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Digitalization in medicine is of major interest since the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic. This article tries to present the induced changes and technical solutions with respect to the different parts in the patient journey. Symptom checkers, new health applications, digital appointment management etc. are described. Apart from the technical and digital possibilities, the changes in the quality of communication additionally have to be mentioned. There is an urgent need for further technical standardization including the interfaces. In many cases further studies must confirm the equivalence of digital applications in comparison to analogue techniques.

ZusammenfassungDie Durchführung von Videosprechstunden wird seit mehreren Jahren als ergänzende Form der medizinischen Versorgung in Ergänzung zu einem persönlichen Arzt-Patienten-Kontakt angesehen und teilweise auch gefördert. Die COVID-19-Pandemie hat der Nutzung von Videosprechstunden ungeahnte Aktualität und Aufmerksamkeit verschafft. Die Kassenärztliche Bundesvereinigung beschloss Sonderregelungen im Rahmen der COVID-19-Pandemie, die bisherige Hindernisse für den Einsatz von Telemedizin und Videosprechstunden (und auch teilweise der herkömmlichen Telefonie) reduziert. Die vorliegende Stellungnahme der DGRh zum Einsatz von Videosprechstunden soll einen Überblick darüber geben, in welcher Form und mit welchen Limitierungen die Videosprechstunde in der Rheumatologie in Deutschland anwendbar ist. Die Stellungnahme skizziert einen Ausblick, wie die Videosprechstunde welche Funktionen zukünftig in der rheumatologischen Versorgung übernehmen kann.

Background: The etanercept biosimilar SB4 is a TNF inhibitor authorised for use as a targeted Biological Disease-Modifying Anti-Rheumatic Drug (bDMARD). Various administration devices have been developed for subcutaneous self-injection of bDMARDs. Objective: This study surveyed patient satisfaction with their experience of using the SB4 pre-filled pen device. Methods: This non-interventional, cross-sectional, multi-centre study enrolled adult rheumatoid arthritis and spondyloarthropathy patients who had been treated for at least three months with the SB4 pre-filled pen. Based on a standardized questionnaire, patients rated general satisfaction, handling, user-friendliness, physical characteristics, and training material received. A total of 492 eligible patients completed questionnaires at 43 centres across Germany between August 2017 and June 2018. Data were analysed descriptively. Pre-defined subgroup analyses by previous therapy and by indication were performed. Results: Overall, 87% (95% CI 83% - 90%) of patients reported being ‘satisfied’ or ‘very satisfied’ with the pen. 89% of patients reported that the pen was ‘simple’ or ‘very simple’ to use. Most patients (87%) self-injected. 93% of patients who received training on the use of the pen were ‘satisfied’ or ‘very satisfied’ with the training provided. In this cross-sectional study, 12 patients reported an Adverse Event (AE) and one patient reported a treatment-related AE (nausea). Conclusion: The results demonstrated a high level of general satisfaction among patients using the SB4 pre-filled pen as well as satisfaction with ease of use for patients who were either naïve to bDMARDs or who had switched to SB4 from other bDMARDs.

In der aktuellen SARS-CoV-2 Pandemie bestehen viele Fragen mit Blick auf die sichere Behandlung von Patienten mit entzundlich-rheumatischen Erkrankungen. Auf viele dieser Fragen gibt es zur Zeit noch keine evidenzbasierte Antwort und das macht die Betreuung der Patienten nicht leicht. Die Deutsche Gesellschaft fur Rheumatologie e. V. (DGRh) will mit diesen ersten Empfehlungen Hilfestellung fur spezielle Belange in der Betreuung von Rheumapatienten angesichts der aktuellen Bedrohung durch SARS-CoV-2 geben. Um den dynamischen weltweiten Erkenntnisgewinn fur unsere Patienten zu nutzen, werden die Empfehlungen regelmasig aktualisiert. Die aktualisierten Versionen der Empfehlungen werden auf der Homepage der DGRh hinterlegt.

ZusammenfassungDie Granulomatose mit Polyangiitis (GPA) und die mikroskopische Polyangiitis (MPA) stellen als primäre nekrotisierende Kleingefäßvaskulitiden die häufigsten ANCA-assoziierten Vaskulitiden dar. Bei diesen, früher oft tödlich verlaufenden Erkrankungen kann heute durch eine Stadien-adaptierte Immunsuppression in den meisten Fällen eine Remission erzielt werden. Diese mündet jedoch nicht in eine Heilung oder medikamentenfreie Langzeitremission. Es bedarf deshalb einer remissionserhaltenden Therapie. Aktuelle randomisierte kontrollierte Studien zeigen, dass unter einer remissionserhaltenden Behandlung mit halbjährlich verabreichtem anti-B-Zell-Antikörper Rituximab weniger Rezidive auftreten als unter der bisherigen Standardmedikation mit Azathioprin bei vergleichbarem Nebenwirkungsprofil. Diese Daten führten 2018 zur Zulassung von Rituximab als remissionserhaltende Therapie der GPA und MPA. Unter einer solchen Rituximab-Therapie muss mit einer Immunglobulindepletion sowie späten Zytopenien mit begleitenden Infekten gerechnet werden. Leitlinien und Expertenempfehlungen zu Infektprophylaxen, Impfungen und Immunglobulinsubstitution werden in diesem Beitrag vorgestellt.

To prevent serious complications such as permanent loss of vision and structural vascular damage, treatment must be initiated quickly in patients with giant-cell arteritis (GCA). So far, usually long-term corticosteroids in cumulative high dosages have been the standard therapy option. However, steroids are often insufficient to achieve adequate disease control and are associated with serious adverse events. Therefore, steroid-sparing therapy options are the focus of interest.

