Search

Background Inhibition of interleukin-1 (IL-1) represents a promising treatment option in adult-onset Still’s disease (AOSD). Canakinumab (CAN) is approved for treatment of systemic juvenile idiopathic arthritis (sJIA) since 2013 and recently for AOSD in Europe. In sJIA, CAN has a proven marked impact on systemic and articular disease activity, but for AOSD RCT data is limited. Objectives Investigation of efficacy and safety of CAN in AOSD patients with joint involvement by means of a multi-centre, double-blinded, randomized, placebo controlled trial. Methods AOSD patients with joint involvement (tender and swollen joint count ≥4 each) were stratified by pre-treatment status with biologics and randomized in a ratio of 1:1 to CAN (4 mg/kg up to a maximum of 300 mg) or placebo (PBO) s.c. every 4wks. The primary endpoint was defined as the proportion of patients with a clinical-relevant reduction (≥1.2) in disease activity as measured by change in disease activity score (DAS28) at week 12. There were two major protocol violations: two PBO patients received blinded CAN at week 4. Therefore, a per-protocol (PP) analysis, which excluded data of these two patients, was performed. Results The study was prematurely stopped with 35 out of 68 planned patients enrolled due to approval of CAN for AOSD by EMA. At enrollment, mean DAS28 represented high disease activity for both: 5.4 in CAN (n=18) and 5.3 in PBO (n=17). In the intention-to-treat analysis, 12 (67%) CAN and 7 (41%) PBO patients met the primary endpoint. The 25.5% [95%CI: -10.3%; 55.9%] difference in the response rate did not achieve statistical significance (p=0.18). Using the PP analysis, the treatment difference for primary endpoint was 33.4% [95%CI: -10.3%; 55.9%, p=0.08]. We observed higher ACR50 (50% CAN vs. 6.7% PBO, p= 0.009), ACR70 (28% CAN vs. 0% PBO, p=0.049) response rates, (PP analysis). Higher mean improvements in the CAN vs PBO group for DAS28(ESR and CRP), ESR, CRP, 66 swollen and 68 tender joint counts at week 12 were observed (PP analysis). There were 47 and 21 non-serious adverse events (AE) reported in CAN and PBO groups, respectively. Two CAN patients experienced an SAE (hepatotoxicity and patellofemoral pain syndrome). Conclusion Although the study was terminated prematurely and the primary endpoint did not achieve statistical significance, treatment of patients with active AOSD with CAN led to improvement of several outcome measures. In addition, safety profile was similar to that reported in sJIA and no unexpected safety issues were observed. This data support the treatment of AOSD patients with CAN. References [1] Ruperto. NEJM (2012): 2396-2406 Disclosure of Interests Claudia Kedor Grant/research support from: Sobi, Consultant for: Novartis, Speakers bureau: Novartis, Roche, Joachim Listing: None declared, Jan Zernicke: None declared, Anja Weis: None declared, Frank Behrens Grant/research support from: AbbVie, Pfizer, Roche, Chugai, Prophylix, Bioline, Novartis, Consultant for: AbbVie, Pfizer, Roche, Chugai, UCB, Bristol-Myers Squibb, Celgene, MSD, Novartis, Biotest, Janssen, Genzyme, Eli Lilly, Speakers bureau: Ad board: AbbVie, Pfizer, Roche, Chugai, UCB, Bristol-Myers Squibb, Celgene, Novartis, Biotest, Janssen, Genzyme, Eli Lilly, Norbert Blank Grant/research support from: SOBI and Novartis, Speakers bureau: Novartis and SOBI, Jorg Henes: None declared, Jorn Kekow: None declared, Andrea Rubbert-Roth Consultant for: Chugai, Eli Lilly, Roche, and Sanofi , Speakers bureau: AbbVie, Bristol-Myers Squibb, Chugai, Hexal/Novartis, Janssen, Eli Lilly, Merck Sharp & Dohme, Pfizer, Roche, and Sanofi , Hendrik Schulze-Koops Grant/research support from: Novartis, Pfizer, Roche, Consultant for: Abbvie, Actelion, Amgen, AstraZeneca, Biogen International, BMS, Celgene, Chugai, GSK, Hospira, Janssen-Cilag, Leo Pharmaceuticals, Lilly, MSD, Medac, Merck, Novartis, Pfizer, Hexal Sandoz, Sanofi, Roche, UCB, Paid instructor for: Abbvie, Actelion, Amgen, AstraZeneca, Biogen International, BMS, Celgene, Chugai, GSK, Hospira, Janssen-Cilag, Leo Pharmaceuticals, Lilly, MSD, Medac, Merck, Novartis, Pfizer, Hexal Sandoz, Sanofi, Roche, UCB, Speakers bureau: Abbvie, Actelion, Amgen, AstraZeneca, Biogen International, BMS, Celgene, Chugai, GSK, Hospira, Janssen-Cilag, Leo Pharmaceuticals, Lilly, MSD, Medac, Merck, Novartis, Pfizer, Hexal Sandoz, Sanofi, Roche, UCB, Eva Seipelt: None declared, Christof Specker: None declared, Eugen Feist Grant/research support from: Pfizer, Roche, Novartis, BMS, Sanofi, Lilly, Consultant for: Pfizer, Roche, Novartis, BMS, Abbvie, Celgene, Sanofi, MSD, Lilly, Speakers bureau: Pfizer, Roche, Novartis, BMS, Abbvie, Celgene, Sanofi, Lilly

The German Society of Rheumatology and the Committee for Student Training investigated what effects the structures in university medicine have on student teaching. In February 2014 a questionnaire was sent to the teaching staff and Deans of each of the 37 medical faculties. Of the locations seven were classified as being independent rheumatological university hospitals and nine universities had a W2/W3/C3 grade professor as head of a department of clinical rheumatology but answerable to superiors. In the 37 faculties in Germany the proportion of lecture hours, the proportion of obligatory lecture hours, the number of hours for practical exercises and the number of hours for bedside teaching were distributed very differently and as a rule higher in universities with academic freedom. Not all medical faculties have obligatory teaching in the field of clinical rheumatology. On average medical students see five patients with rheumatological symptoms during their studies. In summary, over the past years it has not been possible to successfully utilize the great importance of rheumatology for society and the innovation potential of this discipline in order to improve the integration of clinical rheumatology into universities.

