Massimiliano Cazzato

QUEST-RA: quantitative clinical assessment of patients with rheumatoid arthritis seen in standard rheumatology care in 15 countries.

Objective: To conduct a cross-sectional review of non-selected consecutive outpatients with rheumatoid arthritis (RA) as part of standard clinical care in 15 countries for an overview of the characteristics of patients with RA. Methods: The review included current disease activity using data from clinical assessment and a patient self-report questionnaire, which was translated into each language. Data on demographic, disease and treatment-related variables were collected and analysed using descriptive statistics. Variation in disease activity on DAS28 (disease activity score on 28-joint count) within and between countries was graphically analysed. A median regression model was applied to analyse differences in disease activity between countries. Results: Between January 2005 and October 2006, the QUEST-RA (Quantitative Patient Questionnaires in Standard Monitoring of Patients with Rheumatoid Arthritis) project included 4363 patients from 48 sites in 15 countries; 78% were female, >90% Caucasian, mean age was 57 years and mean disease duration was 11.5 years. More than 80% of patients had been treated with methotrexate in all but three countries. Overall, patients had an active disease with a median DAS28 of 4.0, with a significant variation between countries (p<0.001). Among 42 sites with >50 patients included, low disease activity of DAS28 ⩽3.2 was found in the majority of patients in seven sites in five countries; in eight sites in five other countries, >50% of patients had high disease activity of DAS28 >5.1. Conclusions: This international multicentre cross-sectional database provides an overview of clinical status and treatments of patients with RA in standard clinical care in 2005–6 including countries that are infrequently involved in clinical research projects.

Disparities in rheumatoid arthritis disease activity according to gross domestic product in 25 countries in the QUEST–RA database

Objective: To analyse associations between the clinical status of patients with rheumatoid arthritis (RA) and the gross domestic product (GDP) of their resident country. Methods: The Quantitative Standard Monitoring of Patients with Rheumatoid Arthritis (QUEST–RA) cohort includes clinical and questionnaire data from 6004 patients who were seen in usual care at 70 rheumatology clinics in 25 countries as of April 2008, including 18 European countries. Demographic variables, clinical characteristics, RA disease activity measures, including the disease activity score in 28 joints (DAS28), and treatment-related variables were analysed according to GDP per capita, including 14 “high GDP” countries with GDP per capita greater than US

Patient's global assessment of disease activity and patient's assessment of general health for rheumatoid arthritis activity assessment: are they equivalent?

24 000 and 11 “low GDP” countries with GDP per capita less than US

Incident Comorbidity Among Patients with Rheumatoid Arthritis Treated or Not with Low-dose Glucocorticoids: A Retrospective Study

11 000. Results: Disease activity DAS28 ranged between 3.1 and 6.0 among the 25 countries and was significantly associated with GDP (r  =  −0.78, 95% CI −0.56 to −0.90, r2  =  61%). Disease activity levels differed substantially between “high GDP” and “low GDP” countries at much greater levels than according to whether patients were currently taking or not taking methotrexate, prednisone and/or biological agents. Conclusions: The clinical status of patients with RA was correlated significantly with GDP among 25 mostly European countries according to all disease measures, associated only modestly with the current use of antirheumatic medications. The burden of arthritis appears substantially greater in “low GDP” than in “high GDP” countries. These findings may alert healthcare professionals and designers of health policy towards improving the clinical status of patients with RA in all countries.

