New Diagnosis Of Rheumatoid Arthritis Research Articles

Pseudogout, Neutropenia, and Splenomegaly: A Case of Felty Syndrome

Felty syndrome is a rare entity of rheumatoid arthritis, neutropenia, and splenomegaly. Patients typically develop the syndrome years after a diagnosis of rheumatoid arthritis. Here, we present a case of a 76-year-old man with history of chronic calcium pyrophosphate crystal deposition disease, or pseudogout, who presented with neutropenic fever. Given his progressive arthralgias and symmetric synovitis on physical examination, serologies were sent and identified a new diagnosis of rheumatoid arthritis. This, together with exclusion of other causes of neutropenia and discovered splenomegaly, led to diagnosis of Felty syndrome.

Cost-Effectiveness of the Early Arthritis Clinic Organizational Model.

Early diagnosis and tight control improve outcomes of rheumatoid arthritis (RA). However, whether establishing an early arthritis clinic (EAC) is sustainable for national health systems is not known. This analysis aimed to compare effectiveness and costs of an EAC compared to patients followed by the current standard of care. A retrospective study on administrative health databases of patients with a new diagnosis of RA was conducted: 430 patients followed in an EAC were enrolled, and 4 non-EAC controls were randomly matched for each. During 2 years of follow-up, the mean health care costs (outpatient, inpatient, pharmaceutical, and global) and 3 effectiveness measures (number and length of hospitalization and quality of care) of the EAC and non-EAC were estimated. The incremental cost-effectiveness ratio was calculated as well as the cost-effectiveness acceptability curve. The cohorts included patients with a mean age of 55.4 years, and 1,506 patients (70%) were female. The mean pharmaceutical (2,602 versus 1,945 euros) and outpatient (2,447 versus 1,778 euros) costs were higher in the EAC cohort. Conversely, a higher rate of non-EAC patients had a low adherence to quality-of-care indicators. The expected number of hospitalizations and the length of stay were statistically significantly higher in the non-EAC versus EAC. Despite an expected increase in outpatient costs (visits and diagnostic tests) and pharmaceutical costs, the reduction in terms of number and length of hospitalizations and the higher adherence to international quality-of-care guidelines support the effectiveness of the EAC model.

Evaluation of a multidisciplinary care model to improve quality of life in rheumatoid arthritis: a randomised controlled trial.

Health-Related Quality of Life (HR-QOL) is an important patient-reported domain in patients with rheumatoid arthritis (RA). The uptake of multidisciplinary team (MDT) care in RA is generally low, due to initial high demand for resources. We hypothesised that whilst pharmacological treatments are effective in controlling disease activity, a multipronged intervention in an MDT may have a positive impact on HR-QOL. This was a single-centre randomized parallel group, single-blind controlled trial of MDT vs. usual care in an established RA clinic. Data were collected through face-to-face questionnaires, medical records review, and joint counts by a blinded assessor at 0, 3 and 6months. Adult RA patients were randomly assigned in a single visit to a 6-member MDT (rheumatologist, nurse, social worker, physiotherapist, occupational therapist, and podiatrist) or usual care. MDT providers prescribed medications and counselled patients on managing flares, medication adherence, coping, joint protection, exercise, footwear. The primary outcome was minimal clinically important difference (MCID) in HR-QOL (increase in European QOL-5-Dimension-3-Level, EQ-5D-3L by 0.1) at six months. 140 patients (86.3% female, 53.4% Chinese, median (IQR) age 56.6 (46.7, 62.4) years); 70 were randomized to each arm. Median (IQR) disease duration was 5.5 (2.4, 11.0) years and disease activity in 28 joints (DAS28) was 2.87 (2.08, 3.66). 123 patients completed the study. Twenty-six (40.6%) MDT vs. 23 (34.3%) usual care patients achieved an MCID in EQ-5D-3L, OR 1.3 (0.6, 2.7). In multivariable logistic regression, baseline EQ-5D-3L was the only predictor of achieving MCID. There was more disease modifying anti-rheumatic drug escalation in MDT (34.4% vs. 19.4%). Patients with high disease activity were more likely to achieve MCID in the MDT arm. A single visit by stable patients with low disease activity to an MDT failed to achieve MCID in the EQ-5D-3L; however, did achieve small but significant improvements in the EQ-5D-3L, DAS28, pain, coping and self-efficacy. To be sustainable, MDT care should be targeted at patients with high disease activity or those with a new diagnosis of RA. The study is registered on ClinicalTrials.gov, identifier: NCT03099668.