Objective Autoantibodies against CD74 (anti-CD74) are associated with ankylosing spondylitis (AS). The present multicenter study, the International Spondyloarthritis Autoantibody (InterSpA) trial, was undertaken to compare the sensitivity and specificity of anti-CD74 and HLA-B27 in identifying patients with nonradiographic axial spondyloarthritis (axSpA). Methods Patients ages 18-45 years with inflammatory back pain of ≤2 years' duration and a clinical suspicion of axSpA were recruited. HLA-B27 genotyping and magnetic resonance imaging of sacroiliac joints were performed in all patients. One hundred forty-nine patients with chronic inflammatory back pain (IBP) not caused by axSpA served as controls, and additional controls included 50 AS patients and 100 blood donors whose specimens were analyzed. Results One hundred patients with inflammatory back pain received a diagnosis of nonradiographic axSpA from the investigators and fulfilled the Assessment of SpondyloArthritis international Society (ASAS) criteria. The mean age was 29 years, and the mean symptom duration was 12.5 months. The sensitivity of IgA anti-CD74 and IgG anti-CD74 for identifying the 100 axSpA patients was 47% and 17%, respectively. The specificity of both IgA anti-CD74 and IgG anti-CD74 was 95.3%. The sensitivity of HLA-B27 was 81%. The positive likelihood ratios were 10.0 (IgA anti-CD74), 3.6 (IgG anti-CD74), and 8.1 (HLA-B27). Assuming a 5% pretest probability of axSpA in chronic back pain patients, the posttest probability, after consideration of the respective positive test results, was 33.3% for IgA anti-CD74, 15.3% for IgG anti-CD74, and 28.8% for HLA-B27. A combination of IgA anti-CD74 and HLA-B27 results in a posttest probability of 80.2%. Conclusion IgA anti-CD74 may be a useful tool for identifying axSpA. The diagnostic value of the test in daily practice requires further confirmation.

ZUSAMMENFASSUNG: Am 21.12.2020 hat die EMA mit der mRNA-Vakzine BNT162b2 der Firmen BioNTech/Pfizer (Comirnaty®, BioNTech, Mainz, Deutschland; Pfizer, New York City, NY, USA) den ersten Impfstoff gegen SARS-CoV‑2 in der Europäischen Union zugelassen, am 06.01.2021 folgte bereits der zweite (Moderna®, Moderna, Cambridge, MA, USA). Viele weitere Zulassungen werden in Kürze folgen. Mit der Verfügbarkeit der Impfung sind bei Patienten/-innen genau wie bei Ärzten/-innen viele Fragen aufgekommen, die in diesem Hot-Topic-Artikel so aktuell wie möglich adressiert werden sollen. ABSTRACT: On 21 December 2020 the European Medicines Agency (EMA) approved the mRNA vaccine BNT162b2 (Comirnaty®, BioNTech, Mainz, Germany; Pfizer, New York City, NY, USA) as the first vaccine against SARS-CoV‑2 in the European Union and the second vaccine (Moderna®, Moderna, Cambridge, MA, USA) followed on 6 January 2021. Many more approvals will follow within a short period of time. With the availability of the vaccination many questions have arisen for patients as well as for physicians, which are addressed as up to date as possible in this hot topic article.

ZusammenfassungDer Ausbruch der COVID-19-Pandemie geht mit tief greifenden Einschnitten im Alltag und im Berufsleben einher – sowohl gesamtgesellschaftlich als auch speziell im Gesundheitswesen. Im Fokus der Pandemieeindämmung haben sich vielerorts rheumatologische Routineabläufe verändert. Um den entsprechenden Infektionsschutz der Patienten und des medizinischen Personals gewährleisten zu können, wurde hier verstärkt Telemedizin (insbesondere Telefon- und Videosprechstunde) eingesetzt. Weiterhin stehen durch die Digitale-Gesundheitsanwendungen-Verordnung (DiGAV) voraussichtlich in den kommenden Monaten neue, abrechnungsfähige telemedizinische Anwendungsmöglichkeiten wie Apps und Wearables zur Verfügung. Der Artikel soll einen Überblick über telemedizinische Versorgungsmöglichkeiten in der Rheumatologie (mit besonderem Fokus auf die Videosprechstunde) geben. Weiterhin wird Bezug auf die vorhandene Evidenzlage sowie Chancen und Limitation der Telemedizin im Fachgebiet genommen.

Einleitung: Die Riesenzellarteriitis (RZA) und Takayasu – Arteriitis (TAK) sind Großgefäßvaskulitiden (GGV), die zu schweren Komplikationen wie Erblindung, Organ- und Extremitätenischämien und bei einem Teil der Erkrankten zum Tod führen können. Glukokortikoid-assoziierte[zum vollständigen Text gelangen Sie über die oben angegebene URL], Deutscher Rheumatologiekongress 2020, 48. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 34. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh)

A few days after the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection was declared a pandemic, the German Society for Rheumatology (DGRh) compiled the first group of recommendations for the care of patients with inflammatory rheumatic diseases in light of SARS-CoV-2/coronavirus disease 2019 (COVID-19). These first recommendations were based on an expert consensus and were largely non-evidence-based. Now that the first scientific data from registries, cross-sectional studies, case reports and case series are available, the DGRh has developed a timely update. This update is based on a literature search of publications available through 15 June 2020 and addresses preventive measures (such as hygiene measures or vaccinations) and the use of immunomodulatory/immunosuppressive drugs. Driven by the commitment to let patients benefit from these new evidence-based recommendations as quickly as possible, the DGRh published the update in German on its homepage and in the Zeitschrift für Rheumatologie immediately after completion. Here we report the key recommendations to make them available to the international community, provide the scientific methodology used to develop the recommendations, give additional thoughts and advice for the management of patients with rheumatic diseases during the COVID-19 pandemic and discuss our recommendations in the context of other international recommendations.

