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OBJECTIVE@#To analyze the differences of clinical manifestations and laboratory features between primary Sjögren's syndrome (pSS) patients with positive and negative anti-Sjögren's syndrome type B (SSB) antibody.@*METHODS@#The clinical data of pSS patients hospitalized in Department of Rheumato-logy and Immunology, Peking University Third Hospital were retrospectively analyzed to investigate the differences of clinical and laboratory features between anti-SSB positive and negative groups. The t test, Mann-Whitney U test, Chi-square test and Fisher's exact probability were used for analysis.@*RESULTS@#A total of 142 pSS patients were enrolled in this study, including 137 females and 5 males with a mean age of (54.8±13.3) years. The anti-SSB positive group included 44 patients accounting for 31.0% of the pSS patients. The anti-SSB positive pSS patients were younger at disease onset and at visit [age at visit: (50.9±14.5) years vs. (56.5±12.4) years; age at onset: (42.2±14.8) years vs. (49.5±15.3) years, P < 0.05]. The patients with anti-SSB positive more frequently presented with rash (29.5% vs. 14.3%, P < 0.05), enlargement of parotid glands (27.3% vs. 8.2%, P < 0.05), renal tubular acidosis (15.9% vs. 4.2%, P < 0.05), immune thrombocytopenia (9.1% vs. 1.0%, P < 0.05), rheumatoid factor (RF) positive (85.0% vs. 49.4%, P < 0.05), higher RF and antinuclear antibody (ANA) titers (median: 89.8 IU/mL vs. 20.5 IU/mL; median: 320 vs. 160, P < 0.05), anti-Sjögren's syndrome type A (SSA) antibody positive (97.7% vs. 64.3%, P < 0.05), elevation of γ globulin (71.4% vs. 38.5%, P < 0.05), higher levels of IgG (median: 21.0 g/L vs. 15.6 g/L, P < 0.05), higher proportions of CD3-CD19+ cells [(21.0±11.9)% vs. (13.7±9.6)%, P < 0.05] and lower proportions of CD3+ cells [(67.2±14.4)% vs. (76.6%±13.1)%, P < 0.05] than those negative. However, the anti-SSB positive group was less likely to show anti-mitochondrial antibodies (AMA)-M2 positivity (10.5% vs. 35.6%, P < 0.05). Glucocorticoids (90.9% vs. 73.5%, P < 0.05) and immunosuppressants (54.5% vs. 36.7%, P < 0.05) were more frequently used in anti-SSB positive pSS patients than those negative.@*CONCLUSION@#The anti-SSB positive pSS patients were younger at disease onset while more frequently presenting with various symptoms, higher levels of other antibodies and activation of B cells than those negative. Glucocorticoids and immunosuppressants were more frequently used, indicating that anti-SSB positive group presented with a more severe clinal phenotype.
Subject(s)
Adult , Aged , Female , Humans , Male , Middle Aged , Antibodies, Antinuclear , Immunosuppressive Agents , Retrospective Studies , Rheumatoid Factor , Sjogren's Syndrome/complicationsABSTRACT
Abstract Background Currently, only a few studies have described the general characteristics of patients with primary Sjögren's syndrome (pSS) who tested negatives for anti-SSA and anti-SSB antibodies. We aimed to further investigate the clinical characteristics of these patients in a large sample. Methods Data from patients with pSS who were treated at a tertiary hospital in China between 2013 and 2022 were retrospectively analyzed. Clinical characteristics of the patients were compared between those with and without anti-SSA and anti-SSB antibody negativity. Factors associated with anti-SSA and anti-SSB negativity were identified by logistic regression analysis. Results Overall, 934 patients with pSS were included in this study, among whom 299 (32.0%) tested negative for anti-SSA and anti-SSB antibodies. Compared with patients testing positive for anti-SSA or anti-SSB antibodies, that testing negative for the two antibodies had a lower proportion of females (75.3% vs. 90.6%, p < 0.001) and thrombocytopenia (6.7% vs. 13.6%, p = 0.002), but a higher proportion of abnormal Schirmer I tests (96.0% vs. 89.1%, p = 0.001) and interstitial lung disease (ILD) (59.2% vs. 28.8%, p = 0.001). Anti-SSA and anti-SSB negativity was positively associated with male sex (odds ratio [OR] = 1.86, 95% confidence interval [CI]: 1.05, 3.31), abnormal Schirmer I tests (OR = 2.85, 95% CI: 1.24, 6.53), and ILD (OR = 2.54, 95% CI: 1.67, 3.85). However, it was negatively related to thrombocytopenia (OR = 0.47, 95% CI: 0.24, 0.95). Conclusion Approximately one third of pSS patients had anti-SSA and anti-SSB negativity. pSS patients testing negative for anti-SSA and anti-SSB showed a higher risk of abnormal Schirmer I tests and ILD, but a lower risk of thrombocytopenia.