Effect of Chinese Herbal Medicines on Hearing Loss Risk in Rheumatoid Arthritis Patients: Retrospective Claims Analysis

Objectives: Patients with rheumatoid arthritis (RA) are at a higher risk of extra-articular manifestations, especially hearing loss (HL). Although Chinese herbal medicines (CHM) are proven safe and effective treatments for inflammatory conditions, the effect of CHM use on HL in RA patients is unknown. This cohort study aims to determine the relationship between CHM use and the subsequent risk of HL among RA patients.Methods: From health insurance claims data in Taiwan, a total of 6,905 persons aged 20–80 years with newly-diagnosed RA in 2000–2009 were identified. Of these, we recruited 2,765 CHM users and randomly selected 2,765 non-CHM users who matched with the users by the propensity score. Both cohorts were followed up until the end of 2012 to estimate the incidence of HL. Cox proportional hazards regression was used to estimate the adjusted hazard ratio (HR) for HL.Results: The incidence of HL was lower in the CHM users than in the comparison cohort (8.06 vs. 10.54 per 1,000 person-years) (adjusted HR, 0.77; 95% CI, 0.63–0.94). Those who received CHM for more than 2 years had the greatest benefit against the onset of HL, with over 50% risk reduction. Prescriptions of Hai Piao Xiao, Yan Hu Suo, San-Qi, Huang Qin, Dang Shen, Jia-Wei-Xiao-Yao-San, Shu-Jing-Huo-Xue-Tang, and Dang-Gui-Nian-Tong-Tang were found to be associated with a reduced risk of HL.Conclusions: Our findings suggest that adding CHM to conventional therapy may reduce the subsequent risk of HL in RA patients. Prospective randomized trials are recommended to further clarify whether the association revealed in this study supports such a causal relationship.

THU0124 FACTORS ASSOCIATED WITH THE RISK OF MAJOR ADVERSE CARDIOVASCULAR EVENT IN PATIENTS WITH RHEUMATOID ARTHRITIS: A NATIONWIDE, POPULATION-BASED, CASE-CONTROL STUDY