Background:Spondyloarthritis (SpA) is a severe chronic inflammatory disease, which affects quality of life and functional status. It frequently occurs in women of childbearing age. Active disease and TNFi discontinuation at early pregnancy were found to be risk factors for flares during pregnancy (1).Objectives:To compare disease activity during pregnancy in patients with or without bDMARD exposure at conception and during pregnancy and to assess pregnancy outcomes.Methods:RHEKISS is a prospective longitudinal cohort study including patients with confirmed diagnose of inflammatory rheumatic disease. Pregnant patients are eligible to be enrolled until the 20th week of gestation regardless of drug treatment. During observation, information on treatment, disease and pregnancy course, and outcome is collected from rheumatologists and patients. For this analysis, pregnancies of patients with SpA were selected and stratified into three groups according to their exposure to bDMARDs.Results:Of 140 SpA pregnancies included, 74 (53%) were not exposed to bDMARDs at conception (group 1), 38 (27%) were exposed to bDMARDs at conception, but not during pregnancy (group 2) and 28 (20%) were continuously exposed to bDMARDs at conception and during pregnancy (group 3). Certolizumab (50%), Adalimumab (20%), Etanercept (8%) and Infliximab (8%) were the most frequently prescribed bDMARDs at beginning of pregnancy. Baseline characteristics according to treatment exposure are shown in Table 1. Frequency of flares was highest in group 2: 21%, 38%, and 39% of patients flared during the 1st, 2nd, and 3rd trimester. These rates were 20%, 25%, and 21% in group 1 and 8%, 20%, and zero in group 3. The difference in flare rates was also mirrored in the course of physician assessed global disease activity (Figure 1). Whereas patients in group 1 seemed to have a quite stable disease activity during pregnancy, those who were in group 2 had an increasing activity of disease during pregnancy with an even higher increase of disease activity after giving birth. Patients in group 3 had the lowest disease activity.Of 137 singleton pregnancies, 130 (95%) ended in live birth. Of 6 spontaneous abortions 2 were in every of the three groups. One pregnancy in group 1 was terminated in gestational week 22 due to suspect malformation. One baby of the triple pregnancy was born and two aborted. All babies of the twin pregnancies were born healthy.Conclusion:SpA patients treated with bDMARDs at conception are not at higher risk for adverse pregnancy outcomes. Our results in a larger patient population confirmed that discontinuation of bDMARDs after conception is associated with increased disease activity during pregnancy and after birth and a higher risk of flares.References:[1]van den Brandt S et al., Arthritis Res Ther. 2017; 19(1):64.Table 1.Baseline characteristics; numbers are n (%) if not otherwise specified; * value at beginning of pregnancy: first 22 weeks after conceptionParameterno bDMARD at conception (group 1)n=74bDMARD at conception anddiscontinuedduring pregnancy(group 2)n=38bDMARD at conception andcontinuedduring pregnancy (group 3)n=28Totaln=140Singleton72 (97)37 (97.4)28 (100)137 (97.9)Twin1 (1.4)1 (2.6)02 (1.4)Triple1 (1.4)001 (0.7)New-York criteria fulfilled21 (33)17 (49)10 (48)48 (40)disease duration in years, mean (SD)6.4 (5.9)7 (4.1)5.8 (4)6.4 (5.1)age*, mean (SD)33.4 (4.9)32.3 (4)31.6 (3.4)32.7 (4.4)severity of illness*: asymptomatic4 (6)0 (0)3 (14)7 (6) mild31 (48)6 (17)4 (19)41 (34) moderate24 (38)21 (60)14 (67)59 (49) severe5 (8)8 (23)013 (11)HLA-B27 positive41 (62)24 (80)15 (75)80 (69)CRP in mg/l *, mean (SD)6.6 (8.2)5.4 (8.2)5.2 (4.9)6 (7.6)CRP >5mg/l *25 (41)9 (30)8 (35)42 (37)physician global* (NRS 0-10), mean (SD)2.6 (2)2.3 (2.5)1.7 (1.4)2.4 (2.1)BASDAI* (0-10), mean (SD)3.2 (2)2.9 (2.3)2.8 (1.5)3.1 (2)patient global* (NRS 0-10), mean (SD)3.3 (2.7)3 (2.8)3 (2.3)3.1 (2.6)Figure 1Course of physician assessed global disease activityDisclosure of Interests:None declared.

