Sicca syndrome in the same family - the importance of anti-Ro60, anti-Ro52 and anti-La antibody profiling in Sjögren's syndrome.

Sicca syndrome, which is typical for Sjögren's syndrome (SS), both primary (pSS) and secondary (sSS), is relatively often comorbid with other autoimmune diseases. The current classification criteria for SS published in 2016 include only anti-SSA (anti-Ro) autoantibody, while the latest literature proposes that anti-Ro60/anti-Ro52 autoantibody profiles should be used instead, as these two types of antibodies correlate with specific clinical symptoms and laboratory test findings. The paper presents the case of a 41-year-old woman suffering from pSS and her three daughters, who were under observation for rheumatic disorders due to sicca symptoms, especially pSS, as well as a discussion on separate determination of anti-Ro60 and anti-Ro52 autoantibodies based on current literature in the PubMed database. When testing with antinuclear antibodies, the Ro60+Ro52+La+ autoantibody profile most closely matches for pSS. Further research is needed to find marker antibodies for SS and quantification methods.

Epidemiological survey of antinuclear antibodies in healthy population and analysis of clinical characteristics of positive population.

BACKGROUND: In China, the incidence of autoimmune diseases is gradually increasing. To decrease the misdiagnosis rate of autoimmune diseases, we conducted an epidemiological investigation about the presence of antinuclear antibody (ANA) in healthy populations and analyzed the clinical characteristics of healthy population with both high titer of ANA and positive anti-SSA and AMA-M2. METHODS: Serum ANA titers were detected by indirect immunofluorescence (IIF), and other 15 types of ANA-specific antibodies were detected by line immunoassays. RESULTS: In 25 110 individuals for routine examination, the positive rate of ANA titer >1:100 was 14.01%, of which the positive rate of female (19.05%) was higher than that of male (9.04%; P < 0.01). The positive rate of ANA titer >1:320 was 5.93%, of which the positive rate of female (8.68%) was higher than that of male (3.21%; P < 0.01). The specific antibodies were detected in 1489 of ANA-positive people with titer >1:320, and the top three detected antibodies were anti-Ro-52 (212), AMA-M2 (189), and anti-SSA (144). The abnormal rate of blood routine test, liver function test, and other clinical indicators in AMA-M2-positive population was significantly different from those in the control group. The abnormal rate of blood routine test, liver function test, and immune index in anti-SSA-positive population was higher than those in control group. CONCLUSION: There was a high prevalence of ANA positive in healthy population. To avoid misdiagnosis, those who had symptoms of abdominal discomfort, pruritus, or fatigue with abnormal results of blood routine and liver function test should be examined for ANA, AMA-M2, anti-SSA as early as possible.

Aseptic meningitis as an initial manifestation of primary Sjögren's syndrome.

Aseptic meningitis is a rare life-threatening complication of primary Sjögren's syndrome (pSS), and its characteristics and prognosis remain unknown. We present our case of aseptic meningitis associated with pSS and reviewed the published literature to elucidate their characteristics and prognosis. An 84-year-old man was admitted to our hospital for fever and disturbance of consciousness. Acute aseptic meningitis was diagnosed based on the results for cerebrospinal fluid and head imaging tests. As an aetiological investigation for his aseptic meningitis, serum anti-Sjögren's-syndrome-related antigen A and anti-Sjögren's-syndrome-related antigen B antibodies were found to be positive, and the biopsy specimen of his labial salivary gland revealed lymphocytic sialadenitis, confirming a diagnosis of pSS. Treatment with moderate-dose glucocorticoid completely improved his aseptic meningitis. Relapse of the disease was not observed during his clinical course over 12 months. Our present case and literature review suggest that aseptic meningitis can be an initial manifestation of pSS and be treatable by immunosuppressive therapy. Thus, early recognition and treatment initiation are critical to prevent the irreversible damage of central nervous system in pSS-associated aseptic meningitis. In aseptic meningitis of unknown origin, pSS should be included in differential diagnoses, and testing for serum anti-Sjögren's-syndrome-related antigen A and anti-Sjögren's-syndrome-related antigen A antibodies may be useful as an initial screening.

