Background:Sjögren’s syndrome (SjS) is a systemic autoimmune disease with a broad clinical presentation from dry syndrome to systemic extraglandular manifestations. The diagnosis can be complex since none of the markers, except anti-Ro, is sufficiently sensitive or specific. Although, minor salivary glands biopsy (MSGB), Schirmer’s test and unstimulated whole salivary flows (UWSF) are the hallmark for the diagnosis of this entity, its use is not widespread in some centers.Objectives:The aim of the study was to analyze the usefulness and safety of the diagnostic protocol for the classification of SjS and the immunological and analytical markers in dry syndrome due to SjS.Methods:Prospective observational study of a cohort of patients with sicca syndrome from a reference center. The diagnostic protocol (Schirmer’s test, UWSF and minimally invasive MSGB) was applied in the same consultation. Demographic, clinical, analytical and histological data were reviewed.Results:Over a period of 6 months, 48 patients with dry syndrome were analyzed, of which 39 women (81.2%). The main suspicion was SjS (39), followed by sarcoidosis (3), IgG4-related disease (2) and other diagnoses (4). The mean age was 59.1±4.4 years. Almost half (45.8%) reported xerostomia and 41.6% xerophthalmia. Recurrent parotidomegaly was described in 6 patients (12.5%) and arthralgias in 12 (25%). Immunologically, 23 (47.9%) presented anti-nuclear antibodies, 13 (27.1%) anti-Ro, 4 (8.3%) anti-La, 12 (25%) rheumatoid factor and 15 (31.2%) low C4. Schirmer test was positive in 32 patients (66.7%), UWSF in 22 (45.8%) and 9 (18.8%) had a focus score ≥1, although 16 (33.3%) had focal lymphocytic sialadenitis in the MSGB. A total of 21 (43,8%) patients were classified according to the 2016 ACR/EULAR criteria. 12 (57.1%) were seropositive SjS and 9 (42.9%) seronegative SjS. MSGB sensitivity was 71% and specificity 96%. Patient reported symptoms were unhelpful to differentiate SjS from other causes of dry syndrome. The number of protocols needed to diagnose a SjS was 2.28 (5.33 in seronegative SjS). Complications associated with the procedure were low (1 of 48) and mild (self-limited paraesthesia). Patients with SjS, unlike those with dry syndrome of another etiology, had more anemia (p<0.001), lymphopenia (p=0.022), ESR (p=0.030), beta-2 microglobulin (p=0.011), ANA (p<0.001), anti-ENA (p=0.006), anti-Ro (p<0.001), low C4 (p<0.001) and hypergammaglobulinemia (p=0.002).Conclusion:Immunological and histological manifestations were more predictive than clinical ones to differentiate SjS from other causes of dry syndrome. MSGB is a simple, sensitive, specific and safe procedure. The application of the diagnostic protocol (Schirmer test, UWSF and MSGB) allowed to standardize the classification of SjS and increased the diagnosis of patients with seronegative SjS.References:[1]Ramos-Casals M, Brito-Zerón P, Bombardieri S On behalf of the EULAR-Sjögren Syndrome Task Force Group, et al. EULAR recommendations for the management of Sjögren’s syndrome with topical and systemic therapies.Annals of the Rheumatic Diseases2020;79:3-18.[2]Guellec D, Cornec D, Jousse-Joulin S, et al. Diagnostic value of labial minor salivary gland biopsy for Sjögren’s syndrome: a systematic review.Autoimmun Rev. 2013;12(3):416–420.Disclosure of Interests:None declared