Background:Although seronegative RA patients often present with substantial disease burden [1], patients are underrepresented in research cohorts or clinical trials. Consequently, less knowledge about this subgroup has been accumulated.Objectives:To describe outcome of seronegative vs. seropositive RA patients treated with sarilumab in regular care in Germany.Methods:The prospective, observational, 24-month single-arm PROSARA study (SARILL08661) is currently running in Germany at 96 sites, aiming to enroll up to 750 RA patients treated with sarilumab. RA patients are prospectively selected at physician’s discretion according to label, and medical history is documented before treatment. This interim analysis included patients with data available up to 24 months. Here we focus on sustained treatment response after 6 and 12 months, respectively. Patients were stratified according to serostatus (RF- AND ACPA- as seronegative or RF+ AND/OR ACPA+ as seropositive). All analyses are descriptive only.Results:To date 473 patients were included in the study, of which 22.2% (n=105) were seronegative and 59.0% (n=279) were seropositive (Table 1). For 89 patients (18.8%) serostatus was not specified.Table 1.Baseline data regarding patient characteristics, prior treatment and disease activityseronegativeseropositivepatients [n] (%)105(22.2)279(59.0)patient characteristicsSex female [n] (%)81(77.1)207(74.2)Age mean [years] (SD)56.6(12.5)59.6(10.9)BMI mean (SD) [kg/m2]29.2(6.7)28(6.0)Smoking history current/former [%]22.1/11.525.5/20.4Time since diagnosis of RA mean [years] (SD)7.9(7.6)11.5(9.9)prior RA treatmentcsDMARD [n] (%)102(97.1)273(97.8)bDMARD (TNFi) [n] (%)54(51.4)167(59.9)bDMARD (non-TNFi) [n] (%)32(30.5)83(29.7)tsDMARD (JAKi) [n] (%)25(23.8)57(20.4)disease activityCRP [mg/l] (SD)12.9(30.9)14.2(31.0)ESR [mm/h] (SD)23.7(20.5)25.3(22.4)SJC mean (SD)5.3(5.5)4.6(4.8)TJC mean (SD)10.2(8.2)7.2(6.6)HAQ-DI mean (SD)1.4(0.7)1.2(0.7)DAS28-ESR mean (SD)5.0(1.5)4.6(1.5)CDAI mean (SD)27.7(14.7)23.0(12.9)Fatigue [VAS] (SD)64.0(25.5)51.1(28.1)The mean time since diagnosis of RA was shorter in seronegative than in seropositive patients (7.9±7.6 years vs. 11.5±9.9 years) (Table 1).At baseline, CDAI score was 27.7±14.7 (n=104) and 23.0±12.9 (n=272) in seronegative and seropositive patients, respectively. After 12 months of sarilumab treatment, CDAI improved to 15.6±12.3 (n=38) and 9.1±8.7 (n=101) in seronegative and seropositive, respectively, for patients with post-baseline data available. At that time, remission/low disease activity according to CDAI was reached by 5.3% (n=2/38) / 39.5% (n=15/38) of seronegative patients, respectively and by 26.7% (n=27/101) / 68.3% (n=69/101) of seropositive patients, respectively (Fig. 1A, B).Figure 1.CDAI (A, B) and HAQ-DI (C) outcomes in seronegative and seropositive patientsPhysical function, assessed by HAQ-DI, was slightly more impaired in seronegative patients (1.4±0.7) than in seropositive patients (1.2±0.7) at baseline. HAQ-DI improved over 12 months to 0.9±0.7 in seropositive patients (n=90) but showed no change in seronegative patients (1.4±0.7; n=37) (Fig. 1C).Safety was consistent with the anticipated profile of IL-6-R-inhibition and no new safety signals occurred. Adverse events and serious adverse events were described in 61.7% and 12.8% of seronegative patients, respectively and in 55.2% and 13.7% of seropositive patients, respectively.Conclusion:Sarilumab treatment ameliorated CDAI in both seronegative (ΔCDAI -17.0±16.5) and seropositive (ΔCDAI -15.4±14.3) patients to the same extent over the course of 12 months, with a higher remission rate in seropositive patients. Functional capacity improved meaningfully in the seropositive cohort but had no significant impact in seronegative patients. The safety profile was consistent with data reported from controlled clinical trials.References:[1]Choi S-T et al. (2018) PLoS ONE 13(4): e0195550.Acknowledgements:We thank Cornelia Kühne (Haldensleben) for substantial contribution to patient recruitment in PROSARA.Disclosure of Interests:Harald Burkhardt Speakers bureau: Sanofi, Pfizer, Roche, Abb- vie, Boehringer Ingelheim, UCB, Eli Lilly, Chugai, Bristol Myer Scribb, Janssen, and Novartis, Consultant of: Sanofi, Pfizer, Roche, Abbvie, Boehringer Ingelheim, UCB, Eli Lilly, Chugai, Bristol Myer Scripps, Janssen, and Novartis, Grant/research support from: Pfizer, Roche, Abbvie, Hans-Peter Tony Consultant of: AbbVie, Astra-Zeneca, BMS, Chugai, Janssen, Lilly, MSD, Novartis, Pfizer, Roche, Sanofi, Peer-Malte Aries Speakers bureau: Abbvie, Biogen, Berlin Chemie, Celgene, GSK, Hexal, Mylan, Novartis, Pfizer, UCB, Consultant of: Abbvie, Celgene, Hexal, Janssen, Medac, Novartis, Pfizer, Sanofi, UCB, Silke Zinke: None declared, Klaus Krueger Speakers bureau: Sanofi, Jonas Ahlers Employee of: Sanofi, Rolf Hecker Employee of: Sanofi, Inka Albrecht Employee of: Sanofi, Stefanie Kalus Consultant of: Sanofi, Oliver Bley Employee of: Sanofi, Patrizia Sternad: None declared, Ann-Dörthe Holst: None declared, Niklas Thomas Baerlecken: None declared, Thilo Klopsch: None declared, Martin Welcker Speakers bureau: Abbvie, Aescu, Amgen, Biogen, Berlin Chemie, Celgene, GSK, Hexal, Mylan, Novartis, Pfizer, UCB, Consultant of: Abbvie, Actelion, Aescu, Amgen, Cel- gene, Hexal, Janssen, Medac, Novartis, Pfizer, Sanofi, UCB, Grant/research support from: Abbvie, Novartis, UCB, Hexal, BMS, Lilly, Roche, Celgene, Sanofi, Eugen Feist Speakers bureau: Novartis, Roche, Sobi, Lilly, Pfizer, Abbvie, BMS, MSD, Sanofi, Consultant of: Novartis, Roche, Sobi, Lilly, Pfizer, Abbvie, BMS, MSD, Sanofi

Objectives COVID-19 outcomes in people with rheumatic diseases remain poorly understood. The aim was to examine demographic and clinical factors associated with COVID-19 hospitalisation status in people with rheumatic disease. Methods Case series of individuals with rheumatic disease and COVID-19 from the COVID-19 Global Rheumatology Alliance registry: 24 March 2020 to 20 April 2020. Multivariable logistic regression was used to estimate ORs and 95% CIs of hospitalisation. Age, sex, smoking status, rheumatic disease diagnosis, comorbidities and rheumatic disease medications taken immediately prior to infection were analysed. Results A total of 600 cases from 40 countries were included. Nearly half of the cases were hospitalised (277, 46%) and 55 (9%) died. In multivariable-adjusted models, prednisone dose ≥10 mg/day was associated with higher odds of hospitalisation (OR 2.05, 95% CI 1.06 to 3.96). Use of conventional disease-modifying antirheumatic drug (DMARD) alone or in combination with biologics/Janus Kinase inhibitors was not associated with hospitalisation (OR 1.23, 95% CI 0.70 to 2.17 and OR 0.74, 95% CI 0.37 to 1.46, respectively). Non-steroidal anti-inflammatory drug (NSAID) use was not associated with hospitalisation status (OR 0.64, 95% CI 0.39 to 1.06). Tumour necrosis factor inhibitor (anti-TNF) use was associated with a reduced odds of hospitalisation (OR 0.40, 95% CI 0.19 to 0.81), while no association with antimalarial use (OR 0.94, 95% CI 0.57 to 1.57) was observed. Conclusions We found that glucocorticoid exposure of ≥10 mg/day is associated with a higher odds of hospitalisation and anti-TNF with a decreased odds of hospitalisation in patients with rheumatic disease. Neither exposure to DMARDs nor NSAIDs were associated with increased odds of hospitalisation.