We thank our colleagues So and Tam from Hong Kong for their comments1 on our recommendations for the management of patients with inflammatory rheumatic diseases (IRD) during the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)/COVID-19 pandemic published in this journal.2 In our manuscript, we have recommended to ensure necessary controls for therapy and disease monitoring; however, we have suggested to always weigh the individual risk of an infection associated with a visit to the doctor’s office (eg, while travelling to and from as well as being present at the medical facility) against the risk of missing controls. In many IRD, temporary prolonged intervals of monitoring patients with stable disease and stable efficacious therapy may well be justified. Thus, in these patients, this measure may help to decrease the risk of getting infected with SARS-CoV-2. However, prolongation of monitoring intervals should be limited in time and as the pandemic continues, patients will have to be monitored again in order to receive appropriate care. …

Was ist neu? Stand der Dinge Innovationen in der Informations- und Kommunikationstechnologie haben in der Rheumatologie eine jahrzehntelange Tradition, die Deutsche Gesellschaft für Rheumatologie hat im Jahr 2018 die Kommission „Digitale Rheumatologie“ gegründet. Digitale Anwendungen in der deutschen Rheumatologie Mobile Datenerfassung rheumatologischer Patienten ist valide möglich. Sie eröffnet damit neuartige Möglichkeiten bei der Umsetzung moderner Behandlungsstrategien. Zum Screening auf entzündlich-rheumatische Systemerkrankungen stehen Interessierten z. B. mit dem „Rheuma-Check“ und dem „Bechterew-Check“ jederzeit digitale Anwendungen zur Verfügung. Die digitalen Angebote sowie moderne Netzwerkstrukturen erlauben eine Triage der Patienten. Mit dem Rhekiss- und dem RABBIT-SpA-Register stehen erstmals vollständig digitalisierte Register zur Verfügung, die kurzfristig Daten zu Fragestellungen liefern, die in klinischen Studien der pharmazeutischen Industrie nicht abgebildet werden. Ausblick Durch die Digitalisierung in der Rheumatologie können wichtige Fragen der Versorgungsforschung zukünftig deutlich schneller beantwortet werden.

Background: Inhibition of interleukin-1 (IL-1) represents a promising treatment option in adult-onset Still's disease (AOSD). Canakinumab is approved for treatment of systemic juvenile idiopathic arthritis and has a marked impact on systemic as well as articular activity of the disease. Objective: To investigate the efficacy and safety of canakinumab in patients with AOSD and active joint involvement by means of a multi-centre, double-blinded, randomized, placebo controlled trial. Methods: Patients with AOSD and active joint involvement (tender and swollen joint count ≥4 each) were stratified by pre-treatment status with biologic DMARDs to canakinumab (4 mg/kg, maximum 300 mg s.c. q4w) or placebo. After approval of canakinumab for AOSD by the European Medicines Agency, recruitment was stopped prematurely with enrollment of 35 out of 68 planned patients. The primary endpoint was the proportion of patients with a clinically-relevant reduction in disease activity at week 12 as determined by the change in disease activity score (ΔDAS28 >1·2). Results: At enrollment, patients had high active disease with a mean DAS28(ESR) of 5·4 in the canakinumab group (n=18, [CI 43·1 ; 85·2]) and 5·3 in the placebo group (n=17, [CI 20·1 ; 65·0]). In the intention-to-treat analysis, 12 (67%) canakinumab and 7 (41%) placebo patients fulfilled the primary outcome criterion (p=0.18). In the per-protocol analysis, significantly higher ACR30 (61% vs. 33% p=0.033), ACR50 (50% vs. 6·7% p= 0.009) and ACR70 (28% vs. 0% p=0·049) response rates were observed in the canakinumab group compared with placebo. Two patients in the canakinumab group experienced an SAE. Conclusion: Although the study was terminated prematurely and the primary endpoint was not achieved, treatment with canakinumab led to an improvement of several outcome measures in AOSD. The overall safety findings were consistent with the known profile of canakinumab. Thus, our data support indication for IL-1 inhibition with canakinumab in AOSD. Trial Registration: EudraCT No. 2011-001027-20. Funding Statement: Novartis Pharma GmbH Deutschland for financial support and study drug provision. Declaration of Interests: CK has received personal advisory board and congress fees from Novartis, Pfizer, Roche, and Sobi. FB has received grants from Abbvie, Pfizer, Roche, Chugai, Janssen and consultant, speaker, or advisory board fees from Abbvie, Pfizer, Roche, Chugai, UCB, BMS, Celgene, MSD, Novartis, Biotest, Janssen, Genzyme, Lilly; Boehringer; and Sandoz. NB has received grants from Novartis and Sobi, and personal fees from Novartis, Sobi, Lilly, Pfizer, Abbvie, BMS, MSD, Actelion, UCB, Boehringer-Ingelheim, and Roche. JH has received grants from Novartis, Roche, Celgene, and advisory board and speaker fees from Novartis, Roche, Pfizer, Abbvie, Sanofi, Boehringer-Ingelheim, and Celgene. ARR has received speaker fees from UCB, and advisory board and speaker fees from Novartis, Roche, Lilly, Pfizer, Abbvie, BMS, Janssen, Sanofi, and Celgene. CS has received personal fess from Abbvie, BoehringerIngelheim, Chugai, Lilly, Novartis, Sobi, UCB, Celgene, Janssen-Cilag, MSD, Pfizer, Roche, UCB, and Toshiba. EF has received advisory board and speaker fees from Novartis, Roche, Sobi, Lilly, Pfizer, Abbvie, BMS, MSD, and Sanofi. The remaining authors declare no competing interests. Ethics Approval Statement: The study was conducted according to the ethical principles of the Declaration of Helsinki. The study protocol and all amendments were reviewed by the Independent Ethics Commission of the State of Berlin (Ethik-Kommission des Landes Berlin) and Independent Ethics Committees for each center (EudraCT No. 2011-001027-20, Protocol No.: CACZ885GDE01T, ethic No 11/0561 – ZS EK 11). All patients provided written informed consent before any assessment was performed.