BackgroundSLICC Damage Index (SDI) index was designed to reflect accrual damage in patients with systemic lupus erythematosus (SLE). It reflects an irreversible change, unrelated to inflammatory activity, that has occurred since the diagnosis of SLE, verified by clinical assessment, and has been present for at least 6 months.ObjectivesOur objective is to determine the factors associated with higher mean SDI and SDI≥1 in patients with SLE in a long-follow-up cohort.MethodsSingle-centre retrospective observational study of SDI in SLE in a Spanish Lupus Cohort (HAPLES cohort: single-center cohort designed for the prospective evaluation of cardiac involvement in SLE). We included 219 SLE patients but 9 were excluded from the study because they did not fulfill the EULAR/ACR 2019 classification criteria.ResultsTwo hundred and ten patients with a mean age at diagnosis of 35.3 years (89.1% women) were analyzed. The mean follow-up time was 15.4 years. Mean SDI was 2.21 (range 0-26). The majority (70,9%) of patients presented an SDI≥1. However, an SDI≥1 was more frequent with longer follow-up (p=0.034): 23.8% in ≤5 years vs 74% in ≥10 years. The mean SDI increases over time (follow-up) (p<0.001): in patients with <5 years since diagnosis is 1.16, between 5-10 years 2.14 and in >10 years of follow-up 2.47. A positive correlation was also found between mean SDI and age (p<0.001), hypertension (p<0.001), diabetes (p=0.004), dyslipidemia (p<0.001), obesity (p=0.045), thrombosis (p<0.001), serositis (p=0.005), renal involvement (p=0.012), neurolupus (p<0.001), cardiac involvement (p<0.001) and antiphospholipid antibodies (aPLs) (p=0.002). A negative (protective) correlation was only found with antimalarials (p=0.002). Patients with SDI=0 (no damage) vs. SDI≥1 (any damage) were compared (Table 1) to evaluate the risk factors of presenting some irreversible accrual damage in the follow-up.Table 1.Damage accrual measured by SDI (SDI=0 vs SDI≥1)SDI=0SDI≥1p valueOR (IC95%)Female sex (%)88.71 %88.51 %p=0.967Late onset SLE (≥50 years) (%)6.45 %23.65 %p=0.0033.43 (1.41-8.32)Obesity (%)6.45 %16.89 %p=0.0452.83 (1.07-7.48)Hypertension (%)24,19 %53.38 %p<0.0012.21 (1.27-3.86)Diabetes (%)1.61 %7.43 %p=0.097Dyslipidemia (%)22.58 %45.27 %p=0.0021.96 (1.10-3.47)Thrombosis (%)3.23 %18.24 %p=0.0046.00 (1.71-21.01)Serositis (%)12.90 %27.03 %p=0.0262.08 (1.01-4.26)Neurolupus (%)8.06 %25.00 %p=0.0053.12 (1.35-7.26)Glomerulonephritis (%)14.52 %24.32 %p=0.114Cardiac involvement (%)15.52 %54.86 %p<0.0013.67 (1.95-6.92)DNAds+ (%)56.45 %63.51 %p=0.338Anti-Sm (%)12.90 %14.86 %p=0.711Anti-Ro (%)29.03 %25.68 %p=0.616Anti-RNP (%)11.29%8.11%p=0.463Antiphospholipid antibodies (%)25.81 %54.05 %p<0.0012.16 (1.25-3.75)LLDAS (%)56.14 %63.51 %p=0.331DORIS (%)41.38%48.65%p=0.347Prednisone (or equivalent) >7.52.94 %12.93 %p=0.0194.33 (1.20-15.67)Antimalarials90.16 %79.73 %p=0.0220.43 (0.17-1.09)Conventional DMARDs (%)37.70%47.97%p=0.175Belimumab (%)1.64%5.41%p=0.223Death (%)0.00 %2.03 %p=0.259ConclusionSDI in our SLE-cohort was correlated with age, vascular risk factors, severe organ involvement, aPLs and steroid use. Only antimalarials were associated with a lower mean SDI. SDI increases significantly with longer follow-up time, especially after the fifth year of follow-up. Prevention and early treatment of the aforementioned risk factors could avoid irreversible organ accrual damage in lupus.References[1]Bruce IN, O’Keeffe AG, Farewell V, et al. Factors associated with damage accrual in patients with systemic lupus erythematosus: results from the Systemic Lupus International Collaborating Clinics (SLICC) Inception Cohort. Ann Rheum Dis. 2015;74(9):1706-1713.[2]Raman L, Yahya F, Ng CM, et al. Early damage as measured by SLICC/ACR damage index is a predictor of hospitalization in systemic lupus erythematosus (SLE). Lupus. 2020;29(14):1885-1891.[3]Johnson SR, Gladman DD, Brunner HI, et al. Evaluating the construct of damage in systemic lupus erythematosus. Arthritis Care Res (Hoboken). 2021;10.1002/acr.24849.Disclosure of InterestsNone declared
