A Case Report of Recurrent Hypokalemic Quadriparesis in the Setting of Distal Renal Tubular Acidosis Preceding Typical Sicca Symptoms in Primary Sjögren's Syndrome.

Sjögren's syndrome (SS) is an autoimmune disorder with glandular and extra glandular manifestations. The extra glandular manifestations include renal symptoms, primarily tubulointerstitial nephritis (TIN), while the glandular component involves the lymphocytic infiltration of exocrine glands. We describe the case of a 28-year-old woman who experienced two bouts of sub-acute onset recurrent flaccid quadriparesis in four months. Following the initial onset of quadriparesis, additional symptoms emerged, including dysphagia, dry mouth, and dry eyes, which raised the suspicion of underlying SS. Examination revealed a positive urine anion gap suggestive of distal renal tubular acidosis (DRTA), high urine pH, and severe hypokalemic hyperchloremic metabolic acidosis. The Schirmer test was positive after five minutes, and an additional workup confirmed positive anti-Ro and anti-La antibodies. A tissue biopsy collected from the sublingual salivary gland indicated lymphocytic infiltration, acinar atrophy, ductal dilatation, and epimyoepithelial cell islands on histological analysis, confirming SS to be the underlying cause of the symptoms. We stress in our conclusion that SS can also initially manifest without the classic symptoms of sicca and that it should be taken into consideration in cases of recurrent hypokalemic flaccid quadriparesis in the context of DRTA.

Are ultrasonographic scoring systems of the salivary gland in primary Sjögren's syndrome suitable for examination of Type2 diabetes mellitus patients with sicca?

OBJECTIVE: This study aimed to compare the salivary gland ultrasonography(SGUS) findings in patients with primary Sjögren's Syndrome (pSS) and diabetes mellitus(DM) patients with sicca symptoms and to examine the relationship between salivary gland ultrasonography (SGUS) findings with clinical and laboratory parameters. METHODS: In this study, 34 patients with pSS and 34 DM patients with sicca symptoms were included. In all patients, bilateral parotid, and submandibular gland ultrasonography (totally 272 glands) was performed by blinded rheumatologist, using the Hocevar and the Outcome Measures in Rheumatology (OMERACT) scoring system. Clinic and ultrasonographic variables were compared between groups. The association between SGUS score and disease duration was analyzed by correlation analysis. RESULTS: Patients with pSS presented significantly higher SGUS scores than patients with DM (the Hocevar score; 20.93(± 9.65) vs. 3.82(± 3.71); p < 0.05, the OMERACT score; 5.96(± 2.30) vs. 2.07(± 1.65); p < 0.05, respectively). In patients with pSS, the submandibular gland scores were significantly higher than the parotid gland scores (right; p < 0.05 vs. left; p < 0.01) while DM patients showed significantly higher parotid gland scores (right; p < 0.05 vs. left; p < 0.05). In pSS patients, the SGUS scores were associated with disease duration (r = 0.57; r = 0.50; p < 0.05), symptom duration (r = 50; r = 0.47; p < 0.05), and the European League Against Rheumatism Sjögren's Syndrome Patient Reported Index (ESSPRI)-dryness score (r = 0.35, r = 0.36; p < 0.05). However, in DM patients, the SGUS scores are highly correlated with the ESSPRI-dryness (r = 0.74, r = 0.72; p < 0.05) and HbA1C level (r = 0.91, r = 0.86; p < 0.05). CONCLUSIONS: This study demonstrated that major salivary gland involvement was more severe and correlated with disease duration, and submandibular gland was dominantly affected in pSS. Contrarily, in DM patients, salivary gland involvement was milder, parotid dominant and related to level of dryness and HbA1C, rather than disease duration when compared to pSS.

Long-term efficacy of sialendoscopy in treating childhood Sjögren's disease with chronological monitoring by salivary gland ultrasonography: A novel approach.

