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ID PMID Title PublicationDate abstract
26482111 Safety of Anti-TNF Therapies in Immune-Mediated Inflammatory Diseases: Focus on Infections 2015 Dec Preclinical Research The efficacy of anti-TNF agents in the treatment of multiple immune-mediated inflammatory diseases (IMIDs) has increased their daily use. However, concerns remain regarding their long-term safety profile. Using a literature-based review of the infectious and malignant complications of anti-TNF biologics in IMIDs including psoriasis, Rheumatoid Arthritis, and inflammatory bowel disease, this review presents current evidence relative to the safety of anti-TNF agents in the context infections and malignancy in adults with IMIDs. Treatment with anti-TNF biologics is an effective treatment option with known risks that can be mitigated by appreciating the safety aspects and via a thorough screening and continuous monitoring of the patient.
26252407 Basic biology and role of interleukin-17 in immunity and inflammation. 2015 Oct Interleukin-17 (also known as interleukin-17A) is a key cytokine that links T-cell activation to neutrophil mobilization and activation. As such, interleukin-17 can mediate protective innate immunity to pathogens or contribute to the pathogenesis of inflammatory diseases, such as psoriasis and rheumatoid arthritis. This review summarizes the basic biology of interleukin-17 and discusses its emerging role in periodontal disease. The current burden of evidence from human and animal model studies suggests that the net effect of interleukin-17 signaling promotes disease development. In addition to promoting neutrophilic inflammation, interleukin-17 has potent pro-osteoclastogenic effects that are likely to contribute to the pathogenesis of periodontitis, rheumatoid arthritis and other diseases involving bone immunopathology. Systemic treatments with anti-interleukin-17 biologics have shown promising results in clinical trials for psoriasis and rheumatoid arthritis; however, their impact on the highly prevalent periodontal disease has not been investigated or reported. Future clinical trials, preferably using locally administered interleukin-17 blockers, are required to implicate conclusivelyinterleukin-17 in periodontitis and, more importantly, to establish an effective adjunctive treatment for this oral inflammatory disease.
25789049 Primary central nervous system lymphoma mimicking recurrent depressive disorder: A case re 2015 Apr Primary central nervous system lymphoma (PCNSL) is a rare subtype of extranodal non-Hodgkin lymphoma, which is limited to the central nervous system. Few studies are available reporting psychiatric symptoms as the initial and dominant presentation of PCNSL. The current study reports the case of a PCNSL patient with a history of major depressive disorder and coexisting rheumatoid arthritis (treated with methotrexate), who initially presented with recurrent depressive disorder that showed no response to antidepressant drug therapy. Magnetic resonance imaging revealed multiple mass lesions in the brain, and pathological examination of the biopsy confirmed the diagnosis of diffuse large B cell lymphoma of the central nervous system. The present case demonstrated that PCNSL may affect mood in the early stages of the disease and thus, clinicians must be aware of this manifestation in patients with depressive disorder co-existing with immunosuppressive conditions, as early detection and appropriate treatment are important prognostic factors for PCNSL.
27133063 Amelioratory effect of flavonoids rich Pergularia daemia extract against CFA induced arthr 2016 May PURPOSE: Pergularia daemia Forsk. (Asclepiadaceae) is a traditionally reported medicinal herb used to treat joint pain and arthritis. However, there are no scientific reports about anti-arthritic activity of P. daemia methanolic extract on rats as animal model. This study identifies bioactive compounds present in the P. daemia methanolic extract and evaluates its anti-arthritic potential in CFA induced arthritic rats. METHODS AND RESULTS: Phytoconstituents of P. daemia extract were examined using LC-ESI/MS method. Anti-arthritic activity of P. daemia extract was determined by various biochemical experiments (RF, ESR and CRP), ultrasonography and histological analysis. LC-ESI/MS analysis resulted in the identification of major flavonoids compounds such as formononetin, qurecetin, chrysoeriol, taxifolin and naringenin. Serum biomarker analysis, after the treatment with PDME (500mg/kg b.w.) revealed that the hemoglobin (11.84±0.42g/dL) and RBC (8.38±0.67million/mm(3)) levels were significantly increased whereas WBC (8.91±0.38thousands/mm(3)), RF (17.94±0.45IU/mL), ESR (7.91±0.12mm/h) and CRP (22.56±0.26mg/L) levels were decreased when compared with the CFA induced arthritic control group. Histology results revealed that treatment with PDME has resulted in significant prevention against bony destruction by decreasing soft tissue swelling and narrowing of joint spaces (250 and 500mg/kg b.w.). CONCLUSION: Anti-arthritic effect of P. daemia might be due to the presence of these bioactive flavonoids. These findings lend pharmacological support to the reported folkloric use of P. daemia in the treatment and management of painful, arthritic inflammatory conditions.
