The Sjögren's Working Group: The 2023 OMERACT meeting and provisional domain generation.

Sjögren's disease (SjD) is a systemic autoimmune exocrinopathy with key features of dryness, pain, and fatigue. SjD can affect any organ system with a variety of presentations across individuals. This heterogeneity is one of the major barriers for developing effective disease modifying treatments. Defining core disease domains comprising both specific clinical features and incorporating the patient experience is a critical first step to define this complex disease. The OMERACT SjD Working Group held its first international collaborative hybrid meeting in 2023, applying the OMERACT 2.2 filter toward identification of core domains. We accomplished our first goal, a scoping literature review that was presented at the Special Interest Group held in May 2023. Building on the domains identified in the scoping review, we uniquely deployed multidisciplinary experts as part of our collaborative team to generate a provisional domain list that captures SjD heterogeneity.

Pregnancy outcomes in 1869 pregnancies in a large cohort from the Spanish Society of Rheumatology Lupus Register (RELESSER).

INTRODUCTION: Obstetric complications are more common in women with systemic lupus erythematosus (SLE) than in the general population. OBJECTIVE: To assess pregnancy outcomes in women with SLE from the RELESSER cohort after 12 years of follow-up. METHODS: A multicentre retrospective observational study was conducted. In addition to data from the RELESSER register, data were collected on obstetric/gynaecological variables and treatments received. The number of term pregnancies was compared between women with pregnancies before and after the diagnosis of SLE. Further, clinical and laboratory characteristics were compared between women with pregnancies before and after the diagnosis, on the one hand, and with and without complications during pregnancy, on the other. Bivariate and multivariate analyses were carried out to identify factors potentially associated with complications during pregnancy. RESULTS: A total of 809 women were included, with 1869 pregnancies, of which 1395 reached term. Women with pregnancies before the diagnosis of SLE had more pregnancies (2.37 vs 1.87) and a higher rate of term pregnancies (76.8% vs 69.8%, p < 0.001) compared to those with pregnancies after the diagnosis. Women with pregnancies before the diagnosis were diagnosed at an older age (43.4 vs 34.1 years) and had more comorbidities. No differences were observed between the groups with pregnancies before and after diagnosis in antibody profile, including anti-dsDNA, anti-Sm, anti-Ro, anti-La, lupus anticoagulant, anticardiolipin or anti-beta-2-glycoprotein. Overall, 114 out of the 809 women included in the study experienced complications during pregnancy, including miscarriage, preeclampsia/eclampsia, foetal death, and/or preterm birth. Women with complications had higher rates of antiphospholipid syndrome (40.5% vs 9.9%, p < 0.001) and higher rates of positivity for IgG anticardiolipin (33.9% vs 21.3%, p = 0.005), IgG anti-beta 2 glycoprotein (26.1% vs 14%, p = 0.007), and IgM anti-beta 2 glycoprotein (26.1% vs 16%, p = 0.032) antibodies, although no differences were found regarding lupus anticoagulant. Among the treatments received, only heparin was more commonly used by women with pregnancy complications. We did not find differences in corticosteroid or hydroxychloroquine use. CONCLUSIONS: The likelihood of term pregnancy is higher before the diagnosis of SLE. In our cohort, positivity for anticardiolipin IgG and anti-beta-2- glycoprotein IgG/IgM, but not lupus anticoagulant, was associated with a higher risk of poorer pregnancy outcomes.

What do patients on methotrexate need and expect at the clinic? An online patient survey.

To verify, via a survey, the experience and needs of patients receiving methotrexate (MTX), their general management and the quality of the information provided by the rheumatologist. We conducted a 51-item online survey between May and July 2020 addressed to persons diagnosed with an immune-mediated disease and treated with MTX (regardless of the route of administration). Recruitment was done via Twitter. We obtained 294 responses, of which 283 were complete and could be analysed. Almost 82% of the respondents were women, 80% resided in Spain, 75% were between 31 and 60 years old, and 57% were active workers. Diseases included psoriasis (41%), lupus, Sjögren's or vasculitides (33%), and rheumatoid arthritis (16%), among others. Eighty per cent had read the leaflet inserted in the package, of whom 62% found it helpful. Only 15% of the respondents reported having been offered additional written material, which was considered barely functional (33 out of 100). Most patients (88%) responded that they had not received advice on any reliable sources to consult on the internet, and those who received it considered it unhelpful (24 out of 100). Patients receiving MTX due to an autoimmune disease demand more and better-quality written or web-based information than what is currently offered at their clinics.