Systemic sclerosis following human cytomegalovirus infection

Objectives To assess (A) determinants of patients global assessment of disease activity (PTGL) and patients assessment of general health (GH) scores of rheumatoid arthritis (RA) patients; (B) whether they are equivalent as individual variables; and (C) whether they may be used interchangeably in calculating common RA activity assessment composite indices. Methods Data of 7023 patients from 30 countries in the Quantitative Standard Monitoring of Patients with RA (QUEST-RA) was analysed. PTGL and GH determinants were assessed by mixed-effects analyses of covariance models. PTGL and GH equivalence was determined by Bland-Altman 95% limits of agreement (BALOA) and Lins coefficient of concordance (LCC). Concordance between PTGL and GH based Disease Activity Score 28 (DAS28), Clinical Disease Activity Index (CDAI) and Routine Assessment of Patient Index Data 3 (RAPID3) indices were calculated using LCC, and the level of agreement in classifying RA activity in four states (remission, low, moderate, high) using κ statistics. Results Significant differences in relative and absolute contribution of RA and non-RA related variables in PTGL and GH ratings were noted. LCC of 0.64 and BALOA of −4.41 to 4.54 showed that PTGL and GH are not equivalent. There was excellent concordance (LCC 0.95–0.99) for PTGL and GH based DAS28, CDAI and RAPID3 indices, and >80% absolute agreement (κ statistics 0.75–0.84) in RA activity state classification for all three indices. Conclusions PTGL and GH ratings differ in their determinants. Although they are individually not equivalent, they may be used interchangeably for calculating composite indices for RA activity assessment.

A case of adult periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome associated with endocapillary proliferative glomerulonephritis

Objective. To assess the prevalence of comorbidity in a cohort of patients with rheumatoid arthritis (RA), treated or not with low-dose glucocorticoids (GC) and who have been followed for at least 10 years. Methods. This was a retrospective study by review of medical records. Results. We identified 365 patients: 297 (81.3%) were GC users (4–6 mg methylprednisolone daily) and 68 (18.7%) were nonusers. We found that fragility fractures occurred in 18.2% of GC users and in 6.0% of GC nonusers (p < 0.02); arterial hypertension in 32.3% of GC users and in 10.4% of GC nonusers (p < 0.0005); acute myocardial infarction in 13.1% of GC users and in 1.5% of the nonusers (p < 0.01). Prevalence of diabetes mellitus, cataract, and infections was comparable. We divided GC users into groups of different duration of GC therapy: < 2, 2–5, and > 5 years; the mean duration of GC treatment was 1.3 ± 0.5, 3.6 ± 1.1, and 12.1 ± 5.1 years, respectively. GC treatment for > 5 years was associated with significantly higher prevalence of fragility fractures (26.6%; p < 0.001 vs the other groups), arterial hypertension (36.7%; p < 0.0002 vs nonusers and GC users < 2 years), myocardial infarction (16.1%; p < 0.01 vs nonusers), and infections (9.7%; p < 0.005 vs the other groups). GC treatment for 2–5 years was associated with a significantly higher prevalence of arterial hypertension (30.0%; p < 0.01) compared to nonusers. Conclusion. Patients with RA treated with low-dose GC compared to patients never treated with GC show a higher prevalence of fractures, arterial hypertension, myocardial infarction, and serious infections, especially after 5 years of GC treatment. The high prevalence of myocardial infarction and fractures in patients with RA suggests that a more accurate identification of risk factors and prevention measures should be adopted when longterm GC treatment is needed.

Dose adjustments and discontinuation in TNF inhibitors treated patients: when and how. A systematic review of literature

Systemic sclerosis (SSc) is a connective tissue disease characterised by skin and visceral organ involvement.1,2 The cause of SSc is still unknown; it has been suggested that one or more factors may be responsible for the disease through a complex pathogenic mechanism.3,4 Immune system dysregulation, collagen hyperproduction by altered fibroblasts, and vascular alterations can variably contribute to SSc development. The presence of Raynauds phenomenon and diffuse microangiopathy suggests that endothelial injury may represent the first step in the pathogenesis of the disease.4 Numerous genetic, environmental, and infectious agents have been proposed as possible triggering factors.3–6 Among these, human cytomegalovirus (HCMV) infection may play a part in the pathogenesis of the SSc owing to its ability to …

AB0925 Liver Involvement in Adult Onset Still's Disease: Retrospective Analysis of 18 Cases

PFAPA is an acronym for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. This syndrome has been usually described in pediatric patients and it generally resolves spontaneously. The endocapillary proliferative glomerulonephritis (EPG) is a glomerular injury characterized by hypercellularity in glomerular lumen and is caused by post-infectious or autoimmune diseases. In this paper, we describe the case of a 35-year-old man affected by PFAPA and EPG. To our knowledge this association has never been reported in the literature before.

