The prevalence of Sjögren's syndrome and sicca symptoms in patients with systemic sclerosis and alpha-smooth muscle actin expression in biopsy specimens from minor salivary glands

Background/aim: This study aimed to investigate the prevalence of sicca symptoms and secondary Sjögren's syndrome (SjS) in patients with systemic sclerosis (SSc). Also this study aimed to evaluate the expression of α-smooth muscle actin (α­SMA) in minor salivary gland (MSG) specimens, a possible marker of fibrosis responsible for myofibroblastic transformation. Materials and methods: Patients with SSc who were followed in Rheumatology outpatient clinic at a university hospital evaluated. The questionnaire of sicca symptoms and classification of SjS were evaluated according to the American­European Consensus Group (AECG) criteria. Histopathologic evaluations were done in MSG specimens investigating the presence of focal lymphocytic sialadenitis and glandular fibrosis, also assessing the expression of α­SMA. Results: This cross-sectional study included 102 patients with SSc [91 females (89%), mean age 52.5 ± 12 years]. In this cohort 76 (75%) patients had sicca symptoms and 36 (35.3%) patients fulfilled the AECG criteria for SjS; all with limited form. Having SjS found to be associated with older age and the presence of positive anti-SS-A antibodies. On histopathologic examinations, glandular fibrosis was observed in 67 (80%) and lymphocytic sialadenitis was detected in 38 (45%) patients; but only 7 samples were positive for α­SMA. Conclusion: This study suggested sicca symptoms were found to be very common among patients with SSc. Also secondary SjS was detected in nearly one-third of patients with SSc; especially in limited subtype. Anti SS-A positivity and older age were detected as predictors for SjS. Histopathologic evaluations showed significant glandular fibrosis but rare α-SMA staining in patients with SSc.

Performance of different criteria sets for inflammatory back pain in patients with axial spondyloarthritis with and without radiographic sacroiliitis.

It is important to recognize inflammatory back pain (IBP) for an early diagnosis of ankylosing spondylitis (AS). Assessment of Spondyloarthritis International Society (ASAS) has recently published new criteria set for diagnosing IBP. In the present study, we evaluated the performance of the new ASAS IBP criteria and to compare the performance of IBP criteria sets in axial spondyloarthritis (axSpA) patients with and without radiographic sacroiliitis. The study sample included a total of 274 patients with a diagnosis of axSpA and 50 patients with a diagnosis of chronic (>3 months) mechanical back pain (MBP). A face-to-face interview by using a standardized questionnaire addressing all the components of IBP was performed. Data about HLA-B27 status and C-reactive protein levels were obtained from the patients' charts. There were significantly more male patients (P < 0.001) in the AS group (68.6 %) than in the non-radiographic axSpA group (29.6 %) and also than in the MBP group (37.5 %). Among the criteria sets, the Calin criteria showed the best sensitivity (91.2 %), and the Berlin criteria showed the best specificity (82.4 %) in differentiation of IBP from MBP. If the morning stiffness item of the Calin criteria was defined as lasting >30 min (Calin 30), the specificity improved (72.9 %), but at a price of loss in sensitivity (82.4 %). In this study, new ASAS criteria for IBP performed almost as good as but not better than the existing criteria sets. Performances of the criteria sets were quite comparable in the differentiation of IBP from MBP in patients with and without radiographic sacroiliitis.

High prevalence of spondyloarthritis and ankylosing spondylitis among familial Mediterranean fever patients and their first-degree relatives: further evidence for the connection.

INTRODUCTION: Familial Mediterranean fever (FMF) is an auto-inflammatory disease characterized by recurrent attacks of fever and serositis. Limited data suggest that the prevalence of sacroiliitis is increased in patients with FMF. In our present study, we assessed the prevalence of spondyloarthritis (SpA), including ankylosing spondylitis (AS), among a cohort of FMF patients and their unaffected first-degree relatives (FDRs). METHODS: The current study cohort comprised a consecutive group of 201 unrelated patients with FMF and 319 FDRs (≥16 years old). These subjects were examined according to a standard protocol. RESULTS: A total of 157 FMF patients (78.1%) and 233 (73%) unaffected FDRs reported back pain. Fifteen FMF patients (7.5%) and nine unaffected FDRs fulfilled the modified New York (mNY) criteria for AS. One additional FDR with AS was identified after review of the medical records. None of the FMF patients with AS was HLA-B27 positive. The allele frequency of M694V among the FMF patients with radiographic sacroiliitis was significantly higher in comparison with those without sacroiliitis (OR 4.3). When compared with the general population, the risk ratios for SpA and AS among the FDRs of our FMF patients were 3.3 (95% CI; 2.0 to 5.5) and for AS 2.9 (95% CI; 1.3 to 6.4), respectively. CONCLUSIONS: Our study suggests that a) factors other than HLA-B27 play a role in the association of FMF and SpA/AS; b) MEFV gene variations may be one of the geographic/region-specific potential pathogenetic links between these two disorders in the Turkish population.

High frequency of inflammatory back pain and other features of spondyloarthritis in patients with rheumatoid arthritis.

