Total body water and sicca symptoms in primary Sjögren's syndrome.

OBJECTIVES: To evaluated the total body water (TBW) among patients with primary Sjögren's syndrome (pSS) and assess its correlation with the severity of oral and ocular sicca symptoms, and some objective sicca variables. METHODS: We included 85 patients and 85 controls matched by sex, age, and body mass index (BMI). We assessed the Schirmer-I test and the non-stimulated whole salivary flow (NSWSF). We evaluated ocular and oral symptoms during the past 15 days using a 0-10 visual analogue scale (VAS) (highest score=worst symptoms). We obtained the TBW by bioelectric impedance analysis. RESULTS: 80% were women (mean age 54.8 years and mean disease duration 11.5 years). TBW was similar in pSS and controls (46.8±4.6 vs. 46.9±4.5, p=0.88). TBW correlated with age (ρ=-0.25, p=0.02), disease duration (ρ=-0.30, p=0.005), BMI (ρ=-0.78, p=0.001) and ocular VAS scale (ρ=-0.28, p=0.01); but not with NSWSF, Schirmer test or oral VAS scale. When comparing patients in the lowest TBW percentile (≤25%) with the remaining patients, the former group was older, had longer disease duration, higher BMI, lower frequency of anti-Ro/SSA and antinuclear antibodies, and higher ocular VAS scores. In the multivariate analysis, the ocular VAS score (OR 1.88, 95% CI 1.08-3.2, p=0.02) and the BMI 1.92 (OR 1.4, 95% CI 1.4-2.66, p=0.0001) remained associated with a lower TBW percentage. CONCLUSIONS: Patients with pSS had similar TBW percentages to control subjects. However, lower TBW percentages in the pSS were associated with higher BMI and also with the most severe ocular symptoms.

Differential Th follicular cell subsets in minor salivary glands of patients with primary Sjögren's syndrome and systemic lupus erythematosus associated with Sjögren's syndrome.

OBJECTIVES: T follicular helper cells (Tfh) have been recognised in minor salivary glands (MSG) of patients with primary Sjögren's syndrome (pSS). Nevertheless, if the Tfh1, Tfh2, Tfh17, Tfr phenotype is different when comparing pSS and associated SS in systemic lupus erythematosus (SLE) is unknown. METHODS: We included MSG biopsies from 8 pSS, 8 SLE/SS patients, 7 SLE patients, and 2 non-SS sicca patients. To determine the subpopulation of Tfh, a double-staining procedure for transcription factor B cell lymphoma 6 (Bcl-6)+/IL-17A+, Bcl6+/IL-4+, Bcl6+/IFN-γ+, and Bcl6+/Foxp3+ cells was performed. We estimated the mean percentage of positively staining cells in four fields per sample. RESULTS: Tfh1, Tfh2, and Tfh17 cells were highly expressed in pSS compared with the rest of the groups; conversely, in patients with SLE/SS predominated, the Tfh17 and in SLE patients the Tfh1 cells. Regulatory Tfh cells (Tfr) were similar in pSS and the rest of the patients. However, the lowest frequency was found in the SLE group. A positive correlation was observed between anti-Ro/SSA autoantibody and Tfh17 subset (r=0.726, p=0.0001); and with the (Tfh2+Tfh17)/Tfh1 ratio (r=0.844, p<0.0001) in the MSG of patients with pSS. CONCLUSIONS: We showed a differential Tfh profile in primary SS and SLE with associated SS. Whether this Tfh differential profile participates in the increased risk of lymphoproliferative disease in pSS compared with associated SS, or other outcomes, is yet to be determined in future studies.

Calidad de vida oral en pacientes con síndrome de Sjögren primario / Oral health-related quality of life in primary Sjögren's syndrome

OBJETIVO: Evaluar la calidad de vida general y oral, y sus correlaciones con el flujo salival no estimulado (FSNE) y los síntomas de xerostomía en pacientes con síndrome de Sjögren primario (SSP). MÉTODOS: Se incluyeron 60 pacientes con SSP y 60 controles pareados por género y ±3 años de edad. Se midió el FSNE y se aplicó cuestionario ESSPRI (Cuestionario reportado de síntomas en Síndrome de Sjögren de la Liga Europea contra el reumatismo [EULAR]). Se utilizó la versión corta de SF-36 para evaluar calidad de vida general, y para la calidad de vida oral el cuestionario XeQoLS; así como 8 preguntas para evaluar síntomas orales (dificultad al hablar, tragar, cantidad de saliva en boca, sequedad de boca, garganta, labios, lengua y nivel de sed) mediante escalas visuales análogas (EVA). RESULTADOS: Observamos peor calidad de vida general (menor puntuación SF-36), oral (mayor puntuación XeQoL) y mayor sintomatología evaluada por EVA en pacientes vs. controles. El XeQoL correlacionó con el FSNE (tau = −0,24, p = 0,008), el ESSPRI (tau = 0,45, p = 0,0001), las preguntas EVA 1-2 y EVA 5-8 y la escala SF-36 (tau = 0,28, p = 0,002). CONCLUSIONES: Los pacientes con SSP tienen peor calidad de vida general y oral que sujetos sanos. El FSNE contribuye en la calidad de vida oral y a su vez la calidad de vida oral impacta en la calidad de vida general. La intervención oportuna de terapia sintomática de xerostomía y la prevención de infecciones, caries y pérdida dental pudiera impactar la calidad de vida de estos pacientes

