The role of albumin-to-globulin ratio in undifferentiated arthritis: Rheumatoid arthritis versus primary Sjögren syndrome

Objectives: This study aims to compare initial albumin-to-globulin ratio (AGR) in patients with rheumatoid arthritis (RA) and primary Sjögren syndrome (pSS) presenting with undifferentiated arthritis (UA) and to investigate whether there was a difference in terms of AGR between the two patient groups and healthy controls. Patients and methods: Between January 2019 and December 2019, a total of 177 patients including 96 RA (10 males, 86 females; mean age: 53.6±10.8 years; range, 21 to 74 years) and 81 pSS (5 males, 76 females; mean age: 53.2±14.1 years; range, 23 to 79 years) and 82 healthy controls (20 males, 62 females; mean age: 50.5±13.6 years; range, 20 to 79 years) were included in this case-control study. Demographic characteristics, albumin, and globulin levels of all participants were recorded. The AGR, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), rheumatoid factor (RF), anti-nuclear antibody (ANA), and anti-citrullinated protein antibodies (ACPA) were assessed. Results: The mean AGR was 1.50±0.16 in the control group, 1.48±0.24 in the RA group, and 1.30±0.23 in the pSS group, indicating a significant difference between the pSS and the other two groups (p<0.001). The receiver operating characteristic analysis revealed that the cut-off value for AGR was 1.39 (area under the curve=0.736) with a sensitivity of 0.642 and a specificity of 0.646 (p<0.001). The ESR and CRP values were higher (p<0.001), and ANA (p<0.001) and RF (p=0.003) positivity were lower in the RA group, compared to the pSS group. Conclusion: This study findings indicate that AGR is a helpful tool in the differential diagnosis of RA and pSS presenting with UA at the time of admission, and Sjögren syndrome should be considered in case of AGR ≤1.39.

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 &lt; 0.001), most extra-glandular manifestations (p &lt; 0.001), and highest median SGUS Score (DAFpos: 8 [IQR 4–10] vs. SG: 2 [1–4] vs. DAFneg 4 [2–5]; p &lt; 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.

Cerebellar degeneration in primary Sjögren syndrome: a case report

Background Cerebellar degeneration is a rare and severe presentation of primary Sjögren syndrome. There are few case reports of cerebellar degeneration associated with different autoimmune diseases, especially with systemic lupus erythematosus and neuro-Behcet’s disease. There are only six patients reported worldwide to be affected by cerebellar atrophy associated with primary Sjögren syndrome. In this report, we describe a patient with primary Sjögren syndrome who presented with ataxia due to cerebellar degeneration. Case presentation We report the case of a 37-year-old Chinese woman with primary Sjögren syndrome who presented with ataxia over 3 months associated with tremor of the limbs. Magnetic resonance imaging of the brain revealed bilateral cerebellar atrophy. Based on the presence of cerebellar signs with magnetic resonance imaging brain findings, she was diagnosed as cerebellar degeneration secondary to primary Sjögren syndrome. She was treated with methylprednisolone, hydroxychloroquine, and two cycles of monthly intravenous cyclophosphamide. Subsequently, she refused further treatment, and her neurological symptoms remained the same upon the last clinic review. Primary cerebellar degeneration is rarely associated with primary Sjögren syndrome. The pathogenesis of the neurological manifestations in primary Sjögren syndrome is unclear. Treatment involves corticosteroids and immunosuppressive agents with no consensus of a specific therapy for the management of primary Sjögren syndrome with central nervous system involvement. Conclusions Cerebellar degeneration is a rare presentation of primary Sjögren syndrome. Early diagnosis and treatment of this condition is needed to ensure a good outcome.

Lipoprotein-associated phospholipase A2 and carotid intima-media thickness in primary Sjögren syndrome

