English (United Kingdom)

https://curated-unify.zendy.io/wp-json/zendy-region/v1/featured_content/oa?rat=en

https://curated-unify.zendy.io/wp-json/zendy-region/v1/highlighted_journal/

Global: Zendy Plus annual

Presents the access of premium content as premium feature

Premium Content

Presents the keyphrase highlighting as premium feature

Keyphrase Highlighting

Presents the summarisation as premium feature

Summarisation

Insights

Presents the pdf analysis as premium feature

PDF Analysis

Presents the zaia usage as premium feature

ZAIA

Global: Zendy Tools annual

Global: Zendy Free Plan

Global: Zendy Tools monthly

Global: Zendy Plus monthly

A rheumatic disorders constitute a diverse those of patients with rheumatoid arthritis (RA) and SS has shown similarities and differences. Consequently, group of illnesses with significant clinical and immunological variability. Their clinical presentation and the terms ‘primary’ and ‘secondary’ SS were proposed for the former and the latter groups of patients. course vary, ranging from subclinical and mild to severe and life-threatening, and from slow and indolent Compared to patients with secondary SS, patients with primary SS present significantly more often parotid to acute and catastrophic. Some of these disorders can be manifested simultaneously in the same individual gland enlargement and systemic manifestations, such as Raynaud’s phenomenon, lymphadenopathy, splenoor, alternatively, one nosological entity may evolve into another. For instance, Sjögren’s syndrome (SS) megaly, purpura and renal tubular acidosis. In addition, primary SS, but not secondary SS, is characterized frequently co-exists with various other rheumatic conditions, whereas a patient with an organ-specific disease by the occurrence of serum autoantibodies against Ro(SSA) (in 60%) and La(SSB) (in 40%) ribonucleo(e.g. myasthenia gravis or thyroiditis) can progress to a systemic autoimmune disorder, such as systemic proteins, and is associated with distinct HLA haplotypes [5, 6 ]. Although the incidence, clinical expression and lupus erythematosus (SLE) or SS. A multi-faceted activation of the immune system occurs in these disserology of SS in various autoimmune diseases have been evaluated [7], comparative studies of well-defined orders, as illustrated by the presence of autoantibodies directed against one or multiple autoantigens and/or groups of patients with SS co-expressed with autoimmune rheumatic diseases other than RA have never mononuclear inflammatory infiltrates in the affected tissues. The interplay of genetic factor(s) and environbeen performed. Despite this fact, during the present decade, the term ‘secondary SS’ has been arbitrarily mental agent(s) is thought to account for the development and clinical course of these diseases. The nature granted to SS occurring in patients with any autoimmune disorder [3]. of such factors, however, remains obscure, whereas the implication of microorganisms has been speculative Today, 20 yr after the original classification, clinical, histopathological and serological evidence implies the for the last three decades [1]. In an effort to establish an avenue of communication existence of at least two additional SS groups. (1) Primary SS overlapping with other autoimmune among clinical investigators, diagnostic/classification criteria have been established for a number of autodisorders. This group includes individuals who clinically express SS and suffer from another defined rheumatic immune rheumatic disorders [2, 3]. Nevertheless, grouping individuals with diverse clinical and immunological disease. These patients express autoantibodies to Ro(SSA) and La(SSB) proteins as frequently as patients manifestations into a disorder carries the risk of masking certain important or differentiating features. with the ‘primary’ disorder. Immunogenetic studies have revealed that the immune response to Ro(SSA) and Recent advances in the clinical, immunological and immunogenetic profile of one of these disorders, namely La(SSB) autoantigens is associated with distinct major histocompatibility complex (MHC) class II alleles, SS, can serve as a paradigm to illustrate the problem and offer some solutions. This autoimmune disorder is regardless of the clinical entity where they occur [8, 9]. Patients with autoimmune disorders who present feacommon and affects primarily females at the fourth to fifth decade of life. It presents with a diverse clinical tures of SS and express anti-Ro(SSA) and anti-La(SSB) responses could qualify for this group [10]. Careful spectrum ranging from disease confined in the exocrine glands to systemic disorder, whereas in a considerable evaluation of the clinical and immunogenetic profile of this group, and in comparison to that of primary or number of patients lymphoid neoplasia ensues. The lungs, kidneys, liver and thyroid gland are frequently secondary SS groups, should be performed. (2) SS with other autoantibody specificities. The affected, and several lines of evidence suggest that epithelial cells are the main targets of destruction [4]. patients of this group are seemingly indistinguishable from primary SS. On closer scrutiny, however, these SS can be found alone or in association with almost all autoimmune rheumatic disorders. The diagnosis is based patients reveal characteristic clinical and serological features. Their autoantibody profile consists of reactivon the criteria put forward by a European multicentre study [3]. Accordingly, all conditions (metabolic, infecities compatible with other autoimmune disorders (namely, autoantibodies to mitochondria, thyroid pertious, degenerative, inflammatory, neoplastic and drug side-effects) that mimic the clinical and histopathological oxidase or centromere) [11, 12]. Most often, these individuals do not express the overt clinical picture of features of SS should be excluded. In the late 1970s, comparison of the clinical, serothe corresponding disorder (i.e. primary biliary cirrhosis, thyroiditis or limited scleroderma). However, the histological and immunogenetic profiles of patients with SS without evidence for another autoimmune disorder and pathological evaluation of liver or thyroid tissues in SS

Lumping or splitting autoimmune rheumatic disorders? Lessons from Sjogren's syndrome