BACKGROUND: Childhood Sjögren's Disease (cSjD) is an underdiagnosed phenomenon with clinical and pathophysiological nuances in contrast to Sjögren's Disease (SjD) in the adult population. While adults typically experience sicca symptoms, children with cSjD often present with recurrent parotitis, diverse autoantibody profiles, and renal and neurological manifestations. Diagnosis and classification in pediatric rheumatology remain controversial due to the reliance on adult-focused diagnostic criteria and the lack of standardized treatment and understanding of outcomes. The purpose of the paper is to propose a multimodal treatment plan and demonstrate the effectiveness of sialendoscopy in the management of cSjD. CASE PRESENTATION: We present the case of a twelve-year-old female diagnosed with cSjD using the 2016 American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) diagnostic criteria for SjD. In addition to medical management, she underwent sialendoscopy with triamcinolone irrigation under sedation and was monitored for progress via salivary gland ultrasonography (SGUS). Over the course of one year, she demonstrated significant improvement in symptoms, with serial SGUS scores gradually decreasing by five points. CONCLUSIONS: This paper proposes a multimodal treatment plan involving sialendoscopy and medical management as a non-invasive and potentially more effective approach for cSjD. Standardized monitoring through SGUS scoring allows objective and quantifiable measurement of treatment progress, enabling better assessment of glandular tissue status. Recurrence is possible, and each cSjD patient may present differently. Nevertheless, our year-long observation of a patient with cSjD demonstrates that sialendoscopy, as seen in adults, can promote remission of recurrent parotitis in children as well.

Time-Dependent Analysis of Sicca Symptoms and Anti-Ro/SSA and Anti-La/SSB Antibodies in Patients with AQP4-IgG-Positive Neuromyelitis Optica Spectrum Disorder.

Anti-aquaporin-4 antibody (AQP4-IgG)-positive neuromyelitis optica spectrum disorder (NMOSD) and Sjögren syndrome (SS) are likely comorbidities. However, the exact effects of age and disease duration on the positivity rates of serum anti-Ro/SSA and anti-La/SSB (anti-SSA/SSB) antibodies and the presence of sicca symptoms in patients with AQP4-IgG remain unknown. In the present study, we evaluated the data from patients with suspected NMOSD who had neurological episodes and tested for serum AQP4-IgG. Associations between the presence of serum AQP4-IgG and SS-related findings were evaluated. The presence of anti-SSA/SSB antibodies [odds ratio (OR), 7.34; 95% confidence interval (CI), 5.71-9.43; p < 0.0001] and that of sicca symptoms (OR, 2.08; 95% CI, 1.67-2.58; p < 0.0001) were both higher in patients with AQP4-IgG (n = 1,651) than in those without AQP4-IgG (n = 2,796). Meanwhile, neither age nor the elapsed time from neurological onset was linked to the prevalence of anti-SSA/SSB antibodies or sicca symptoms, and the prevalence rates of the SS-related factors were elevated since the onset of neurological episodes in those with AQP4-IgG. The frequency of sicca symptoms among those with anti-SSA/SSB antibodies was irrespective of AQP4-IgG (OR, 1.11; 95% CI, 0.67-1.85; p = 0.6892). The measured AQP4-IgG titers did not differ significantly according to the presence of anti-SSA/SSB antibodies (p = 0.2386; Mann-Whitney U test). In summary, age and duration of NMOSD were not the factors producing an elevated prevalence of anti-SSA/SSB antibodies and sicca symptoms in patients with AQP4-IgG, implying that the occurrence of comorbid SS is likely to temporarily precede or synchronize with the onset of AQP4-IgG-positive NMOSD.

[Practicable diagnostics of Sjögren's syndrome in interstitial lung disease-A discussion article]. / Praktikable Sjögren-Diagnostik bei interstitieller Lungenerkrankung ­ ein Diskussionsbeitrag.

Sjögren's syndrome (SjS) is a possible autoimmune cause of interstitial lung disease. The diagnostic pathway for SjS, however, is largely undefined in comparison to other systemic autoimmune diseases. Subjective sicca symptoms, anti-SS-A/Ro antibodies and even ANA as screening tests all have relevant limitations in sensitivity and/or specificity. Against this background, in an interdisciplinary discussion we have developed a consensus for the clarification of SjS, which is presented here for broader discussion. In addition to ANA and anti-SS-A/Ro antibodies, antibodies against alpha-fodrin should be included. Objective measures of dryness, such a Schirmer and Saxon tests are important, as is a salivary gland biopsy in the absence of typical autoantibodies.

Sialendoscopy enhances saliva production of parotid glands in primary Sjögren syndrome patients.