Dermatomyositis With Dual Positive Anti-melanoma Differentiation-Associated Gene 5 and Anti-Sjogren Syndrome-Related Antigen 52 kD Antibodies: A Case Report and Literature Review on Clinical Characteristics.

Anti-melanoma differentiation-associated gene 5 (MDA 5) is one of the subtypes of dermatomyositis associated with rapidly progressive lung disease. MDA 5 carries a high mortality risk due to respiratory failure. The exact pathophysiology is unclear, but it is linked to genetic predisposition and viral triggers with the associated innate response and cytokine production like interleukins IL-1,6,18, tumor necrosis factor-alpha, and interferons. It is usually treated with anti-cytokines, high-dose steroids, immunosuppressants, and plasma exchange. Due to the atypical presentation and rapidity of the disease course, the diagnosis is often delayed. We report a 39-year-old female presenting with rapidly progressive lung disease secondary to an aggressive form of dermatomyositis.

Diagnostic Utility of Minor Salivary Gland Biopsy for Primary Sjögren Syndrome in Patients With Negative Anti-SSA Antibodies.

BACKGROUND: Sjögren syndrome is a systemic autoimmune disease characterized by lacrimal and salivary gland inflammation resulting in dry eyes and mouth. Although it is a common disease, diagnosis can be challenging due to its heterogeneous presentation. A positive minor salivary gland biopsy is mandatory to fulfill the 2016 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for primary Sjögren syndrome in patients who are seronegative for anti-SSA/Ro antibodies. The objective of our study was to evaluate the validity of minor salivary gland biopsy for patients who are SSA antibody-negative yet are suspected of having primary Sjögren syndrome because of compelling symptoms. METHODS: We conducted a retrospective chart review of adult patients with a negative anti-SSA antibody test who underwent minor salivary gland biopsy to assess suspected Sjögren syndrome at Henry Ford Rheumatology Clinics between January 2005 and December 2019. Patient characteristics and clinical features are described. Sensitivity, specificity, positive predictive value, and negative predictive value are assessed. RESULTS: A total of 47 patients were included: 46 (97.9%) females and one (2.1%) male. The mean age was 57.2 ± 13.8 years. There were 14 (29.8%) patients who had a positive minor salivary gland biopsy result and 15 (31.9%) patients who had a final diagnosis of Sjögren syndrome. Minor salivary gland biopsy had 93.3% sensitivity (95% confidence interval (CI): 68%-99.8%), 100% specificity (95% CI: 89.1%-100%), 100% positive predictive value (95% CI: 76.8%-100%), and 97% negative predictive value (95% CI: 84.2%-99.9%). CONCLUSION: The diagnostic value of minor salivary gland biopsy is high for patients who do not have anti-SSA antibodies yet are suspected of having Sjögren syndrome. The results of the study support the consideration of routine minor salivary gland biopsy for identifying Sjögren syndrome in these patients.

Increased Threat of Thyroid Diseases in Patients With Sjogren's Syndrome: A Systematic Review.

Sjogren's syndrome is an autoimmune disorder of the body's exocrine glands; however, it is known to have numerous extra-glandular and endocrine manifestations in the body. Moreover, other autoimmune have also been reported with high prevalence in patients with Sjogren's syndrome, including thyroid diseases. Therefore in this study, we aimed to ascertain the increased risk of developing thyroid disorders in patients with pre-existing Sjogren's syndrome. The systematic review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Online searches on PubMed, PubMed Central (PMC), Google Scholar, and Cochrane were done till 5th June 2022 to filter out studies published in the last twenty years. Based on the inclusion-exclusion criteria, 167 studies were initially selected. They were screened and assessed by quality assessment tools that yielded seven studies, including one meta-analysis, three non-randomized control trials, and three systematic reviews. The study proved that patients with Sjogren's syndrome are at significant risk of developing thyroid disorders, especially autoimmune thyroiditis. This also highlights the need for advanced research and better diagnostic and screening protocols for these patients to reduce the seriousness of the disease.