27846748 Beneficial effects of natural eggshell membrane (NEM) on multiple indices of arthritis in 2017 Sep OBJECTIVES: This study was performed to evaluate the potential efficacy of natural eggshell membrane (NEM) in collagen-induced arthritic rats, a well-established rodent model of inflammation and rheumatoid arthritis. METHODS: Rats with developing type II collagen-induced arthritis (CIA) were treated once daily by oral gavage on study days -14 to 17 with vehicle or NEM (52 mg/kg body weight). Rats were euthanized on study day 17. Efficacy was assessed by daily ankle caliper measurements, ankle diameter expressed as area under the curve (AUC(d0-17)), and histopathologic evaluation of ankles and knees. Serum biomarkers of cartilage function and inflammation [collagen type II C-telopeptide (CTXII), cartilage oligomeric matrix protein (COMP), and alpha-2-macroglobulin (A2M)] were measured by ELISA. RESULTS: Treatment with NEM resulted in significant beneficial effects on the daily ankle diameter measurements and ankle diameter AUC. Ankle and knee histopathology scores were significantly reduced (36% and 43% reduction of summed individual histopathology scores for ankle and knee, respectively; p < 0.05) toward normal for rats given NEM compared to vehicle controls. The percent reduction of serum CTXII, COMP, and A2M in NEM-treated rats ranged from 30% to 72% (p < 0.05). CONCLUSIONS: NEM significantly improved multiple aspects of inflammatory arthritis including inflammation, pannus, cartilage damage, bone resorption, and periosteal bone formation. This study provides further support for the use of CTXII, COMP, and A2M as relevant biomarkers that were responsive to NEM.
25424319 Aldehyde modification and alum coadjuvancy enhance anti-TNF-α autovaccination and mitigat 2015 May Experimental vaccination to induce antibodies (Abs) capable of cytokine antagonism shows promise as a novel immunotherapy for chronic inflammatory disease. We prepared a hybrid antigen consisting of residues 141-235 of rat TNF-α fused to the C-terminus of glutathione-S-transferase (GST), chemically modified to incorporate aldehyde residues, for development of an auto-vaccine eliciting anti-rTNF-α Abs. In rat immunization the soluble aldehyde-modified fusion protein did not generate observable Ab responses. By contrast, vaccination with the aldehyde-modified fusion protein adsorbed on alum induced anti-TNF-α autoAbs with high titer and neutralizing activity. Induction of adjuvant arthritis in rats pre-immunized with unmodified fusion protein or a control protein in alum resulted in severe inflammation and joint damage, whereas the disease induced in rats immunized with the aldehyde-bearing fusion protein in alum was markedly attenuated. Similar results were obtained in a collagen-induced rat arthritis model. Anti-collagen II IgG Ab titers did not deviate significantly in groups pre-immunized with modified fusion protein and control protein, suggesting that anti-TNF vaccination did not skew the immune response related to disease induction. This study demonstrates synergy between particulate alum and protein bound carbonyl residues for enhancement of protein immunogenicity. The antigen-specific co-adjuvant system could prove advantageous for breaking tolerance in emerging auto-vaccination therapies targeting inflammatory cytokines as well as for enhancing a broader category of subunit vaccines. Aldehyde adduction introduces a minimal modification which, together with the established use of alum as a safe adjuvant for human use, could be favorable for further vaccine development.
25341505 MRI pattern of arthritis in systemic lupus erythematosus: a comparative study with rheumat 2015 Feb OBJECTIVE: In this study we aimed to describe the magnetic resonance imaging (MRI) pattern of the distribution of bone marrow edema (BME) and joint erosion in hands and wrists of patients with systemic lupus erythematosus (SLE) with arthritis in comparison with rheumatoid arthritis (RA) and healthy subjects (H). METHODS: SLE patients with arthritis (n = 50), patients with RA (n = 22), and H (n = 48) were enrolled. Every patient underwent a non-dominant hand (2nd-5th metacarpophalangeal joints) and wrist MRI without contrast injection with a low-field extremity dedicated 0.2-Tesla instrument. RESULTS: BME was observed in two SLE patients in the hand (4%) and in 15 in the wrist (13%) versus three (30%), and 14 (63%) RA patients. No BME was found in H. Erosions were observed in the hand in 24 SLE patients (48%), 15 RA patients (68%), and 9 H (18 %); in the wrist, in 41 (82%) SLE, all RA and 47 (97%) H. The cumulative erosive burden in SLE was significantly higher than in H (c = 0.002) but similar to RA patients. CONCLUSIONS: Joint involvement of the wrist in SLE is similar to RA and is not as rare as expected, as shown by the comparison with healthy subjects. On the contrary, the involvement of the hand in SLE is significantly lower compared to RA.
