OBJECTIVE: To analyze the clinical features and prognosis in patients with primary Sjögren's syndrome (pSS) and autoimmune liver diseases (ALD). METHODS: A retrospective analysis of clinical manifestation and prognosis was performed in patients with ALD or without ALD during the three years (February 2014 to December 2017). RESULTS: Totally, 203 patients with pSS were included in this study, 68 patients had ALD (31 patients with autoimmune hepatitis, 37 patients with primary biliary cholangitis), while 135 patients did not have ALD. There were no differences between the two groups regarding age, gender, clinical manifestations, such as dry mouth, dry eyes, pain, fatigue, lymphadenopathy, glandular swelling, cutaneous involvement, lung involvement, and renal involvement, and the incidence rate of other autoimmune diseases, such as autoimmune thyroid disease, rheumatoid arthritis, and vasculitis. There were also no differences in the titer of antinuclear antibody (ANA), the positive rates of anti-Sjögren's syndrome A antibody (SSA), SSA52, and anti-Sjögren's syndrome B antibody (SSB), and at the levels of erythrocyte sedimentation rate and C-reactive protein between the two groups. Most importantly, the pSS patients with ALD had a shorter disease course, a higher positive rate of anti-mitochondrial M2 antibody (AMA-M2) and anti-centromere antibody, a higher level of IgG and IgM, a lower level of complement 3, and a decreased number of blood cells. They also had a higher level of liver related serum index, such as alanine aminotransferase, aspartate aminotransferase, gamma-glutamyl transferase, alkaline phosphatase and total bilirubin, direct bilirubin, indirect bilirubin, a higher incidence rate of liver cirrhosis, an increased death incident (the mortality was 13.24% in the pSS patients with ALD, while 2.96% in the controls, P=0.013), and a worse prognosis. Binary Logistic regression analysis revealed that liver cirrhosis, the EULAR Sjögren's syndrome disease activity index (ESSDAI) scores and the level of total bilirubin were the prognostic factors of mortality in the pSS patients with ALD. The survival curve was estimated by the Kaplan-Meier method. It demonstrated that the pSS patients with ALD had a lower survival rate when compared with the controls. CONCLUSION The patients with both pSS and ALD will suffer from a more severe disease and a higher death incident. We should pay more attention to these patients and provide a better symptomatic treatment for them during clinical practice.
Hepatitis, Autoimmune/epidemiology* ; Humans ; Liver Cirrhosis, Biliary ; Prognosis ; Retrospective Studies ; Sjogren's Syndrome/epidemiology*

OBJECTIVE: To investigate the prevalence of sleep disorders and the relevant determinants in a cohort of primary Sjögren' s syndrome (pSS) patients. METHODS: One hundred and eighty-six pSS patients were included in the study, who were admitted to Peking University People' s Hospital and met the criteria of inclusion and exclusion. Sleep quality was assessed using the Pittsburgh sleep quality index(PSQI).Depression, anxiety were evaluated by patient health questionnaire (PHQ)-9, generalized anxiety disorder(GAD)-7, respectively. The demographic and clinical data were also recorded.Disease activity and damage were evaluated with the European League Against Rheumatism Sjögren's syndrome disease activity index (ESSDAI). According to the PSQI score>7, the pSS patients were divided into 152 cases of sleep disorder group and 34 cases of normal sleep group. Mann-Whitney U test, Chi-square test or Fisher' s exact test, independent samples t test, Spearman correlation analysis and Logistic regression were used for statistical analysis. RESULTS: The prevalence of sleep disturbance (PSQI > 7) was 81.7% (152 / 186) in the pSS patients, and 52.7% (98/186) had moderate or severe sleep disorders (PSQI≥ 11). The mean PSQI score of sleep disordered group was (12.29±3.30), while the normal sleep group PSQI score was (5.50±1.20). The PSQI score, PHQ-9 score and GAD-7 score in the sleep-disordered group were significantly higher than those in the normal sleep group (P=0.000, 0.035, 0.031). The PSQI score in the sleep disordered group were significantly higher than those in the normal sleep group in seven aspects: subjective sleep quality, sleep latency, sleep duration, habitual sleep efficiency, sleep disorders, hypnotic drug use and daytime dysfunction. All of them had statistical significance. According to the results of Spearman correlation analysis, PSQI had significantly positive correlation with course of disease, anxiety, depression score (r=0.151, 0.240, 0.421, P < 0.05), but negatively correlated with C3, C4 (r=-0.021, -0.235, P < 0.05). Logistic analysis identified the course of disease(OR=2.809, 95%CI: 1.21-6.52)and PHQ-9 score(OR=1.422, 95%CI: 1.04-1.94)as predictors of sleep disorders. CONCLUSION The incidence of sleep disorder in the pSS patients was higher, which was closely related to the course of disease, anxiety, depression and other factors. It is critical to assess and manage comprehensively the disease.
Anxiety/etiology* ; Cohort Studies ; Humans ; Sjogren's Syndrome/epidemiology* ; Sleep ; Sleep Wake Disorders/epidemiology*