Background:Blockade of IL-6 signaling by sarilumab has been demonstrated to be an effective treatment approach for rheumatoid arthritis. Due to strict inclusion and exclusion criteria, randomized controlled trials may not represent the heterogeneous RA patient population encountered in regular care.Objectives:The current study investigated the safety and effectiveness of sarilumab in the treatment of RA in regular care in Germany.Methods:The prospective, observational, single-arm 24-months PROSARA study (SARILL08661) is currently running in Germany at 79 sites, aiming to include up to 750 RA patients treated with sarilumab. RA patients are selected at physician discretion and treated according to the label. Study objectives include the documentation of safety and various effectiveness outcomes. This interim analysis included patients with data available up to 12 weeks. All analyses are descriptive only.Results:To date 348 patients were included in the study; of which 265 patients had post-baseline data. The mean age of the patients analyzed was 58.6 years (24-83); 76.3% are female. The mean disease duration was 10.3 years; comorbidities were present in 80.7% of patients. At baseline, 32.5% were biologic naïve. Most common pretreatment with b/ts DMARDs included TNF-inhibitors (TNFi, 56.2%), non-TNFi biologics (29.1%) or JAK-inhibitors (JAKi, 17.4%). At baseline, 49% received sarilumab as monotherapy and 29% in combination with conventional DMARDs (not specified for 22%). After 12 weeks of treatment with sarilumab, the mean DAS28-ESR decreased from 5.0±1.46 to 3.0±1.44 and CDAI from 26.7±13.79 to 13.6±11.4, respectively. DAS28-ESR remission/ low disease activity was achieved in 42.8% [n=77/180 patients with valid data on this parameter]/ 59.4% [n=107/180] of patients; 13.6% [n=28/206] and 49% [n=101/206] of patients reached CDAI remission and low disease activity. Boolean remission was observed in 9.5% [n=19/201] of patients at week 12. HAQ-DI improved from 1.3 at baseline to 1.1 at week 12 (n=195). The mean CDAI improvement was similar for autoantibody-positive (RF and/or ACPA; CDAI -12.5 at week 12) compared to -negative patients (CDAI -15.4 at week 12). Patients switching from JAKi to sarilumab (n=32), were more severely affected, had longer disease duration and received more prior treatments than patients switched from another compound. Of note, similar efficacy was observed among patients that switched from JAKi to sarilumab vs patients switched from other DMARDs; disease activity outcome measures including DAS28, CDAI, TJC, SJC and global assessments improved consistently (Figure 1).Safety was consistent with the anticipated profile of IL-6-R-inhibition and no new safety signals occurred. Adverse events and serious adverse events were described in 33.9% and 6.3% of patients, respectively.Conclusion:Sarilumab administered in regular care demonstrated rapid and clinically meaningful improvement in a general RA patient population including patients switching from JAKi. The safety profile was consistent with data reported from controlled clinical trials.Disclosure of Interests:Eugen Feist Consultant of: Novartis, Roche, Sobi, Lilly, Pfizer, Abbvie, BMS, MSD, Sanofi, Speakers bureau: Novartis, Roche, Sobi, Lilly, Pfizer, Abbvie, BMS, MSD, Sanofi, Peer-Malte Aries Consultant of: Sanofi, Speakers bureau: Sanofi, Silke Zinke: None declared, Harald Burkhardt Grant/research support from: Pfizer, Roche, Abbvie, Consultant of: Sanofi, Pfizer, Roche, Abbvie, Boehringer Ingelheim, UCB, Eli Lilly, Chugai, Bristol Myer Scripps, Janssen, and Novartis, Speakers bureau: Sanofi, Pfizer, Roche, Abbvie, Boehringer Ingelheim, UCB, Eli Lilly, Chugai, Bristol Myer Scripps, Janssen, and Novartis, Inka Albrecht Employee of: Sanofi, Oliver Bley Employee of: Sanofi, Michael Obermeier: None declared, Patrizia Sternad: None declared, Martin Welcker Grant/research support from: Abbvie, Novartis, UCB, Hexal, BMS, Lilly, Roche, Celgene, Sanofi, Consultant of: Abbvie, Actelion, Aescu, Amgen, Celgene, Hexal, Janssen, Medac, Novartis, Pfizer, Sanofi, UCB, Speakers bureau: Abbvie, Aescu, Amgen, Biogen, Berlin Chemie, Celgene, GSK, Hexal, Mylan, Novartis, Pfizer, UCB, Cornelia Kühne Grant/research support from: Novartis, Amgen, Roche/Chugai, Pfizer, Celgene, AbbVie, Sanofi, Ann-Dörthe Holst: None declared, Niklas Thomas Baerlecken: None declared, Hans-Peter Tony Consultant of: AbbVie, Astra-Zeneca, BMS, Chugai, Janssen, Lilly, MSD, Novartis, Pfizer, Roche, Sanofi

To assess safety, effectiveness and onset of effect of rituximab (RTX) in routine clinical treatment of severe, active rheumatoid arthritis (RA). Prospective, multi-centre, non-interventional study in rheumatological outpatient clinics or private practices in Germany. RTX-naive adult patients were to receive RTX according to marketing authorisation and at their physician’s discretion. Also according to their physician’s discretion, patients could receive a second cycle of RTX (re-treatment = treatment continuation). Major outcome was the change in Disease Activity Score based on 28-joints count and erythrocyte sedimentation rate (DAS28-ESR) over 24 weeks and during 6 months of re-treatment. Overall, 1653 patients received at least one cycle RTX; 99.2% of these had received disease-modifying antirheumatic drugs (DMARD) pre-treatment and 75.5% anti-tumor necrosis factor(TNF)‑α pre-treatment. After a mean interval of 8.0 months, 820 patients received RTX re-treatment. Mean DAS28-ESR decreased from 5.3 at baseline to 3.8 after 24 weeks (−1.5 [95% confidence interval, CI: −1.6; −1.4]), and from 4.1 at start of cycle 2 to 3.5 at study end (change from baseline: −1.8 [95% CI: −2.0; −1.7]). Improvements in DAS28-ESR and Health Assessment Questionnaire (HAQ) score occurred mainly during the first 12 weeks of RTX treatment, with further DAS28-ESR improvement until week 24 or month 6 of re-treatment. Improvements in DAS28-ESR and EULAR responses were more pronounced in seropositive patients. RF was a predictor of DAS28-ESR change to study end. Safety analysis showed the established profile of RTX. RTX was safe and effective in a real-life setting with rapid and sustained improvement in RA signs and symptoms.