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Objective To explore the relationship between the clinical features,serological markers and European League Against Rheumatism SS Disease Activity Index (ESSDAI) scores of primary Sj(o)gren's syndrome (SS).Methods We enrolled 106 patients,who fulfilled the 2002 classification criteria for primary SS from December 2008 to January 2015,to evaluate the relationship among the clinical characteristics,laboratory features,serological variables and ESSDAI scores.According to serological variables,the prognosis was subdivided into three distinct groups:favourable (no serological markers),intermediate (one serological marker) and poor (two or more serological markers).These data were analyzed by Chi-square test and variance analysis.Results The mean ESSDAI score of 106 pSS patients was (11±7).ESSDAI score was categorized according to the EULAR-SS recommendations as low activity,moderate activity and high activity (scores of 0-4,5-13 and ≥14,respectively),and the positive rate of antinuclear antibody (ANA) 1:100 (6 cases,37.5％;37 cases,66.1％;32 cases,94.1％) in three different ESSDAI levels was statistically different (x2＝18.110,P＜0.01).Those with positive ANA 1:100[positive (13±7) and negative (7±4)],anti-SSA antibody postive (12±7) and negative (9±7),anti-RNP antibody (positive 16±9 and negative 10±6) had higher ESSDAI scores than those with negative ones (F=8.812,P=0.0001;F=3.862,P=0.024;F=5.786,P=0.004).No statistical difference in ESSDAI means were found between patients with positive anti-SSB antibody,rheumatoid factor (RF),FS level,dry mouth,Raynoud's phenomenon and psychosomatic diseases.The ESSDAI scores of favourable group,intermediate group and poor group were significantly different (8±5,10±7,14±7,F=8.715,P=0.000 1).In comparison with the other two groups,the poor pSS patients had a higher frequency of positive ANA 1:100 (15 cases,55.6％;20 cases,57.1％;40 cases,90.9％),anti-SSA antibody(11 cases,0.7％;23 cases,41.1％;36 cases,81.8％),anti-SSB antibody (6 cases,2 2.2％;13 cases,37.1％;23 cases,52.3％),anti-RNP antibody (0 case,0;2 cases,5.7％;9 cases,20.5％) (x2=17.408,P=0.002;x2=14.306,P=0.006;x2=12.330,P=0.015;x2=1 1.482,P=0.022).Conclusion Patients with two or more serological markers may have higher ESSDAI score,and which in turn may associate with poor prognosis.
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ObjectiveTo explore the pathogenesis, clinical manifestations, diagnosis, and treatment of autoanti-body-associated congenital heart block.MethodsThe clinical data of one child with autoantibody-associated congenital heart block was retrospectively analyzed.ResultsIn 24 week gestation, fetal bradycardia had been found by routine fetal echocar-diography. After birth, the anti-SSA/Ro antibodies and anti-SSB/La antibodies were positive in both infant and her mother. The diagnosis of autoantibody-associated congenital heart block was conifrmed. Intravenous immunoglobulin at 1 g/kg was adminis-trated. At 6 months follow-up, the electrocardiogram suggested type I second degree atrioventricular block.ConclusionIn the fetus or neonates found to have bradycardia and excluded the cardiac structural abnormalities, the autoimmune antibody should been tested and early intervention should been promoted.
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Objective To investigate the detection rate of anti-SSA60 and SSB antibodies in sera of patients with systemic lupus erythematosus (SLE).The correlation of anti-SSA and SSB antibodies with SLE clinical outcome was also investigated.Methods This study included 251 cases of SLE diagnosed in our hospital between 2007 and 2010.ELISA and double immunodiffusion method was used to detect the sera antiSSA60 and SSB antibodies.The patients were closely monitored for three years in terms of clinical and laboratory parameters and the presence of associated Sj(o)gren' s syndrome (SS).Statistical analysis were performed using student t test or x2 test.Results ① The detection rate of anti-SSA60 antibody in serum of patients with SLE was 65.3％.The detection rate of anti-SSB antibody in serum of patients with SLE was 28.3％; ② During the three-year follow up,patients with anti-SSA60 (29.3％,48 cases) or SSB antibodies (35.2％,25 cases) were more likely to have dry mouth and eyes and later developed SS (P＜0.05); ③ Patients with anti-SSA60 antibody were more likely to develop serositis (20.7％ vs 8.0％),neuropsychiatric lupus erythematosus (NPLE)(18.9％ vs 8.0％),and hematuria (35.4％ vs 21.8％)(P＜0.05).Patients with negative anti-SSB antibody were more likely to have fever (43.7％ vs 57.8％,x2=4.082,P＜0.05); ④ Patients positive for anti-SSB antibody were also positive for anti-Sm antibody (50.7％ vs 32.8％,x2=6.956,P＜0.05);⑤ Younger patients were more likely to have anti-SSA60 and SSB antibodies in their sera (P＜0.05); ⑥Patients positive for anti-SSA60 antibody had higher SLE disease activity index (SLEDAI) than patients with negative anti-SSA60 antibody [(17±9) vs (15±7),t=2.389,P＜0.05].Patients positive for anti-SSB antibody had higher level of IgG [(18±7) vs (16±6) g/L,t=2.304,P=0.023],and lower level of CRP than patients negative for anti-SSB antibody [(14±20) vs (21±33) mg/L,t=-2.173,P=0.031].Conclusion Patients positive for anti-SSA60 antibody have higher SLEDAI and more severe clinical outcomes.Patients with antiSSA or anti-SSB antibody are more likely to develop dry mouth and eyes which eventually leads to SS.