We investigate the clinical characteristics of Sjogren's syndrome (SS). METHODS: We. retrospectively reviewed 59 patients with SS in the period from January 19: To 86 to January 1997. European Epidemiology Center criteria was used to diagnose SS. OBJECTIVES AND RESULTS: Thirty-one primary SS and 28 secondary SS patients were included in this study. There were 57 females and 2 males, the mean age was 45. 2+12.3 years (24 to 70 years). Patients with secondary SS were associated with rheumatoid arthritis(n=10), systemic lupus erythematosus(n=7), scleroderma(n=5), mixed connective tissue disease(n=3), and myositis(n=3). The common presenting symptoms were dry mouth in 92% and dry eyes in 90% of patients. Other symptom and signs included fatigue(27%), lymphadenopathy(22%) and parotid gland swelling (10%). The commonly presented autoantibodies were antinuclear antibody(83%) and rheumatoid factor(57%). Anti-Ro was positive in 49%(25/51) and anti-La in 37%(19/51). Anti-La positivity was significantly higher in primary SS(52%) compared to secondary SS(21%) (p<0.05). Schirmer test was positive in 98% (44/45), salivary radioscintigraphy in 74%(25/34), and lower lip biopsy in 47%(16/34). Extraglandular manifestations, such as Raynaud's phenomenon, vasculitis, glomerulonephritis, vaginal dryness, renal tubular acidosis, and primary biliary cirrhosis, were more frequently presented in primary SS compared to secondary SS (p<0. 05). CONCLUSION: The sex, age, and clinical manifestation of primary SS were similar with secondary SS, but extraglandular manifestations and anti-La antibody positivity were more frequent in primary SS.
Acidosis, Renal Tubular ; Autoantibodies ; Biopsy ; Connective Tissue ; Diagnostic Tests, Routine ; Epidemiology ; Female ; Glomerulonephritis ; Humans ; Lip ; Liver Cirrhosis, Biliary ; Male ; Mouth ; Parotid Gland ; Retrospective Studies ; Sjogren's Syndrome* ; Vasculitis

OBJECTIVETo investigate the risk factors of community-acquired pneumonia (CAP) in patients with primary Sjogren's syndrome (pSS).

METHODSThe clinical data were collected from 121 inpatients with pSS and univariate analysis and logistic regression were conducted to analyze the risk factors of CAP.

RESULTSThe incidence of CAP in the 121 patients with pSS was 27.3%. Age, disease course, low while blood cells, low complement levels, liver and kidney dysfunction, low albumin, hyperglobulinaemia, renal tubule acidosis, interstitial lung disease (ILD) and immunosuppressive agents were closely related to CAP in these patients. Logistic regression analysis identified ILD, low complement levels and hyperglobulinemia as the risk factors for CAP in patients with pSS.

CONCLUSIONVigorous control of pSS and minimizing the risk factors may prove effective to lower the incidence of CAP in patients with pSS.

Adolescent ; Adult ; Aged ; China ; epidemiology ; Community-Acquired Infections ; epidemiology ; etiology ; Female ; Humans ; Incidence ; Logistic Models ; Male ; Middle Aged ; Pneumonia ; epidemiology ; etiology ; Retrospective Studies ; Risk Factors ; Sjogren's Syndrome ; complications ; Young Adult

OBJECTIVETo evaluate the sensitivity and specificity of international classification criteria (2002) for primary Sjögren's syndrome (pSS) and the role of lower lip biopsy in diagnosis of pSS in Chinese patients.

METHODSPatients who were diagnosed by the experts/rheumatologists as pSS during 1990-2002 from the Department of Rheumatology, Peking Union Medical College Hospital were retrospectively collected as experimental group. Patients who were diagnosed as non-pSS connective tissue diseases or non-connective tissue diseases served as control group. Those with a history of head-neck radiation, hepatitis C virus infection, AIDS, lymphoma, sarcoidosis, graft versus host disease (GVHD), and anti-acetylcholine drug use were exempted. Both groups were required to complete questionnaires about symptoms such as dry eyes and dry mouth, and complete the objective tests of keratoconjunctivitis and xerostomia including Schirmer test, corneal staining, unstimulated salivary flow, sialography, lower lip biopsy, and antinuclear antibodies (including anti-SSA/SSB antibodies) test.