25680227 Sjögren's syndrome complicated by interstitial cystitis: A case series and literature rev 2015 Jul OBJECTIVES: To characterize the interstitial cystitis (IC) associated with Sjögren's syndrome (SS). METHODS: Report of three new cases. Only cases fulfilling the American-European consensus criteria for SS and the European Society for the Study of Interstitial Cystitis criteria with positive histological findings for IC were included. RESULTS: Thirteen cases of SS and IC have been reported in women, including the three reported here, with a mean age of 54 years. SS appeared first in 77% (n=10) of cases, a mean of 6.6 years before IC. The symptoms of IC included pollakiuria (n=11), lower abdominal pain (n=8), urinary urgency (n=5), painful micturition (n=6), hematuria (n=3) and dysuria (n=3). Urinary dilatation occurred in three cases, leading to acute renal failure in two patients. The diagnosis of IC was confirmed by anatomical evidence of cystitis inflammation on bladder biopsy in all (n=13) patients. Treatment was reported for nine patients, seven of whom (78%) received corticosteroid treatment, which was partially or completely effective in six cases. Immunosuppressive treatment was added in three cases (cyclosporine, n=2; azathioprine, n=1; cyclophosphamide, n=1). Local bladder treatments were performed, with hydraulic distension in five cases and DMSO instillation in one patient. A urinary catheter was inserted in the two cases of acute obstructive renal failure. CONCLUSIONS: Urinary symptoms without infection should lead the physician to consider a diagnosis of IC in SS patients. Urinary dilatation may occur, leading to acute obstructive renal failure. Corticosteroid treatment may be effective and local treatments have been tried.
24820476 Effect of laser acupuncture on salivary flow rate in patients with Sjögren's syndrome. 2015 Aug Sjögren's syndrome (SS) is a multisystem autoimmune disease characterized by hypofunction of the salivary and lacrimal glands, frequently relieved with symptomatic treatments, such as saliva substitutes, eye lubricants, and cholinergic stimulators. The aim of this pilot randomized placebo-controlled study was to estimate the effects of laser acupuncture on salivary flow rates in patients with severe hyposalivation due to SS. A prospective cohort of 26 female patients affected by SS has been evaluated. The laser therapy equipment used was the Pointer Pulse, emitting light in the red visible spectrum (650 nm), with a power of 5 mW and an irradiation time of 120 s per acupoint, in an area of 3.14 mm(2) (fluence = 19.2 J/cm(2), power density = 0.16 W/cm(2), total dose = 0.6 J). The following acupuncture points were stimulated bilaterally: LI 2 Erjian, ST 5 Daying, ST 6 Jiache, ST 7 Xiaguan, SI 19 Tinggong, and BL 13 Feishu. True laser acupuncture led to a significantly higher amount of saliva production, measured after the end of the protocol (5 weeks), and during the 6-month follow-up period. The results are stable from the end of the protocol until the 3rd month of follow-up; during the last control, a slight but significant decrease in production has also been shown. This preliminary study proposes laser acupuncture as a possible treatment for improving salivary flow rates in patients with SS, but further validation on a larger sample is still necessary.