Background:Few population-based studies have assessed factors associated with major adverse cardiovascular evet (MACE) among patients with rheumatoid arthritis (RA).Objectives:The study aimed to assess risk factors of MACE in RA patients in Taiwan.Methods:This study used the 2003–2013 Taiwanese National Health Insurance Research Database to identify 115,143 newly-diagnosed RA patients aged 20 years or more from 2004 to 2012. MACE was defined as having an inpatient visit with a diagnosis of myocardial infarction (ICD-9-CM 410.x except 410.2) with the receipt of coronary artery bass graft or percutaneous coronary intervcention/percutaneous transluminal coronary angioplasty, or having an inpatient visit with a diagnosis of ischemic stroke (ICD-9-CM 433-436 except 433.0 and 434.0). We firstly identified 108,319 RA patients who had no history of MACE before RA diagnosis date. From these patients, we identified 7,580 RA patients who developed MACE in the follow-up period and 100,739 who did not develop MACE in the follow-up periods. The index dates for MACE cases were the date of first inpatient visit for MACE. We finally selected 5,994 MACE cases and 23,976non-MACE controls matching (1:4) for age, sex and disease duration. We examined the associations of MACE with comorbidities, use of biological and conventional synthetic disease modifying antirheumatic drugs (DMARDs), nonsteroidal anti-inflammation drug (NSAID), corticosteroid, antiplatelet agent, anticoagulant, statin and non-statin lipid lowering agents within one year before the index date using conditional logistic regression analyses with a full model and a parsinomious model (only considering the covariates with a p value <0.05 in the univarialbe analysis), shown as odds ratios (ORs) with 95% confidence intervals (CIs).Results:We selected 5,994 MACE cases and 23,976 non-MACE controls. The mean ± SD age was 69.19±11.62 years and 18,925 (63.15%) subjectes were female. The mean ± SD disease duration was 3.44±2.21 years. Using multivariable conditional logistic regression analysis, the risk of MACE was associated with use of abatacept (OR, 0.12; 95% CI, 0.02–0.90), methotrexate (OR, 0.69; 95% CI, 0.62–0.76), ciclosporin (OR, 1.41; 95% CI, 1.06–1.86), NSAID (OR, 1.36; 95% CI, 1.27–1.45), antiplatelet agent (OR, 2.48; 95% CI, 2.32–2.64), non-statin lipid lowering agents (OR, 1.46; 95% CI, 1.28–1.65), and comorbidities including hypertension (OR, 1.23; 95% CI, 1.16–1.32), diabetes (OR, 1.28; 95% CI, 1.19–1.38), ischemic heart disease (OR, 1.22; 95% CI, 1.12–1.33) and chronic obstructive pulmonary disease (COPD) (OR, 1.13; 95% CI, 1.04–1.24) in the parsinomious model.Conclusion:This population-based case-control study revealed that in newly-diagnosed RA patients, use of abatacept and methotrexate were associated with a decreased risk of MACE development, while use of ciclosporin, NSAID, antiplatelet agents and non-statin lipid lowering agents, and comorbidities including hypertension, diabetes, ischemic heart disease and COPD were associated with an increased risk of MACE development.

EP29 An evaluation of the use of baseline pulmonary function tests in the detection of interstitial lung disease in patients newly diagnosed with rheumatoid arthritis

Abstract Background Interstitial lung disease (ILD) is a serious extra-articular manifestation of rheumatoid arthritis (RA). Risk factors include smoking, the presence of rheumatoid factor (RF) and anti-cyclic citrullinated peptide antibodies (CCP). Pulmonary function tests (PFT) show reduced carbon monoxide diffusion capacity (DLCO) early and reduced forced vital capacity (FVC) later in disease. HRCT is the gold standard diagnostic test while chest X-ray (CXR) has low sensitivity. PFT are routinely performed in the majority of RA patients at baseline at our tertiary centre. The aim of this study was to evaluate the frequency of abnormal PFT, specificity for ILD and influence on subsequent decision-making in patients newly diagnosed with RA. Methods A retrospective analysis was undertaken of patients with a new diagnosis of RA between January 2016 and December 2017. Patients meeting the ACR (2010) criteria for RA, with baseline PFT data available were included. Clinic letters and the hospital electronic records were used to obtain the data. Results 139 patients were included in the data analysis (Table 1). 23 patients had DLCO <70% predicted, while 7 patients had an FVC <80% predicted. Patients with abnormal PFT were more likely to be older, female, seropositive and to have smoked. Of the patients with DLCO <70%, CXR was abnormal in 6 patients with changes suggesting ILD in 2 patients. 13 patients had HRCT and 7/13 patients had evidence of ILD and 6/13 patients had significant emphysema on CXR or HRCT. 1 patient with DLCO of 82% had changes of ILD on a CT scan organised for another reason. Methotrexate was commenced in 19/23 patients with DLCO<70% and discontinued in 2 patients for respiratory reasons. Conclusion This evaluation suggests baseline PFT are more sensitive than baseline CXR in detecting ILD but that a DLCO <70% is not specific for this diagnosis. The abnormal PFT lead to HRCT being requested in 13/24 patients, of whom 7 had ILD which had not been identified by CXR in 5 patients. Baseline PFT are also useful as a reference point in patients who go on to develop respiratory symptoms at a later point in their illness. Disclosures A. Haque None. R. Kilding None. R. Smith None. S. Khalid None. R. Sandler None. M. Cox None. T. Hendry None. A. Flores-martin None. K. Lindop None. J. Maxwell None.

