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Aim: To describe the clinical and laboratory features of Sjögren's syndrome (SS) with renal tubular acidosis (RTA) from published literature. Methods: A systematic search of indexed publications in all languages till December 2021 identified cases of SS with RTA (SS‐RTA) and were included if either antibody (anti‐SSA/anti‐SSB) or salivary gland histopathology were positive. Results: There were 440 cases of SS‐RTA (63.9% from Asia, 95.5% women). The median (range) age was 37 (6‐78) years. Only 7.7% had a previous diagnosis of SS. Oral or ocular sicca symptoms were present in 63.7%. Positive ocular tests, oral tests, anti‐SSA, anti‐SSB and salivary gland histopathology were reported in 256/331 (77.3%), 123/128 (96%), 382/407 (93.9%), 298/379 (78.6%), and 246/268 (91.8%), respectively. Hypokalemic paralysis (HP) was the presenting feature in 63.6%; 25% had multiple episodes of HP and 8.4% had respiratory paralysis. Type 1, type 2, combined type 1 & 2, and type 4 RTA was seen in 388, 8, 38, and 3 patients, respectively. Proximal dysfunction and RTA complications were infrequently evaluated. Fanconi syndrome, nephrogenic diabetes insipidus, proteinuria, and low estimated glomerular filtration rate were found in 45, 21, 178, and 157, respectively. Nephrocalcinosis, renal stones, and osteomalacia were reported in 92, 79, and 72, respectively. Tubulointerstitial nephritis was found in 142 out of 152 renal biopsies. Conclusion: SS‐RTA is an early manifestation of SS characterized by younger age and subclinical sicca symptoms. Although evaluated less frequently, oral sicca signs and salivary gland biopsy have a high positive yield. HP is the most common presentation. RTA is mostly distal; proximal dysfunction and complications were infrequently assessed. [ABSTRACT FROM AUTHOR]

Background: B cells contribute significantly to the pathogenesis of primary Sjögren's syndrome (pSS). Free light chains (FLCs) are generated during the production of immunoglobulins (Igs) and are surrogates of B cell activity. We hypothesized that salivary FLCs and salivary Igs could represent salivary gland inflammation and therefore, serve as biomarkers in pSS. Methods: Patients >18 years old fulfilling the American College of Rheumatology / European League Against Rheumatism (EULAR) 2016 criteria for pSS and age‐matched healthy and disease controls (sicca non‐pSS, rheumatoid arthritis, systemic lupus erythematosus) were recruited for this cross‐sectional study. FLCs in saliva and serum were measured by immunoturbidimetry. Serum and salivary Igs were measured by nephelometry and enzyme‐linked immunosorbent assay, respectively. Area under the receiver operator characteristic curve was determined. The factors influencing the serum and salivary FLCs in pSS were determined using backward linear regression. Results: A total of 78 patients with pSS, 76 healthy controls and 62 disease controls were recruited. Median EULAR SS disease activity index (interquartile range) was 1 (3.75). Serum FLCκ and FLCλ, salivary FLCλ, serum and salivary IgG, salivary IgM was significantly higher in the pSS group compared to the controls. Areas under the curve for salivary FLCλ, serum FLCκ, serum and salivary IgG were 0.75, 0.72, 0.78 and 0.77, respectively. Regression analysis showed that salivary FLCκ, salivary FLCλ and salivary IgG were associated with positive salivary gland histopathology. Use of immunosuppressants and glucocorticoids was associated with lower values of salivary parameters. Conclusion: Salivary FLCλ and salivary IgG were significantly different between pSS and control groups and could be potential non‐invasive biomarkers in pSS. These findings should be confirmed in larger longitudinal studies. [ABSTRACT FROM AUTHOR]

Objective To estimate levels of salivary and serum free light chains ( FLCs) and explore its utility as a biomarker in primary Sjögren's syndrome ( pSS). Methods Patients with pSS classified by American European Consensus group 2002 or American College of Rheumatology 2012 criteria between January 2015 and August 2015 were included. Healthy staff and non-first degree relatives of patients constituted controls. Serum and salivary FLCs were measured by immunoturbidometry using FREELITE™ Human Kappa(κ) and Lambda(λ) Free Kit (Binding site, Birmingham, UK), on a Roche Modular P800. FLCs were compared between cases and controls using the Mann-Whitney U-test. The receiver operator characteristic curve was constructed to analyze the discriminating ability of salivary and serum kappa and lambda FLCs. Results Salivary and serum FLCs were assayed in 15 patients and 13 patients, respectively, and in 15 controls. Median age of cases and controls was 34 years. Salivary kappa and lambda FLCs were higher in pSS as compared to controls ( P < 0.05 and P < 0.001, respectively). Serum kappa and lambda FLCs were also higher in pSS (both P < 0.05). Salivary lambda levels were higher in pSS with ocular signs; serum kappa and lambda levels were higher in those with ocular symptoms. A cut off of ≥ 1.1 mg/L for salivary lambda FLC had a sensitivity and specificity of 73.3% and 93.3%, respectively, for the diagnosis of pSS. Serum kappa FLC ≥ 30 mg/L had a sensitivity and specificity of 92.3% and 73.3%, respectively. Conclusion Serum and salivary FLCs and in particular the latter, are potential biomarkers in pSS. Larger studies are required for validating the findings. [ABSTRACT FROM AUTHOR]

Objective This study is aimed to determine the reliability of the Box and Block (B&B) Test for manual dexterity of upper extremity function in patients with rheumatoid arthritis ( RA) and to compare the results with age- and sex-matched healthy controls, and also with available normative data. Methods The reliability of B&B Test was assessed within and between testers using the intraclass correlation coefficient ( ICC) in patients with RA attending rheumatology clinics of Christian Medical College Hospital, India. The dexterity scores of patients were then compared with age- and sex-matched controls and the Mathiowetz's population-based normative data by Student's independent t-test. Results The interrater and intrarater reliability of the B&B Test in patients with RA ( n = 60) ranged from 0.92 to 0.97 and 0.91 to 0.95, respectively. The dexterity scores in patients with RA were lower as compared to the control group (dominant hand 54.87 vs. 68.18, P < 0.001; contralateral hand 52.65 vs. 65.6, P < 0.001) and population-based normative score (dominant hand 54.87 vs. 80.02, P < 0.001; contralateral hand 52.65 vs. 77.23; P < 0.001). The control group scores were also lower than the normative data. Higher age of patient, longer disease duration and higher disease activity reflected by Disease Activity Score of 28 joints ( DAS-28) also correlated well with lower dexterity score. Conclusions The B&B Test is a reliable tool for assessing upper extremity function in patients with RA and the dexterity scores are lower for RA patients. The scores had correlation with age, disease duration and disease activity. [ABSTRACT FROM AUTHOR]