Estimated glomerular filtration rate is a marker of mortality in the European Scleroderma Trials and Research Group (EUSTAR) database.

OBJECTIVES: The study aim was to evaluate the estimated glomerular filtration rate (eGFR), its association with clinical disease and its predictive ability with respect to mortality in SSc patients from the European Scleroderma Trials and Research Group (EUSTAR) database. METHODS: SSc patients from the EUSTAR database who had items required for the calculation of eGFR at a baseline visit and a second follow-up visit available were included. A cut-off eGFR value of 60 ml/min was chosen for all SSc patients, and 30 ml/min for those with scleroderma renal crisis (SRC). Cox regression and competing risk analysis were performed to evaluate the use of eGFR as a predictive factor of mortality. RESULTS: A total of 3650 SSc patients were included in this study. The median serum level of creatinine and the mean of eGFR were 0.8 mg/dl (interquartile range = 0.6-0.9) and 86.6 ± 23.7 ml/min, respectively. The eGFR was significantly lower in patients with pulmonary hypertension. Overall survival (OS) was significantly reduced in SSc patients with eGFR < 60 ml/min compared with patients with eGFR ≥ 60 ml/min [OS at 5 years 0.763 (95% CI: 0.700, 0.814) vs 0.903 (95% CI: 0.883, 0.919; P < 0.001)]. In multivariable analysis, OS was associated with male gender (P < 0.01), systolic pulmonary arterial pressure (sPAP) (P < 0.001) and eGFR (P < 0.001). The cumulative incidence of deaths due to SSc was associated with increased sPAP (P < 0.001) and reduced eGFR (P < 0.05). The OS at 5 years of 53 SRC patients was not significantly different between SSc patients with eGFR > 30 ml/min and those with eGFR <30 ml/min. CONCLUSION: eGFR represents a predictive risk factor for overall survival in SSc. The eGFR, however, does not represent a risk factor for death in SRC.

Fever of unknown origin: a challenging case.

We report a case of Cogan's syndrome presenting as fever of unknown origin in a 31-year-old woman who was admitted to the hospital with a 7-week history of fever, night sweats and other constitutional symptoms. The diagnosis remained elusive despite numerous investigations, and the patient subsequently developed rash, episcleritis, dizziness and sensorineural hearing loss. While initially thought to be a postinflammatory response to a previous infection, confirmation of the rash as a vasculitis together with the audiovestibular and ocular involvement led to a clinical diagnosis of Cogan's syndrome. This was further corroborated by resolution of her symptoms once immunosuppressive therapy was instituted. Early recognition of Cogan's syndrome is crucial to reducing the risk of serious complications through the timely initiation of treatment.

Congenital tufting enteropathy and chronic arthritis: a clinical and radiological perspective.

Congenital tufting enteropathy is a rare condition which presents in early infancy. It is a condition which should be suspected in infants who present with diarrhoea soon after birth. A rare association with arthritis has been observed with a handful of cases documented in the literature. Our case differs as the arthritis described is erosive in nature, a feature which is not present in other cases.

Cost Effectiveness of TNF-α Inhibitors in Rheumatoid Arthritis.

Background. TNF-α inhibitors have shown to be effective in reducing disease activity and improving the quality of life. Due to the high costs associated with acquisition of this treatment, this study was undertaken to evaluate the ICER of TNF-α antagonists (etanercept, adalimumab, and infliximab) in improving the quality of life. Methods. The HAQ and SF-36 were administered at phases 1, 2, and 3, in order to assess the improvement in the QOL. Suppression of disease activity was assessed through the DAS-28. Results. Statistically significant improvements (P < 0.05) were noted for the SF-36 and HAQ after 3 months and for the DAS-28 after 6 months of TNF-α inhibitor therapy. The mean ICER per 10% improvement in the HAQ, DAS-28, and SF-6D were €1976.5, €2086.5, and €2316.4, respectively, following 6 months of TNF-α intervention. Most favorable ICERs were reported from a patient who had to undergo surgical intervention whilst on DMARD therapy. Conclusion. Significant improvement was observed in patients' quality of life, after a short timeframe of 6 months. Such data is useful information in the light of convincing policy makers, in terms of providing access to the medications to individual patients on national health service schemes.

Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database.

OBJECTIVES: To determine the causes and predictors of mortality in systemic sclerosis (SSc). METHODS: Patients with SSc (n=5860) fulfilling the American College of Rheumatology criteria and prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) cohort were analysed. EUSTAR centres completed a structured questionnaire on cause of death and comorbidities. Kaplan-Meier and Cox proportional hazards models were used to analyse survival in SSc subgroups and to identify predictors of mortality. RESULTS: Questionnaires were obtained on 234 of 284 fatalities. 55% of deaths were attributed directly to SSc and 41% to non-SSc causes; in 4% the cause of death was not assigned. Of the SSc-related deaths, 35% were attributed to pulmonary fibrosis, 26% to pulmonary arterial hypertension (PAH) and 26% to cardiac causes (mainly heart failure and arrhythmias). Among the non-SSc-related causes, infections (33%) and malignancies (31%) were followed by cardiovascular causes (29%). Of the non-SSc-related fatalities, 25% died of causes in which SSc-related complications may have participated (pneumonia, sepsis and gastrointestinal haemorrhage). Independent risk factors for mortality and their HR were: proteinuria (HR 3.34), the presence of PAH based on echocardiography (HR 2.02), pulmonary restriction (forced vital capacity below 80% of normal, HR 1.64), dyspnoea above New York Heart Association class II (HR 1.61), diffusing capacity of the lung (HR 1.20 per 10% decrease), patient age at onset of Raynaud's phenomenon (HR 1.30 per 10 years) and the modified Rodnan skin score (HR 1.20 per 10 score points). CONCLUSION: Disease-related causes, in particular pulmonary fibrosis, PAH and cardiac causes, accounted for the majority of deaths in SSc.