OBJECTIVE: Primary Sjögren syndrome (pSS) is a chronic systemic autoimmune disorder that harms exocrine glands located mainly in the oral and ocular regions. pSS patients often complain about pain and mouth dryness. The aim of this retrospective study was to evaluate the influence of parotid glands' sialendoscopy on salivary flow in pSS patients and to assess the tissue characteristics of the parotid glands during the sialendoscopy procedure. METHOD AND MATERIALS: Twenty-six pSS patients (52 glands) treated with sialendoscopy for their parotid glands between the years 2017 and 2019 were included. The unstimulated whole saliva (UWS) flow rate was obtained 2 weeks before intervention (T1) and 3 months post intervention (T2). For 20 patients, UWS was measured 6 months post intervention (T3); and for 11 patients, UWS was measured 12 months post intervention (T4). Patients were asked about their oral quality of life before and after sialendoscopy. RESULTS: UWS was significantly higher at T2, T3, and T4 compared to T1 (P = .002, P = .01, and P = .04 respectively). In total, 22 patients (84.6%) reported substantial improvement of their oral quality of life at T2. Of the 52 glands, 33 (63.5%) exhibited avascularity in the walls of the Stensen duct, 33 (63.5%) had strictures, and in 8 (15.4%) mucus plugs were present. No major complications were observed in this study except for one patient who suffered from duct perforation, which during follow-up was spontaneously healed. CONCLUSION: This study indicates a positive effect of sialendoscopy on salivary flow rate and oral quality of life in pSS patients. Sialendoscopy should be considered as a vital tool in treating pSS patients. (Quintessence Int 2023;54:234-240; doi: 10.3290/j.qi.b3609681).

Clinical and laboratory evaluation of sicca complaints: distinctive aspects of primary, secondary and non-Sjogren syndrome.

BACKGROUND: Sjögren Syndrome (SS) is a systemic autoimmune disease with a wide spectrum of manifestations that can lead to misdiagnosis. This study describes and compares demographic, clinical, serological, and histopathological data from subjects with SS and non-Sjögren Syndrome (NSS). It also details specific features within the primary SS (pSS) and secondary SS (sSS) groups identifying sub-groups. METHODS: The sample included individuals referred to an academic medical center in Brazil for investigation of SS from 2012 to 2020. Patients were retrospectively classified as primary SS (pSS), secondary SS (sSS), or NSS, based on the American-European Consensus Group criteria (AECG-2002), after multi-professional clinical and laboratory evaluation. RESULTS: A total of 676 individuals were screened and 510 (75.4%) completed the assessments; 198 patients were classified as pSS, 149 as sSS, and 163 as NSS. Symptoms and glandular dysfunction tests were similar in the groups. Concerning pSS, extraglandular manifestations were present in 59% of patients; the elderly had more dry symptoms and peripheral neurological disorders; and 2.5% developed non-Hodgkin lymphoma. In sSS, each overlap promoted distinct clinical and laboratory variants. Several alternative diagnoses were identified as a cause of sicca complex in NSS group. CONCLUSIONS: The diagnosis of SS remains a challenge behind dryness. Up to 31% of the suspected cases had other conditions associated to the symptoms. Histopathological analysis of LSG and SSa determined the diagnostic. Aging in pSS and overlap disease in sSS were responsible for distinct phenotypes and characteristic sub-groups in SS.

Familial Neuromyelitis Optica and Sjogren's Overlap Syndrome-A Rare Case Report.

Familial cases of NMO are rare; and there are very few reports in association with Sjogren's syndrome. To report a familial case of NMO-Sjogren's Overlap syndrome. Mother and daughter presented with phenotypically different neurological episodes; mother had recurrent optic neuritis and myelitis with brainstem episode while the daughter had diencephalic presentation. Both were found to have AQP4 positivity with Ro-52 antibodies and positive Schirmer's test. They responded to steroids and plasma exchange. NMO associated with Sjogren's syndrome association suggests that apart from ethnicity both may have similar genetic predisposition and HLA-linkage.

Instruments for Outcome Evaluation of Specific Domains in Primary Sjögren's Syndrome.