The diagnosis of ANCA-associated vasculitides (AAV) consists of clinical symptoms, results of technical procedures and a characteristic histology. The diagnostic workup should focus on the prominent clinical symptoms and should involve an interdisciplinary team of specialists. The therapy is divided into induction and maintenance treatment. Cyclophosphamid remains standard therapy for the induction of remission, whereas azathioprine, methotrexate and leflunomide are often used for maintenance therapy. The introduction of a stage-adapted treatment has contributed to a significant improvement of the long-time prognosis of patients with AAV.

Die haufig noch uncharakteristischen Symptome bei Erstmanifestation einer Vaskulitis wie Myalgien, Fieber und Gewichtsverlust fuhren nicht selten zu Verzogerungen in der Diagnosestellung. Organspezifischere Symptome wie Hamoptysen, Dyspnoe, Epistaxis, Odeme oder Organinfarkte treten hinzu, wenn als Folge der Vaskulitis bereits eine Funktionsminderung der betroffenen Organsysteme besteht. Eine gezielte serologische Diagnostik, insbesondere die Bestimmung von anti-neutrophilen zytoplasmatischen Antikorpern (ANCA) oder Kryoglobulinen kann eine fruhe Diagnosestellung von Kleingefasvaskulitiden erleichtern. Moderne bildgebende Verfahren wie die Magentresonanztomographie, Computertomographie, Positronenemissionstomographie und Sonographie sind fur die Fruhdiagnose von Vaskulitiden bedeutsam, da sie eine Diagnose oft noch subklinischer Manifestationen ermoglichen und die Auswahl eines Ortes zur Gewinnung einer Biopsie erleichtern. Die bioptische Sicherung ist immer noch der Goldstandard in der Diagnostik der Vaskulitiden. Funktionell relevante Spatschaden als Folge einer Vaskulitis konnen durch eine fruhzeitig eingeleitete Therapie in ihrer Schwere deutlich reduziert werden. Voraussetzung hierfur ist die moglichst fruhe und prazise Diagnosestellung.

Erst die chemische Modifikation der naturlichen Steroide in den 50er Jahren konnte die notwendigen strukturellen Voraussetzungen fur die biologische Aktivitat der Glucocorticoide (GC) nachweisen. Wahrend die delta-4,3-keto-11-beta, 17-alpha,21-trihydroxyl Konfiguration Voraussetzung fur die GC-Aktivitat ist, konnte durch die kunstliche Einfuhrung einer zusatzlichen Doppelbindung in Position 1 und 2 die GC-Aktivitat um das 4fache erhoht werden. Von diesen synthetischen GC ist das Prednison das am haufigsten verwendete GC in der medikamentosen Therapie. Aufgrund des raschen Wirkungseintritts sind die GC in der Therapie der Vaskulitis inzwischen unverzichtbar geworden. Die Dosis, Therapiedauer und Applikationsform richten sich nach der Diagnose, Krankheitsstadium bzw. -ausdehnung sowie Krankheitsaktivitat. Bei der Abwagung zwischen dem therapeutischen Nutzen und therapiebedingten Nebenwirkungen der GC sind die Erfahrungen aus den Ende der 80er Jahre publizierten Kohortenstudien von entscheidender Bedeutung. Fur die Arteriitis temporalis zeigte sich, dass zur Remissionsinduktion auch GC-Dosen von unter

Objective Analyses of families with multiple autoimmune disorders have revealed a functional polymorphism, 620W, in the intracellular tyrosine phosphatase gene PTPN22 as a predisposing factor for type 1 diabetes, seropositive rheumatoid arthritis, systemic lupus erythematosus, and Hashimoto thyroiditis, and the presence of the PTPN22 protein appears to herald the development of autoantibodies in these disorders. This study therefore examined whether the functionally relevant PTPN22 polymorphism is associated with Wegener's granulomatosis (WG). Methods A population-based study was performed for the PTPN22 polymorphism in 199 patients with WG and in 399 healthy individuals. The R620W variation was investigated by simple restriction fragment-length polymorphism analysis. Results The PTPN22 620W allele frequency was significantly increased in antineutrophil cytoplasmic antibody (ANCA)–positive WG patients compared with healthy controls (P < 0.001). The association was particularly striking in patients with kidney, lung, eye, and peripheral nervous system involvement (i.e., those with generalized WG). Conclusion The PTPN22 620W allele appears to be involved in the pathogenesis of WG, and ANCA positivity seems to be the hallmark.

Initially, intravenous immunoglobulins (IVIgs) were used as replacement therapy in primary and secondary antibody-deficiency syndromes. The clinical use of IVIg has been extended during the past decade to a wide variety of clinical conditions, such as infectious processes, neuroimmunological diseases, and different systemic autoimmune diseases. The mode of action of IVIg is complex, involving modulation of the Fc receptors, interference with the complement and cytokine network, and effects on the activation and differentiation of T and B-cells. Kawasaki disease (KD) was one of the first diseases within the group of primary vasculitides in which IVIg were used. Today, there is a clear evidence of benefit for IVIg in the treatment of coronary artery abnormalities related to KD. Subsequently, various reports have suggested a beneficial effect in other vasculitides; however, there are few data from controlled studies. For antineutrophil cytoplasmic antibody-associated vasculitis (AAV) one placebo-controlled and several open-label studies have shown a beneficial effect on the disease activity in patients with Wegener's granulomatosis or microscopic polyangiitis refractory to standard therapy with prednisone and cyclophosphamide. For other vasculitides, such as polyarteritis nodosa or Henoch-Schonlein purpura, only case reports have described an inhibition of a disease progression by IVIg so far. However, the effect was partly only temporary. In conclusion, KD and AAV are the only vasculitides with a definite beneficial use of IVIg. For other vasculitides, the efficacy of IVIg has not been proven properly but may be useful in single cases.