Consumption of Meat and Dairy Products Is Not Associated with the Risk for Rheumatoid Arthritis among Women: A Population-Based Cohort Study.

Diet has gained attention as a risk factor for the development of rheumatoid arthritis (RA), especially with regards to food of animal origin, such as meat and dairy products. By using data from national patient registers and dietary data from a large prospective population cohort, the Swedish Mammography Cohort, we aimed to investigate whether the consumption of meat and dairy products had any impact on the risk of subsequent development of RA. During 12 years of follow-up (January 2003–December 2014; 381, 456 person-years), 368 patients with a new diagnosis of RA were identified. No associations between the development of RA and the consumption of meat and meat products (hazard ratio [HR] for the fully adjusted model: 1.08 [95% CI: 0.77–1.53]) or the total consumption of milk and dairy products (HR for the fully adjusted model: 1.09 [95% CI: 0.76–1.55]) were observed. In conclusion, in this large prospective cohort of women, no associations were observed between dietary intake of meat and dairy products and the risk of RA development.

Is incident rheumatoid arthritis interstitial lung disease associated with methotrexate treatment? Results from a multivariate analysis in the ERAS and ERAN inception cohorts

ObjectivesTo assess predictive factors for rheumatoid arthritis interstitial lung disease (RA-ILD) in two early rheumatoid arthritis (RA) inception cohorts with a focus on methotrexate (MTX) exposure.DesignMulticentre prospective early RA inception...

Treatment delays for patients with new-onset rheumatoid arthritis presenting to an Australian early arthritis clinic.

Early treatment ensures optimal outcomes in rheumatoid arthritis (RA) yet there are limited data in Australia quantifying treatment delays in clinical practice. To quantify treatment delays in new RA patients and to explore factors influencing delay and resultant patient outcomes. Data were obtained for 88 patients presenting with a new diagnosis of RA to an early arthritis clinic (EAC) in Australia between 2008 and 2015. Date and details of symptom onset, initial general practitioner (GP) presentation, GP referral and review at EAC were collected. Patient demographics and clinical features were analysed for outcomes and features predictive of delay. Median overall delay from symptom onset to rheumatology review was 26.4 weeks. Patient delay (8.7 weeks) was the longest delay and predicted overall delay. Delays in GP referral and time to EAC review were 4 and 8.4 weeks respectively. Increased overall delay was predicted by lower fatigue and disease activity scores (DAS28) and increased tender joint counts (TJC). Patient delay was increased by socioeconomic disadvantage. Increased GP delay was associated with lower DAS28 and higher TJC and ESR. Patients seen within 16 weeks had greater improvement in DAS28 and probability of remission at 6 months. In this Australian EAC setting, patient delay was the greatest contributor to RA treatment delay. Delays in treatment were associated with lower disease severity and socioeconomic disadvantage. Remission was more likely after prompt initiation of treatment.