FRI0383 Laser speckle contrast imaging (LASCA) is a valid aid in the differential diagnosis of raynaud’s phenomenon

Objectives To review the available evidence concerning the possibility of discontinuing and/or tapering the dosage of TNF inhibitors (TNFi) in RA patients experiencing clinical remission or low disease activity. Methods A systematic review of the literature concerning the low dosage and discontinuation of TNFi in disease-controlled RA patients was performed by evaluation of reports published in indexed international journals (Medline via PubMed, EMBASE), in the time frame from 8 April 2013 to 15 January 2016. Results We analysed the literature evaluating the efficacy and the safety of two different strategies using TNFi, decreasing dosage or discontinuation, in patients experiencing clinical remission or low disease activity. After the analysis of online databases, 25 references were considered potentially relevant and 16 references were selected. The majority of data concerned etanercept and adalimumab. Results suggested the induction of stable clinical remission or low disease activity by using TNFi followed by a dosage tapering and/or discontinuation of such drugs may be associated with the maintenance of a good clinical response in a subset of patients affected by early disease. Conclusion RA patients treated early with TNFi and achieving their therapeutic clinical targets seem to maintain their clinical response after tapering or discontinuing TNFi. These data may allow physicians a more dynamic and tailored management of RA patients.

Systemic sclerosis: Demographic, clinical, and serologic features and survival in 1,012 Italian patients

Background Adult-onset Stills disease (AOSD) is a multisystem inflammatory disease of unknown etiology typically characterized by high fever, arthralgias/arthritis and transient cutaneous rash. Liver involvement has been reported in AOSD, however only few studies have described it comprehensively. Objectives To describe the frequency and clinical presentation of liver involvement in a single center cohort of AOSD. Methods Retrospective observational study including unselected patients with a diagnosis of AOSD made according to the Yamaguchi criteria and admitted at our University Hospital between 2009 and 2013. Demographic, clinical and serological data were retrieved from patients files including: fever, evanescent rash, sore throat, arthritis, myalgias, pleuritis, pericarditis, pneumonitis, lymphadenopathy, splenomegaly, hepatomegaly leucocytosis >15,000/μl and high serum ferritin levels. The severity of liver-associated enzymes was based on the degree of elevation and was stratified as mild (<2 times normal), moderate (2-5 times normal), and severe (>5 times normal). Severe liver dysfunction was defined as having all of the following criteria: total bilirubin >3 mg/dL; albumin <3.2 g/dL; and prothrombin time >3 seconds prolonged. Medications used, response to treatment and long-term outcomes were also recorded. Comparison in terms of continuous data were determined using independent sample t tests or Mann–Whitney tests, and in terms of proportions using contingency table analysis and chi square test. Results Eighteen patients (9 F: 9M; mean age (SD) =37 (12) years, mean follow-up=31 (15) months) were included in the study. Twelve patients out of 18 (66.6%) presented liver test abnormalities whereas hepatomegaly occurred in 4/18 (22.2%) cases. No correlation was found between liver involvement and gender or age at the diagnosis. A mild elevation of liver enzymes was found in 4/12 cases, a moderate alteration in other 4 patients, and a severe cytolysis in the other 4. A liver biopsy was performed in 3/12 patients revealing acute hepatitis with necrosis and accompanying non specific inflammatory infiltration. Three patients presented a liver acute failure concurrently with a diffuse intravascular coagulation (DIC) and, in one case, with a fatal macrophagic activation syndrome (MAS). Prednisone therapy induced a fast improvement of liver function in all the cases but one. Hydroxychloroquine, methotrexate and cyclosporine were the most common steroid sparing agents adopted during the follow-up. Conclusions Our findings outlined the high frequency of liver involvement in AOSD. Despite generally mild or moderate, severe acute hepatitis can occur during the disease course especially in those patients with DIC or MAS features. Treatment with systemic corticosteroid therapy is generally able to control liver function during AOSD, however steroid sparing agents are crucial in maintaining a long- term remission. Larger prospective studies could clarify the pathogenetic role of macrophagic activation in AOSD severe liver involvement, leading to develop new concepts for treatment modalities. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.4541