anti-SSB Antibodies Research Articles

Lupus néonatal et transmission materno-fœtale des anticorps anti-SSA/Ro et anti-SSB/La

Neonatal lupus (NL) is a rare syndrome caused by placental transfer of maternal anti-SSA/Ro (60 and 52kDa) or anti-SSB/La antibodies. The aim of this study was to evaluate the clinical and biological profile of NL at the neonatal unit of Sfax, Tunisia, over a 10-year period. Six mother-NB pairs (two sets of twins and two sisters) had positive ANA by transplacental transmission during the study period. The ANA pattern was speckled and the NBs' sera titer was half that of their mothers'. Anti-SSA, anti-Ro52, and anti-SSB were found in 100%, 33%, and 50% of the mothers' sera, respectively. The transmission of anti-SSA was observed in four pregnancies out of six, anti-Ro52 in two pregnancies out of two, and anti-SSB in one pregnancy out of three. The patients' clinical records showed that two NBs had a congenital heart block: one with anti-SSA, whose mother had Sjögren syndrome, and another with anti-SSA, anti-SSB, anti-Ro52, and anti-mitochondrial antibodies (M2 type), whose mother had no diagnosis at the child's birth (cutaneous erythema and positive ANA with the same profile). Cutaneous signs (erythema, petechia) were described in three NBs out of six. The two sets of fraternal twins had cutaneous signs with the same ANA titer and profile (no anti-SSA transmission from their mother with lupus and anti-phospholipid syndrome). The two sisters' (two pregnancies 3 years apart) mother had Sjögren syndrome, one of them had heart block with positive anti-SSA, and the other was asymptomatic with anti-SSA and anti-Ro52. The same mother had a history of three pregnancies with two NBs who died of heart block.

Clinical characteristics of 95 children with systemic lupus erythematosus

Objective To investigate the clinical features of children with systemic lupus erythematosus(SLE). Methods Ninety-five patients hospitalized in Wuxi Children's Hospital from Jan.2003 to Dec.2013 were analyzed retrospectively.They were diagnosed as SLE for the first time.The clinical features of SLE between boys and girls were compared.Chi-square test was used for statistical analysis. Results Of the 95 cases with SLE, 12 were boys and 83 were girls.Clinical manifestations showed 69 cases with facial rash (72.6%), 43 cases with fever (45.3%), 31 cases with arthritis (32.6%), 24 cases with serositis (25.3%), 23 cases with oralulcer (24.2%), 13 cases with allergy to sunlight (13.7%), 11 cases with nervous system damage (11.6%). Laboratory examination: 94 cases(98.9%) with anti-nuclear antibody positive, 91 cases(95.6%) with decreased C3 complement, 71 cases(74.7%) with anti-dsDNA antibody positive, 71 cases(74.7%) with decreased C4 complement, 69 cases(72.6%) with anti-SSA antibody positive, 62 cases(65.3%) with anti-Sm antibody positive, 52 cases (54.7%) wiht proteinuria, anti-SSB antibody po-sitive 49 cases(51.6%), 46 cases (48.4%) with decreased leukocyte, 38 cases(40.0%) with anti-nucleosome antibody, 38 cases (40.0%) with anemia, 26 cases(27.4%) with hematuresis, 14 cases(14.7%) with decreased platelet, 6 cases(6.3%) with injury of renal function.Renal injury, anti-Sm antibody and anti-nucleosome antibody of male children with SLE were significantly higher than those of female children(χ2=9.989, 4.224, 4.070, all P<0.05). The positive rate of anti-SSB antibody in female children with SLE was significantly higher than that of male children(χ2=3.885, P<0.05). Conclusions Male childre with SLE are easy to appear the renal injury, proteinuria is the most common.Anti-Sm antibody is a risk factor for the high incidence of renal injury in male children with SLE.Anti-nucleosome antibody is a risk factor for the high incidence of SLE disease activity in male children than that of female children.The risk of future Sjogren's syndrome in female children with SLE is higher than that of male children. Key words: Systemic lupus erythematosus; Clinical features; Child

Outcomes of fetal echocardiographic surveillance in anti-SSA exposed fetuses at a large fetal cardiology center.