29741821 Profile of Rheumatological Manifestations in Leprosy in a Tertiary Care Hospital from Hima 2016 Jan The rheumatological manifestations of leprosy occur singly or in varying combinations, particularly during lepra reactions. Despite being third most common, these remain under diagnosed and under reported. This study has been carried out to study the spectrum of rheumatological manifestations in leprosy patients. One hundred consecutive patients of leprosy presenting during January to December 2013 were studied for rheumatological manifestations. Complete hemogram, serum biochemistry, urinalysis, rheumatoid factor, ASO titer, C-reactive protein, ANA, and x-rays for hands, feet, chest and involved joints were performed. These 100 (M:F 66:34) patients aged between 16-80 years had indeterminate (2 patients), TT (4 patients), BT (26 patients), BB (2 patients), and LL leprosy (32 patients). 27 patients had rheumatological manifestations; arthritis involving large or small joints in 23 patients being the commonest. 7 of 24 patients in type-1 lepra reaction had enthesitis in 3 patients and oligoarthritis in 4 patients. Rheumatoid arthritis-like polyarthritis was noted in 19 patients with type-2 reaction. Tenosynovitis, dactylitis, bony changes were also noted. Except for one case, these features were present in patients having lepra reactions. Rheumatoid factor in 14, ANA in 15, C-reactive protein in 45 cases was positive. ASO was positive 34 cases. Symmetrical polyarthritis involving small joints of hands and feet, oligoarthritis, enthesitis and dactylitis are common in leprosy particularly with borderline leprosy, type-2 lepra reaction especially in the presence of positive RAfactor.
27383445 Sjögren syndrome. 2016 Jul 7 Sjögren syndrome (SjS) is a systemic autoimmune disease that primarily affects the exocrine glands (mainly the salivary and lacrimal glands) and results in the severe dryness of mucosal surfaces, principally in the mouth and eyes. This disease predominantly affects middle-aged women, but can also be observed in children, men and the elderly. The clinical presentation of SjS is heterogeneous and can vary from sicca symptoms to systemic disease (characterized by peri-epithelial lymphocytic infiltration of the affected tissue or the deposition of the immune complex) and lymphoma. The mechanism underlying the development of SjS is the destruction of the epithelium of the exocrine glands, as a consequence of abnormal B cell and T cell responses to the autoantigens Ro/SSA and La/SSB, among others. Diagnostic criteria for SjS include the detection of autoantibodies in patient serum and histological analysis of biopsied salivary gland tissue. Therapeutic approaches for SjS include both topical and systemic treatments to manage the sicca and systemic symptoms of disease. SjS is a serious disease with excess mortality, mainly related to the systemic involvement of disease and the development of lymphomas in some patients. Knowledge of SjS has progressed substantially, but this disease is still characterized by sicca symptoms, the systemic involvement of disease, lymphocytic infiltration to exocrine glands, the presence of anti-Ro/SSA and anti-La/SSB autoantibodies and the increased risk of lymphoma in patients with SjS.
26960645 Adult-onset Still's disease with myocarditis and hemophagocytic lymphohistiocytosis: Rare 2016 Jan Adult-onset Still's disease (AOSD) is a rare inflammatory disorder of unknown etiology characterized by fever, evanescent pink salmon rash, arthritis, and multiorgan involvement. Here, we report an unusual manifestation of AOSD in a 40-year-old male who presented to our hospital with pyrexia of unknown origin and rash of 3 weeks duration. All his serological investigations and imaging studies were unremarkable. He was fulfilling clinical and laboratory criteria as per Yamaguchi for AOSD and was managed for the same. Our patient did not respond well to the treatment, had a downhill course, and succumbed to his illness. Autopsy confirmed myocarditis and florid bone marrow reactive hemophagocytosis as the cause of his death.
26505667 Temperatures of the Ocular Surface, Lid, and Periorbital Regions of Sjögren's, Evaporativ 2016 Jan PURPOSE: To compare the temperatures of the ocular surface, eyelid, and periorbital skin in normal eyes with Sjögren's syndrome (SS) eyes, evaporative dry eyes (EDE), and aqueous deficient dry eyes (ADDE). METHODS: 10 eyes were analyzed in each age-matched group (normal, SS, EDE, and ADDE). A noninvasive infrared thermal camera captured two-dimensional images in three regions of interest (ROI) in each of three areas: the ocular surface, the upper eyelid, and the periorbital skin within a controlled environmental chamber. Mean temperatures in each ROI were calculated from the videos. Ocular surface time-segmented cooling rates were calculated over a 5-s blink interval. RESULTS: Relative to normal eyes, dry eyes had lower initial central OSTs (SS -0.71°C, EDE -0.55°C, ADDE -0.95°C, KW P<.0001) and lower central upper lid temperatures (SS -0.24°C, ADDE -0.51°C, and EDE -0.54°C, KW P<.0001). ADDE eyes had the lowest initial central OST (P<.0001), while EDE eyes had the lowest central lid temperature and lower periorbital temperatures (P<.0001). Over the 5-s interblink interval, the greatest rate of temperature loss occurred following eyelid opening, but varied by group (normals -0.52, SS -0.73, EDE -0.63, and ADDE -0.75°C/s). The ADDE group also had the most substantial heat loss over the 5-s interblink interval (-0.97°C). CONCLUSIONS: Differences in OST may be related to thermal differences in lids and periorbita along with an altered tear film. Thermography of the ocular surface, lids, and surrounding tissues may help to differentiate between different etiologies of dry eye.