Response to local corticosteroid injections in carpal tunnel syndrome with normal conduction studies.

INTRODUCTION: Nerve conduction studies (NCS) have been considered as the gold standard in carpal tunnel syndrome (CTS) diagnosis, despite correlation between clinical symptomatology and NCS severity has shown to be poor. In fact, clinical symptoms precede NCS changes in months or years. Few papers have been published about the clinical response to treatment of clinically typical CTS, but with normal NCS (NNCS). OBJECTIVE: To compare the clinical response to local corticosteroid injections (LCI) in clinically typical CTS, with NNCS and abnormal NCS (ANCS). METHOD: We included patients older than 18, with typical CTS symptoms (ongoing daily nocturnal pain/paresthesias in hand, at least during 3 months). Follow-up was done at 3, 6 and 12 months. Primary outcome was the visual analog scale for pain (p-VAS), comparing NNCS CTS wrists with ANCS CTS wrists. Statistic signification was established by the Student's t test, Mann-Whitney's "U", χ2 test and Yates' correction. RESULTS: We included 44 wrists in the NNCS group, and 83 in the ANCS group. There was no statistical significance between data in both groups, except in the 12-month follow-up, where the NNCS group achieved better results than the ANCS group in the 20% response (p=0.006). There was a trend toward a better 50% response in the 12-month follow-up. CONCLUSIONS: Our data suggest that LCI are similarly effective in both CTS with NNCS and ANCS. Nonetheless, there is a mild better effect in NNCS than in ANCS at 12-month follow-up.

Use of parenteral methotrexate in rheumatic diseases: A systematic review.

OBJECTIVE: To analyse the efficacy, adherence, patient satisfaction, safety, pharmacodynamics and cost-effectiveness of parenteral methotrexate (MTX) in patients with rheumatic diseases. METHODS: A systematic review of literature was carried out in Medline, Embase and Cochrane Central from the beginning until June 2019. Studies including adult patients with rheumatic diseases being treated with parenteral MTX were identified and data on efficacy, adherence, satisfaction, safety, pharmacokinetics, and cost-effectiveness analysed. As for the designs, systematic reviews, clinical trials, or observational studies were permitted, including cross-sectional and small-sample studies if they were pharmacokinetic studies. RESULTS: Out of 4160 identified articles, 80 articles were finally included. The efficacy profile of parenteral MTX seems useful in general and in those patients with insufficient response to oral MTX. The parenteral route does not seem to increase the rate or severity of adverse events due to the use of MTX. The use of parenteral MTX is an appropriate way to reduce costs in patients with inadequate response to oral MTX. Adherence and satisfaction are favoured by training programmes in the use of the parenteral route. The results in rheumatic diseases other than rheumatoid arthritis (RA) are very scarce and do not enable obtaining conclusive data. CONCLUSIONS: Parenteral MTX can be an alternative to the use of oral MTX, due to its profile of efficacy, safety, adherence and pharmacoeconomic results, especially in those patients with RA.

Biologic therapy in refractory neurobehçet's disease: a multicentre study of 41 patients and literature review.