Echocardiography screening in anti-SSA antibody exposed fetuses is controversial. The aim of this study is to evaluate utility of fetal echocardiography in anti-SSA exposure. Echocardiograms performed over 9 years for maternal anti-SSA exposure were reviewed for atrioventricular (AV) block, cardiomyopathy, arrhythmias, effusion, valve abnormalities, or other abnormalities identified by the echocardiographer. Fetuses with AV block referred to our institution and subsequently found to be anti-SSA exposed were also identified. Six hundred thirty six echocardiograms were performed on 140 fetuses (Cohort 1) of 134 women screened for maternal anti-SSA +/- anti-SSB antibodies. No fetuses developed second or third-degree AV block or cardiomyopathy (odds ratio 0.1, CI 0.0051 to 1.9410, p = 0.13). Dexamethasone was administered to three fetuses for sinus bradycardia, echogenicity near AV node, and ventricular systolic dysfunction with valve regurgitation; all normalized. Screening echocardiograms identified: sinus bradycardia (n = 1), PR prolongation (n = 5), premature atrial contractions (n = 3), valve regurgitation (n = 24), echogenic myocardium (n = 4), and pericardial effusion (n = 1). Isolated tricuspid regurgitation and first-degree AV block did not progress. Nine cases of SSA-mediated AV block (Cohort 2) were referred after heart block developed. Serial fetal echocardiography in anti-SSA exposed fetuses did not detect AV block. In rare cases, dexamethasone treatment may have affected disease course.

AB0543 Systemic Manifestations in the Primary SjÖGren's Syndrome: about 31 Cases

BackgroundPrimary Sjögren's syndrome (pSS) is a systemic disorder characterized by lymphocytic infiltration and progressivedestruction of exocrine glands. The inflammatory process extends beyond the exocrine glands and can potentially affect any...

Patient with Neuromyelitis Optica Spectrum Disorder Combined with Sjögren's Syndrome Relapse Free Following Tacrolimus Treatment

Tacrolimus, a novel immunosuppressant agent, has been widely used in organ transplantation and autoimmune diseases. We herein present a case of neuromyelitis optica spectrum disorder (NMOSD) combined with Sjögren's syndrome (SS) successfully treated with tacrolimus. This patient repeatedly presented with recurrent longitudinally extensive transverse myelitis. Her NMO-IgG and anti-SSA and anti-SSB antibodies were seropositive. Considering the frequency of relapses and severe disability, tacrolimus was initiated after failure of intravenous cyclophosphamide. Her status was steady for over 36 months after tacrolimus treatment. This report indicates that tacrolimus may be a potentially effective immunosuppressant for NMOSD with systemic autoimmune diseases.

Chinese SLE treatment and research group registry: III. association of autoantibodies with clinical manifestations in Chinese patients with systemic lupus erythematosus.

We investigated the characteristics of Chinese SLE patients by analyzing the association between specific autoantibodies and clinical manifestations of 2104 SLE patients from registry data of CSTAR cohort. Significant (P < 0.05) associations were found between anti-Sm antibody, anti-rRNP antibody, and malar rash; between anti-RNP antibody, anti-SSA antibody, and pulmonary arterial hypertension (PAH); between anti-SSB antibody and hematologic involvement; and between anti-dsDNA antibody and nephropathy. APL antibody was associated with hematologic involvement, interstitial lung disease, and a lower prevalence of oral ulcerations (P < 0.05). Associations were also found between anti-dsDNA antibody and a lower prevalence of photosensitivity, and between anti-SSA antibody and a lower prevalence of nephropathy (P < 0.05). Most of these findings were consistent with other studies in the literature but this study is the first report on the association between anti-SSA and a lower prevalence of nephropathy. The correlations of specific autoantibodies and clinical manifestations could provide clues for physicians to predict organ damages in SLE patients. We suggest that a thorough screening of autoantibodies should be carried out when the diagnosis of SLE is established, and repeated echocardiography annually in SLE patients with anti-RNP or anti-SSA antibody should be performed.

Association of Genes in the NF‐κB Pathway with Antibody‐Positive Primary Sjögren's Syndrome