PSY148 - PRIMARY NON-ADHERENCE AMONG NEWLY DIAGNOSED RHEUMATOID ARTHRITIS PATIENTS

To describe the prescription and use of conventional and biologic disease modifying anti-rheumatic drugs (DMARD) use within one year following a new rheumatoid arthritis (RA) diagnosis. The IBM MarketScan Explorys Claims-EMR Data Set was used to identify adult patients with newly diagnosed RA from 2012-2017; the new diagnosis of RA was the index event. Patients were required to have two RA diagnoses at least 14 days apart, continuous enrollment for one year pre- and post-index, and EMR activity during the one-year follow-up period. Patients with prior RA diagnosis or use of an RA biologic were excluded. The primary outcomes were presence and timing of a prescription claim and/or physician order for a conventional or biologic DMARD. A total of 2,732 adults met the study inclusion criteria. Overall, 58.1% of patients received a conventional or biologic DMARD within one year of their new RA diagnosis. An additional 4.7% of newly diagnosed patients had a physician order for a DMARD but no corresponding fill or treatment receipt. Overall, 279 (10.2%) patients started a new biologic within one year post-index and an additional 32 patients (1.2%) had a physician order for a biologic DMARD but no corresponding fill. Put differently, 10.3% of newly diagnosed RA patients with a new physician prescription for an RA biologic do not receive that biologic. Of these patients, approximately two-thirds (62.5%) did receive a conventional DMARD, driven by methotrexate (70.0%). Overall, 56.8% of patients used a conventional DMARD and an additional 4.9% had a physician order for a conventional DMARD but no corresponding fill; non-fill rates were similar between methotrexate and the other conventional DMARDs. Clinical treatment guidelines recommend early and aggressive treatment of RA, however, one-in-ten patients prescribed a biologic DMARD and one-in-twelve prescribed a conventional DMARD do not receive one.

Methodology of a new inflammatory arthritis registry: TReasure

The TReasure registry, created in 2017, is an observational multicenter cohort that includes inflammatory arthritis patients. This article reviews the methodology and objectives of the TReasure registry established to collect data from rheumatoid arthritis (RA) and spondyloarthritis (SpA) patients. Fifteen rheumatology centers in Turkey will contribute data to the TReasure database. The actual proprietor of the database is the Hacettepe Rheumatology Association (HRD) and Hacettepe Financial Enterprises. Pharmaceutical companies that operate in Turkey (in alphabetical or er), Abbvie, Amgen, BMS, Celltrion Healthcare, Novartis, Pfizer, Roche, and UCB, support the TReasure registry. TReasure is a web-based database to which users connect through a URL (https://www.trials-network.org/treasure) with their unique identifier and passwords provided for data entry and access. TReasure records demographic and clinical features, comorbidities, radiology and laboratory results, measures of disease activity, and treatment data. TReasure will provide us with various types of data, such as a cross-sectional view of the current nationwide status of the patients currently receiving these treatments, and retrospective data as much as allowed by the participating centers’ records. Finally, a high-quality prospective dataset will be built over the ensuing years from patients with a new diagnosis of RA or SpA.

Comparative evaluation of treatment patterns and healthcare utilization of newly-diagnosed rheumatoid arthritis patients by anti-cyclic citrullinated peptide antibody status

Background: Anti-cyclic citrullinated peptide (CCP) antibody positivity is an established diagnostic factor for severe disease activity and joint damage and a prognostic factor for aggressive disease in rheumatoid arthritis (RA).Objective: To compare RA-related treatment, healthcare utilization, and joint erosion between anti-CCP-positive and anti-CCP-negative RA patients.Methods: Newly-diagnosed RA patients were identified from the Henry Ford Health System database between January 1, 2009 and December 31, 2014; the date of the first RA diagnosis within the study period was the index date. Baseline anti-CCP test was used to categorize patients as anti-CCP-positive or anti-CCP-negative, and outcomes were evaluated in the 6 months post-index.Results: There were 217 anti-CCP-positive and 191 anti-CCP-negative RA patients included in the study. A higher proportion of anti-CCP-positive patients were initiated on RA treatment than anti-CCP-negative patients (70.5% vs 23.0%; p < .0001). More anti-CCP-positive patients received methotrexate (73.2% vs 56.8%; p = .0374), while more anti-CCP-negative patients received hydroxychloroquine (31.8% vs 13.1%; p = .0037) in first-line therapy. A higher proportion of anti-CCP-negative patients were tested for rheumatoid factor (RF) and erythrocyte sedimentation rate (ESR). Of those tested, there were more positive test results in the anti-CCP-positive cohort compared to the anti-CCP-negative cohort (RF: 84.4% vs 18.2%, p < .0001; C-reactive protein [CRP]: 69.7% vs 48.3%, p = .0008; and ESR: 89.5% vs 53.9%, p < .0001). Outpatient utilization predominated, with more anti-CCP-positive patients having any outpatient physician office visit (96.3% vs 77.5%, p < .0001) and a higher mean number of visits (5.3 vs 2.5, p < .0001) than anti-CCP-negative patients. Among anti-CCP-positive (n = 113) and anti-CCP-negative (n = 58) patients with imaging results, more anti-CCP-positive patients had joint erosion compared to anti-CCP-negative patients (18.6% vs 8.6%; p = .0858); however, statistical significance was not reached.Conclusion: RA patients with positive anti-CCP antibodies had higher degrees of inflammation and disease activity as indicated by laboratory results, which likely contributed to their higher rates of healthcare utilization, joint erosion, and proportions of RA treatment.