OBJECTIVES: To assess efficacy and safety of biologic therapy (BT) in neurobehçet's disease (NBD) refractory to glucocorticoids and at least one conventional immunosuppressive drug. METHODS: Open-label, national, multicentre study. NBD diagnosis was based on the International Consensus Recommendation criteria. Outcome variables were efficacy and safety. Main efficacy outcome was clinical remission. Other outcome variables analysed were glucocorticoid-sparing effect and improvement in laboratory parameters. RESULTS: We studied 41 patients [21 women; age 40.6 (10.8) years]. Neurological damage was parenchymal (n = 33, 80.5%) and non-parenchymal (n = 17, 41.5%). First BTs used were infliximab (n = 19), adalimumab (n = 14), golimumab (n = 3), tocilizumab (n = 3) and etanercept (n = 2). After 6 months of BT, neurological remission was complete (n = 23, 56.1%), partial (n = 15, 37.6%) and no response (n = 3, 7.3%). In addition, median (IQR) dose of oral prednisone decreased from 60 (30-60) mg/day at the initial visit to 5 (3.8-10) mg/day after 6 months (P < 0.001). It was also the case for mean erythrocyte sedimentation rate [31.5 (25.6)-15.3 (11.9) mm/1st h, P = 0.011] and median (IQR) C-reactive protein [1.4 (0.2-12.8) to 0.3 (0.1-3) mg/dl, P = 0.001]. After a mean follow-up of 57.5 months, partial or complete neurological remission persisted in 37 patients (90.2%). BT was switched in 22 cases (53.6%) due to inefficacy (n = 16) or adverse events (AEs) (n = 6) and discontinued due to complete prolonged remission (n = 3) or severe AE (n = 1). Serious AEs were observed in two patients under infliximab treatment. CONCLUSIONS: BT appears to be effective and relatively safe in refractory NBD.

Long-term Outcome of Local Steroid Injections Versus Surgery in Carpal Tunnel Syndrome: Observational Extension of a Randomized Clinical Trial.

Background: In a previous paper, we have demonstrated that: (1) local injection of corticosteroids for carpal tunnel syndrome (CTS) is as effective as decompressive surgery, at 1-year follow-up; and (2) surgery has an additional benefit in the 2-year follow-up. In this study, we assess the long-term outcomes of both therapies in an observational extension of the patients originally enrolled in our randomized clinical trial. Methods: Patients were included in an open, randomized clinical trial, comparing injections versus surgery in CTS. After the end of the clinical trial, patients received the treatment prescribed by their general practitioner or specialist. Therapeutic failure was defined as the need of any new therapeutic intervention on the involved wrist. Comparison between groups was made using Cox multiple regression analysis. Estimation of the accumulated incidence of new therapeutic failure was made considering the withdrawal as a competitive risk (Gooley's test). Results: Of 163 randomized wrists at the beginning of the study, only 148 were available at the final follow-up. The mean follow-up was 6.3 and the median was 5.9 years. In the long-term follow-up, the accumulated incidence of therapeutic failure in the surgery group was 11.6% versus 41.8% in the injection group. The Cox multiple regression analysis showed a risk of failure associated with injection group of 4.5 (95% confidence interval [CI], 2.1-9.8; P < .0001). Conclusions: In long-term follow-up, surgery seems more effective than local corticosteroid injections in primary CTS. Nonetheless, about 58% of the patients in the injection group will not need further therapeutic interventions during the follow-up.

Patient's experiences of the barriers and facilitators to Methotrexate.

OBJECTIVE: To investigate the barriers and facilitators of adherence to methotrexate (MTX) in people with rheumatic diseases and to explore the experience of shared decision-making. METHODS: A qualitative study was carried out. People diagnosed with inflammatory arthritides or systemic autoimmune diseases and who were treated with MTX were invited to participate in focus groups. The discourse was coded and synthesised with a content analysis approach. RESULTS: The groups included 12 representative patients (rheumatoid arthritis, spondylarthritis, and systemic lupus erythematosus, taking either oral or subcutaneous MTX). Four main themes were identified: (1) drug-related aspects (package insert, adverse events, administration, and difficulties with treatment); (2) patient-physician relationship; (3) social environment (lack of visibility of rheumatic diseases and the support of patient associations); and (4) medication and medical care practicalities (information, reliable sources, and expanding knowledge in other health areas). CONCLUSIONS: Aspects identified might help improve adherence, including quality information, especially on adverse events, the role of the setting, and shared decision-making.

Response to Local Corticosteroid Injections in Carpal Tunnel Syndrome With Normal Conduction Studies.