Antiphospholipid syndrome (APS) is characterized by recurrent arterial or venous thromboembolism or pregnancy loss in association with antibodies directed against anionic phospholipids or against plasma proteins bound to anionic phospholipids. It occurs in 15-30 % of patients suffering from systemic lupus erythematosus (secondary APS) but can also occur without an underlying connective tissue disease (primary APS). The reason for the huge variety of clinical manifestations is thromboembolic vaso-occlusive disease and not vasculitis in blood vessels of different sizes and sites (e.g. deep vein thrombosis, pulmonary embolism and cerebrovascular disease). Accordingly, fetal abortion is also caused by infarctions of blood vessels in the placenta typically after the tenth week of gestation.Establishing the correct diagnosis of APS is not easy. Diagnosis should not rely on only slightly or temporarily elevated levels of AP antibodies as thrombosis might occur due to other reasons even in patients with AP antibodies. The estimation of the risk of thrombotic complications as well as the type and intensity of a suitable anticoagulation prophylaxis are challenging in the treatment of patients with APS.Antiplatelet agents (aspirin), heparin and vitamin K antagonists are used for therapy. Immunosuppression is not effective for the prevention of thromboembolic complications; therefore, not only problem issues on laboratory diagnostics but also current therapeutic recommendations are presented.

Einleitung: Eine deutschsprachige Leitlinie zur Diagnostik und Therapie der ANCA-assoziierten Vaskulitiden sollte erstellt werden. Methoden: Eine Expertenkommission aus 13 Mitgliedern wurde von der DGRh mit der Erstellung der Leitlinie beauftragt. Für eine systematische Literaturrecherche wurden[zum vollständigen Text gelangen Sie über die oben angegebene URL], 45. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 31. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), 27. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR)

Based on both historical development and functional advantages, such as in emergencies, hierarchical aspects dominate all levels of medicine. Doubtlessly, the high degree of respect for experts and their opinions may impede exchange between different levels of hierarchy. At medical conferences, discussions usually take place between experts, while patients, young doctors and students only listen and rarely actively participate. For the 11th European Lupus Meeting in Dusseldorf in March 2018, we have tried the fishbowl method to increase participation at all levels. Fishbowl is an interactive and dynamic technique with a group of discussants sitting in an inner circle that contains an additional empty chair and surrounded by the audience in an outer circle.1 The empty chair can be occupied by any member of the outer circle at any time to join the discussion immediately. After the additional discussant of the auditorium has made his/her statement, he or she will leave the chair again to create an opportunity for another member of the audience (figure 1). Figure 1 The fishbowl method. A group …

Objective The objective of this article is to evaluate the safety and clinical outcome of rituximab treatment in systemic lupus erythematosus (SLE) patients refractory to standard of care therapy in a real-life setting in Germany. Methods The GRAID registry included patients with different autoimmune diseases who were given off-label treatment with rituximab. Data on safety and clinical response were collected retrospectively. In SLE patients, clinical parameters included tender and swollen joint counts, fatigue, myalgia, general wellbeing, Raynaud’s and the SLEDAI index. Laboratory tests included dsDNA antibody titres, complement factors, hematologic parameters and proteinuria. Finally, the investigators rated their patients as non-, partial or complete responders based on clinical grounds. Results Data from 85 SLE patients were collected, 69 female and 16 male, with a mean disease duration of 9.8 years. The mean follow-up period was 9.6 ± 7.4 months, resulting in 66.8 patient years of observation. A complete response was reported in 37 patients (46.8%), partial response in 27 (34.2%), no response in 15 (19.0%). On average, major clinical as well as laboratory efficacy parameters improved substantially, with the SLEDAI decreasing significantly from 12.2 to 3.3 points. Concerning safety, one infusion reaction leading to discontinuation of treatment occurred. Infections were reported with a rate of 19.5 (including six severe infections) per 100 patient years. Conclusion With the restrictions of a retrospective data collection, the results of this study confirm data of other registries, which suggest a favourable benefit-risk ratio of rituximab in patients with treatment-refractory SLE.

Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease with a high prevalence in females of childbearing age. Pregnancy in SLE nowadays has favorable outcomes for the majority of women. However, flares of disease activity, preeclampsia, fetal loss, and preterm birth are well-known risks in such pregnancies. Anti-SS-A(Ro)/SS-B(La) antibodies put fetuses at risk for congenital heart block and neonatal lupus. Several risk factors for adverse pregnancy outcomes have been identified. Women with antiphospholipid antibodies or antiphospholipid syndrome and lupus nephritis represent a group with high risk for obstetric complications. Factors such as appropriate preconception counseling and medication adjustment, strict disease control prior to pregnancy, and intensive surveillance during and after pregnancy are essential to improve pregnancy outcome. The aim of this review article is to update on the medical care of pregnancy in these women to ensure the best maternal and fetal prognosis.

Background Approximately 1.5 million adults in Germany suffer from an inflammatory rheumatological condition. The most common among these are rheumatoid arthritis and spondyloarthritis-above all axial spondyloarthritis, including ankylosing spondylitis (Bekhterev's disease) and psoriatic arthritis. These systemic inflammatory diseases often affect the heart as well. Methods This review is based on pertinent articles retrieved by a selective literature search, on current European guidelines, and on the authors' clinical experience. Results Rheumatic inflammation of cardiac structures can manifest itself as pericarditis, myocarditis, or endocarditis. The heart valves and the intracardiac conduction system can be affected as well, leading to AV block. Functional sequelae, e.g., congestive heart failure, can arise as a consequence of any inflammatory rheumatic disease. The long-term mortality of rheumatic diseases is elevated predominantly because of the increased risk for cardiovascular comorbidities. The cardiovascular risk profile should therefore be re-evaluated regularly (e.g., at 5-year intervals) in cooperation with the patient's primary care physician. The cardiovascular manifestations of rheumatic disease, such as pericarditis, myocarditis, and vasculitis, are treated initially with high-dose glucocorticoids and then over the long term with maintenance drugs such as methotrexate and azathioprine. Biological agents are sometimes used as well. Conclusion In patients with inflammatory rheumatic diseases, the elevated cardiovascular risk should be kept in mind and preventive measures should be initiated early. This subject should be further studied in controlled trials so that the treatment options for patients with cardiac involvement can be evaluated.

