Saliva levels of caspase-1, TNF-α, and IFN-γ in primary Sjögren's syndrome: oral mucosal abnormalities revisited

Background/aim: Abnormalities in oral mucosal immunity contribute to complex pathogenesis of primary Sjögren's syndrome (pSjS). We aimed to measure saliva and serum levels of caspase-1, tumor necrosis factor-alpha (TNF-α) and interferon gamma (IFN-γ) in patients with pSjS. Materials and methods: We studied 43 pSjS patients fulfilling the AECG criteria and 30 age/sex-matched healthy controls, as well as 39 rheumatoid arthritis (RA) patients as a disease control group. ESSDAI scores were less than seven in all patients with pSjS, indicating low disease activity. Quantitative analyses were made in serum and whole saliva samples. The statistical analysis was performed using SPSS 19.0. Results: While no significant difference was found in serum measurements, saliva levels of TNF-α and caspase-1 were significantly higher in pSjS patients versus healthy controls when using the Mann-Whitney U test. On the other hand, in the pSjS group, saliva levels of TNF-α and caspase-1 were also significantly higher compared to the RA group using Student's t-test. In the pSjS group, those parameters did not show any correlation with disease duration, seropositivity, and smoking. Conclusion: Despite low disease activity, saliva TNF-α and caspase-1 levels were found to be significantly higher in the pSjS group, which may suggest a possible advantage of local anticytokine treatments in selected cases.

Segmental arterial mediolysis mimics systemic vasculitis.

Segmental arterial mediolysis (SAM) is a rare, nonarteriosclerotic, noninflammatory vascular disease and mostly affects medium-to-large sized abdominal arteries as well as presents with hemorrhages in the abdominal cavity. We report the case of a patient with SAM of the celiac, right renal, jejunal branch of the superior mesenteric, left gastric, and splenic arteries who was diagnosed by excluding other causes and in whom transcatheter embolization was performed in two different sessions, but he died because of an undefined reason. SAM mimics systemic vasculitis and causes abdominal pain; it should be considered because abdominal hemorrhage or arterial infarction can result in death.

Therapy resistant idiopathic scleredema: an underlying pathology not always present.

Scleredema is a rare connective tissue disorder of unknown pathogenesis. Three types of scleredema have been described, based on its association with postinfection, monoclonal gammopathy and diabetes mellitus. We report herein a case of scleredema which the diagnosis didn't get specified. The patient was followed regularly for 13 years and did not respond to various combinations of immunosuppressants and psoralen plus ultraviolet A therapy. Treatment of scleredema is quite difficult and of limited success. At present, there is no proved treatment for this disease.

Autoimmune thyroid disease in ankylosing spondylitis.

Although autoimmune thyroid disease is well known to be associated with primary Sjögren's syndrome (SjS) and with various autoimmune diseases, it is less clear whether a similar association also exists for ankylosing spondylitis (AS). Therefore, we investigated the frequency of autoimmune thyroid disease in patients with AS. In this cross sectional study, 80 patients with AS fulfilling the 1984 Modified New York Criteria and 80 healthy subjects, age and sex-matched with AS patients, were included. As the positive control group, 62 female patients with primary SjS were also studied. All cases underwent thyroid ultrasonography (USG) by a single endocrinologist. Thyroid function tests and thyroid autoantibodies were measured. The diagnosis of Hashimoto's thyroiditis (HT) was made if the patient had thyroid autoantibody positivity plus at least one of the following criteria: diffuse goiter with physical examination, abnormality in thyroid function tests, and parenchymal heterogeneity with USG. The chi-squared test and Fisher's exact test were used to compare cases and controls. The p values <0.05 were considered statistically significant. The frequencies of parenchymal heterogeneity with USG (30 vs 11.3 %, p = 0.045), thyroid autoantibody positivity (13.8 vs 2.5 %, p = 0.017), and concomitant diagnosis of HT (10 vs 1.3 %, p = 0.034) were significantly higher in AS group compared to healthy controls. Among AS patients having HT, subclinical hypothyroidism was detected only in a single patient. Frequency of autoimmune thyroid disease was significantly higher in AS group, compared to healthy controls. Prospective studies are needed to see the clinical relevance of these findings and outcome in the long term.

Cigarette smoking in primary Sjögren's syndrome: positive association only with ANA positivity.

Smoking is well known to contribute to the pathogenesis and severity of some systemic autoimmune rheumatic diseases and especially to the production of certain autoantibodies. Primary Sjögren's syndrome (pSS) is an autoimmune disease, affecting primarily the exocrine glands. It may also cause extraglandular involvement in some cases. In this study, we aimed to determine the frequency of smoking habits in our cohort of pSS patients and to investigate whether the frequencies of autoantibody positivity and extraglandular involvement were significantly different between patients with and without smoking. In this cross-sectional study, 207 patients with pSS (F/M 203/4), fulfilling the United States-European Consensus Criteria, and 602 healthy controls (F/M 534/68) were included. Patients and controls were classified into five groups: never smokers, current smokers, former smokers; ever smokers, and passive smokers. The χ(2) and Kruskal-Wallis tests were used for statistical analysis; a p value of less than 0.05 was accepted as statistically significant. While the frequency of current smokers was significantly lower in the pSS group compared with the healthy controls (11.6 vs 22.3%), the frequencies of former smokers (30.4 vs 11.8%), ever smokers (42.0 vs 34.1%), and passive smokers (47.3 vs 37.5%) were significantly higher in the pSS group compared with the healthy controls. In pSS patients, only antinuclear antibody (ANA) positivity was significantly associated with smoking habits, while there was no significant association with other autoantibodies or with the presence of extraglandular involvement. We found that in pSS patients smoking was significantly associated only with ANA positivity. Unlike the deleterious effects of smoking upon disease severity and anti-cyclic citrullinated protein (CCP) antibody production in rheumatoid arthritis, we could not find any association of smoking with extraglandular involvement and/or anti-Ro/anti-La antibody positivity in pSS. These results are indeed in line with the limited number of previous studies reported in the literature. Further studies with higher numbers of pSS patients are required to confirm the seemingly negative association of smoking with pSS.