The aim of this study was to investigate the frequency of patients with rheumatoid arthritis (RA) who have inflammatory back pain (IBP) and meet the existing classification criteria for ankylosing spondylitis (AS) and spondyloarthritis (SpA). We included 167 patients fulfilling the ACR 1987 revised criteria for RA. After obtaining a medical history and performing a physical examination, standard pelvic X-rays for examination of the sacroiliac joints (SIJ) were ordered in all patients. A computed tomography (CT) or magnetic resonance imaging (MRI) of SIJ was performed in patients with suspected radiographic sacroiliitis and MRI of SIJ in those who have IBP but no radiographic sacroiliitis. IBP was defined according to both Calin and experts' criteria. The modified New York (mNY) criteria were used to classify AS, both ESSG and Amor criteria for SpA and ASAS classification criteria for axial SpA. There were 135 female and 32 male patients with a mean age of 54.8 years. The mean disease duration was 9.8 years. RF was positive in 128 patients (79.2 %) and anti-CCP in 120 patients (81.1 %). Twenty-eight patients with RA (16.8 %) had IBP (Calin criteria), and four (2.4 %) had radiographic sacroiliitis of bilateral grade 3. Three patients (1.8 %) fulfilled the mNY criteria for AS, 31 (18.6 %) ESSG and 26 (15.6 %) Amor criteria for SpA. Nine patients (five with MRI sacroiliitis) (5.3 %) were classified as having axial SpA according to new ASAS classification criteria. This study suggests that the prevalence of SpA features in patients with RA may be much higher than expected.

Familial Mediterranean fever: risk factors, causes of death, and prognosis in the colchicine era.

We assessed the risk factors and causes of death in patients with familial Mediterranean fever (FMF) in an era when colchicine is the standard therapy for all patients.This study included all FMF patients who had presented to any of the internal medicine, rheumatology, or nephrology clinics at Dokuz Eylul University Hospital between 1992 and 2009. Of the 650 patients with FMF identified, 587 (90.3%) had either a face-to-face (n = 380) or telephone (n = 193) interview, or were confirmed as deceased. A structured questionnaire was used to obtain socioeconomic and demographic data, presenting and cumulative clinical features, and disease severity scores.During the follow-up period mortality was analyzed by calculating age- and sex-standardized mortality ratio (SMR) according to the mortality statistics of the Turkish population. Factors predictive of mortality were evaluated using Kaplan-Meier and Cox proportional hazard models. Sixty-three (9.7%) patients whose initial demographic and major clinical characteristics were similar to the rest of the group could not be contacted during the study period.Most (94.2%) patients were on colchicine at the time of the study. Thirty-seven (6.3%) patients had biopsy-verified amyloidosis, and 44 (7.5%) had renal disease. During a median follow-up of 6 years, 14 patients (9 women) died, and amyloidosis and its related complications were the leading causes of death in 7 patients. Univariate analysis revealed that increasing age, coronary heart disease, hypertension, renal disease, and amyloidosis were associated with mortality. However, Cox regression analysis showed amyloidosis as the only significant predictor of mortality (p < 0.001). The overall patient survival rate was not significantly different from the age- and sex-matched Turkish general population (SMR, 1.48; 95% confidence interval, 0.817-2.49).Our findings suggest that although the survival of FMF patients in the colchicine era is comparable to that of the general population, renal involvement still predicts mortality.

Down-regulation of adiponectin in patients with familial Mediterranean fever during attack-free period.

To evaluate the circulating levels of adipokines (leptin and adiponectin) and ghrelin in patients with familial Mediterranean fever (FMF) and also to assess the relationships between these molecules and disease-related parameters. Forty-eight FMF patients in attack-free period (31 men, [M], 17 women, [F], mean age 35.8 ± 8.6 years, and a mean body mass index [BMI] of 24.7 ± 3.1) and 40 age-, sex-, and BMI-matched healthy controls (24 M, 16 F, mean age 35.5 ± 8.5 years, and a mean BMI of 24.5 ± 2.8) were included in the study. Patients and controls with a history of any other chronic diseases and obese or underweight subjects were excluded. High-sensitive C-reactive protein (hs-CRP), leptin, adiponectin, and total ghrelin concentrations were studied. Age, sex, BMI, waist circumference, and smoking status were similar between FMF patients and controls (P > 0.05). Adipose tissue-derived molecules including leptin, and adiponectin were lower than healthy controls but only adiponectin levels reached the statistically significance (16.7 ± 8.9 ng/ml vs. 27.7 ± 15.9 ng/ml, P < 0.001) and leptin concentrations just missed significance (25.2 ± 16.2 ng/ml vs. 34.9 ± 27.2 ng/ml, P = 0.051). Ghrelin concentrations were not different between the groups. Adiponectin levels were significantly and negatively correlated with hs-CRP (P < 0.05, r = -0.24). The results of this study suggest that low-grade chronic inflammation during attack-free period in FMF patients may suppress adiponectin production or low levels of adiponectin might contribute to subclinical inflammation in these patients.