Primary Sjögren's syndrome (pSS) is a systemic autoimmune disorder characterized by very heterogeneous features. The spectrum of this disorder may vary from benign but disabling symptoms such as dryness, due to lachrymal and salivary involvement, pain and fatigue, to systemic, potentially severe, manifestations that may involve any organ. In recent decades, the arrival of biotechnological therapy has offered new opportunities for the treatment of this-until now-orphan disease. Currently, the possible use of these new drugs in therapeutic trials has made it necessary to have reliable outcome measures to evaluate their efficacy in this disease. A great effort has been made in multicenter, often multinational, studies to develop and validate instruments capable of assessing the different disease-related features. The adoption in therapeutic trials of the newly developed outcome measures aimed at assessing systemic features and patient reported symptoms has often yielded disappointing results. These negative data have been ascribed, on the one hand, to the trial design not being completely appropriate, and, on the other hand, to the fact that a single instrument may be not sufficient to cover the great clinical heterogeneity of the disease features. There is now growing belief that composite end points that include instruments that are able to assess the various aspects of the disease may be more properly and successfully used in future therapeutic trials.

The Diagnostic Performance of Early Sjögren's Syndrome Autoantibodies in Juvenile Sjögren's Syndrome: The University of Florida Pediatric Cohort Study.

Objectives: The aim of this study was to evaluate the clinical validity of early Sjögren's syndrome (SS) autoantibodies (eSjA), which were originally marketed for early diagnosis of SS, for juvenile SS (JSS) in a recently identified pediatric cohort. Methods: A total of 105 symptomatic subjects with eSjA results available were evaluated at the Center for Orphaned Autoimmune Disorders at the University of Florida and enrolled for this study. JSS diagnosis was based on the 2016 ACR/EULAR SS criteria. Demographic/clinical/laboratory parameters were compared between JSS (n = 27) and non-JSS (n = 78) for % positivity, sensitivity, and specificity of eSjA (SP1, anti-salivary protein; CA6, anti-carbonic anhydrase VI; PSP, anti-parotid secretory protein) and classic SS-autoantibodies (cSjA; ANA, SSA/SSB, RF, and others) either alone or in combination. Associations between eSjA and diagnostic/glandular parameters were also determined by Fisher's exact test. Results: Compared to non-JSS, JSS patients exhibited sicca symptoms demonstrating reduced unstimulated salivary flow rate (USFR) and abnormal glandular features revealed by salivary gland ultrasound (SGUS). Among cSjA, ANA demonstrated the highest sensitivity of 69.2%, while SSA, SSB, and RF showed around 95% specificities for JSS diagnosis. The % positive-SSA was notably higher in JSS than non-JSS (56% vs. 5%). Of eSjA, anti-CA6 IgG was the most prevalent without differentiating JSS (37%) from non-JSS (32%). Sensitivity and specificity of eSjA were 55.6 and 26.9%, respectively. Autoantibodies with potentially applicable specificity/sensitivity for JSS were seen only in cSjA without a single eSjA included. There were no associations detected between eSjA and focus score (FS), USFR, SSA, SGUS, and parotitis/glandular swelling analyzed in the entire cohort, JSS, and non-JSS. However, a negative association between anti-PSP and parotitis/glandular swelling was found in a small group of positive-SSA (n = 19, p = 0.02) whereas no such association was found between anti-PSP-positive compared to anti-PSP-negative. JSS and non-JSS groups differed in FS, USFR, and EULAR SS Patient Reported Index Dryness/Mean in CA6/PSP/ANA, SP1, and SSA-positive groups, respectively. Additionally, a higher FS was found in RF-positive than RF-negative individuals. Conclusions: eSjA underperformed cSjS in differentiating JSS from non-JSS. The discovery of clinical impact of eSjA on early diagnosis of JSS necessitates a longitudinal study.

Multimodal Assessment and Characterization of Sicca Syndrome.