Rare Case of Central Pontine Myelinolysis: Etiological Dilemma.

Central nervous system (CNS) involvement in Sjogren's syndrome (SS) has a broad spectrum of presentations. We present a 33-year-old with sudden onset, rapidly progressive quadriplegia, severe dysarthria, bilateral facial palsy, bulbar palsy, and hypernatremia. The MRI of the brain revealed hyperintensity in the central pons diffusion-weighted imaging, T2-weighted imaging, and fluid-attenuated inversion recovery (FLAIR) without abnormal contrast enhancement, consistent with central pontine myelinolysis. However, there was no antecedent history of hyponatremia with rapid correction. The patient responded excellently to sodium correction and pulse methylprednisolone therapy and was erroneously diagnosed as idiopathic hypernatremic osmotic demyelination. One year later, she presented with vague constitutional symptoms, renal tubular acidosis type-1 (distal), hypokalemia with associated myopathy. Subsequent testing for anti-Sjögren's-syndrome-related antigen A (SSA)/Ro autoantibodies and a biopsy of the minor salivary gland established the diagnosis of primary Sjogren syndrome (pSS). Remission was achieved with oral prednisolone after her discharge. Neurological signs can be the initial presentation that precedes the classical systemic manifestations of multisystem autoimmune disorders like pSS. In the event of osmotic demyelination, when antecedent hyponatremia with rapid correction is not there, we suggest evaluating for possible autoimmune etiology.

Treatment and follow-up of fetuses that developed congenital heart block due to autoantibody in two cases.

OBJECTIVE: Autoantibody-related congenital heart block (ACHB) is a passively acquired autoimmune disease. This study aimed to examine the pathogenesis, clinical manifestations, and treatment of ACHB. METHOD: The clinical data of two fetuses with first-degree ACHB were retrospectively analyzed. RESULTS: Two pregnant women were strongly positive for anti-Sjögren's syndrome-related antigen A (SSA) antibody. Among these two cases, one had a prolonged atrioventricular (AV) interval at 28+3 weeks in utero, while the other had a prolonged AV interval at 24+6 weeks in utero. After prenatal intervention, one patient recovered to normal, while one fetus continued to have ACHB after treatment with dexamethasone and intravenous immunoglobulin. Furthermore, the two neonates were positive for anti-SSA antibody and were diagnosed with ACHB. CONCLUSION: The pathogenesis of ACHB is closely correlated with anti-SSA/Ro antibody and anti-SSB/La antibody from the mother, and is affected by fetal susceptibility. Early screening and early intervention for ACHB are important.

Evaluation of cardiac function and systolic dyssynchrony of fetuses exposed to maternal anti-SSA and anti-SSB antibodies using two-dimensional speckle tracking imaging / 中华超声影像学杂志

Objective:To evaluate the cardiac function and systolic dyssynchrony of fetuses exposed to maternal autoimmune antibodies (anti-SSA/Ro60, anti-SSA/Ro52 and anti-SSB/La) by using two-dimensional speckle tracking imaging (2D-STI).Methods:A total of 52 pregnant women with singleton pregnancy in the Affiliated Hospital of Inner Mongolia Medical University from July 2018 to November 2020 were selected. Eighteen fetuses of mothers with autoimmune antibodies were enrolled as autoimmune disease (AD) group and 34 fetuses of healthy mothers without antibodies were included as control group. Maternal baseline characteristics, fetoplacental Doppler parameters, and conventional echocardiographic data of two groups were prospectively collected. The systolic global and regional longitudinal strain of left and right ventricles (LV and RV) and the time to peak strain of regional myocardium were measured using 2D-STI. The differences in time to peak strain between the LV free wall and RV free wall (two-chamber dyssynchrony, 2C-DYS) and between the septum and LV free wall (one-chamber dyssynchrony, 1C-DYS) were also calculated.Results:There were no significant differences between the two groups in conventional systolic and diastolic functional parameters for the LV and RV(all P>0.05). The myocardial deformation parameters and 2C-DYS obtained by 2D-STI showed no statistical differences between two groups(all P>0.05). However, 1C-DYS was significantly more prolonged in the AD group than control group[28.50(13.50, 39.25)ms vs 19.50(8.00, 29.25)ms, P=0.042]. Conclusions:LV systolic mechanical dyssynchrony in fetuses of mothers with autoimmune antibodies suggests in-utero subclinical damage of the cardiac conduction system.

