thrombophilias.

Download Full-text

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

The Journal of Rheumatology ◽

10.3899/jrheum.200247 ◽

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.

Download Full-text

Prognostic markers of lymphoma development in primary Sjögren syndrome

Lupus ◽

10.1177/0961203319857132 ◽

2019 ◽

Vol 28 (8) ◽

pp. 923-936 ◽

Cited By ~ 16

Author(s):

S Retamozo ◽

P Brito-Zerón ◽

M Ramos-Casals

Keyword(s):

Risk Factors ◽

Lupus Erythematosus ◽

Activity Index ◽

Sjögren Syndrome ◽

Systemic Autoimmune Disease ◽

Low Grade ◽

Sjogren Syndrome ◽

Close Link ◽

Systemic Involvement ◽

Primary Sjögren Syndrome

Sjögren syndrome is a systemic autoimmune disease that principally affects women between the fourth and sixth decades of life who present with sicca symptomatology caused by dryness of the main mucosal surfaces. The clinical spectrum of Sjögren syndrome extends from dryness to systemic involvement. Since 1978, Sjögren syndrome has been closely associated with an enhanced risk of lymphoma, one of the most severe complications a patient may develop. Primary Sjögren syndrome patients have a 10–44-fold greater risk of lymphoma than healthy individuals, higher than that reported for systemic lupus erythematosus and rheumatoid arthritis. The close link between lymphoma and Sjögren syndrome is clearly exemplified by the very specific type of lymphoma arising in Sjögren syndrome patients, mainly low-grade B-cell lymphomas (predominantly a marginal zone histological type) with primary extranodal involvement of the major salivary glands (overwhelmingly parotid), with a primordial role of cryoglobulinemic-related markers (both clinical and immunological). The most recent studies support a higher number of risk factors detected in an individual leads to a higher lymphoma risk. A close follow-up of high-risk groups with longitudinal assessments of all known risk factors, including cryoglobulin-related markers and EULAR Sjögren's syndrome disease activity index measurement in particular, is mandatory.

Download Full-text

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

Nutrients ◽

10.3390/nu13062072 ◽

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.

Download Full-text

P083 Primary Goujerot- Sjögren Syndrome: a pediatric case report

Rheumatology ◽

10.1093/rheumatology/keab722.075 ◽

2021 ◽

Vol 60 (Supplement_5) ◽

Author(s):

Djohra Hadef ◽

Samy Slimani ◽

Yamina Ouchen

Keyword(s):

Autoimmune Disease ◽

Clinical Signs ◽

Lymphocytic Infiltration ◽

Initial Therapy ◽

Sjögren Syndrome ◽

Lower Limbs ◽

Systemic Autoimmune Disease ◽

Sjogren Syndrome ◽

Organ Systems ◽

One Year

Abstract Background Gourgerot-Sjögren syndrome (GSS) is a systemic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands leading to dryness of the mucous membranes. It may also involve other organs and organ systems. GSS can be primary (pGSS) or secondary due to other autoimmune diseases. pSGS mainly affects women over 40 years old, but can occur at any age. GSS is rare in children and is often secondary to other diseases. We report the case of a 12-year-old girl with primary Gougerot-Sjögren Syndrome Methods and results A 12-year-old girl from Batna (Algeria) presented to her pediatrician in 2016 for a rash with arthralgia. She had no relevant past medical history and is the child of a non-consanguineous marriage. Clinical examination found a well-nourished child with a purpuric rash on her lower limbs. She complained of arthralgia without clinical signs of arthritis. The child also complained of a sensation of a foreign body and burning in both eyes. The eye exam was normal except for a positive Schirmer's test. There were no signs of parotitis. Laboratory tests showed an erythrocyte sedimentation rate (ESR) of 84 mm the first h and a negative C-reactive protein (CRP). She was also Leukopenic. Serological testing resulted in a positive Anti SS-A and Anti SS-B. The abdominal pelvic ultrasound was normal. The patient was diagnosed with primary Gougerot-Sjögren Syndrome. Artificial tears and hydroxychloroquine were started as initial therapy. One year later we added an immunosuppressant (Methotrexate) due to the persistence of clinical signs. Conclusion Our case is particular in that primary Gougerot-Sjögren Syndrome is rare in children. Routine follow- up with this patient is important to determine whether it is indeed pGSS with pediatric onset or GSS secondary to another autoimmune disease.

Download Full-text