On behalf of the Steering Committee of the German Society for Rheumatology, in 2016 the Interdisciplinary Commission on Healthcare Quality updated the 2008 memorandum on rheumatological healthcare in Germany. The update considers changes in therapeutic strategies, treatment targets as well as current structures in healthcare and the political framework. It concentrates on examination of the need for rheumatologists with a background in internal medicine and determines the gap between needs and supply. The internist rheumatologist is responsible for the care of patients with inflammatory rheumatic diseases and contributes to the care of patients with severe forms of other musculoskeletal diseases. At least 2 internist rheumatologists are needed for the outpatient care of 100,000 adult inhabitants, equivalent to 1350 rheumatologists in Germany. With currently 776 rheumatologists, we have little more than half of what we need. The German Society for Rheumatology calls for specific requirements planning for rheumatologists in outpatient care in order to decrease the deficit. In acute inpatient care we need specialized hospitals and wards that ensure a high quality of treatment for patients with complex diseases. We need up to 50 beds per 1 million inhabitants. At least 2 full-time internist rheumatologists and 3 further physicians are needed per 30 beds. In inpatient and outpatient rehabilitation we need 40 beds or outpatient places per 1 million inhabitants with at least 1 full-time rheumatologist and 1 further physician. In order to reduce the existing deficits and to cover the increasing future need for rheumatologists, more emphasis has to be laid on primary and secondary education. Chairs for internal rheumatology are needed at each medical university and more positions for postgraduate training in rheumatology should be provided. In all segments of healthcare the treatment aims should be jointly defined between patients and physicians. The patients should be treated in an interdisciplinary network, comprising other medical specialties, health professionals as well as patient organizations.

Objective. MMF is teratogenic and needs to be replaced before pregnancy. This change may lead to flares. Our aim was to determine the risk of renal flares in women with LN who switched treatment from MMF to AZA before conception and to evaluate the outcome of their pregnancies. Methods. Medical records of women with LN counselled for pregnancy wish were reviewed. Women receiving treatment with either MMF or AZA (control group), with inactive lupus (SLEDAI4 4) and quiescent LN (serum creatinine

Objective.Rituximab (RTX) therapy is a treatment option in patients with refractory antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We investigated the tolerability and clinical efficacy of RTX in a cohort of patients with refractory AAV.Methods.Clinical and safety data of patients with AAV treated with RTX were retrospectively assessed from the data of a German national registry.Results.In total, 58 patients were included in this analysis (50/58 with granulomatosis with polyangiitis; 8/58 with microscopic polyangiitis who received at least 1 cycle, 17 patients who received 2 cycles, and 3 patients who received 3 cycles of RTX). Response was classified as complete and partial in 22 (40%) and in 29 cases (52.7%), respectively. Four patients (7.3%) were classified as nonresponders.Conclusion.RTX was well tolerated with good clinical efficacy in patients with refractory AAV.

Digital clubbing is usually secondary to different acquired diseases. Primary hypertrophic osteoarthropathy (PHO) is a rare hereditary disorder with variable digital clubbing as the most prominent feature, subperiosteal new bone formation, and arthropathy. Recently, mutations in the 15-hydroxy-prostaglandin dehydrogenase (15-PGDH) encoding gene HPGD were found to cause PHO. Here, we identified three unrelated families with different mutations in the prostaglandin transporter (PGT) encoding gene SLCO2A1 which presumably result in reduced metabolic clearance by 15-PGDH due to diminished cellular uptake of prostaglandin E2 (PGE2) by mutant PGT. In two consanguineous families, homozygous mutations, an intragenic deletion that results in frameshift and a missense mutation, are associated with a severe PHO phenotype. In a third family, a heterozygous carrier of a stop mutation presents with isolated digital clubbing. Thus, our study further supports the importance of PGE2 metabolism in the pathogenesis of digital clubbing and PHO. Hum Mutat 33:660–664, 2012. © 2012 Wiley Periodicals, Inc.

Objective To analyze the inquiries sent to an online ask-the-rheumatologist service in order to identify the users' needs and requirements. Methods The official web site of the German Competence Network Rheumatology (www.rheumanet.org) provided expert information for patients, relatives, and physicians. We analyzed the content of 1,133 inquiries posted over 5 years and the experts' answers were blinded for analyses. Results Patients (60.0%), relatives (24.3%), and physicians (15.7%) addressed the experts. Inquiries were predominantly sent by women (62.2%). Distinct rheumatic diseases were mentioned in 40.5% of the inquiries, and 16.3% reported musculoskeletal symptoms without a definite diagnosis. The number of questions ranged from 1–7 per inquiry (mean ± SD 1.58 ± 0.9). Of the inquiries, 33.2% contained personal histories, 24.9% searched for a rheumatologist nearby, and 11.6% asked for a “second opinion.” The questions covered a wide range of interests, including medication (30.8%), diagnosis-related issues (15.7%), laboratory tests (6.9%), (treatment) guidelines (6.2%), sexual and reproductive health issues (4.1%), and clinical trials (3.4%). In more than 50% of the inquiries, the information requested from the experts was already at least partly published on the web site. The experts' answers covered the users' questions completely in 91.8%, partly in 6.1%, and not at all in 2.1%. Conclusion A standardized medical web site providing tailored and trustworthy information for all user groups gains from an ask-the-expert service. Only such an interactive online application is able to satisfy users' actual demands: searching for specific individualized information on the internet. Therefore, an ask-the-expert service contributes to optimized patient care.

The current European Society for Immunodeficiencies (ESID) registry was established 10 years ago in 2004, when the system was moved from a paper-based to an online system. The purpose of the registry is to collect data on European patients with primary immunodeficiencies (PIDs) and their treatment, with the aim of building an easily accessible database for use by physicians and researchers. During the 10 years for which the current registry has been operational, the number of patients registered has grown substantially; after 4 years, more than 7000 patients were registered and, as of 25 June, 19 355 patients are registered in Europe as having a PID. Longitudinal data (20 or more data sets, documented every 6 months) is now available for some of the patients in the registry. Children younger than age 15 years represent two-thirds of this cohort. More than half the patients (57%) have an antibody disorder, and this is also the group with the greatest number of adult patients. Of the 19 355 registered, treatment data are available on approximately 14 000, of whom 6476 receive immunoglobulin (Ig) treatment; 4239 patients are receiving intravenous immunoglobulin (IVIg) treatment and approximately 2181 receive subcutaneous immunoglobulin (SCIg). One of the interesting comparisons arising from the registry data is the difference in minimum prevalence of PIDs between countries. In France, for example, 6·058 cases are documented per 100 000 inhabitants; much higher than in Switzerland (4·157 per 100 000) or Germany (2·105 per 100 000). This is most probably because France has a very efficient documenting system, whereby designated documenters, financed by local grants in France, visit centres to enter patients' data into the registry. Thus, the more efficient the documenting system, the higher the quality of data collected. Physicians and researchers can apply for access to the registry data by writing to ESID with details of their specific project, or with a proposal to collect new and different data on patients with specific diseases. Despite the large number of patients in the registry and the large amount of data collected, only 23 papers have been published, which is an average of more than two per year over 10 years. However, considering that more than 200 physicians and researchers have access to the database, this still means that the registry is one of the most under-used data sets of its kind in Europe. In a recent registry publication we analysed the subset of common variable immunodeficiency (CVID) subjects 1. There are more than 4000 CVID subjects in the registry, but because not all have complete data sets, the cohort described in this paper included 2212 subjects. As CVID has a variable clinical presentation, we investigated the frequencies of different disease phenotypes in the cohort 1. The most common clinical features were pneumonia (32%) and autoimmunity (29%). Other features included splenomegaly (26%), bronchiectasis (23%) and granulomatous disease (10%), and a further 10% had enteropathy. Surprisingly, 5% of patients had solid tumours and 4% had meningitis/encephalitis [conditions associated more usually with X-linked agammaglobulinaemia (XLA)]. Less frequent clinical features were lymphoma, found in 3% of the patients, splenectomy in 2% and lobectomy in 1%. With regard to how efficiently patients are being diagnosed, CVID has been described as having a bi-modal age of manifestation, with a peak in diagnoses between the ages of 5 and 10 years, a trough around the age of 20, and then another peak between 30 and 40 years. However, when patients are asked about the onset of their symptoms the reported age is lower, with most patients experiencing recurrent infections in childhood, and fewer than half manifesting after age 20 (Fig. 1). In some countries, the delay between onset of symptoms and CVID diagnosis is greater than others, the average being 4·1 years, ranging from 1·8 years in Poland to 7 years in France 1. Figure 1 (a) Age at onset of symptoms in the total cohort (n = 1914), among female patients (n = 985) and among male patients (n = 929). (b) Age at diagnosis in the total cohort (n = 2134), among ... We showed that adult CVID cases were diagnosed more promptly than paediatric cases (aged