Primary Sjögren's syndrome (SS) is a systemic autoimmune inflammatory disease characterized by focal lymphocytic infiltrates in the lachrymal and salivary glands and autoantibodies against the SSA/Ro and SSB/La antigens. Experimental studies have shown an activation of NF-κB in primary SS. NF-κB activation results in inflammation and autoimmunity and is regulated by inhibitory and activating proteins. Genetic studies have shown an association between multiple autoimmune diseases and TNFAIP3 (A20) and TNIP1 (ABIN1), both repressors of NF-κB and of IKBKE (IKKε), which is an NF-κB activator. The aim of this study was to analyse single nucleotide polymorphisms (SNPs) in the IKBKE, NFKB1, TNIP1 and TNFAIP3 genes for association with primary SS. A total of 12 SNPs were genotyped in 1105 patients from Scandinavia (Sweden and Norway, n = 684) and the UK (n = 421) and 4460 controls (Scandinavia, n = 1662, UK, n = 2798). When patients were stratified for the presence of anti-SSA and/or anti-SSB antibodies (n = 868), case-control meta-analysis found an association between antibody-positive primary SS and two SNPs in TNIP1 (P = 3.4 × 10(-5) , OR = 1.33, 95%CI: 1.16-1.52 for rs3792783 and P = 1.3 × 10(-3) , OR = 1.21, 95%CI: 1.08-1.36 for rs7708392). A TNIP1 risk haplotype was associated with antibody-positive primary SS (P = 5.7 × 10(-3) , OR = 1.47, 95%CI: 1.12-1.92). There were no significant associations with IKBKE, NFKB1 or TNFAIP3 in the meta-analysis of the Scandinavian and UK cohorts. We conclude that polymorphisms in TNIP1 are associated with antibody-positive primary SS.

Clinical characteristics of children with positive anti-SSA/SSB antibodies

This study aimed to characterize the manifestations of clinical symptoms and signs, primary rheumatic diseases, and other autoantibodies in pediatric patients with positive anti-SSA and/or anti-SSB antibodies. Subjects under age 18 with positive anti-SSA and/or anti-SSB antibodies were screened and enrolled in a tertiary hospital in Taiwan. Data were collected via medical records,including age, gender, onset of the primary rheumatic disease, clinical symptoms and signs, and the medication used. Schirmer test for Sjögren's syndrome (SS) screening was performed in all enrolled patients. Among twenty enrolled subjects, seventeen of them had systemic lupus erythematosus; four of them were diagnosed as SS with positive Schirmer test. In addition to antinuclear antibodies and anti-DNA antibodies, other common autoantibodies were anti-RNP antibodies (50 %) and anti-Sm antibodies(30 %). The most common symptoms were arthritis (60 %)followed by malar rash (40 %). In conclusion, we observed that a low proportion of childhood SS (4/20) exists in our patients with positive SSA and/or anti-SSB antibodies. It is suggested that clinicians should focus more on the clinical symptoms in these patients, rather than undertaking invasive diagnostic interventions to rule out Sjögren's syndrome.

Potential contribution of interleukin-33 to the development of interstitial lung disease in patients with primary Sjogren’s Syndrome

ObjectiveTo determine whether interleukin (IL)-33 and soluble ST2 (sST2) are associated with primary Sjogren’s Syndrome (pSS). MethodsSerum levels of IL-33 and sST2 in 110 pSS patients and 78 healthy controls were measured by enzyme-linked immunosorbent assay (ELISA). Immunoglobulins, rheumatoid factors (RF), antinuclear antibody (ANA), anti-SSA/RO-52 antibody, anti-SSB antibody and erythrocyte sedimentation rate (ESR) were measured by standard laboratory techniques. Interstitial lung disease (ILD) was identified on high-resolution computed tomography (HRCT). Disease activity in pSS was scored with the European League Against Rheumatism Sjogren’s Syndrome Disease Activity Index (ESSDAI). ResultsSerum levels of IL-33 and sST2 were significantly elevated in pSS patients, especially in patients with ILD. There was significant positive correlation between IL-33 and RF, anti-SSB antibody. ConclusionIL-33/sST2 may be involved in the pathogenesis of pSS and partly contribute to the ILD in pSS patients.

FRI0303 Validation of eular primary sjögren’s syndrome disease activity and patient indexes

ObjectivesTo validate the EULAR scores for assessment of primary Sjögren’s Syndrome (SS): the EULAR SS Disease Activity Index (ESSDAI), the EULAR SS Patient Reported Index (ESSPRI).MethodsProspective international validation study with...

AB0846 Smoking and alcohol consumption may increase the development of anti-jo-1 antibodies in patients with idiopathic inflammatory myopathies

BackgroundIdiopathic Inflammatory Myopathies (IIMs) are a group of rare systemic autoimmune diseases, characterized by chronic inflammation of the muscle, resulting symmetric proximal weakness, characteristic rashes and other systemic features. The...

FRI0278 Risk factors for mortality in patients with lupus nephritis: a long-term study at a single center in japan

BackgroundLupus nephritis (LN) is a major cause of morbidity and mortality in patients with systemic lupus erythematous (SLE). Previous reports showed age (>50 years), nonwhite race, and renal dysfunction at...