RESULTSA total of 330 pSS patients were included in experimental group and 185 non-pSS patients in control group. The mean age of both groups matched (47.8 +/- 10.9 years vs. 46.2 +/- 13.6 years, P > 0.05). The sensitivities of the criteria in pSS patients with lower lip biopsy and in pSS patients without lower lip biopsy were 89.2% and 87.2%, respectively; the overall sensitivity was 88.5%. The specificity was 97.3%. A total of 11.3% pSS patients with negative anti-SSA/SSB anti- bodies were diagnosed as pSS by lower lip biopsy.

CONCLUSIONThe international classification criteria (2002) for pSS is feasible in Chinese patients. It has high sensitivity and specificity, and may serve as diagnosis criteria in routine clinical practice.

Adolescent ; Adult ; Aged ; Biopsy ; Child ; China ; epidemiology ; Evaluation Studies as Topic ; Female ; Humans ; Lip ; pathology ; Male ; Middle Aged ; Practice Guidelines as Topic ; standards ; Sensitivity and Specificity ; Sjogren's Syndrome ; classification ; diagnosis ; epidemiology ; Surveys and Questionnaires

Objective: To investigate the relationship between positive anti-Ro/Sjögren syndrome antigen type A (SSA) antibody and anti-La/Sjögren syndrome antigen type B (SSB) antibody in pregnant women and neonatal adverse outcomes. Methods: This study was a retrospective study, and 145 deliveries of 136 anti-Ro/SSA and anti-La/SSB antibody positive pregnant women were selected who had prenatal examination and delivered in Peking University First Hospital from January 2017 to June 2022. According to whether adverse neonatal outcomes occurred, 145 deliveries were divided into adverse outcome group (26 cases) and no adverse outcome group (119 cases). According to the time when anti-Ro/SSA and anti-La/SSB antibodies were found positive, 145 deliveries were divided into the antibody positive during pregnancy group (69 cases) and the pre-pregnancy antibody positive group (76 cases). The pregnancy outcomes, treatment and maternal and infant antibody levels of pregnant women between the adverse outcome group and no adverse outcome group, between antibody positive during pregnancy group and the pre-pregnancy antibody positive group were compared. Results: (1) Most of the pregnant women with positive anti-Ro/SSA and anti-La/SSB antibodies were diagnosed as undifferentiated connective tissue disease, accounting for 40.4% (55/136), followed by Sjogren's syndrome (25.0%, 34/136), systemic lupus erythematosus (23.5%, 32/136), antiphospholipid antibody syndrome (6.6%, 9/136), idiopathic thrombocytopenic purpura (1.5%, 2/136), and 4 cases were not diagnosed. (2) The titers of anti-Ro/SSA and anti-La/SSB antibodies in the first trimester and the second trimester were compared, and there were no statistical significances (all P>0.05). (3) The proportion of high level anti-Ro/SSA antibody (>100 kU/L), positive level of anti-La/SSB antibody and positive rate of anti-La/SSB antibody in the adverse outcome group were higher than those in the no adverse outcome group, and the birth weight of newborns and live birth rate in the adverse outcome group were lower than that in the no adverse outcome group, all with statistical significances (all P<0.05). The anti-Ro/SSA antibody level, the proportion of drug treatment (hydroxychloroquine, glucocorticoid, gamma globulin), the incidence of fetal growth restriction (FGR), the rate of preterm birth, and the positive level of anti-Ro/SSA and anti-La/SSB antibodies in newborns were compared between the two groups, and there were no statistically significant differences (all P>0.05). (4) The anti-Ro/SSA antibody level of pregnant women in the pre-pregnancy antibody positive group, the proportion of hydroxychloroquine and glucocorticoid treatment, and the anti-Ro/SSA antibody positive rate of newborns were higher, while the incidence of FGR and gamma globulin treatment rate of newborns in the antibody positive during pregnancy group were higher, respectively, and the differences were statistically significant (all P<0.05). The levels of anti-La/SSB antibodies in pregnant women, anti-Ro/SSA and anti-La/SSB antibodies in newborns, the positive rate of anti-La/SSB antibodies in newborns and the incidence of adverse outcomes were compared between the antibody positive during pregnancy group and the pre-pregnancy antibody positive group, and there were no statistical significances (all P>0.05). Conclusions: High concentrations of anti-Ro/SSA antibodies and co-positive anti-La/SSB antibodies during pregnancy may increase the incidence of adverse neonatal outcomes. There is no significant difference in the incidence of adverse neonatal outcomes between antibody positive pregnant women and antibody positive pregnant women who were first found during pregnancy after comprehensive treatment in the rheumatology and immunology department.
Infant, Newborn ; Humans ; Female ; Pregnancy ; Sjogren's Syndrome/drug therapy* ; Pregnant Women ; Hydroxychloroquine/therapeutic use* ; Retrospective Studies ; Glucocorticoids ; Premature Birth/epidemiology* ; Lupus Erythematosus, Systemic/drug therapy* ; Pregnancy Outcome ; gamma-Globulins