Sjögren syndrome

Abstract

S jögren syndrome is a chronic, systemic disorder of an autoimmune nature. It is characterized by lymphocytic infiltration of the exocrine (mainly salivary and lacrimal) glands and by remarkable B-cell hyperactivity. The latter is manifested by hyper gam maglobulinemia and the presence of serum autoantibodies, including antinuclear antibodies, rheumatoid factor, cryoprecipitable immunoglobulins and antibodies against two ribonucleoproteinic complexes named Ro/SSA and La/SSB; these antibodies are considered hallmarks of the disease. 1,2Although their exact role is not known, recent data suggest that antibodies against the Ro52 component of the Ro/SSA antigen (a 52kD protein that inhibits proinflammatory responses) might inhibit its regulatory function.3 The condition is nine times more common among women than among men, with peak onset during menopause.1 A recent review suggests that the prevalence ranges from 0.1% to 4.8%, 1 with rates increasing with advanced age.4,5 Heterogeneity in inclusion criteria, ethnic origin, sample size and sex distribution between studies contributed to the observed variability.Although Sjögren syndrome is classically considered to be localized disease of the exo crine glands, mainly manifested with oral and ocular dryness, it also has a wide range of systemic clinical manifestations that affect essentially any organ system, and a small number of cases are complicated by the development of non-Hodgkin lymphoma.1,6 Secondary Sjögren syndrome is associated with an established connective-tissue disease.As is the case for many autoimmune diseases, the primary etiopathogenetic events are not known. The current hypothesis is that an interplay between environmental contributors (e.g., viruses, stress, hormones) and the patient's genetic background can lead to inflammatory responses against epithelial tissues. In this review, we summarize the current evidence, mostly from observational, open-label and randomized clinical trials, for the clinical manifestations, diagnosis and management of Sjögren syndrome (Box 1). What are the clinical features of Sjögren syndrome?The clinical features of Sjögren syndrome can be largely divided into those related to exocrine dysfunction (glandular) and those that affect organs other than the exocrine glands (extraglandular or systemic). The latter can be further divided into nonspecific features, those characterized by periepithelial infiltrates in parenchymal organs and those that result from immunocomplex deposition as a result of B-cell hyperactivity. 7,8 Most patients with Sjögren syndrome (about 90%) have an indolent benign course; however, a small but important number of cases (5%-10%) are complicated by immunocomplex pathology and lymphoid neoplasia, both of which are associated with high mortality (a 3.25-fold increase compared with the general population). 6,7,9

Sex differential association of dermatomyositis with Sjögren syndrome

“…Reportedly, phenotypes (e.g., myositis) may be altered in patients with concurrent anti-SSA antibodies, hallmarks of Sjögren syndrome. 13 These alterations include perivascular inflammation, 15 myositis deterioration, malignant disease, 32 left ventricular diastolic dysfunction, 33 interstitial lung disease 34 and death. 32 Sjögren syndrome combined with other autoimmune diseases was associated with increased T-cell activation, 35 Raynaud phenomenon, 36 thyroid disease, 37 cardiac electrical instability 38 and lymphoma, 39 more so than with the respective autoimmune diseases alone.…”

“…Although an association with Sjögren syndrome has been shown in other autoimmune diseases, including rheumatoid arthritis, 6 systemic lupus erythematosus 7 and systemic sclerosis, 8 data on Sjögren syndrome in patients with dermatomyositis are sparse and mostly limited to case reports. [9][10][11] Both dermatomyositis and Sjögren syndrome share common autoantibodies (e.g., anti-poly(U)-binding-splicing factor, 12 antinuclear antibodies, 13,14 anti-Sjögren-syndrome-related antigen A [anti-SSA], 13,15 anti-Sjögren -syndrome-related antigen B [anti-SSB] 13,16 ) and genetic polymorphisms (e.g., interferon regulatory factor 5 [IRF5 rs4728142]). 17,18 In addition, patients with Sjögren syndrome have a 4-fold higher mortality, 19 with surviving patients incurring a lifetime of increased medical expenses, among other costs.…”

Síndrome de intestino irritable y autoinmunidad

“…El Síndrome de Sjögren es una enfermedad autoinmune sistémica crónica que presenta infiltrado linfoplasmocitario en glándulas exocrinas (glándulas lagrimales y salivales) y que se manifiesta clínicamente por el complejo sicca: xerostomía y xeroftalmia 28,29 . El complejo sicca es común en la FM (hasta el 70% de los pacientes con FM presentan SII), por lo cual un estudio analizó la prevalencia de sicca y FM en 45 pacientes con SII atendidos en un nivel secundario de atención y 46 controles sanos 29 .…”

Alteraciones Motoras Esofágicas en Pacientes con Síndrome de Sjögren: Estudio de Casos y Controles

“…El síndrome de Sjögren primario (SSp), es una entidad nosológica de origen autoinmune, progresión lenta, que afecta 20 veces más a mujeres que a hombres (Alamanos et al, 2006;Patel & Shahane, 2014) y que evoluciona con potencial afectación de diversos órganos y sistemas, como el tracto respiratorio (Kreider & Highland, 2014), endocrino (Mavragani et al, 2012), renal (Evans et al, 2015), digestivo (Mavragani & Moutsopoulos, 2014), etc.…”