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.

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.

Dexamethasone intravitreal implant in the treatment of macular edema secondary to necrotizing retinitis.

PURPOSE: To describe our experience with the use of a sustained-release dexamethasone implant in three patients with recalcitrant macular edema that developed after necrotizing retinitis in the context of the previously treated virus. CASE DESCRIPTION: Two immunocompetent patients presented with unilateral acute retinal necrosis (ARN) due to Varicella-Zoster (VZV). The other, an immunocompromised patient, presented with unilateral cytomegalovirus (CMV) necrotizing retinitis. The diagnoses were confirmed by anterior chamber polymerase chain reaction (PCR) and all were treated with oral valganciclovir and intravitreal ganciclovir (2 mg/0.1 ml). Infection was controlled but two of them required pars plana vitrectomy. Between 2 and 4 months after the resolution of signs of infection, resistant macular edema (RME) developed, and an intravitreal dexamethasone device was implanted after anterior chamber PCR had been negative. Functional and anatomical improvement was achieved, with the resolution of the edema accompanied by improvement in visual acuity in all patients. There was no evidence of reactivation at two years. No cataract or ocular hypertension was observed. One patient required two additional dexamethasone implants. CONCLUSION: Dexamethasone intravitreal implant could be considered as an option for the treatment of macular edema developed after ARN. Care should be taken to avoid reactivation and patients need to be properly informed.

Autologous Pericranium Graft for Necrotizing Scleritis.

PURPOSE: The purpose of this study was to describe the clinical outcome of scleral staphyloma secondary to necrotizing scleritis treated with autologous pericranial grafting. CASE REPORT: A 63-year-old woman with necrotizing scleritis and choroidal protrusion underwent homologous scleral grafting, which failed. The patient's medical management was optimized for control of her systemic inflammation, and she underwent a second surgery consisting of autologous pericranium grafted to the parietal region. A 3- × 2-cm tissue patch was harvested and sutured over the staphyloma borders with 8/0 polyglactin sutures. Complete re-epithelialization was observed at 3 weeks, the graft was well integrated, and no signs of inflammation were observed at 18 months. CONCLUSIONS: The pericranium offers a viable alternative for scleral thinning because of necrotizing scleritis.

Cancer in rheumatoid arthritis: occurrence, mortality, and associated factors in a South European population.

OBJECTIVES: To estimate the prevalence, incidence, mortality, and predictors of cancer in patients with rheumatoid arthritis (RA). METHODS: We compared the incidence of cancer and the mortality by cancer in a cohort of 789 randomly selected RA patients (1999-2005) with the expected ones in the general population. We estimated standardized incidence ratios (SIR) and standardized mortality ratios (SMR) by indirect age and sex standardization. Additionally, we analyzed by generalized linear models the association of various predictors with cancer incidence, obtaining incidence rate ratios (IRR) with 95% confidence intervals (CI). RESULTS: The SIR of cancer in RA is 1.23 (95% CI: 0.78-1.85). By cancer type, there is an increased risk of leukemia, non-Hodgkin's lymphoma, and lung cancer in RA compared with the general population of the same sex and age. The SMR of cancer is 1.0 (95% CI: 0.53-1.7). By cancer type, RA patients with lung or kidney cancer have higher mortality than expected. Being male, elderly, with longstanding disease, and having used any cytotoxic drugs apart from methotrexate are confirmed as predictive factors for cancer. Additional independent predictors are increases in blood leukocyte counts (IRR per 3000 u/mm3 increase: 1.88 (95% CI: 1.6 -2.1)) and decreases in serum hemoglobin (IRR per 2 g/l decrease: 1.88 (95% CI: 1.19 -2.94)). CONCLUSIONS: The overall incidence and mortality of cancer in RA is not greater than the expected, although there is an increased risk of hematopoietic and lung cancers in RA patients compared with the general population. Hemoglobin and leukocyte counts may help to identify RA patients at risk for cancer.

[Factors related to temporal incapacity in patients with fibromyalgia]. / Factores relacionados con la incapacidad temporal en pacientes con fibromialgia.

OBJECTIVE: To know the sociodemographic, clinical and working conditions characteristics related with temporary disability (TWD) in patients with fibromyalgia syndrome (FS). PATIENTS AND METHOD: Patients diagnosed with FS who met the American College of Rheumatology's criteria, attending an outpatient clinic for at least three months prior were included. We performed a standard clinical protocol with sociodemographic, clinical and working conditions, dates and number of TWD during the last year. All patients were asked to complete a questionnaire with 40 items [Fibromyalgia impact questionnaire (FIQ) inclusive]. RESULTS: The participants were 51 women with FS, 32 of whom needed one or more TWD during the last year. The mean TWD was 83.73 days (DS 98). There was not a statistically significant relationship between the TWD and sociodemographic characteristics in a bivariate analysis. There is a significant trend with hard physical work and with the presence of triggering factors. There was evidence of a significant statistical relationship between TWD with a lack of response to selective serotonin re-uptake inhibitors (SSRIs). We noticed that the highest marks in the FIQ have a direct relationship with patients in a TWD situation. CONCLUSIONS: The lack of response to SSRIs was related with TWD process. The patients in TWD situation scored the highest FIQ. There is a trend towards an increase in the TWD with jobs that required physical effort.