The Italian registry of aggressive rheumatoid arthritis -- the GIARA project.

Objective. In 1999, the Italian Society of Rheumatology started a project to determine the prevalence and clinical characteristics of aggressive rheumatoid arthritis (ARA). Methods. For I year, all patients with RA for 2 to 2 to 1.5, female sex, and RF positivity. Conditions other than RA were recorded in about 50% of the patients, and only 30%-40% were taking disease modifying antirheumatic drugs. Conclusion. In an Italian RA population, the GIARA (Gruppo Italiano Artrite Reumatoide Aggressiva) criteria for ARA were met by 15% of the patients with disease duration of 2 years, but erosions were seen in 35%. Upon referral, most of the RA patients were inadequately treated and had other conditions

Development of the autoinflammatory disease damage index (ADDI)

OBJECTIVES: Autoinflammatory diseases cause systemic inflammation that can result in damage to multiple organs. A validated instrument is essential to quantify damage in individual patients and to compare disease outcomes in clinical studies. Currently, there is no such tool. Our objective was to develop a common autoinflammatory disease damage index (ADDI) for familial Mediterranean fever, cryopyrin-associated periodic syndromes, tumour necrosis factor receptor-associated periodic fever syndrome and mevalonate kinase deficiency. METHODS: We developed the ADDI by consensus building. The top 40 enrollers of patients in the Eurofever Registry and 9 experts from the Americas participated in multiple rounds of online surveys to select items and definitions. Further, 22 (parents of) patients rated damage items and suggested new items. A consensus meeting was held to refine the items and definitions, which were then formally weighted in a scoring system derived using decision-making software, known as 1000minds. RESULTS: More than 80% of the experts and patients completed the online surveys. The preliminary ADDI contains 18 items, categorised in the following eight organ systems: reproductive, renal/amyloidosis, developmental, serosal, neurological, ears, ocular and musculoskeletal damage. The categories renal/amyloidosis and neurological damage were assigned the highest number of points, serosal damage the lowest number of points. The involvement of (parents of) patients resulted in the inclusion of, for example, chronic musculoskeletal pain. CONCLUSIONS: An instrument to measure damage caused by autoinflammatory diseases is developed based on consensus building. Patients fulfilled a significant role in this process

Prenatal exposures and exposomics of asthma

This review examines the causal investigation of preclinical development of childhood asthma using exposomic tools. We examine the current state of knowledge regarding early-life exposure to non-biogenic indoor air pollution and the developmental modulation of the immune system. We examine how metabolomics technologies could aid not only in the biomarker identification of a particular asthma phenotype, but also the mechanisms underlying the immunopathologic process. Within such a framework, we propose alternate components of exposomic investigation of asthma in which, the exposome represents a reiterative investigative process of targeted biomarker identification, validation through computational systems biology and physical sampling of environmental medi

A comparison of cyclosporine, sulfasalazine, and symptomatic therapy in the treatment of psoriatic arthritis

To compare the efficacy and tolerability of cyclosporine (CSA) with that of symptomatic therapy (ST) alone and sulfasalazine (SSZ) in the treatment of psoriatic arthritis (PsA)

Short- and long-term considerations concerning the management of plaque psoriasis with low-dose cyclosporin

In an open multicenter study, cyclosporin (CsA) at low doses (3 mg/kg/day adjusted during the course of treatment on the basis of clinical response and tolerability up to a maximum of 5 mg/kg/day) was given to 293 evaluable patients with severe plaque-form psoriasis (M/F 215/78, aged 19–80 years, 2–53 years from diagnosis) in order to evaluate its safety and efficacy over a median follow-up of 7 months of treatment and 3 months after treatment. All patients were unsatisfactory responders to conventional topical therapy and had indications for systemic treatment. Patients entered the study only if they were within the normal range for renal an hepatic function and blood pressure, and were free of any clinically obvious immunodeficiencies, malignancies or blood dyscrasia. All gave their informed consent. After remission (defined as reduction ≥ 75% of the body area involved and an improvement of at least 2 points on a 4-point scale for desquamation, erythema and infiltration) CsA was slowly tapered off (0.5 mg/kg/day every 2 weeks) until total discontinuation or the reappearance of signs of the disease; the dose of CsA was also varied in the case of any important modification in renal and hepatic function of blood pressure. As concomitant treatment, white petrolatum was allowed, as well as specific local therapy after CsA discontinuation. Considerable improvement ( > 50% reduction in the skin area affected) was observed in 98% and only 2% (5 patients) did not respond. Clinical remission was achieved in 225 patients (77%): of these, 73% after a median of 2 months at CsA doses of 2.5–3.49 mg/kg/day, 8% after 4 months at doses < 2.49 mg/kg/day and 19% after 3 months at doses ≥ 3.5 mg/kg/day. After remission, the gradual withdrawal of the drug over a period of 3 months (0.5 mg/kg/day every 2 weeks) allowed control over the disease (the absence of relapse) to be maintained for a median of 8 months in 133 patients (59%). The topical therapies permitted after remission and during the maintenance phase (steroids, inert topical agents and exposure to UVB radiation) were used in less than 50% of cases. Disease relapse (the reappearance of skin involvement over more than 50% of the area affected at baseline) occurred in 92 of the 225 patients achieving remission, 24 of whom relapsed a median of 6 months after remission, when CsA had been completely withdrawn; the remaining relapses occurred about 4 months after remission, during the gradual withdrawal of the drug. In none of the patients was any ‘rebound’ effect observed. In 11 patients who relapsed twice during the course of observation, the administration of successive cycles of equal doses of CsA proved to be equally efficacious. The adverse events reported by 26% of the patients were mild or moderate and reversible with the adjustment of posology or discontinuation of the treatment (3% of cases). In conclusion, CsA at 3 mg/kg/day given to carefully selected and closely monitored severe psoriatic patients produces constantly favourable results within 2-3 months. After remission, it seems advisable to withdraw the drug gradually, to evaluate the usefulness, effectiveness and tolerability of a low-dose maintenance regimen aimed at preventing the recurrence of the disease or an intermittent therapy which allows a relapse-free period of 6–8 months in 70% of patients