AB0297 Does Delay of Therapy Affect Outcomes of Early RA in A T2T Clinic?

BackgroundVery early referral/start of therapy within 3-4 months vs. delayed referral has been shown beneficial for short- and long-term outcomes in rheumatoid arthritis (RA). However, treatment strategy in these studies...

SAT0459 Significant association between periodontitis and risk of rheumatoid arthritis: A nationwide, population-based, case–control study

BackgroundAlthough rheumatoid arthritis (RA) is associated with periodontitis, it remains unclear whether periodontitis is a significant risk factor for RA.ObjectivesTo investigate RA risk based on a history of periodontitis.MethodsThe study...

Biological therapy for rheumatoid arthritis: where are we now?

Since the introduction of targeted biological therapies, the implications of a new diagnosis of rheumatoid arthritis have changed dramatically. There are now several therapeutic options available for these patients and the target of treatment - remission - is now a realistic goal.

Assessment of synovitis in the knee of patients with newly-diagnosed rheumatoid arthritis using high frequency and color Doppler ultrasonography

To investigate the value of high frequency and color Doppler ultrasonography in detection of synovitis and the intra-articular vascularization in the knee joint of patients with newly-diagnosed rheumatoid arthritis (RA). Forty-one patients (30 women, 11 men) with newly-diagnosed RA were recruited to a cross sectional study (RA group). Forty-one age and gender-matched healthy volunteers were used as control group. The thickness of hydatid fluid, synovium hyperplasia, color flow imaging, peak systolic velocity (PSV), end diastolic velocity (EDV), resistance index (RI), venous blood flow and intra-articular perfusion were evaluated by high frequency and color Doppler ultrasonography. Totally 91.46% knee joints with synovial hyperplasia (> 2 mm) were found in 41 patients with RA (75/82 knee joints), and the thickness of the synovial membrane was 2.2 - 19.7 mm (average 6.3 ± 3.4 mm). In aspect of blood flow, the percentage of 0 to 3 grade were 18.67% (14/75), 29.33% (22/75), 45.33% (34/75) and 6.67% (5/75), respectively; the results of arterial blood were indicated with PSV (10.82 ± 3.71 cm/s), EDV (3.86 ± 1.12 cm/s) and RI (0.61 ± 0.07), while the average of venous blood velocity was 2.72 ± 1.02 cm/s. Joint effusion was found in 69 joints (84.15%) with the anteroposterior diameter 2.4 - 16.1 mm (average 6.9 ± 3.2 mm). The thickness of synovial membrane was 1.2 - 1.8 mm (average 1.4 ± 0.4 mm) and no significant difference were observed in joint effusion, signal of blood flow and thickness of synovial membrane in the control group. High frequency and power Doppler ultrasonography may be a valuable and optimal clinical tool to accurately and objectively detect synovial hyperplasia, vascular pannus formation and joint effusion in the knee joint of patients with RA.

