Primary Sjögren syndrome and development of another autoimmune rheumatic disease during the follow-up

Abstract

Background Primary Sjögren syndrome (pSS) is a chronic autoimmune disease with its main target being exocrine glands, and is the connective tissue disease more frequently associated with other autoimmune diseases. The aim of this study was to assess the frequency of another autoimmune rheumatic disease (ARD) developed in primary Sjögren syndrome (pSS) patients and to describe it’s clinical, serological and histologic characteristics. Materials and methods This is a retrospective cohort study. Data of patients with pSS diagnosis (American-European criteria 2002), included in the GESSAR database (Grupo de Estudio Síndrome de Sjögren, Sociedad Argentina de Reumatología) were analyzed. The development of a second ARD was registered during the follow up. Results 681 patients were included, 94.8% female. The mean age was 54 (SD 14) years and mean age at diagnosis of 50 (SD 13) years. The mean follow-up was 4.7 (SD 4.9) years; 30 patients (4.41%, CI 95%: 3.1–5.7) developed a second ARD during the follow up, incidence rate was 9.1/1000 patients-year (IR 95%: 5.8–12.4/1000 patients-year), the most frequent being rheumatoid arthritis (RA). 96% out of these 30 patients had xerophthalmia, 86.2% xerostomia, 92% positive Schirmer test, 88.24% positive Rosa Bengala test, lisamine green or Ocular Staining Score, 81.2% positive unstimulated salivary flow, 82.1% Ro(+) and 33.33% La(+). Minor salivary gland biopsy had been performed in 14 of the 30 patients, 12 with positive results. There were no statistically significant differences respect baseline characteristics when comparing the patients who developed another ARD to the ones that did not. Conclusions Of all the patients analyzed, 4.4% presented another ARD during their follow-up. It is important to be aware of this, to make an early and proper diagnosis and treatment of our patients.

Increased Risk of Developing Systemic Lupus Erythematosus and Rheumatoid Arthritis in Patients with Primary Sjögren’s Syndrome—A Secondary Cohort Analysis of Nationwide, Population-Based Claims Data

“…These findings are consistent with previous studies. Those who had arthralgias and arthritis and positive serology for rheumatoid factor or anti-cyclic citrullinated peptide antibodies were more likely to develop RA [ 15 ]. Kuo et al showed that the risk of pSS and other autoimmune diseases, such as RA or SLE, was higher in the relatives of patients with pSS [ 3 ].…”

“…In addition, a meta-analysis showed that patients with pSS might have a higher risk of developing RA [ 14 ]. In a retrospective cohort study of 681 patients with pSS, 1% ( n = 5) developed SLE and 2.1% ( n = 14) developed RA after 4.7 years of follow-up [ 15 ]. On the other hand, one study suggested that pSS patients with articular manifestations might just resemble RA, but it differed in the absence of structural damage [ 16 ].…”

Sjogren's syndrome: a neurological perspective

“…31 In the original description of the disease by Henrik Sjögren in 1933, 13 (68.4%) out of 19 patients also had RA. 28 Rodriguez et al 31 described that among 681 SS patients prospectively followed for a mean of 4.7 years, 30 developed a second autoimmune rheumatic disease, mostly RA, followed by scleroderma and SLE. SS seems to influence the character and phenotype of the accompanying disease, either improving (less central nervous system symptoms in SLE) or exacerbating its course (lymphoma in RA).…”

“…30 SS is also frequently associated with other rheumatic diseases, in ∼30% of the cases, such as RA, SLE, scleroderma, and dermatopolymyositis. 31 In the original description of the disease by Henrik Sjögren in 1933, 13 (68.4%) out of 19 patients also had RA. 28 Rodriguez et al 31 described that among 681 SS patients prospectively followed for a mean of 4.7 years, 30 developed a second autoimmune rheumatic disease, mostly RA, followed by scleroderma and SLE.…”