Background. An increased CD4⁺ CD25⁺ T-cell population is observed in Wegener's granulomatosis (WG). This T-cell population is not well characterized yet and their contribution to the disease pathogenesis remains obscure. Methods. Thirty patients with WG and 18 healthy controls (HC) were included in this study. The disease activity and extension were measured by the Birmingham Vasculitis Activity Score (BVAS) and the Disease Extent Index (DEI). Lymphocytes from peripheral blood were analysed by FACS for the expression of CD4, CD25, CD134 and GITR. Cytokine expression in these subsets was assessed too. Nasal, lung and renal tissues from WG patients were immunohistochemically stained for CD3 and CD134. Results. The percentage of CD134⁺ as well as GITR⁺ expressing CD4 ⁺CD25⁺ lymphocytes was increased in patients as compared to HC (37 ± 12% versus 27 ± 8%, P = 0.005; 18 ± 9% versus 11 ± 6%, P = 0.003). The expression of CD134 and GITR showed a significant correlation with disease activity (r = 0.5, P = 0.009; r = 0.55, P = 0.001). Most of these displayed the phenotype of effector memory T-cells (94 ± 4% and 91 ± 6%). CD134 T-cells were found in tissues affected by WG. Conclusions. CD4⁺CD25⁺ effector memory T-cells expressing CD134 and GITR seem to play a role in disease mechanisms, as suggested by their close association with disease activity and their participation in inflammatory process. © The Author [2008]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

B-cell-dependent autoantibody production is a hallmark of systemic lupus erythematosus (SLE) which requires costimulatory molecules. The aim of the study was to analyse the expression of costimulatory molecules on B cells in patients with SLE. Twenty-six patients with SLE (four male, 22 female, mean age 46 +/- 15 years) as defined by the American College of Rheumatology criteria and 13 healthy controls (three male, 10 female, mean age 43 +/- 15 years) were included in the study. In a subgroup analysis, SLE patients were divided according to renal involvement due to SLE (10 with and 16 patients without renal involvement). Clinical disease activity was assessed according to the systemic lupus erythematosus disease activity index (SLEDAI). Blood B-cell populations were analysed by FACS for the cell surface marker expression of CD27, CD38, CD71, CD80, CD86 and CD137 ligand. The expression levels of CD71, CD80 and CD86 on B cells were significantly enhanced in SLE patients when compared with healthy controls (27 +/- 3% versus 11 +/- 2%, P = 0.0003, 55 +/- 2% versus 28 +/- 4%, P0.0001, 34 +/- 3% versus 12 +/- 2%, P0.0001). CD86 expression was significantly elevated in patients with renal involvement when compared with patients without renal disease (43 +/- 6% versus 28 +/- 3%, P0.05). There was a significant correlation between the expression levels of CD80 and CD86 on CD19(+) B cells and disease activity. Moreover, prednisone dose significantly correlated with SLEDAI (r = 0.5, P = 0.02) and with the expression levels of CD86 (r = 0.47, P = 0.02). A pathological B-cell population is associated with disease activity and renal involvement in SLE which are obviously resistant to therapy with medium doses of prednisone.

Background: Adult-onset Still’s disease (AOSD), a rare autoinflammatory disorder, resembles systemic juvenile idiopathic arthritis (SJIA). The superimposable systemic clinical features of AOSD and SJIA suggest both clinical phenotypes represent the same disease continuum with different ages of onset. To further characterize the similarity between AOSD and SJIA at the molecular level, 2 previously identified response gene sets in SJIA were used to investigate how genes that respond to interleukin (IL)-1β inhibition with canakinumab in SJIA patients behave in AOSD patients with active disease prior to IL-1β targeting therapy, relative to healthy subjects. Findings: All genes downregulated in SJIA patients following canakinumab treatment were upregulated in most patients with active AOSD prior to canakinumab treatment, relative to healthy subjects. A few patients with milder AOSD had expectedly gene-expression patterns that resembled those in healthy subjects. Comparison of the gene-expression patterns with neutrophil counts showed a correlation between elevated neutrophil numbers and upregulation of canakinumab-responsive genes. Correspondingly, most genes upregulated following canakinumab treatment in patients with SJIA patients were downregulated in the majority of AOSD patients. Conclusions: These results further support the concept of a Still’s disease continuum that includes both a pediatric/juvenile onset (SJIA) and adult onset (AOSD) form.

Adult-onset Still's disease (AOSD) is a rare auto-inflammatory disorder resembling a similar pediatric syndrome known as systemic juvenile idiopathic arthritis (SJIA).[1] The superimposable systemic and clinical features in SJIA and AOSD suggest that both clinical phenotypes represent a disease continuum with a pediatric (SJIA) and more adult-onset (AOSD).[2] Analyses of gene expression profiles may be useful not only for disease classification, diagnosis, and prognosis, but also to identify disease specific treatment effects that counteract the underlying pathological mechanisms. Here, we address the question: How do genes that respond to canakinumab treatment in SJIA patients[3] behave in AOSD patients with active disease relative to healthy controls and prior to IL-1 targeting therapy?