INTRODUCTION: Nerve conduction studies (NCS) have been considered as the gold standard in carpal tunnel syndrome (CTS) diagnosis, despite correlation between clinical symptomatology and NCS severity has shown to be poor. In fact, clinical symptoms precede NCS changes in months or years. Few papers have been published about the clinical response to treatment of clinically typical CTS, but with normal NCS (NNCS). OBJECTIVE: To compare the clinical response to local corticosteroid injections (LCI) in clinically typical CTS, with NNCS and abnormal NCS (ANCS). METHOD: We included patients older than 18, with typical CTS symptoms (ongoing daily nocturnal pain/paresthesias in hand, at least during 3 months). Follow-up was done at 3, 6 and 12 months. Primary outcome was the visual analog scale for pain (p-VAS), comparing NNCS CTS wrists with ANCS CTS wrists. Statistic signification was established by the Student's t test, Mann-Whitney's "U", χ2 test and Yates' correction. RESULTS: We included 44 wrists in the NNCS group, and 83 in the ANCS group. There was no statistical significance between data in both groups, except in the 12-month follow-up, where the NNCS group achieved better results than the ANCS group in the 20% response (p=0.006). There was a trend toward a better 50% response in the 12-month follow-up. CONCLUSIONS: Our data suggest that LCI are similarly effective in both CTS with NNCS and ANCS. Nonetheless, there is a mild better effect in NNCS than in ANCS at 12-month follow-up.

Nódulo reumatoide benigno en sóleo. Reporte de un caso / Benign rheumatoid nodule in the soleus muscle. Case report

RESUMEN Los nódulos reumatoides benignos son nódulos subcutáneos, idénticos en su morfología e histología a los que ocurren en el transcurso de la artritis reumatoide (AR), que aparecen en personas sin síntomas de artritis y con factor reumatoide (FR) negativo. Se presenta el caso de un varón de 46 arios de raza caucasiana que consultó por tumoración en la pierna derecha. Destacaba negatividad de factor reumatoide, anticuerpos anti péptido citrulinado, anticuerpos antinucleares y anticuerpos anticitoplasma de neutrófilo, así como normalidad de los reactantes de fase aguda. Una ecografía musculoesquelética mostró una tumoración sólida de 3,5 x 1,5 cm, de márgenes bien definidos, en músculo sóleo, con una estructura heterogénea hipoecoica con vascularización intra y perilesional. La resonancia magnética evidenció una tumoración de carácter fibroso sin datos sugestivos de agresividad. Una biopsia guiada por ecografía mostró histología característica de nódulo reumatoide. Se discuten la clínica, el diagnóstico, el diagnóstico diferencial y el tratamiento de los nódulos reumatoides benignos.

ABSTRACT Benign rheumatoid nodules are subcutaneous nodules, identical in morphology and histo logy to those that occur in the course of rheumatoid arthritis. They appear in people without symptoms of arthritis and with negative rheumatoid factor. The case is presented of a 46-year-old Caucasian man who consulted for a tumour inthe right leg. He was negative for rheumatoid factor, anti-citrullinated protein antibodies, antinuclear antibodies, and antineutrophil cytoplasmic antibodies, as well as for acute phase reactants. A musculoskeletal ultrasound showed a solid tumour of 3.5 × 1.5 cm with well-defined margins in the soleus muscle, and with a heterogeneous hypoechoic structure with intra- and peri-lesional vascularisation. The magnetic resonance imaging showed a fibrous tumour with no signs suggestive of aggressive growth. An ultrasound-guided biopsy sho wed the characteristic histology of the rheumatoid nodule. The clinical picture, diagnosis, differential diagnosis, and treatment of benign rheumatoid nodules are discussed.

Use of Parenteral Methotrexate in Rheumatic Diseases: A Systematic Review. / Revisión sistemática del uso de metotrexato por vía parenteral en enfermedades reumáticas.

OBJECTIVE: To analyse the efficacy, adherence, patient satisfaction, safety, pharmacodynamics and cost-effectiveness of parenteral methotrexate (MTX) in patients with rheumatic diseases. METHODS: A systematic review of literature was carried out in Medline, Embase and Cochrane Central from the beginning until June 2019. Studies including adult patients with rheumatic diseases being treated with parenteral MTX were identified and data on efficacy, adherence, satisfaction, safety, pharmacokinetics, and cost-effectiveness analysed. As for the designs, systematic reviews, clinical trials, or observational studies were permitted, including cross-sectional and small-sample studies if they were pharmacokinetic studies. RESULTS: Out of 4160 identified articles, 80 articles were finally included. The efficacy profile of parenteral MTX seems useful in general and in those patients with insufficient response to oral MTX. The parenteral route does not seem to increase the rate or severity of adverse events due to the use of MTX. The use of parenteral MTX is an appropriate way to reduce costs in patients with inadequate response to oral MTX. Adherence and satisfaction are favoured by training programmes in the use of the parenteral route. The results in rheumatic diseases other than rheumatoid arthritis (RA) are very scarce and do not enable obtaining conclusive data. CONCLUSIONS: Parenteral MTX can be an alternative to the use of oral MTX, due to its profile of efficacy, safety, adherence and pharmacoeconomic results, especially in those patients with RA.