Three cases of pigmented cosmetic dermatitis-like eruptions associated with primary Sjögren's syndrome or anti-SSA antibody.

Pigmented cosmetic dermatitis-like (Riehl's melanosis-like) pigmentation was reported in three of 27 patients with primary Sjögren's syndrome. But case reports of such eruptions are rare. We describe three cases of such eruptions associated with primary Sjögren's syndrome or anti-SSA antibody and possible associations with specific types of human leukocyte antigen (HLA) and infiltrating lymphocytes. These middle-aged Japanese women had reticular facial pigmentation and histopathological examination revealed interface dermatitis, melanophages, and dense lymphocytic infiltration around hair follicles and sweat ducts. HLA typing revealed common antigenic equivalents or genetic typing of HLA-A2, DR52, DPA1(02:02) and DPB1(05:01). Immunohistochemical staining revealed major subsets of T cells to be CD8 and CD45RO. Some Foxp3- and few IL17-positive cells were found in strong contrast to the major CD4 subset of infiltrated T cells in annular erythema associated with Sjögren's syndrome. Apparently, our patients' pigmentation represented a specific etiology associated with primary Sjögren's syndrome or anti-SSA antibody.

Primary Sjögren's syndrome accompanied by pleural effusion: a case report and literature review.

Sjögren's syndrome (SS) is a systemic autoimmune disease characterized by the infiltration of lymphocytes in exocrine glands, specifically the salivary and lacrimal glands, resulting in the typical symptoms of xerophthalmia and xerostomia. SS may be accompanied by pleural effusion when the lung is involved, but this occurrence has been reported in only 10 cases in the literature. We report the case of a 42 year-old woman with severe bilateral pleural effusion for eight years. Primary Sjögren's Syndrome was finally diagnosed based on the presence of xerophthalmia and xerostomia, biopsy of the minor salivary glands, and positive anti-SS-A antibody in the serum and pleural effusion. Biopsy of the parietal pleura through video-assisted thoracoscopy revealed infiltration of lymphocytes. The patient had a long history of pleural effusion without clear etiology. Malignant disease was first suspected because of abnormal density lesion on the left lung and malignant cells found on cytology, but PET-CT revealed no malignant lesion. Examinations did not support infection, malignant tumor, pulmonary sarcoidosis, or other connective tissue diseases. This data could be useful for the future study of pleural effusion in SS.

[Evolution of autoantibodies profile in systemic lupus erythematosus according to age and clinical manifestations]. / Profil des réponses auto-anticorps suivant l'âge et la symptomatologie clinique chez des patients atteints de lupus érythémateux.