We report a case of amyloidosis in association with hyperimmunoglobulinemia D syndrome (HIDS). The patient showed typical clinical features of HIDS. He had crescentic glomerulonephritis progressing to end-stage renal disease at age 13 years. Eight years later, he developed an AA-type amyloidosis with extensive involvement of the intestine, respiratory tract, and thyroid gland. These unusual complications of HIDS seriously challenge the assumption that the disease is associated with a good prognosis.

SummarySystemic lupus erythematosus (SLE) is characterized by a deviation of the immune system that involves T cell-dependent autoantibody production. The aim of this study was to investigate the role of co-stimulatory markers on T cells in this disease. Twenty-eight patients with SLE as defined by the American College of Rheumatology (ACR) criteria and 11 healthy controls were included into the study. Eleven patients had biopsy-proven lupus nephritis while 17 patients had no clinical evidence of lupus nephritis. Clinical disease activity was assessed according to the systemic lupus erythematosus disease index (SLEDAI). CD4+ T cell populations in the peripheral blood were analysed for the expression of co-stimulatory markers CD45RO, CD70, CD80, CD86, CD137, CD137L, CD134, CD152, CD154 and ICOS. SLE patients showed an increased frequency of peripheral CD4+ T cells expressing high levels of CD80, CD86 and CD134 compared to healthy controls (7·1 ± 1·5%versus 1·7 ± 0·9%; P

ZusammenfassungEs wird ein Fall zur Differenzialdiagnose der Crohn-Arthritis vorgestellt, welcher nach 13-wöchiger Symptomatik und diversen Behandlungsversuchen auf eine lokale Knochenstoffwechselstörung bei Osteomalazie aufgrund eines Vitamin-D-Mangels zurückgeführt werden konnte. Eine entsprechende „osteologische“ Therapie mit Bisphosphonaten und Vitamin D war im Gegensatz zu vorherigen Behandlungen mit Kortikosteroiden klinisch erfolgreich und führte auch zu einer Normalisierung der kernspintomographischen und szintigraphischen Knochenveränderungen.

Objective: Antiphospholipid syndrome (APS) is a rare coagulation disorder associated with recurrent arterial and venous thrombotic events. We analysed our experience with five APS patients who underwent cardiac surgery. In three of them the diagnosis of APS had been established before surgery, two patients were diagnosed after surgery. Methods: From March 1999 to March 2004 five patients with APS underwent cardiac surgery using cardiopulmonary bypass (CPB). We retrospectively reviewed their clinical data, operative and postoperative courses, and the longterm results. Results: Procedures performed were heart and lung transplantation (patient 1), endoventriculoplasty and CABG (patient 2), biventricular resection of endoventricular fibrosis and thrombus (patient 3), mitral valve repair repair and coronary artery bypass grafting (CABG, patient 4), and mitral valve replacement with closure of a patent foramen ovale (patient 5). There were three perioperative deaths (patients 1, 2 and 3), two of three patients in whom the diagnosis was known before surgery, survived (patients 4 and 5). In these patients, only half the dose of protamin (patient 4) and no protamin at all (patient 5) was applied to reduce the probability of postoperative thromboembolic complications. At 1 year follow up, only patient 4 had survived, patient 5 had died of the complications of intestinal thromboembolism. Conclusions: Patients with APS undergoing cardiac surgery belong to a high risk subgroup. Thus, though rare, APS can be a critical issue in cardiac surgery. Some of the cardiac patients with unexplained perioperative thromboembolic complications, such as graft occlusion, may turn out to have an undiagnosed APS. q 2005 Elsevier B.V. All rights reserved.

We report the clinical and bioptic findings for a 57-year-old woman with severe chloroquine-induced myopathy. Since 1989, she had been suffering from systemic lupus erythematosus (SLE) with renal involvement and undergone periods of treatment with azathioprine and cyclophosphamide. Additional therapy with chloroquine (CQ) was started because of arthralgia. At the same time, slightly increased creatine kinase (CK) levels were noted. Myositis was suspected, and the patient was treated with steroids. The CK increase persisted, however, and she developed progressive muscular weakness and muscular atrophy. Routine controls revealed markedly elevated CK levels of 1,700 U/l. The neurological and electrophysiological findings were not typical of myositis. Thus, muscle biopsy of the deltoid muscle was performed in order to exclude polymyositis or toxic myopathy. As it revealed chloroquine-induced myopathy, medication was stopped. Discriminating between primary SLE-induced affection of the musculoskeletal system and drug-induced side effects is important for appropriate treatment of SLE patients.

OBJECTIVE On behalf of the "Systemic Inflammatory Rheumatic Diseases Network" comprehensive, nationwide horizontal and vertical cross-linking of research and care is to be developed for the first time. The quality of scientific work and patient care is to be increased in the medium term through this improved communication and co-operation. Our objective was to determine what hardware and software are avail- able to the physicians involved, with a view to the Internet being used as a basis for communication and documentation within the network. METHODS A survey was carried out among 723 active members of the German Rheumatology Society (DGRh). Data on the hardware and software used and on Internet access were collected using a unilateral questionnaire. RESULTS The response rate among the addressed rheumatologists was 55.3%, with 64.1% of members in private practice replying. Of those responding 85% have Internet access, with rheumatologists in private practice using the Internet significantly less frequently at work than those working at a hospital (42% vs 80%). The latter accordingly reported a higher proportion of medical Internet usage (69% vs 52%, p

Methotrexate is beneficial in rheumatoid arthritis and has been used in small studies of patients with systemic lupus erythematosus. We describe a patient with severe subacute cutaneous lupus erythematosus refractory to therapy with antimalarials and corticosteroids. Treatment with methotrexate resulted in complete clearing of the skin lesions without any side effects. (J Am Acad Dermatol 2002;46:600-3.)

Antimalarials represent the first line in treatment of cutaneous lupus erythematosus (LE). However, some patients show no improvement on monotherapy with chloroquine or hydroxychloroquine. A 30-year-old female patient had treatment-resistant cutaneous LE exhibiting features of both LE tumidus and subacute cutaneous LE. Previously, the patient had been unsuccessfully treated with chloroquine, hydroxychloroquine, dapsone, and azathioprine, each in combination with variable doses of prednisolone. However, the LE lesions increased during these therapeutic regimens. A combination of chloroquine and mepacrine therapy led to improvement and then total clearing after 4 months of treatment.