OBJECTIVE: To assess health-related quality of life (HRQoL) and oral health-related quality of life, and correlate them with unstimulated whole salivary flow (UWSF) and oral sicca symptoms in patients with primary Sjögren's syndrome (PSS). METHODS: We included 60 patients with PSS and 60 healthy controls matched according to gender and age (±3 years). We measured the UWSF and scored the European League Against Rheumatism (EULAR) Sjögren's Syndrome Patient Reported Index (ESSPRI). We assessed the short version of the SF-36 as a generic measurement of HRQoL and the Xerostomia Quality of Life Scale (XeQoLS) questionnaire to evaluate oral quality of life. We evaluated oral symptoms using an 8-item Visual Analogue Scale (VAS) questionnaire. RESULTS: We observed a poorer HRQoL (lower scores in SF-36) and oral quality of life (higher scores in XeQoLS), as well as a greater severity of symptoms in the VAS questionnaire upon comparing patients vs. controls. The XeQoL correlated with the UWSF (Tau = −0.24, P = .008), the ESSPRI (Tau =0.45, P = .0001), VAS 1-2 and VAS 5-8 and the SF-36 score (Tau = −0.28, P = .002). CONCLUSIONS: Patients with PSS had a poorer HRQoL and oral quality of life than controls. UWSF contributes to the oral quality of life which, in turn, has an impact on HRQoL. Symptomatic treatment of xerostomia as well as the prevention of infections, decay and tooth loss would help to improve the oral quality of life in these patients

Oral health-related quality of life in primary Sjögren's syndrome. / Calidad de vida oral en pacientes con síndrome de Sjögren primario.

OBJECTIVE: To assess health-related quality of life (HRQoL) and oral health-related quality of life, and correlate them with unstimulated whole salivary flow (UWSF) and oral sicca symptoms in patients with primary Sjögren's syndrome (PSS). METHODS: We included 60 patients with PSS and 60 healthy controls matched according to gender and age (±3 years). We measured the UWSF and scored the European League Against Rheumatism (EULAR) Sjögren's Syndrome Patient Reported Index (ESSPRI). We assessed the short version of the SF-36 as a generic measurement of HRQoL and the Xerostomia Quality of Life Scale (XeQoLS) questionnaire to evaluate oral quality of life. We evaluated oral symptoms using an 8-item Visual Analogue Scale (VAS) questionnaire. RESULTS: We observed a poorer HRQoL (lower scores in SF-36) and oral quality of life (higher scores in XeQoLS), as well as a greater severity of symptoms in the VAS questionnaire upon comparing patients vs. controls. The XeQoL correlated with the UWSF (τ = -0.24, P = .008), the ESSPRI (τ =0.45, P = .0001), VAS 1-2 and VAS 5-8 and the SF-36 score (τ = -0.28, P = .002). CONCLUSIONS: Patients with PSS had a poorer HRQoL and oral quality of life than controls. UWSF contributes to the oral quality of life which, in turn, has an impact on HRQoL. Symptomatic treatment of xerostomia as well as the prevention of infections, decay and tooth loss would help to improve the oral quality of life in these patients.

Prevalence and associations of anti-phosphatidylserine/prothrombin antibodies with clinical phenotypes in patients with primary antiphospholipid syndrome: aPS/PT antibodies in primary antiphospholipid syndrome.

OBJECTIVE: The clinical significance of anti-phosphatidylserine/prothrombin (aPS/PT) in antiphospholipid syndrome (APS) is still controversial. We assessed the prevalence of aPS/PT antibodies, their association with other anti-phospholipid antibodies (aPL) and with different APS clinical phenotypes. METHODS: We included 95 primary APS patients according to the Sydney classification criteria, and patients with thrombocytopenia and/or hemolytic anemia who also fulfilled the serological APS criteria. We tested aCL, anti-ß2GP-I and aPS/PT antibodies (both IgG and IgM isotypes) and lupus anticoagulant (LA). We used χ2 test, Spearman's correlation coefficient, Mann-Whitney U test and logistic regression. RESULTS: Seventy-seven percent of patients had thrombosis, 50% hematologic involvement and 25% obstetric events (non-exclusive groups). Twenty patients had only hematologic features. The prevalence of IgG and IgM aPS/PT antibodies was 61% and 60%, respectively. Patients with LA+ had a higher prevalence and higher titers of IgG and IgM aPS/PT antibodies. aPS/PT antibodies correlated with aPL antibodies including LA. IgG aPS/PT antibodies were associated with thrombosis (OR 8.6 [95% CI 2.13-33.8, p = 0.002]) and pure hematologic features (OR 0.2, CI 95% 0.05-0.97, p = 0.004). IgM anti-ß2GP-I antibodies conferred high risk for both hematologic (OR 7.9, 95% CI 1.88-34.61, p = 0.006) and thrombotic involvement (OR 7.4, 95% CI 1.76-31.12, p = 0.006). CONCLUSIONS: aPS/PT antibodies were highly prevalent and correlated with other aPL antibodies. IgG aPTS/PT conferred a high risk for thrombosis, but not for pure hematologic involvement. aPS/PT antibodies may be a useful serological tool in the diagnosis and phenotypic characterization of APS patients.