Clinical features and auto-antibodies profile of 35 Senegalese patients' diagnosed systemic lupus erythematosus (SLE) were analyzed after measurement of antinuclear antibodies (ANA) by IFI, detection of Abs anti-DNA native by ELISA and evaluation of antibodies anti-Sm, anti-RNP, anti-SSA anti-SSB, anti-CCP2, anti-J0, and anti-Scl70 levels by immunodot. Mean age of 33 yrs (18-50 yrs) and sex ratio (F/M) of 16 were found. The most frequent clinical features were rheumatic (88.7%) and cutaneous (79.4%) disorders. ANA and anti-DNAn Abs were detected in 85.7% and 62.5% of the patients respectively. Abs anti-RNP, anti-Sm, anti-SSA, anti-SSB and anti-CCP2 were detected in 30 to 70% of patients. In young patients, the levels of anti-DNAn and anti-Sm Abs were higher than in patients older than 40 yrs (P<0.05). In addition, associations of cutaneous and rheumatic symptoms were characterized by high levels of anti-DNAn, anti-SSA and anti-SSB Abs. Our study shows the interest of a measurement of anti-DNAn, anti-SSA and anti-SSB Abs during the follow of SLE patients particularly in those presenting both rheumatic and cutaneous symptoms.

Analysis on results difference of two methods for detecting 146 cases of anti-nuclear antibody / 国际检验医学杂志

Objective To investigate the results difference of the indirect immunofluorescence (IIF) and immunoblotting (LIA) for detecting ant-inuclear antibodies (ANA) and clinical value .Methods One hundred and forty-six ANA detection specimens of IIF negative and LIA positive were collected and performed the detection of HBV antibodies and anti-HCV antibodies .Results (1) In 146 specimens of IIF (-)LIA (+ ) ,the positive specimen numbers of single anti-Ro52 antibody ,anti-SSA antibody and anti-AMA-M2 antibody were 69 cases ,42 cases and 15 cases respectively ,which of anti-RNP antibody ,anti-PCNA antibody and PM-Scl antibody were 3 cases ,5 cases and 2 cases respectively ,the combined 2-item positive was in 8 cases .(2)Among positive specimens of single anti-Ro52 antibody ,HBsAg(+ ) ,anti-HBe(+ ) ,anti-HBc(+ ) model and HBsAg(+ ) ,HBeAg(+ ) ,HBcAb(+ ) model of hepatitis B ,and hepatitis C were 51 cases ,4 cases and 7 cases respectively ,7 cases were non-hepatitis patients .(3) Among positive specimens of single ant-SSA antibody ,6 cases were hepatitis B patients and 36 cases were the patients with non-hepatitis B .Conclu-sion Anti-Ro52 antibody and anti-SSA antibody are easier to be missed by the IIF detection .Anti-Ro52 antibody positive has a cer-tain relation with small three positive of hepatitis B .

The significance of serological markers and European League Against Rheumatism SS Disease Activity Index score in patients with primary Sj(o)gren's syndrome / 中华风湿病学杂志

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.

The diagnostic value of anti-SSA antibody in primary Sj(o)gren's syndrome / 中华内科杂志

Objective According to international classification criteria (2002) on Sjogren' s syndrome, labial pathology was still considered as a major criterion for diagnosis. Standard labial biopsy was hard to be carried out in China. This study is to evaluate whether the invasive labial biopsy could be replaced by noninvasive detection of serum anti-SSA antibody. Methods 181 Chinese patients with the initial diagnosis of primary Sjogren's syndrome in Peking Union Medical College Hospital (PUMCH) were enrolled in Sjogren's International Collaborative Clinical Alliance (SICCA). All patients received standard labial biopsies (area of salivary gland tissues≥4 mm~2) and focal score (FS) of focal lymphatic sialadenitis were confirmed by pathologists from school of stomatology,University California of San Francisco (UCSF). Anti-SSA antibodies in sera of all patients were detected by double immunodiffusion (DID), Western blot in PUMCH and by enzyme-linked immunosorbent assay (EIJSA) in central laboratory of SICCA. The correlation between labial pathological findings and serum anti-SSA antibody was studied by X~2 test and the concordance was calculated by unweighted Kappa. Results(1)Bivariate analysis revealed strong associations of FS > 1 with the presence of anti-SSA antibody by DID (83.9% vs 42. 0%, P <0. 0001). The accordance between FS and antibody detection by DID was fine with a kappa value of 0. 432. However, there were 16. 1% false-positive antibody reports and 42.0% false-negative antibody reports. (2)FS > 1 was strongly associated with the presence of anti-SSA antibody by Western blot (83.0% vs 51.7%, P < O. 0001). But the accordance between FS and antibody detection by Western blot was only fair with a kappa value of 0. 316. There were 17.0% false-positive antibody reports and 51.7% false-negative antibody reports. (3)FS > 1 was strongly associated with the presence of anti-SSA antibody by ELISA (81.5% vs 38.6%, P <0. 0001). The accordance between FS and antibody detection by EI,ISA was fine with a kappa value of 0.427. There were 18.5% false-positive antibody reports and 38. 6% false-negative antibody reports. Conclusion In Sjogren's syndrome, labial biopsy with FS > 1 finding is strongly associated with anti-SSA antibody. Positive results of anti-SSA antibodies by DID or ELISA may indicate FS > 1, thus labial biopsy could relatively be avoided, negative results may need further standard labial biopsy procedure to confirm the diagnosis of Sjogren's syndrome.