Antinuclear antibodies in general population: what does that mean? (P4535)

Abstract Antinuclear antibodies (ANAs), the serologic hallmarks of autoimmunity are frequently seen in patients with systemic autoimmune diseases. We screened 2223 Caucasian normal control and 143 SLE sera for ANA, anti-chromatin, anti-Ro and anti-SSB antibodies. Further, we employed protein array screening to detect antibodies against over 90 different auto-antigens. Our data show that about 26% of the normal population are ANA positive, with 17% (382 of 2223) having moderate (20-40 ELISA units) and 9% (197 of 2223) having high (&amp;gt;40 ELISA units) ANA antibody levels. Chromatin ELISA and Crithidia luciliae immunofluorescence tests (CLIFT) confirmed the absence of anti-chromatin antibodies in the ANA positive fraction of the normal population. Results of Anti-Ro-52 ELISA showed that 6.5% (3/46) moderate ANA NCs, 40.4% (17/42) high ANA NCs and 26.3% (25/95) SLEs test positive for anti-Ro-52 antibodies. Interestingly, a very strong correlation was observed between ANA and anti-Ro-52 antibodies in the high ANA (Pearson r =0.69, p&amp;lt;0.0001) and SLE group (Pearson r =0.66, p&amp;lt;0.0001). We also investigated ANA positivity as a function of age in the normal population and found that correlation between ANA and Ro-52 antibodies was stronger in subjects under 50 year of age (Pearson r =0.75, p&amp;lt;0.0001) than that of those above 50 year (Pearson r =0.57, p&amp;lt;0.0001). This observation was also consistent in SLE patients (Pearson r = 0.76, p&amp;lt;0.0001). Protein array results will be discussed.

Specificity of anti-SSB as a diagnostic marker for the classification of systemic lupus erythematosus

The aim of the present study was to investigate the sensitivity and specificity of anti-Sjögren’s syndrome type B (SSB) antibodies for diagnosing systemic lupus erythematosus (SLE) and to understand the correlation between anti-SSB antibodies and the clinical manifestations of SLE. A line immunoassay (LIA) was used to detect the presence of serum anti-SSB antibodies in SLE patients. The clinical manifestations of the patients were recorded to enable their correlation with the serum anti-SSB antibodies to be analyzed. In 25.7% of the 74 SLE patients, the serum was positive for anti-SSB antibodies, whereas only 3.3% of the 30 control cases were positive. The specificity of anti-SSB antibodies for detecting SLE was 96.7%. In anti-SSB antibody-positive SLE patients, the incidence of cheek erythema, alopecia, serositis, secondary Sjögren’s syndrome (sSS), leukocytopenia, elevated immunoglobulin (Ig)G and positive presence of anti-Sjögren’s syndrome type A (SSA)60 or anti-SSA52 antibodies was higher than in the anti-SSB antibody-negative group (P<0.05). Anti-SSB antibodies are important for the diagnosis of SLE and are associated with cheek erythema, alopecia, serositis, sSS, leukocytopenia, the elevation of IgG and positive presence of anti-SSA60 or anti-SSA52 antibodies.

Characteristics of Sjogren's syndrome in rheumatoid arthritis

To compare features of SS in RA with primary SS and RA without SS. Patients hospitalized between January 2007 and December 2010 were retrospectively studied. Seventy-four cases of overlap RA and SS (RA/SS) among 509 cases of RA were identified. Cases of SS (n = 187) detected during the same period acted as controls. Among those with RA/SS, there were 46 cases of RA-onset SS and 12 cases of SS-onset RA. Sixteen patients had simultaneous-onset RA and SS. Compared with RA without SS, RA/SS patients had more severe arthritis; a higher incidence of haematological abnormality, fever and rash; and a higher frequency of RF, ANAs and anti-SSA and anti-SSB antibodies (P < 0.05). Compared with primary SS, RA/SS patients were older, had more severe arthritis, anaemia and lung involvement; a lower incidence of fever, rash, leucopenia, thrombocytopenia and hyperthyroidism; and a higher frequency of RF, anti-keratin antibody, anti-perinuclear factor and anti-cyclic citrullinated antibodies (P < 0.05). Compared with RA and primary SS, RA/SS patients had higher disease activity scores of both RA and SS. RA/SS patients have distinctive features, with more complications and systemic involvement. In addition, disease activity is higher in RA/SS.