26197421 Systemic lupus erythematosus: An update for ophthalmologists. 2016 Jan Systemic lupus erythematosus (SLE) is a life-threatening multisystem inflammatory condition that may affect almost any part of the eye. We provide an update for the practicing ophthalmologist comprising a systematic review of the recent literature presented in the context of current knowledge of the pathogenesis, diagnosis, and treatment of this condition. We review recent advances in the understanding of the influence of genetic and environmental factors on the development of SLE. Recent changes in the diagnostic criteria for SLE are considered. We assess the potential for novel molecular biomarkers to find a clinical application in disease diagnosis and stratification and in the development of therapeutic agents. We discuss limited forms of SLE and their differentiation from other collagen vascular disorders and review recent evidence underlying the use of established and novel therapeutics in this condition, including specific implications regarding monitoring for ocular toxicity associated with antimalarials.
27615015 Upper gastrointestinal bleeding in a patient with Sjögren syndrome. 2016 Sep A 68-year-old female patient with Sjögren syndrome was admitted with asthenia, anorexia and weight loss. On the third day of admission, she developed severe hematemesis. Urgent upper gastrointestinal endoscopy (UGE) showed multiple gastric and duodenal ulcers, some with active bleeding requiring endoscopic therapy. The anatomopathologic examination was compatible with gastric and duodenal infiltration by a diffuse CD20+ large B cell lymphoma with germinal center B cell-like morphology. And the patient was referred for chemotherapy with rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone. Although seldom observed, the risk of lymphoma in Sjögren syndrome is up to 44 times higher than the general population and rises 7 years after diagnosis. We present a brief case report with interesting iconography.
26550578 Contribution of Genetic Factors to Sjögren's Syndrome and Sjögren's Syndrome Related Lym 2015 We aimed to summarize the current evidence related to the contributory role of genetic factors in the pathogenesis of Sjögren's syndrome (SS) and SS-related lymphoma. Genes within the major histocompatibility complex (MHC) locus previously considered conferring increased susceptibility to SS development have been also revealed as important contributors in recent genome wide association studies. Moreover, genetic variations outside the MHC locus involving genes in type I interferon pathway, NF-κB signaling, B- and T-cell function and methylation processes have been shown to be associated with both SS and SS-related lymphoma development. Appreciating the functional implications of SS-related genetic variants could provide further insights into our understanding of SS heterogeneity, allowing the design of tailored therapeutic interventions.
26412810 Rate, risk factors and causes of mortality in patients with Sjögren's syndrome: a systema 2016 Mar OBJECTIVES: There is conflicting evidence regarding prognosis in patients with primary SS (pSS). The aim of this study was to estimate the rate, risk factors and causes of mortality in patients with pSS through a systematic review and meta-analysis. METHODS: Through a systematic review of multiple databases through October 2014, we identified cohort studies reporting relative risk (compared with standardized population), risk factors and causes of mortality in patients with pSS. We estimated summary risk ratios (RRs) with 95% CIs using random effects model. RESULTS: We identified 10 studies with 7888 patients (91% females) with pSS, of whom 682 patients died over a median average follow-up of 9 years. The pooled standardized mortality ratio in patients with pSS was 1.38 (95% CI 0.94, 2.01). Leading causes of mortality were cardiovascular diseases, solid-organ and lymphoid malignancies and infections; however, it is unclear whether these observed causes were overrepresented in patients with pSS as compared with the general population. Risk factors associated with increased mortality were advanced age at diagnosis [RR 1.09 (95% CI 1.07, 1.12)], male sex [RR 2.18 (95% CI 1.45, 3.27)], parotid enlargement [RR 1.81 (95% CI 1.02, 3.21)], abnormal parotid scintigraphy [RR 2.96 (95% CI 1.36, 6.45)], extraglandular involvement [RR 1.77 (95% CI 1.06, 2.95)], vasculitis [RR 7.27 (95% CI 2.70, 19.57)], anti-SSB positivity [RR 1.45 (95% CI 1.03, 2.04)], low C3 [RR 2.14 (95% CI 1.38, 3.32)] and C4 [RR 3.08 (95% CI 2.14, 4.42)] and cryoglobulinaemia [RR 2.62 (95% CI 1.77, 3.90)]. CONCLUSION: pSS is not associated with an increase in all-cause mortality as compared with the general population. However, a subset of patients with extraglandular involvement, vasculitis, hypocomplementaemia and cryoglobulinaemia may be at increased risk of mortality and require close follow-up.