Clinical study on anticentromere antibody and anti-SSA antibody in patients with Primary Sjogren's Syndrome / 中国综合临床

Objective To investigate the clinical and laboratory characteristics of anticentromere antibody (ACA)and anti-SSA antibody expressions in patients with Primary Sj(o)gren's Syndrome (PSS). Methods Twelve PSS patients with ACA positive but SSA negative(ACA PSS)and 19 PSS patients with SSA positive but ACA negative(SSA PSS)were enrolled into the study and classified into two groups. We compared the age,laboratory data,occurrence of Raynaud's phenomenon(RP),and histological changes in minor labial salivary glands biopsies of the patients from two group. Results The mean age of the ACA PSS group(68.4 ± 7.9)years was significantly higher than that of the SSA PSS(54. 6 ± 16. 2)years group(P ＜ 0. 05). Serum IgG level of ACA PSS group(17. 89 ±4. 08)g/L was close to the normal range,which was significantly lower than that of SSA PSS(27.90 ±6. 72)(P ＜0. 01). Leukocytopenia was less frequently observed in ACA PSS than in SSA PSS(P ＜ 0. 05),the difference between two groups was statistically significant. We also found more frequent RP in the ACA PSS group than SSA PSS group(P ＜ 0. 05). Conclusions Our data confirm that ACA positive PSS differs from SSA positive PSS at several clinical respects and laboratorial examinations.

Clinical study on anti—SSA antibody by using dot immunobinding assay / 中国免疫学杂志

Using human spleen purified SSA antigen,we set up a new technique of dot immunobinding assay(DIBA)for detection of anti—SSA antibody.The specificity of DIBA is better than that of double immunodiffusion method.The positive rate of anti—SSA antibody in Sjogren syndrome is 76.9%,while that in systemic lupus erythematosus is 33.3%.We found that anti—SSA antibody and RF usually appeared in same patient with Sjo-gren syndrome or rheumatoid arthritis.

Interstitial lung disease in patients with Sjgren′s syndrome / 中华风湿病学杂志

Objective To investigate the prevalence and clinical characteristics of Sj?gren′s syndrome-interstitial lung disease (SS-ILD). Methods 136 patients with SS were studied. Anti-SSA and Anti-SSB antibodies were measured by Western blot. The inpatients had chest X ray, chest HRCT and pulmonary function examined. Results ①pSS-ILD patients with postive anti-SSA antibody were proned to have interstitial lung disease and the ILD were more severe. ②HRCT showed that sSS-ILD were more severe than that of pSS-ILD. ③Lung capacity of pSS-ILD decreased more frequently than sSS-ILD. sSS-ILD mainly had venti-latory function abnormalities. The lung function impairment of both were dominated by small airways dysfunction and decrease of TLCO. Conclusion SS patients should be examined by HRCT and lung function tests should be performed in the course of the disease to find out and treat ILD.