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Neonatal lupus erythematosus (NLE) is an autoimmune disease that is associated with transplacental passage of maternal autoantibodies that are reactive to SSA/Ro and SSB/La antigens. Cardiac involvement, hematologic abnormality and hepatic disease may occur in the infants suffering with NLE, in addition to the characteristic skin lesions. We report here on a case of NLE in a 4-week-old female infant who was born to an asymptomatic mother, and the baby displayed the characteristic clinical and histological features of cutaneous NLE with transient anemia and hepatitis. Both the infant and mother were positive for anti-SSA/Ro and anti-SSB/La. There have been 18 case reports of NLE in the Korean literature, including 7 case reports in the dermatological field. We describe herein another case of NLE that showed transient anemia and hepatitis, and we also review the case reports of NLE in the Korean literature.
Subject(s)
Female , Humans , Infant , Anemia , Autoantibodies , Autoimmune Diseases , Hepatitis , Lupus Erythematosus, Systemic , Mothers , Skin , Stress, PsychologicalABSTRACT
Objective To establish a new,rapid,simple and reliable assay for detecting autoantibody SSB.Methods A new dot immunogold filtration assay(DIGFA) was developed,in which the recombinant SSB protein expressed in Pichia pastoris was bound to nitrocellulose(NC) membrane and colloidal gold-labeled staphylococus protein A(SPA) was used as an indicator.Results The sensitivity and specificity of DIGFA were 100% and 98.75%,respectively.The agreement between DIGFA and ENA dot assay was 99.01%.Conclusion DIGFA for detecting autoantibody SSB is a good,rapid,simple and accurate assay for clinical diagnosis.
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PURPOSE: Neonatal lupus is characterized by congenital complete heart block(CCHB), cutaneous rash, and laboratory abnormalities in infants born to mothers with systemic lupus erythematosus(SLE). This study aims to examine the incidence of CCHB and clinical outcome in neonates born to mothers with SLE. METHODS: The study group consisted of 49 neonates, born from 57 pregnancies of 55 women with SLE, diagnosed at Hanyang University Hospital for the period between January 1997 and January 2005. Clinical and laboratory data were retrospectively identified from medical record. RESULTS: There were 5(8.8 percent) spontaneous abortions and one(1.8 percent) still births among 57 pregnancies of 55 mothers. Of 49 live births, 15(26.3 percent) were premature and eight(12.3 percent) were small for their gestational age. There was one(1.8 percent) CCHB suspected during pregnancy on fetal echocardiograpy in a fetus of mother with systemic lupus erythematosus and the fetus was not born by artificial abortion because of mother. There was no CCHB among EKG findings of 49 newborns. Laboratory testing showed hematologic abnormalities among 25.6 percent(10/39) of the babies. 5.1 percent(2/39) and 7.7 percent(3/39) of them were diagnosed as neutropenia, and thrombocytopenia was seen respectively. Anti-SSA(Ro) and antiphospholipid antibodies were predictive factors for prematurity(P=0.003, P=0.049). Anticardiolipin antibodies were predictive factors for ventilatory care(P=0.018). CONCLUSION: The incidence of CCHB among neonates born to mothers with SLE, which was measured in this study, was lower than that in earlier studies. A high incidence of hematologic abnormalities was found in our study. It is suggested that careful examination should be made of skin for the diagnosis of neonatal lupus.
Subject(s)
Female , Humans , Infant , Infant, Newborn , Pregnancy , Abortion, Spontaneous , Antibodies, Anticardiolipin , Antibodies, Antiphospholipid , Diagnosis , Electrocardiography , Exanthema , Fetus , Gestational Age , Heart , Incidence , Live Birth , Lupus Erythematosus, Systemic , Medical Records , Mothers , Neutropenia , Parturition , Retrospective Studies , Skin , ThrombocytopeniaABSTRACT
OBJECTIVE: To determine whether GSTM1, GSTT1 and GSTP1 polymorphisms are associated with susceptibility or disease manifestations in patients with SLE. METHODS: Two hundred eighty-six SLE patients who fulfilled the American College of Rheumatology (ACR) criteria were compared with 271 cases of age and sex matched controls to examine association between GST genotypes and susceptibility to SLE. The effect of genotype on SLE manifestations was assessed using the comparison of ACR diagnostic criteria. GST gene polymorphisms were determined by a multiplex polymerase chain reaction and antibodies to SS-A and SS-B were determined by double immunodiffusion. RESULTS: No association was found in the comparison of GSTM1 null, GSTT1 null, GSTP1 Ile105--Subject(s)
Humans
, Antibodies
, Exanthema
, Genotype
, Glutathione Transferase
, Glutathione
, Heterozygote
, Immunodiffusion
, Lupus Erythematosus, Systemic
, Multiplex Polymerase Chain Reaction
, Nephritis
, Psychotic Disorders
, Rheumatology