Clinical analysis of neuropsychiatric systemic lupus erythematosus involving the central nervous system

Background Neuropsychiatric systemic lupus erythematosus (NP-SLE) presents with a wide variety of clinical manifestations, which is often difficult to diagnose with a high mortality. This study aims to investigate the clinical features of NP-SLE involving the central nervous system (CNS) and the differential diagnoses between CNS NP-SLE and intracranial infections. Methods The clinical manifestations, serum immunological features, cerebrospinal fluid (CSF) examinations (including intracranial pressure, leukocyte count, protein, glucose and chloride), CT and (or) MRI and electroencephalogram (EEG) data of 23 NP-SLE patients with CNS involved were retrospectively reviewed. Results Nine patients presented with diffuse manifestations, while 14 patients presented with focal manifestations. Serum analysis showed the positive rates of immunoglobulins anti-nuclear antibody (ANA), anti-double stranded DNA antibody (dsDNA), anti-Sm, anti-ribosmal P protein, anti-SSA and anti-SSB antibodies were 21/22, 7/22, 1/14, 2/14, 9/14 and 3/14 respectively. Patients with decreased serum C3 accounted for 14/20 while patients with decreased serum C4 accounted for 5/20. Besides, patients with increased CSF leukocyte count and microalbumin took up 5/12 and 7/12, while patients with decreased glucose and chloride levels took up 5/12 and 6/12. All 23 patients presented abnormal CT and (or) MRI and 6 patients presented abnormal EEG. Conclusion Serum immunological levels, CT and (or) MRI and EEG examinations contributed to the diagnosis of NP-SLE involving CNS. Although CSF analyses were slightly abnormal, the increase of leukocyte count and average microalbumin was not obvious, and the mean values of glucose and chloride were in the normal range, suggesting that the CSF examinations were helpful for the differential diagnoses from intracranial infections. Glucocorticoids and immunosuppressive drugs were remarkably effective for CNS NP-SLE patients.

Sj�gren's Syndrome Complicated with Thrombotic Thrombocytopenic Purpura: A Case Report and Literature Review

The association of Sjogren's syndrome (SS) with thrombotic thrombocytopenic purpura (TTP) is rare, but it carries a high death rate and can be easily misdiagnosed. We report a case of TTP with SS and the related literature is reviewed. A previously healthy 72-year-old woman presented with an acute onset of marked thrombocytopenia and then dizziness, fatigue, fever and fainting. Schirmer's test was bilaterally positive, associated with positive Rose Bengal corneal staining. Radionuclide imaging of the glands showed abnormalities. The presence of anti-SSA and anti-SSB antibodies confirmed the diagnosis of SS. The patient was treated successfully with plasma exchange therapy, plasma infusion, glucocorticoid, and immunosuppressive agents. Her condition has remained stable after 6 months of follow-up. Only a few cases of SS complicated with TTP have been described in the literature. Rheumatologists should pay attention to this special condition because it is rare and has a high mortality rate. Keywords Sjogren's syndrome, Thrombotic thrombocytopenic purpura, Autoimmune

Infliximab Induced Systemic Lupus Erythematosus: A Case Report

A 41-year-old Hispanic female was diagnosed with Crohn's disease in 1998, and was treated with Mesalamine and 6 Mercaptopurine. She also required the use of prednisone for 6-8 weeks at least once a year. Her disease was complicated by perianal fistulae and recurrent perianal abscesses. In 2001, she was started on Infliximab (5 mg/kg) every 8 weeks. However, it was stopped in 2003 for financial reasons. She continued to use 5 ASA and 6-mercaptopurine. In addition, prednisone and Flagyl were used for treatment of acute flare-ups. Infliximab was restarted in January 2009 due to frequent flare-up and inability to control perianal disease. In April 2009, the patient presented to the clinic complaining of right toe pain and swelling, and this was preceded with multiple episodes of large and small joint pain and swelling, with lower extremity tingling. She denied skin rashes, oral ulcers, or photosensitivity. The physical exam was remarkable for right toe swelling and tenderness with normal range of motion. Serology was positive for ANA (1:320), mildly elevated anti DsDNA (1:20), increased anti-B2 lipoprotein antibodies (29.4), low C4 and mildly decreased C3. Anti-Smith, anti SSA, anti SSB, anti RNP and anti-histone antibodies were negative. Rheumatoid factor and urinalysis were normal. Correlation of the history, examination, and laboratory findings in the context of patient's exposure to infliximab, the diagnosis of TNF-alpha antagonist induced Lupus syndrome (TAILS) was made. Treatment included stopping infliximab. Patient was started on hydroxychloroquine (200 mg twice daily). Arthralgia resolved 6 months after stopping infliximab. Her symptoms of lower extremity tingling also partially resolved with gabapentin in 12 months. Serology and complement levels were found to be normal. Discussion: The occurrence of TAILS is estimated to be 0.5-1% with infliximab, and etanercept being the most common agents. Onset of symptoms ranges from 10 days to 54 months after exposure. In our patient, symptoms started 3 months after restarting infliximab. Presenting symptoms can widely vary involving musculoskeletal system, neuropathy, and neuropsychiatric disorders. Arthritis is the most common presenting disorder. Peripheral neuropathy was found in 5 out of 32 patients receiving infliximab, and neuropsychiatric symptoms developed in 3% of patients receiving either infliximab or etanercept. Our patient presented with arthralgia, arthritis and peripheral neuropathy. Anti-neutrophil antibodies were found to be positive in the majority of patients and in our patient as well. In a prospective controlled study, only 20% of patients receiving infliximab were positive for anti-double stranded DNA, and it was mildly elevated in our patient. Anti-histone antibodies are positive in 95% of patients with drug induced lupus, and only in 17-57% of patients with TAILS. Anti-histone antibodies were negative in our patient. Resolution of symptoms was found in most of the patients after stopping the drug in 3 weeks to 6 months. Our patient's arthritis resolved after 6 months, and it required the addition of hydroxychloroquine. Peripheral neuropathy required more time to resolve (12 months).