History A 63-year-old man developed a Churg-Strauss syndrome with predominantly motor-sensory polyneuropathy. Initial treatment with cyclophosphamide and steroids achieved complete remission. Subsequent relapse with marked retinal vasculitis at first was refractory to the standard treatment. Renewed remission was obtained with additional infliximab, and was maintained with azathioprine for 12 months before the patient again presented with symmetrical polyarthritis. Investigations Clinical examination revealed a symmetrical polyarthritis involving the joints of the hand and fingers. The acute-phase parameters were raised, the rheumafactor was highly positive. Radiology showed early erosions in the bones of the hand. There were no indices of renewed activity of the Churg-Strauss syndrome. Treatment and course These findings indicated sero-positive rheumatoid arthritis and methotrexate was started, later supplemented with sulfsalazine and hydroxychloroquine because of continuing signs of activity. Infliximab was again given because of further progression, but a severe infusion reaction developed during the second infusion. After changing to etanercept remission of the rheumatoid arthritis was achieved. Conclusion The development of rheumatoid arthritis during remission achieved with azathioprine in Churg-Strauss syndrome of four-year duration is very rare. Repeated dosis of infliximab at long intervals greatly increases the risk of an intolerance.

Background Making the diagnosis of axial spondyloarthritis (axSpA) may be difficult. Antibodies against CD74 have been shown to be present in 2/3 of patients with long established axial SpA. Objectives InterSpA is an international multicenter study, conducted to compare the sensitivity and specificity of anti-CD74 and HLA-B27 in patients with axSpA of recent onset. Methods Patients between 18 and 45 years suffering from inflammatory back pain (IBP) for maximally 2 years were recruited. The presence of ankylosing spondylitis, additional inflammatory rheumatic disorders and biologic therapy were exclusion criteria. MRI of sacroiliac joint was performed in all patients; HLA-B27 was detected by genotyping and anti-CD74 using a CE certified kit of Aesku Diagnostics (Wendelsheim, Germany). The sensitivity of anti-CD74 and HLA-B27 were calculated in patients fulfilling the imaging arm of ASAS criteria, in all patients fulfilling ASAS criteria and in 100 blood donors. Both the MRI reading as well as the laboratory procedures were performed blinded. Results 205 patients suffering from IBP were recruited. There were 40 recruiting failures. The remaining 165 patients had a mean age of 29.4 years, a mean duration of IBP of 12.6 months, 50% of the patients were female. Sacroiliitis was diagnosed by the local and an independent expert reader in 71/123 (57.7%). So far complete data sets are available of 91 patients. Of these, 16 fulfilled the ASAS criteria of axSpA by a pathologic MRI only, 40 by both MRI and presence of HLA-B27 and 20 by HLA-B27 only. The sensitivities of IgA anti-CD74, IgG anti-CD74 and HLA-B27 were 71.4%, 26.8% and 75% in the 56 axSpA patients with a pathologic MRI, 73.7%, 23.7% and 81.6% in all 76 patients fulfilling ASAS criteria, and 3%, 5% and 8% in the blood donors. The likelihood ratios are 23.8 (IgA anti-CD74), 5.4 (IgG anti-CD74) and 9.4 (HLA-B27) when considering the patients with a pathologic MRI only, and 24.6 (IgA anti-CD74), 4.7 (IgG anti-CD74) and 10.2 (HLA-B27) when considering all patients fulfilling ASAS criteria. Conclusions Considering their specificity and sensitivity, IgA anti-CD74 is a useful addition to our diagnostic tools for axSpA. Acknowledgement This study is funded by AbbVie Deutschland GmbH & Co. KG. AbbVie contributes to study logistics and site management. The authors determined study design, conduct of the study and the content of the publication. No payments were made to the authors for writing this manuscript. Disclosure of Interest E. Riechers Grant/research support from: AbbVie GmbH, N. T. Baerlecken Grant/research support from: AbbVie GmbH, X. Baraliakos: None declared, K. Achilles-Mehr Bakhsh: None declared, P. M. Aries: None declared, B. Bannert: None declared, K. Becker: None declared, J. F. Brandt-Jurgens: None declared, J. Braun: None declared, B. Ehrenstein: None declared, H. Euler: None declared, M. Fleck: None declared, R. Hein: None declared, K. Karberg: None declared, L. Kohler: None declared, T. Matthias: None declared, R. Max: None declared, A. Melzer: None declared, D. Meyer-Olson: None declared, J. Rech: None declared, K. Rockwitz: None declared, M. Rudwaleit: None declared, E. Schweikhard Employee of: Aesku GmbH, J. Sieper: None declared, C. Stille: None declared, U. von Hinuber: None declared, P. Wagener: None declared, H. Weidemann: None declared, S. Zinke: None declared, T. Witte Grant/research support from: AbbVie GmbH

HISTORY AND ADMISSION FINDINGS A 28-year-old man, known to have abnormal intestinal magnesium absorption, presented with recurrent cerebral seizures. Despite daily intravenous sulphate infusions, magnesium concentration remained inadequate. Physical examination was unremarkable. INVESTIGATIONS Serum magnesium concentration was markedly reduced to 0.48 mmol/l. The parenteral magnesium tolerance test indicated reduced enteric magnesium absorption of < 20%. Absolute magnesium concentration in 24-hour urine was normal at 6.3 mmol/24 h, but high in proportion to the hypomagnesaemia. All other laboratory data were within normal limits. TREATMENT AND COURSE In addition to the known intestinal malabsorption the patient also had isolated renal loss of magnesium. It was only by continuous magnesium infusion with a recently developed portable subcutaneous pump system that normal serum magnesium concentrations were attained and all symptoms disappeared. CONCLUSION This patient has combined magnesium transport defects, which could not be effectively treated by conventional methods, but required continuous magnesium infusion with an indwelling subcutaneous infusion pump. This case serves as an example of the way in which subcutaneous infusion can provide physiological substitution of electrolytes.

The definition of outcome parameters has improved the care of vasculitis patients dramatically in recent decades. In the first phase of joint European studies, disease stages and activity were defined. In the second and third phases results of the randomized and controlled trials were summarized and published as European recommendations for the care of small and large vessel vasculitis. Irrespective of the type of vasculitis, inducing remission, maintaining remission and preventing disease- and therapy-related complications are the main outcome criteria.