Update of the Position Paper of the Spanish Society of Rheumatology on Biosimilar Drugs. / Actualización del Documento de posicionamiento de la Sociedad Española de Reumatología sobre fármacos biosimilares.

In 2015 the Spanish Society of Rheumatology (Sociedad Española de Reumatología [SER]) published its position paper on biosimilar drugs. In this update, the SER, continues to manifest its unequivocal commitment to the sustainability of the health system of our country and is aligned with the measures that, without reducing quality of care, are aimed at ensuring its continuity. Since the publication of the previous position paper, the European Commission has authorized new biosimilar drugs, which provides an excellent opportunity to advance the efficiency of health care. In this new scenario of increased therapeutic offer of biologics, the SER considers it crucial to preserve the freedom of prescription of physicians who prescribe drugs based exclusively on the characteristics and individual circumstances of each patient, without forgetting the economic aspects there of.

Treat-to-target strategy in patients with rheumatoid arthritis: Audit of adherence from real world clinical data.

INTRODUCTION: The current paradigm of the management of rheumatoid arthritis (RA) recommends achieving a state of remission or low disease activity through the treat-to-target strategy. Our study assesses adherence to this strategy. METHOD: Patients with RA (ACR-EULAR 2010 criteria) were included. From each centre, 19 patients were randomly selected. Clinical histories (CH) were assessed by independent auditors, checking compliance with predefined quality criteria. The study was approved by ethics committees. RESULTS: We included 856 patients (mean age 54 years; 71% women). The use of a combined index (CI) was recorded in 61% of cases. Visits were recorded every 4 weeks using a CI in 4% of CH while attempts were made to achieve remission. Monitoring of disease activity every 6-8 months after reaching the target was recorded in 73% of cases. CONCLUSIONS: The implementation of the treat-to-target strategy is barely recorded in patients with RA in routine clinical practice.

Protocolised odontological assessment of patients with primary Sjögren's syndrome. / Evaluación protocolizada odontológica en el paciente con síndrome de Sjögren primario. Proyecto EPOX-SSp: metodología y objetivos.

OBJECTIVE: The aim of this study is to describe the findings of a protocolised odontological evaluation of patients with primary Sjögren's syndrome (pSS) treated in rheumatology units in the Community of Madrid. METHODS: Multicentric descriptive study in which pSS patients classified according to the American-European consensus of 2002 criteria were included. We collected the demographic, clinical and serological data of each patient. A complete oral examination was performed and salivary flow and the CAOD caries index were collected. The patients completed the visual analogue scale for xerostomia, the Oral Health Impact Profile-14 questionnaire and an oral health questionnaire. RESULTS: Sixty-one patients were recruited. Ninety-eight percent were women and the mean age of the patients was 57 years. Pathological oral signs (unstimulated salivary flow/salivary glands sialography/scintigraphy) were present in 52.5% of the patients, anti-Ro/anti-La were positive in 90.2%. Ninety-two percent of the patients reported xerostomia and 61% of the patients suffered from hyposialia. Thirty-five patients presented oral mucosa lesions. CAOD index was 16.97±7.93 and visual analogue scale for xerostomia was 46.69±14.43. The results of the OHIP-14 questionnaire were 23.13±14.16. Patients with pathological oral signs obtained a significantly higher Oral Health Impact Profile-14 score (P=.03). We also found that patients with peripheral nervous system involvement obtained a significantly higher Oral Health Impact Profile-14 score (P=.001) CONCLUSIONS: The presence of xerostomia in this cohort of pSS patients was high and hyposialia was present in 61% of the patients. Oral lesions appeared in more than half of the subjects. Oral health had a negative impact on the quality of life of patients with pSS, being higher in those with pathological objective oral signs and in those with peripheral nervous system involvement.