Paralisia Por Hipocaliémia: Manifestação Inaugural da Síndrome de Sjögren Primário

The Sjögren's syndrome is a systemic autoimmune disorder characterized by chronic inflammation of the exocrine glands with extraglandular manifestations in up to 25% patients. Renal involvement occurs in 18.4-67% of cases, with tubulointerstitial nephritis being the most frequent pathology. We present the case of a 37 year-old woman admitted because of generalized grade 2 muscle weakness which developed over a week. We detected: hypokalemia, rhabdomyolysis, urinary pH 6.5, proteinuria and metabolic acidemia. The laboratory tests suggestive of distal renal tubular acidosis with hypokalaemia led to the diagnosis of lymphoplasmocytic tubulointerstitial nephritis, which was confirmed by renal biopsy, and to a clinical suspicion of Sjögren's syndrome. Primary Sjögren's syndrome was diagnosed in this patient based on the following criteria: xerophthalmia, xerostomia, sialadenitis, positive anti-SSA and anti-SSB antibodies, and absence of criteria for lupus and rheumatoid arthritis. During hospitalization, the patient developed deep vein thrombosis. Tests showed positive antiphospholipid antibodies and the diagnosis of secondary antiphospholipid syndrome was made. She was treated with potassium, bicarbonate, steroids, ramipril and warfarin. The authors wish to highlight the extraglandular manifestations and in particular the rarity of hypokalemic paralysis as the presenting manifestation of primary Sjögren's syndrome.

Preliminary study of the relationship between tumor like Sjögren's syndrome and malignant lymphoma

To investigate the clinical and laboratory characteristics of tumor like Sjögren's syndrome (TLSS) patients and non-tumor like Sjögren's syndrome (NTLSS) and the incidence of lymphoma in patients of Sjögren's syndrome (SS). A retrospective analysis was carried out in 199 primary SS (including TLSS) patients who were recruited in Peking University School and Hospital of Stomatology from 1998 to 2010. Clinical and laboratory information were collected. The patients were divided into two groups: TLSS (n = 25) and NTLSS (n = 174). Clinical and laboratory characteristics were compared between these two groups by a statistical analysis. Of the 25 TLSS patients, 23 had enlargements of parotid glands and 2 had enlargements of submandibular glands. There were significant differences of salivary scintigraphy appearance (P = 0.018), hypergammaglobulinemia (P = 0.014), rheumatoid factor positive rate (P = 0.001), formation of the ectopic germinal centers (P = 0.014), double positive rate of anti-SSA antibody and anti-SSB antibody (P < 0.001) between the TLSS and NTLSS patients. Among the 25 TLSS patients, 3 developed lymphomas, accounting for 1.5% (3/199) of the total 199 patients and 12% (3/25) of the TLSS patients. Lymphoma subtypes included one diffused large B-cell lymphoma and two mucosa-associated lymphoid tissue lymphoma. There was no lymphoma detected in NTLSS patients. There are clinical and laboratory differences between TLSS and NTLSS patients, with a more tendency to develop lymphomas in TLSS patients.