26289759 [A case of motor dominant neuropathy and focal segmental glomerulosclerosis associated wit 2015 A 49-year-old woman was admitted to our hospital with gradually progressive weakness of the limbs for about 20 days. She presented with weakness of the limbs, predominantly in the proximal portion, and slight dysesthesia of the limbs, predominantly in the distal portion. Repeated nerve conduction examination revealed axonopathy dominantly in the motor neurons. Therefore, we suspected her as having Guillain-Barré syndrome, and initiated intravenous administration of high-dose immunoglobulin. However, her symptoms progressed gradually and finally she found it difficult to walk. Her urine analysis simultaneously demonstrated albuminuria, and a kidney biopsy indicated focal segmental glomerulosclerosis. At that point, laboratory examination showed high levels of anti SS-A antibody and salivary gland biopsy revealed infiltration of a significant number of lymphocytes around the gland, which led to the diagnosis of Sjögren's syndrome. We considered the etiology of the neural and renal dysfunction as due to the inflammatory mechanism associated with Sjögren's syndrome. Therefore, we administered a second course of immunoglobulin therapy and steroid therapy, which included both pulse and oral administration. Her neurologic symptoms and albuminuria improved rapidly after steroid therapy. The present case indicates that both motor dominant neuropathy and focal segmental glomerulosclerosis can occur in patients with Sjögren's syndrome.
27511335 Minor salivary gland evaluation: Sjögren's syndrome. 2016 Jan 5 BACKGROUND/AIM:  We aimed to analyze the value of 3 serial sections, spaced 200 µm apart, for quantification of lymphocyte and plasma cell foci in minor salivary gland biopsy (MSGB). MATERIALS AND METHODS:  Labial MSGBs from 69 patients with Sjögren's syndrome (SS) and scleroderma were used for this study. Each sample was prepared as 3 serial sections spaced 200 µm apart. Lymphocytic and plasma cell focus score (LFS, PFS) were determined for each section, and the diagnostic results were compared to those obtained from a single section. RESULTS: For 22 of the 69 patients, all 3 sections were scored at <1 and interpreted as inconclusive for the presence of SS. For 20 cases, all 3 sections were scored at ≥1 and interpreted as diagnostic for SS. In the remaining 27 cases, the score was found to vary between sections. Plasma cell foci were observed in 11 cases, with 5 cases exhibiting a PFS of ≥1. Of those 5 cases, 4 also had a LFS of ≥1. CONCLUSION:  Assessment of 3 serial sections in MSGB has the potential to improve accuracy of SS diagnosis by detecting specific features that may not have been detected in a single section. We concluded that data about the PFS require further evaluation.
26179502 Anti-centromere antibody-positive Sjögren's syndrome: A distinct clinical subgroup? 2015 Sep AIM: To investigate whether patients with Sjögren's syndrome (SS) can be distinguished based on the positivity of anti-centromere antibody (ACA), and if so, whether the subgroups differ in their clinical and laboratory features. METHODS: Eleven patients with ACA-positive and 71 patients ACA-negative SS were examined. All patients had minor salivary gland biopsy; sociodemographic data, glandular and extraglandular manifestations, and laboratory findings, including autoantibodies, complement and immunoglobulin levels, were analyzed. European League Against Rheumatism SS disease activity index (ESSDAI) and SS disease damage index (SSDDI) were also measured. RESULTS: The prevalence of ACA among SS patients was 13.4%. ACA-positive SS patients had a higher prevalence of Raynaud's phenomenon, sclerodactyly and autoimmune thyroiditis and a lower prevalence of anti-SSA/Ro and anti-SSB/La antibodies compared to ACA-negative patients. Disease activity was higher in ACA-positive patients than in ACA-negative patients, but the damage index did not differ between the two groups. None of the patients who originally had ACA evolved to having full-blown systemic sclerosis. CONCLUSION: Patients with SS who have ACA differ from classic SS patients in several clinical and laboratory parameters. ACA should be considered one of the pathogenically relevant autoantibodies for SS.