Ein 25jahriger turkischer Patient stellte sich mit einer seit funf Jahren bestehenden „Schwellung” im Bereich, der Sprunggelenke beidseitig vor, die zuvor als HLA-B27-negative Spondylarthropathie mit peripherer Gelenkbeteiligung klassifiziert worden war. Die Beschwerdesymptomatik zeigte auf unterschiedliche Therapieversuche ein nur unzureichendes Ansprechen. Nach serologischer und radiologischer Diagnostik wurde als Ursache der Arthropathie eine β-Thalassaemia minor diagnostiziert. Klinik, Diagnose und Differentialdiagnostik der β-Thalassaemia minor werden diskutiert.

Objective: We evaluated the feasibility of electronic data capture of self-administered patient questionnaires using a Tablet PC for integration in routine patient management; we also compared these data with results received from corresponding paper–pencil versions. Methods: Standardised patient questionnaires (FFbH/HAQ, BASDAI, SF-36) were implemented in our documentation software. 153 outpatients (rheumatoid arthritis, systemic lupus erythematosus, spondyloarthritis) completed sets of questionnaires as paper–pencil and electronic versions using a Tablet PC. The quality and validity of data obtained using a Tablet PC and the capability of disabled patients to handle it were assigned; patients’ experiences, preferences and computer/internet use were also assessed. Results: Scores obtained by direct data entry on the Tablet PC did not differ from the scores obtained by the paper–pencil questionnaires in the complete group and disease subgroups. No major difficulties using the Tablet PC occurred. 62.1% preferred remote data entry in the future. Seven (4.6%) patients felt uncomfortable with the Tablet PC due to their rheumatic disease. Conclusions: Self-administered questionnaires via Tablet PC are a facile and capable option in patients with rheumatic diseases to monitor disease activity, efficacy and safety assessments continuously. Tablet PC applications offers directly available data for clinical decision-making improves quality of care by effective patient monitoring, and contributes to patients’ empowerment.

Programmed death (PD)-1 is a cell death receptor that, upon stimulation, leads to apoptosis. Previous studies have shown alteration of PD-1 expression on T cells and PD-1 genes in patients with systemic lupus erythematosus (SLE). The aim of this study was to assess the expression of this receptor on effector T cells in patients with SLE.In this study we enrolled 32 SLE patients and 31 healthy controls. T cells from peripheral blood were analysed by flow cytometry for the expression of PD-1. Interferon (IFN)-γ and interleukin (IL)-17-producing cells were investigated for the expression of this co-stimulatory marker.Percentages of CD4(+) T cells expressing PD-1 were significantly increased in patients with SLE compared to healthy controls. The percentage of PD-1 expression was correlated with the production of INF-γ (r = 0.83, p0.0001). We also investigated the production of IL-17 by PD-1(+) CD3(+) T cells. Inactive patients (3.2 ± 1.2% vs. 5.9 ± 3.5%, p = 0.002) and patients without lupus nephritis (LN) (3.2 ± 1.5% vs. 5.9 ± 3.5%, p = 0.005) showed lower levels of IL-17 compared to healthy controls.We have demonstrated increased expression of PD-1 on CD4(+) T cells in SLE patients and an association between PD-1 expression on CD4(+) T cells and IFN-γ expression on CD3(+) T cells. We have also shown that there is an altered subset of PD-1(+) T cells in inactive patients and patients without LN producing lower amounts of IL-17.

The German Society of Rheumatology approved new German guidelines for the sequential medical treatment of rheumatoid arthritis (RA) based on the European League Against Rheumatism (EULAR) recommendations for the management of RA published in 2010. An update of the EULAR systematic literature research was performed in Medline, Embase, and Cochrane databases. Meta-analyses, controlled trials, cohort studies, and registry data addressing traditional and biologic disease-modifying antirheumatic drugs, glucocorticoids, and treatment strategies published between January 2009 and August 2011 were included. Two reviewers independently evaluated and compared the additional data that had been published after the time limit set by the EULAR recommendations. A national guideline working group developed an adapted set of recommendations. The new German guidelines were accepted by vote using an informal Delphi approach. Twelve recommendations and the resulting updated treatment algorithm were developed and approved as a practical orientation for rheumatologists. These recommendations are based on a successive treatment with traditional and biologic disease-modifying drugs depending on the individual progress of the disease and distinct patient characteristics. The German guidelines have been developed on the basis of the internationally well-recognized EULAR recommendations. In addition, more recent evidence from a systematic literature research was considered. They have been developed and approved by a group of national experts aiming at guidance for rheumatologists to reach best medical practice.

Background: The pathogenesis of Sneddon's syndrome is unclear. This study addresses the question whether cerebral thromboembolism may be involved in the pathogenesis of the neurologic complications of the disorder. The study consisted of 13 patients with Sneddon's syndrome defined by both generalized livedo reticularis and a history of one or more cerebrovascular ischemic events; none had clinical or Doppler ultrasonographic evidence of atherosclerosis. Methods: Transcranial Doppler microembolic monitoring of the middle cerebral artery; blood screening for antiphospholipid antibodies (lupus anticoagulant, anticardiolipin antibodies). Results: Five patients (38%) showed clinically silent microembolism at transcranial Doppler monitoring, with individual microembolic event rates of the middle cerebral artery between 2 per hour and 33 per hour. In this group, the time since the last ischemic symptom was significantly shorter than in the eight patients without microemboli. Antiphospholipid antibodies were detected in three patients (23%), all of whom belonged to the microemboli-positive group. Conclusions: These data suggest that the detectability of both clinically silent cerebral microembolism and antiphospholipid antibodies may provide paraclinical evidence of active disease in patients with Sneddon's syndrome. The results support the notion that an immune-mediated prothrombotic state facilitating the formation of arterial thrombi with subsequent cerebral embolization, and/or triggering in situ thrombosis of cerebral vessels, plays a pathogenetic role in the neurologic manifestations of this disorder.