Objectives: This study aims to evaluate serum lipoprotein-associated phospholipase A2 (Lp-PLA2) level and carotid intima-media thickness in primary Sjögren syndrome (pSS) as an indicator of atherosclerosis. Patients and methods: Between July 2019 and July 2020, a total of 33 female pSS patients (mean age: 44.5±11.2 years; range, 23 to 60 years) and 37 female age- and sex-matched healthy individuals (mean age: 40.9±7.2 years; range, 25 to 54 years) were included. Carotid intima-media thickness and serum Lp-PLA2 levels were measured in the patient and control groups. Results: The patients had a higher median serum Lp-PLA2 of 560 (range, 108 to 1,222) ng/mL vs. 328 (range, 0 to 1,280) ng/mL in the controls (p=0.024) and a similar mean intima-media thickness of carotid artery (0.64±0.14 mm vs. 0.62±0.15 mm, respectively; p=0.595). Serum Lp-PLA2 was positively correlated with platelet count (r=0.411, p=0.018) and negatively correlated with erythrocyte sedimentation rate (r=-0.409, p=0.018). The mean value of carotid intima-media thickness was positively correlated with disease duration (r=0.316, p=0.074) and was negatively correlated with the level of leucocyte (r=-0.458, p=0.007). Conclusion: Our study suggests that the patients of pSS have a potential risk of atherosclerotic cardiovascular disease, independent of traditional cardiovascular risk factors and disease severity.

A primer Sjögren-szindróma pszichológiai vonatkozásai

Összefoglaló. A primer Sjögren-szindróma (pSS) krónikus autoimmun betegség, melynek elsődleges tünetei az exokrin mirigyeket érintő autoimmun folyamat következtében létrejövő szem- és szájszárazság, szisztémás manifesztációi között pedig a leggyakoribbak a szellemi és fizikai fáradtság és az ízületi fájdalmak. A betegség lélektani vonatkozásait nagy érdeklődés övezi; közleményünk a vonatkozó kutatási eredményeket foglalja össze a hátterükben húzódó pszichoneuroimmunmodulációs háttér rövid bemutatásával. A pSS pszichológiai tényezői közt a legrégebben vizsgáltak a depresszió és a szorongás. Ezek jelentős hatással vannak a betegek életminőségére, jóllétére, kognitív funkcióira, sőt a betegség aktivitására is. Bemutatjuk továbbá a stressz és a megküzdési stratégiák jellegzetes mintázatait pSS-ben, amelyek közt a betegségre jellemző maladaptív stratégiák a legjelentősebbek a patogenezis megértése és a kezelés szempontjából. Ilyenek a tagadás, a hasítás és a helyettesítés. Kitérünk a Sjögren-szindrómát kísérő személyiségjellemzőkre is, amely ígéretes terület, de kevés adat áll rendelkezésünkre; az eddigi vizsgálatok alapján a neuroticismus a leginkább pSS-re jellemző tényező. A kognitív funkciók érintettségének összefoglalása és az egyéb pszichológiai tényezők (szellemi fáradtság, szexualitás, testképzavar, életminőség) összegzése után egyértelműen levonható a következtetés, hogy a Sjögren-szindróma nemcsak biológiai, hanem pszichés, pszichoszociális és szociális jelenségszinteken is okoz zavarokat, tüneteket. Ezért rendkívül fontos a betegség biopszichoszociális szemléletű kezelése, a betegek aktív, intencionális részvétele a pSS-sel való megküzdésben. Orv Hetil. 2021; 162(39): 1558–1566. Summary. Primary Sjögren syndrome (pSS) is a chronic, autoimmune disorder. Primary symptoms are ocular and oral dryness as a of an autoimmune process affecting the exocrine glands. The most common systemic manifestations of the disease are mental and physical fatigue and arthralgia. Psychological features of pSS are studied with great interest; the present publication reviews the results of the related investigations alongside with the possible psychoneuroimmunomodulatory background. Among psychological factors in pSS, depression and anxiety have been studied the longest. These impact significantly the quality of life, wellbeing, cognitive functions and disease activity of the patients. Afterwards, we introduce the characteristic patterns of stress and coping mechanisms in pSS, among which maladaptive strategies, typical for the disorder are the most important regarding the pathogenesis and the therapy. These pSS-linked maladaptive strategies are denial, disengagement and focus and venting of emotions. As next, we give a summary about personality characteristics in pSS, which is a promising field to study, and yet very few related data are available; based on them, neuroticism seems to be the most common personality factor in Sjögren’s. After summarizing briefly cognitive functions and other psychological features (mental fatigue, sexuality, body image disturbance and quality of life), it is evident that pSS is determined not only by biological but also by psychological, psychosocial and social disturbances. Hence, treating pSS patients with a biopsychosocial perspective is crucial and so is the active and intentional participation of patients in their recovery. Orv Hetil. 2021; 162(39): 1558–1566.