Background: Sicca syndrome represents a heterogeneous group of conditions, such as Sjögren syndrome, causing xerophthalmiaand xerostomia. This study characterizes in depth patients with Sicca syndrome and evaluates salivary gland ultrasound (SGUS). Methods: Principal component analysis and hierarchical clustering of clinical parameters, such as ESSPRI, ESSDAI and laboratory data, were performed on all referrals for assessment of Sicca symptoms between October 2018 and March 2021. SGUS and labial gland biopsies were compared across groups. Results: A total of 583 patients were assessed. Objective dryness was confirmed in 73% of the patients. Cluster analysis identified 3 groups with post-hoc analysis confirming distinct phenotypes: Somatic Group (283/583; 49%) with more frequent symptoms but limited objective dryness; Dry Without Autoimmune Features (DAFneg, 206/584; 35%), and Dry With Autoimmune Features (DAFpos, 94/584;16%). DAFpos patients had highest autoantibody titers (anti-SSA(Ro) 240 vs. 3.6 vs. 3.8; p < 0.001), most extra-glandular manifestations (p < 0.001), and highest median SGUS Score (DAFpos: 8 [IQR 4-10] vs. SG: 2 [1-4] vs. DAFneg 4 [2-5]; p < 0.001). No tangible correlation with primary Sjögren syndrome criteria was observed. Discussion: SGUS score correlated with a subset of patients with Sjögren syndrome, identified in the DAFpos cluster. This study highlights heterogeneity within sicca and, indeed, Sjögren syndrome, highlighting the need for further studies.

Aseptic meningitis as an initial presentation of Sjögren syndrome: a report of two cases and literature review.

Sjögren syndrome (SS) is one of several collagen vascular diseases that occasionally involve the central nervous system. We report two cases of SS involving young patients who initially presented with aseptic meningitis. A male with recurrent AM was found to have anti-Ro/SSA and La/SSB antibodies in a screening test for autoimmune process. A minor salivary gland biopsy revealed lymphocytic infiltrations compatible with SS, although the patient did not exhibit sicca symptoms. A female presenting with AM and polyarthritis also reported xerophthalmia. Anti-Ro/SSA antibody testing and a positive result in a minor salivary gland biopsy led to the diagnosis of SS. In the literature review, we found that AM or aseptic meningoencephalitis (AME) preceded or had a concomitant onset with SS in approximately 70% of cases. Screening for anti-Ro/SSA antibody, as well as systemic assessment for rheumatic symptoms, may be useful for diagnosing AM/AME of unknown etiology.

[Severe polyneuropathy in primary Sjögren's syndrome : Sjögren's syndrome should be considered in patients with motor neuropathy]. / Schwere Polyneuropathie bei primärem Sjögren-Syndrom : Bei Patienten mit motorischen Neuropathien auch an Sjögren-Syndrom denken!

A 64-year-old male patient developed over a period of 20 years a peripheral neuropathy symmetrically affecting the upper and lower limbs. The histological examination of a sural nerve biopsy revealed a severe axonal neuropathy. Despite extensive laboratory investigations including immunological and metabolic tests the origin could not be identified. Finally, a Schirmer test revealed xerophthalmia. A subsequent salivary gland biopsy from the lower lip revealed a grade III lymphocytic inflammation according to Chisholm and Mason and confirmed the diagnosis of Sjögren's syndrome.

Sicca Symptoms, Oral Health Conditions, Salivary Flow and Oral Candida in Sjögren's Syndrome Patients.

This study aimed to investigate the relationships among sicca symptoms, oral health conditions, salivary profiles and oral Candida in Sjögren's syndrome (SS) patients. Eighty-five SS patients (mean age = 50.5) and 40 healthy non-SS individuals (mean age = 51.4) were recruited. They self-completed the Sicca Symptoms Inventory (SSI). Decayed, missing and filled surface (DMFS) scores, salivary flow rates, pH and oral Candida colonization were determined. Mean SSI summary scores of SS patients and non-SS individuals were 11.1 and 5.4 respectively (p < 0.001). The most prevalent sicca symptoms in SS patients were eye irritation (93%), dry throat or nose (88%) and need of fluid for mouth wetting (88%). SS patients had significantly lower whole salivary flow rates than the non-SS individuals. Candida strains were isolated from over 60% of SS patients but not in non-SS patients. C. albicans was the predominant species. SSI summary score was negatively correlated to salivary flow rates while SSI summary and domain scores were positively correlated to the number of filled surfaces (FS) and DMFS scores and oral Candida counts. In conclusion, SS patients had more severe sicca symptoms than non-SS individuals. SSI scores were negatively correlated to the salivary flow rates but positively correlated to caries experience and oral Candida colonization.

Sjögren Syndrome in Systemic Lupus Erythematosus: A Subset Characterized by a Systemic Inflammatory State.