Efficacy of belimumab in Primary Sjögren's syndrome: A systematic review.

OBJECTIVE: To evaluate the efficacy and safety of belimumab in patients with Primary Sjögren's syndrome (pSS). METHODS: The search included manuscripts assessing the efficacy or safety of belimumab in patients with pSS (American-European Consensus Criteria 2002) published between 2004 and 2017 in MEDLINE, EMBASE or Cochrane databases. Two reviewers independently selected the articles, extracted data and evaluated the quality of the evidence following Scottish Intercollegiate Guidelines Network (SIGN) recommendation grades. RESULTS: Out of 135 citations, only 3 articles were included. All of them publishing results from the same study at different time points including 28 patients. At week 28 improvement was reported for visual analogue scale (VAS) dryness score and glandular manifestations in 37% and 77% of patients, respectively, which persisted at week 52 (W52). Belimumab was well tolerated and safely administered. CONCLUSION: Published evidence to determine the efficacy of belimumab in pSS is limited. Belimumab seems to be effective to reduce systemic activity, parotid enlargement, lymphadenopathies, articular manifestation and B cell biomarkers.

Clinically significant renal involvement in primary Sjögren's syndrome is associated with important morbidity: data from the Spanish Sjögrenser cohort.

OBJECTIVES: To investigate the prevalence, associated factors, and effects of primary overt renal disease on morbidity in patients with primary Sjögren's syndrome (pSS). METHODS: All patients in the Sjögrenser (registry of adult pSS patients of the Spanish Society of Rheumatology) cohort were retrospectively investigated for the presence of clinically significant renal involvement directly related to pSS activity. RESULTS: Of the 437 patients investigated, 39 (9%) presented overt renal involvement during follow-up. Severe renal disease necessitating kidney biopsy was relatively rare (2%). Renal involvement may complicate pSS at any time during the disease course and is associated with severe disease (indicated by higher scores of involvement, activity, and damage), systemic multiorgan involvement, and a higher frequency of lymphoma. Multivariate analysis showed that older age (odds ratio [OR] 1.03, 95% confidence interval [CI] 1.00-1.07), higher European League Against Rheumatism Sjögren's Syndrome Disease Activity Index scores (OR 1.1, CI 1.03-1.18), serum anti-La/SSB positivity (OR 6.65, CI 1.41-31.372), and non-vasculitic cutaneous involvement (OR 5.47, 1.03-29.02) were independently associated with this complication. Chronic renal failure developed in 23 of 39 patients (59%); only 1 of them progressed to end-stage renal disease necessitating renal replacement therapy. Patients with overt renal disease showed higher Sjögren's syndrome disease damage index scores, higher rates of hospitalisation due to disease activity and higher rates of clinically relevant comorbidities. CONCLUSIONS: Overt renal involvement in pSS is not uncommon. Although it usually shows a favourable prognosis, is associated with significant morbidity.

Digestive involvement in primary Sjögren's syndrome: analysis from the Sjögrenser registry.

OBJECTIVES: Digestive involvement (DI) has been reported in 10-30% of primary Sjögren's syndrome (pSS) patients, and few studies have systematically analysed the prevalence of DI in pSS patients. The aim of this study was to describe DI prevalence in pSS patients from the Sjögrenser Study, and to analyse its clinical associations. METHODS: All patients included in the Sjögrenser study, a Spanish multicentre randomised cohort, containing demographic, clinical and histologic data, have been analysed retrospectively. Patients were classified according to the presence of DI (oesophageal, gastric, intestinal, hepatic and pancreatic), and we have performed DI clinical associations, descriptive statistics, Student t or χ2 test, and uni and multivariate logistic regression. RESULTS: From 437 included patients, 95% were women, with a median age of 58 years, 71 (16.2%) presented DI: 21 (29.5%) chronic atrophic gastritis, 12 (16.9%) oesophageal motility dysfunction, 3 (4.2%) lymphocytic colitis, 18 (25.3%) primary biliary cholangitis, 15 (21.1%) autoimmune hepatitis, 7 (9.8%) pancreatic involvement and 5 (7%) coeliac disease. Half of them developed DI at the same time or after pSS diagnosis. Patients with DI were significantly older at pSS diagnosis (p=0.032), more frequently women (p=0.009), presented more autoimmune hypothyroidism and C3 hypocomplementaemia (p=0.040), and were treated more frequently with glucocorticoids, immunosuppressant and biologic therapies. Patients with pancreatic involvement presented more central nervous system and renal involvement, Raynaud's phenomenon, lymphoma and C3/C4 hypocomplementaemia. CONCLUSIONS: DI is frequent in Sjögrenser patients, mainly in the form of autoimmune disorders, and seem to be associated with a more severe phenotype. Our results suggest that DI should be evaluated in pSS patients, especially those with more severe disease.