We aimed to assess the relationship between bone inflammation in multi-pinhole single-photon emission computed tomography (MPH-SPECT) and synovitis detected by magnetic resonance imaging (MRI) in early rheumatoid arthritis patients.MPH-SPECT with technetium dicarboxypropanedisphosphonate (Tc-99mDPD) and 3 Tesla MRI were performed in 10 early rheumatoid arthritis patients. Eighty finger joint sites were assessed for increased osteoblastic activity using visual and region-of-interest (ROI) analysis. Presence of joint inflammation in MRI was investigated using the subscores of the rheumatoid arthritis MRI score.Tc-99mDPD uptake was increased in 38 (47.5%) and 22 (27.5%) joint sites as determined by visual and ROI analysis, respectively. A total of 32 (84.2%) sites with increased bone metabolism showed a normal MRI bone signal. The MPH-SPECT uptake ratio was elevated only in the subgroup with severe synovitis (P0.001).In early rheumatoid arthritis, molecular imaging with MPH-SPECT detects higher rates of inflammatory bone involvement compared to MRI. Our preliminary data suggest that osteitis is related to severe synovitis.

Objectives To evaluate the utility of sequentially acquired, post hoc fused, magnetic resonance imaging (MRI) and multi-pinhole single photon emission computed tomography (MPH-SPECT) with technetium-99m-labeled disphosphonates (Tc99m-DPD) for the identification of finger joints with later erosive progression in early rheumatoid arthritis (ERA) patients. Methods Ten consecutive ERA patients prospectively underwent MPH-SPECT and MRI of metacarpophalangeal (MCP) joints prior to and after 6 months methotrexate therapy. Tc99m-DPD uptake was measured at proximal and distal MCP sites using regional analysis. The course of joint pathologies was scored according to the Rheumatoid Arthritis MRI Score (RAMRIS) criteria. Results The frequency of increased Tc99m-DPD uptake, synovitis and bone marrow edemadecreased under MTX therapy; but the number of bone erosions increased. Joints with progressive and new erosions on follow-up had a higher baseline Tc99m-DPD uptake (2.64 ± 1.23 vs. 1.43 ± 0.91) ( p = 0.02). Conclusions Joints with erosive progression are characterized by an early increased Tc99m-DPD uptake, even in absence of MRI bone pathologies. Tc99m-DPD MPH-SPECT might thus be of additional value to morphological MRI for the identification of RA patients with a high risk for erosive progression.

Pachydermoperiostosis as the primary form of hypertrophic osteoarthropathy is a rare hereditary disorder with a number of characteristic findings, e.g. periosteal hypertrophy, digital clubbing and pachydermia. Further typical symptoms include arthritic manifestations in all major joints. Literature review showed that therapeutical approaches usually focus on medical therapy alleviating chronic polyarthritis by controlling inflammation. In the case presented in this report anti-inflammatory therapy appeared to have been insufficient since knee arthritis persisted. Arthroscopic synovectomy and radiosynoviorthesis were performed consecutively. Postoperative and follow-up examinations indicate that this combined therapy reduces pain, increases range of motion and improves overall mobility, and is thus of lasting benefit to the patient.

Objective. Persistent T-cell activation is frequently observed in granulomatosis with polyangiitis (GPA, formerly known as Wegener's granulomatosis). T-cell activation is usually balanced by negative costimulatory molecules. The negative costimulator programmed death receptor-1 (PD-1) and its relevance to T-cell immunity have not been studied so far in GPA. Thus it is the aim of the study to characterize the role of PD-1 in GPA. Methods. Thirty-two patients suffering from GPA and 19 age-matched healthy controls (HCs) were enrolled. T-lymphocyte subsets from peripheral blood were analysed by flow cytometry for the expression of PD-1. The frequency of memory T cells and T cells producing pro-inflammatory cytokines was determined. Renal biopsies from GPA patients were stained for CD3 and PD-1. Results. PD-1 expression was increased on T-helper cells (Th cells) from GPA patients as compared with HCs. In addition, parameters of persistent T-cell activation and production of pro-inflammatory cytokines were positively associated with numbers of PD-1(+) Th cells in patients but not in HCs. Latent infection with CMV seemed to enhance PD-1 expression on CD4(+) and CD4(+)CD25(-) T cells. Interestingly, expression of PD-1 on CD4(+)CD25(+)T cells was inversely correlated with relapse rate. Importantly, lesional T cells were mostly lacking PD-1. Conclusions. The expression of the negative costimulator PD-1 is altered in GPA and might counterbalance persistent T-cell activation.

Objective. Lymphocytes of patients with rheumatoid arthritis (RA) have diminished receptor density; thus, patients with RA should show partial resistance to glucocorticoids. We investigated the glucocorticoid sensitivity of lymphocytes in RA patients compared with healthy subjects. Methods. We determined the effects of glucocorticoids on lymphocyte proliferation and cytokine release. Results. Proliferation and cytokine release were inhibited in RA patients to the same extent as in healthy controls. Conclusion. Diminished receptor density in RA patients does not result in glucocorticoid resistance.

Objective To assess cartilage glycosaminoglycan content and cartilage thickness in the metacarpophalangeal (MCP) joints of patients with early rheumatoid arthritis (RA) and healthy volunteers. Methods After review board approval and informed consent were obtained, 22 subjects were prospectively enrolled (9 patients with early RA [7 women and 2 men with a mean ± SD age of 49 ± 13 years; range 25–68 years] and 13 healthy volunteers [10 women and 3 men with a mean ± SD age of 51 ± 12 years; range 25–66 years). In a total of 44 MCP joints of the index and middle fingers, measurements of cartilage thickness and delayed gadolinium-enhanced magnetic resonance imaging (MRI) of cartilage (dGEMRIC) index (T1 [msec]) were obtained using the variable flip-angle method and a 3T MR scanner. MRIs were evaluated for bone edema, erosions, and synovitis (using the RA MRI Scoring criteria). Student's t-test was used to test the significance of differences between groups. Results The mean ± SD dGEMRIC index was 497 ± 86 msec in healthy volunteers and was significantly lower in the early RA group (421 ± 76 msec) (P = 0.042). There was no joint space narrowing seen on standard radiographs. No significant difference was found between cartilage thickness in patients with early RA and that in controls (index finger mean ± SD 1.27 ± 0.23 mm in RA patients versus 1.46 ± 0.34 mm in controls [P = 0.16] and middle finger 1.26 ± 0.23 mm in RA patients versus 0.97 ± 0.47 mm in controls [P = 0.10]). No significant correlation was noted between cartilage thickness and dGEMRIC index (R = 0.36, P = 0.88 in RA patients; R = 0.156, P = 0.445 in controls). Conclusion Our findings indicate that cartilage damage is present in the MCP joints of patients with early RA despite the absence of joint space narrowing on standard radiographs and MRI. Cartilage damage in RA can be imaged with dGEMRIC.