OBJECTIVE: An often-neglected subset of patients with systemic lupus erythematosus (SLE) is those with secondary Sjögren syndrome (SLE-sSS). Further, primary SS overlaps and can be difficult to delineate from SLE. To shed light on the SLE-sSS subset, we investigated a large and well-characterized SLE cohort, comparing patients with SLE-sSS and SLE patients without SS (SLE-nonsSS) and controls. METHODS: We included 504 consecutive patients with SLE, fulfilling the 1982 revised American College of Rheumatology criteria, and 319 controls from the general population, matched for age and sex to the first 319 patients. SLE-sSS was defined according to the American-European Consensus Criteria (AECC). A thorough clinical examination, including subjective and objective quantifications of sicca symptoms, was performed in all participants. Autoantibodies and 20 selected cytokines were measured by luminex and multiplex analysis, respectively. RESULTS: SLE-sSS, as defined by AECC, occurred in 23% of the patients with SLE. In comparison to SLE-nonsSS, the SLE-sSS group was older and more frequently female. Leukopenia and peripheral neuropathy were more frequent and nephritis less frequent. Circulating levels of 6/20 investigated proinflammatory cytokines [tumor necrosis factor-α, interleukin (IL) 6, monocyte chemoattractant protein 4, macrophage inflammatory protein 1ß, IL-12/IL-23p40, and interferon γ-induced protein 10], total IgG, anti-SSA/Ro52, anti-SSA/Ro60, anti-SSB/La antibodies, and rheumatoid factor (IgM and IgA) were higher in the SLE-sSS group (p < 0.05 for all comparisons). CONCLUSION: The frequency of SLE-sSS increased with age and affected roughly one-quarter of all patients with SLE. Despite less internal organ involvement, a systemic inflammatory state with high levels of proinflammatory cytokines is present in the SLE-sSS subgroup. This is a novel observation that may affect future understanding and treatment of the SLE-sSS subset.

Comparison of Effect on Sicca Symptoms of Anticentromere Antibody-positive Sjögren Syndrome and Primary Sjögren Syndrome Alone.

OBJECTIVE: To determine whether positive anticentromere antibody (ACA) serology affects the severity of sicca symptoms in patients with primary Sjögren syndrome (pSS). METHODS: Evaluation to detect subjective and objective sicca symptoms included questionnaires, physical examination, and pathology. Cases of pSS were classified according to the 2002 American-European Consensus Group (AECG) criteria. All patients were evaluated for presence of anti-Ro, anti-La, and ACA serology. Patients with pSS were categorized into ACA+ SS and ACA-SS. The groups were compared for measures of severity of oral and ocular sicca. RESULTS: The pSS group had 446 patients, of whom 26 were ACA+ SS. Subjective ocular sicca measured 7.0 ± 2.4 (out of 10) in ACA+ SS and 6.4 ± 2.6 in ACA-SS (p = 0.197). Objective ocular sicca measured 3.2 mm ± 1.8 mm/5 min in ACA+ SS and 4.2 mm ± 4.4 mm/5 min in ACA-SS (p = 0.038). Subjective oral sicca measured 8.5 ± 1.4 in ACA+ SS and 6.7 ± 2.4 in ACA-SS (p < 0.001). Objective oral sicca measured 0.1 ml ± 0.2 ml/15 min in ACA+ SS and 0.4 ml ± 1.0 ml/15 min in ACA-SS (p < 0.001). Only 35% of ACA+ patients with SS were anti-Ro-positive or anti-La-positive compared with 77% of the ACA-patients with SS (p < 0.001). There was no significant difference in minor salivary gland fibrosis or focus scores between ACA+ SS and ACA-patients with SS. CONCLUSION: ACA+ SS is associated with more severe objective ocular sicca and more severe subjective and objective oral sicca compared to ACA-SS. The majority of ACA+ patients with SS meet AECG criteria for pSS despite negative serology for anti-Ro/La antibodies.

Neurological Involvement as the Initial Manifestation in Primary Sjögren's Syndrome - A Case Report.