A case of normal-pressure hydrocephalus associated with rheumatoid arthritis

Rheumatoid arthritis may cause central nervous system complications by means of various mechanisms. We describe the case of a patient with a new diagnosis of rheumatoid arthritis (RA) complicated by normal-pressure hydrocephalus. After treatment with prednisone, the patient improved remarkably as regards mental status, urinary control and gait. We suggest that normal-pressure hydrocephalus may occur as an extra-articular manifestation of RA, caused by a pathogenic inflammatory mechanism. We analyse previous case reports describing a relationship between RA and normal-pressure hydrocephalus, and discuss potential mechanisms underlying this association.

C-Reactive Protein in the Prediction of Rheumatoid Arthritis in Women

The purpose of this study was to examine whether levels of C-reactive protein (CRP), a sensitive marker of disease activity in rheumatoid arthritis (RA), are associated with increased risk of subsequent RA. Eligible subjects were 39 876 healthy women from the Women's Health Study, a completed randomized trial of aspirin and vitamin E in cardiovascular disease and cancer prevention, begun in 1992. We included 27 939 women who provided blood samples at baseline that could be assayed for CRP. During 9.9 years of follow-up, 398 women reported a new diagnosis of RA. Of these, 90 cases were confirmed on medical chart review using American College of Rheumatology criteria. In age-adjusted analysis, the relative risks for developing confirmed, incident RA associated with increasing tertiles of CRP (first, second, and third) were 1.00 (reference value), 0.94 (0.54-1.61), and 1.29 (0.78-2.12) (P = .30 for trend). Further adjustment for randomized treatment, age, body mass index, and smoking demonstrated corresponding relative risks of 1.00 (reference value), 0.95 (0.55-1.65), and 1.33 (0.77-2.30) (P = .48 for trend). When we examined whether CRP levels predicted incident RA within 4 years, between 5 to 8 years, and 9 or more years after CRP measurement, we found no significant associations for any time period. In this prospective study of healthy women, a single CRP level did not predict increased risk of RA. Furthermore, CRP measurement closer to the time of diagnosis was not predictive. The consistency of this effect throughout different time periods from diagnosis suggests that CRP does not have a large effect in predicting incident RA.

Long-Term Outcomes in Difficult-to-Treat Patients With Recurrent Pericarditis

Patients with many recurrences of acute pericarditis are commonly alarmed by the fear of constriction. We studied their long-term outcome and the possible presence of systemic diseases. Sixty-one Italian patients (36 men) were followed for an average of 8.3 years according to a predefined protocol, including testing for autoimmune diseases and familial Mediterranean fever. Symptomatic pericarditis lasted from 1 to 43 years (mean 5.4 years). Fifty-two patients had been referred to us after failure of previous therapies, including steroids. We observed 378 attacks with a mean of 1.6 per patient per year and 156 hospital admissions. Thirteen patients had a post-cardiac injury syndrome. In 43 (70.5%), the pericarditis remained idiopathic, whereas we made a new diagnosis of rheumatoid arthritis in 1 and of Sjogren's syndrome in 4 patients, but in these patients pericarditis represented the dominant clinical manifestation. Cardiac tamponade occurred during the initial attacks in 4 patients (6.5%) but never recurred. Pleural effusions were present during the first attack in 22 patients (36.0%) and liver involvement in 5 (8%). No patients developed constrictive pericarditis. Echocardiographic examination produced no evidence of chronic myocardial disease. Response to therapy was good. Thirty-one patients (50.8%) are in sustained remission, without any therapy; their total observation period has averaged 10.3 years. In idiopathic patients, antinuclear antibodies were present in 56.2% and anti-Ro/SSA in 8.3%. Mutations linked to familial Mediterranean fever were absent. In conclusion, in this large series of difficult patients with recurrent acute pericarditis and a very long follow-up, the long-term prognosis is good.