BackgroundSystemic lupus erythematosus (SLE) is a highly heterogeneous systemic autoimmune disease with multiorgan manifestations. There are disparities in its manifestations conditioned by sex, age of onset, and serological characteristics. The ethnic origin of the patients may be a conditioning factor for different organic manifestations, immunological markers, and outcomes.ObjectivesOur objective was to evaluate the clinical and serological differences in Caucasian vs. Latin American patients in a multiethnic Spanish single-center SLE cohort.MethodsSingle-centre retrospective observational study from a Spanish Lupus Cohort of adult SLE patients fulfilling the 2019 EULAR/ACR Classification Criteria. Only patients of Caucasian or Latin American origin were included.ResultsThe study included 205 patients: 186 (90.7%) Caucasian and 19 (9.3%) Latin American. The mean age at diagnosis was 35.5 years. The female/male ratio was 9:1 in the Caucasian group and 19:1 in the Latin American group (ns). Serous and cutaneous involvement was similar between groups. The presence of arthralgia was also similar (ns) but there were statistically significant differences with the presence of arthritis (p=0.008). Severe hematologic manifestations were also more frequent in Latin American patients but only statistically significant for autoimmune hemolytic anemia (AIHA). Lupus nephritis and end-stage renal disease (ESRD) were two-fold and four-fold more common in Latin American patients. Among the immunological findings: hypocomplementemia, Anti-Sm, and anti-histones were differential markers between the two groups. Anti-RNP was also more frequent in the Latin American group (ns). Regarding treatments, there were no relevant differences between steroids and antimalarials. There were also no disparities in outcomes measured by activity (SLEDAI), cumulative damage (SDI), low activity (LLDAS), remission (DORIS), or death in our cohort.Table 1.Caucasian vs. Latin American SLE patientsCaucasianLatin Americanp-valueOdds ratio (IC95%)Serositis (%)22.58 %26.32 %p=0.712Skin involvement (%)65.59 %52.63 %p=0.261Arthritis (%)24.32 %52.63 %p=0.0082.39 (1.06-5.38)AIHA (%)0.00 %5.26 %p=0.00228.69 (1.13-728.60)Lymphopenia (%)34.95 %52.63 %p=0.084Immune thrombocytopenia (%)4.30 %10.53 %p=0.230Neurolupus (%)20.97 %10.53 %p=0.278Glomerulonephritis (%)18.82 %42.11 %p=0.0182.51 (1.05-6.02)ESRD (%)3.76 %15.79 %p=0.0204.20 (1.01-17.58)Low C3 (%)54.30 %89.47 %p=0.0031.65 (0.82-3.31)Low C4 (%)49.46 %84.21 %p=0.0041.70 (0.84-3.46)Low vitamin D (%)11.29 %26.32 %p=0.0612.33 (0.79-6.89)High DNAds (%)61.29 %63.16 %p=0.404Anti-Sm (%)12.37 %36.84 %p=0.0042.98 (1.13-7.85)Anti-Ro (%)26.34 %31.58 %p=0.624Anti-RNP (%)8.06 %21.05 %p=0.0632.61 (0.79-8.66)Anti-histones (%)4.30 %21.05 %p=0.0034.89 (1.35-17.77)Antiphospholipid antibodies (%)45.16 %42.11 %p=0.799Steroids (%)88.71 %94.74 %p=0.419Antimalarials (%)94.05 %89.47 %p=0.436SLEDAI≥6 (%)34.41%26.32%p=0.477SDI ≥1 (%)70.43 %68.42 %p=0.855LLDAS (%)60.99 %66.67 %p=0.637DORIS (%)48.09 %38.89 %p=0.456Death (%)1.61 %0.00 %p=0.577ConclusionIn our multiethnic Spanish single-center cohort, SLE patients of Latin American origin had a higher frequency of arthritis, glomerulonephritis, and AIHA. They also presented a higher frequency of hypocomplementemia, anti-Sm, and anti-histones at the immunological level. However, these clinical and serological differences did not correlate with outcomes of activity, damage, remission, or mortality. Future studies are needed to assess the definitive effect of ethnicity on SLE.References[1]Gómez-Puerta JA, Pons-Estel GJ, Quintana R, et al. A longitudinal multiethnic study of biomarkers in systemic lupus erythematosus: Launching the GLADEL 2.0 Study Group. Lupus. 2021;961203320988586.[2]Pons-Estel GJ, Catoggio LJ, Cardiel MH, et al. Lupus in Latin-American patients: lessons from the GLADEL cohort. Lupus. 2015;24(6):536-545.Disclosure of InterestsNone declared
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