Associated factors to serious infections in a large cohort of juvenile-onset systemic lupus erythematosus from Lupus Registry (RELESSER).

OBJECTIVE: To assess the incidence of serious infection (SI) and associated factors in a large juvenile-onset systemic lupus erythematosus (jSLE) retrospective cohort. METHODS: All patients in the Spanish Rheumatology Society Lupus Registry (RELESSER) who meet ≥4 ACR-97 SLE criteria and disease onset <18 years old (jSLE), were retrospectively investigated for SI (defined as either the need for hospitalization with antibacterial therapy for a potentially fatal infection or death caused by the infection). Standardized SI rate was calculated per 100 patient years. Patients with and without SI were compared. Bivariate and multivariate logistic and Cox regression models were built to calculate associated factors to SI and relative risks. RESULTS: A total of 353 jSLE patients were included: 88.7% female, 14.3 years (± 2.9) of age at diagnosis, 16.0 years (± 9.3) of disease duration and 31.5 years (±10.5) at end of follow-up. A total of 104 (29.5%) patients suffered 205 SI (1, 55.8%; 2-5, 38.4%; and ≥6, 5.8%). Incidence rate was 3.7 (95%CI: 3.2-4.2) SI per 100 patient years. Respiratory location and bacterial infections were the most frequent. Higher number of SLE classification criteria, SLICC/ACR DI score and immunosuppressants use were associated to the presence of SI. Associated factors to shorter time to first infection were higher number of SLE criteria, splenectomy and immunosuppressants use. CONCLUSIONS: The risk of SI in jSLE patients is significant and higher than aSLE. It is associated to higher number of SLE criteria, damage accrual, some immunosuppressants and splenectomy.

Prevalence and factors associated with osteoporosis and fragility fractures in patients with primary Sjögren syndrome.

This study aimed at determining socio-demographic and clinical factors of primary Sjögren syndrome (pSS) associated with osteoporosis (OP) and fragility fracture. SJOGRENSER is a cross-sectional study of patients with pSS, classified according to American European consensus criteria developed in 33 Spanish rheumatology departments. Epidemiological, clinical, serological and treatment data were collected and a descriptive analysis was conducted. Bivariate and multivariate analyses were performed using a binomial logistic regression to study the factors associated with OP and fragility fracture in pSS. 437 patients were included (95% women, with a median age of 58.6 years). 300 women were menopausal (76.4%). Prevalence of OP was 18.5% [in men (N = 21) this measured 19%]. A total of 37 fragility fractures were recorded. In the multivariate analysis, there was an association between OP and age: in the 51-64 age range (menopausal women), the OR measured 9.993 (95% CI 2301-43,399, p = 0.002); In the age > 64 years group, OR was 20.610 (4.679-90.774, p < 0.001); between OP and disease duration, OR was 1.046 (1.008-1085, p = 0.017); past treatment with corticosteroids, OR 2.548 (1.271-5.105, p = 0.008). Similarly, an association was found between fragility fractures and age: in the 51-64 age group, OR measured 5.068 (1.117-22,995, p = 0.035), age > 64 years, OR was 7.674 (1.675-35,151, p < 0.009); disease duration, OR 1.049 (CI 1.003-1097, p < 0.036) and the ESSDAI index, OR 1.080 (1.029-1134, p = 0.002). Patients with pSS can develop osteoporosis and fragility fractures over the course of the disease. Age, corticosteroids treatment and disease duration were associated with the development of OP. Disease duration and ESSDAI were associated with the development of fractures in patients with pSS.