BACKGROUND: Sjögren's syndrome is an autoimmune disease characterised by exocrinopathy mainly involving the salivary and lacrimal glands. In addition, it is a multisystemic condition (i.e., affecting multiple organs and systems). Neurological involvement has been reported in ~20% of cases, with peripheral manifestations being the most frequent. METHODS: We analysed four cases in which neurological manifestations were the first symptoms of Sjögren's syndrome. RESULTS: In all four cases, neurological symptoms preceded sicca symptoms. In addition, immunosuppressive treatment with steroids and, in some cases, cyclophosphamide showed improvement. CONCLUSION: Neurological involvement in Sjögren's syndrome is common and often occurs as the first clinical manifestation. Since evidence is limited, more studies are required in order to determine appropriate diagnostic methods and treatments for each manifestation of Sjögren's syndrome.

Unusual presentation Of Sjögren-associated neuropathy with plasma cell-rich infiltrate.

INTRODUCTION: Sjögren syndrome is thought to be a lymphocyte-driven process. Peripheral nervous system involvement occurs in about 20%-25% of patients. A sensory-predominant, large-fiber peripheral neuropathy is most common, and it is usually associated with a subacute to chronic presentation. METHODS: We report a rare case of an acute Sjögren-associated, sensory predominant, length-dependent peripheral neuropathy mimicking Guillain-Barré syndrome. The patient presented with sensory ataxia preceded by fever and polyarthralgia. She gave a history of years of dry eyes and dry mouth. RESULTS: She had a positive Shirmer test, abnormal salivary gland scan, and positive SS-A and SS-B antibodies. A sural nerve biopsy showed an unusual, dense, non-IgG4, polyclonal, plasma-cell perivascular infiltrate. The patient responded to treatment with weekly pulse intravenous methylprednisolone. CONCLUSIONS: Sjögren syndrome can present with acute-onset, sensory predominant peripheral neuropathy. The role of plasma cells in Sjögren syndrome is unexplored and deserves further study. Muscle Nerve 55: 605-608, 2017.

Secondary Sjogren's Syndrome in 83 Patients With Rheumatoid Arthritis.

UNLABELLED: Sjogren syndrome (SS) can occur alone, primary Sjogren syndrome, or in association with other rheumatic diseases, secondary Sjogren syndrome (sSS), such as Rheumatoid arthritis (RA). The occurrence of Sjogren syndrome with RA makes it course worse and increases high morbidity and mortality of RA. In this exploratory study we aim to determine the prevalence of sSS (diagnosed based on the revised version of American-European consensus Group Classification Criteria: AUCG-criteria), sicca symptoms (dry eye, dry mouth), positive autoantibody tests (Anti RO or Anti-LA antibodies), UWSFR (Unstimulated Whole Salivary Flow Rate), Schirmer and Lissamine test. In this cross-sectional study, eighty three consecutive RA patients (according to American College of Rheumatology criteria 1987) who were visited at rheumatology clinic of Razi General Hospital located in the north of Iran entered into our study. Our exclusion criteria was a positive history of past head and neck radiation treatment, Hepatitis C infection, acquired immunodeficiency disease (AIDS), pre-existing lymphoma, sarcoidosis, graft versus host disease, use of anticholinergic drugs (including neuroleptics, antidepressants, antihypertensive and parasympatholytics). They examined with UWSFR by a rheumatologist and with Schirmer test and Lissamine test by an ophthalmologist. Participants were 90.4% female with the mean age 48.3±13 years. Duration of RA was in 36.1% less than 5 years, in 22.9% 5-10 years, in 12.1% 11-15 years and in 28.9% more than 15 years. Our results demonstrated that the prevalence of sSS was 5.9% (CI:0.6%-10.5%). Number of 27.7% of RA patients positively responded to at least one question about sicca symptoms. Among objective tests, only Positive UWSFR and Lissamine test were significantly more common in RA patients with sSS in comparison to ones without sSS (P<0.001, P=0.01 respectively). In RA patients, we found a linear trend between sicca symptoms and aging (P=0.02). In patients with sicca symptoms, among tests that used for assessing decrease in saliva or tear production, only USWFR significantly more common (P=0.01). IN CONCLUSION: In RA population in North of Iran prevalence of sSS was less than 10%. In them, a significant linear trend existed between aging and sicca symptoms. Among objective tests of AUCG-criteria (except for lip biopsy that was not performed in the current study) only UWSFR and Lissamine test were significantly more common in patients with sSS in comparison ones without it.