Conventional immunosuppressive treatment of systemic vasculitides has improved their often fatal outcome, but is burdened by cytotoxic side effects and frequent relapses. Recent advances in the therapy of systemic vasculitides with biologicals have helped to establish new options for patients resistant to conventional treatment. Moreover, early intervention aiming to interfere with specific targets important in the break of tolerance and/or persistence of the autoimmune response might further improve the prognosis of autoimmune vasculitides such as antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV). In vitro and in vivo studies suggest that the interaction of ANCA and cytokine (TNF-alpha, IL-1)-primed neutrophils results in premature neutrophil activation and degranulation, subsequent endothelial cell damage, and further leukocyte recruitment. For one of the AAV, Wegener's granulomatosis, recent ex vivo data have provided evidence that WG-granulomata might provide the necessary "proinflammatory environment" for the break of tolerance and display features of lymphoid-like tissue neoformation, in which autoimmunity to "Wegener's autoantigen" proteinase 3 PR3 could be sustained. Blocking TNF-alpha and eliminating autoreactive B cells seem promising treatment targets to interfere with these fundamental disease processes. While the recombinant TNF-alpha receptor/IgG1 fusion protein etanercept, in addition to standard therapy with subsequent tapering of standard medications, was found to be not effective for maintenance of remission, open clinical studies suggest a beneficial effect of the anti-TNF-alpha antibody infliximab in addition to standard therapy for the induction of remission in patients with refractory AAV. Peripheral B cell depletion with the anti-CD20 antibody rituximab also induced remissions in AAV in uncontrolled trials.

Inherited autosomal-dominant mutations in the tumour necrosis factor receptor superfamily 1A ( TNFRSF1A ) gene encoding the tumour necrosis factor receptor p55 (TNF-R1) are the cause of an auto-inflammatory syndrome that is characterized by periodic fever attacks, aseptic peritonitis, arthritis, meningitis, conjunctivitis, pleuritis and skin rash (OMIM #142680). The most common TNFR-associated periodic syndrome (TRAPS)-like disease that is associated with a R92Q mutation, however, occurs sporadically, with later onset (median 23 years vs 7 years with other mutations) and a milder and often oligosymptomatic course.1,2 Intriguingly, carriers of the R92Q allele bear a slightly increased risk for some other diseases, such as myocardial infarction,3 increased carotid intima-media thickness,3 thrombotic complications in Behcet’s disease4 and early synovitis.1 Due to the low …

Antineutrophil cytoplasmatic autoantibodies (ANCA) with specificity to Proteinase 3 (PR3-ANCA) are serological marker for Wegener's granulomatosis (WG) and are involved in the pathophysiology of the associated vasculitis. PR3 is a neutral serine protease that is localized in the cytoplasmic granules of neutrophils and the lysosomes of monocytes. Priming and apoptosis of neutrophils results in an increased expression of the membrane bound PR3, making them accessible for ANCA. In vitro studies have shown that simultaneous binding of ANCA to PR3 and Fcγ-RII receptors expressed on the cell surface of previously primed neutrophils causes degranulation and an oxidative burst of the neutrophils. Additionally, recently it was demonstrated that ANCA generated against murine PR3 had significant pro-inflammatory effects in wild-type mice, but did not induce a vasculitic disease reminiscent of human ANCA–associated vasculitis. In the clinical setting, the proof of PR3-ANCA is a strong indication for WG but per se does not allow the diagnosis of WG. The sensitivity of ANCA testing is related to the extent, severity, and activity of disease at the time of sampling. The best diagnostic performance is obtained when IIF is combined with PR3-ANCA ELISA (the diagnostic sensitivity and specificity increased up to 73 and 99%, respectively). Even when there is a good correlation between PR3-ANCA ELISA levels and disease activity during the course of the disease, changes cannot be only the basis for therapeutical decisions.

Objective: To investigate the safety and efficacy of rituximab (RTX) in patients with refractory Wegener’s granulomatosis (WG). Patients and methods: Eight consecutive patients with active refractory WG were included. In all patients disease activity had persisted despite standard treatment with cyclophosphamide and prednisolone, as well as tumour necrosis factor α blockade 3 months before inclusion in the study. Patients had particular granulomatous manifestations like retro-orbital granulomata (n = 5), nodules of the lungs (n = 1), and subglottic stenosis (n = 2). RTX was given intravenously every 4th week in combination with the standard treatment in five patients and with methotrexate in two others. Disease extent and activity were monitored clinically by interdisciplinary care, immunodiagnostics (ANCA serology, B cells by flow cytometry), and magnetic resonance imaging. Results: Beneficial response and a reduction in disease activity were seen in three patients, two of whom went into complete remission. In three other patients, disease activity remained unchanged while the disease progressed in the remaining two patients. In all patients peripheral blood B cells fell to zero during treatment with RTX. cANCA titres remained unchanged in all except one patient. Conclusion: In this pilot study, B lymphocyte depletion was not associated with a change of the ANCA titres or obvious clinical improvement of refractory granulomatous disease in patients with WG. Further studies are needed to evaluate the role of RTX in WG.

The diagnosis of ANCA-associated vasculitides (AAV) consists of clinical symptoms, results of technical procedures and a characteristic histology. The diagnostic workup should focus on the prominent clinical symptoms and should involve an interdisciplinary team of specialists. The therapy is divided into induction and maintenance treatment. Cyclophosphamid remains standard therapy for the induction of remission, whereas azathioprine, methotrexate and leflunomide are often used for maintenance therapy. The introduction of a stage-adapted treatment has contributed to a significant improvement of the long-time prognosis of patients with AAV.

Background A 63-year-old man with a 4-year history of Wegener's granulomatosis presented with hemoptysis, palatal ulceration and sensorimotor polyneuropathy. Investigations Physical examination, serologic testing, proteinase 3 enzyme-linked immunosorbent assay, IgG and IgM specific to Epstein–Barr virus enzyme-linked immunosorbent assay, MRI of the middle face, an ear, nose and throat consultation, immunohistochemical staining of an esophageal sample obtained from esophagogastroduodenoscopy, polymerase chain reaction of cytomegalovirus (CMV) DNA from esophageal and blood samples, and measurement of pp65 early antigen. Diagnosis Wegener's granulomatosis with progressive palatal ulceration and osseous destruction complicated by CMV-related esophagitis during induction treatment. Management Induction treatment with cyclophosphamide pulse therapy was discontinued and antiviral therapy with ganciclovir was started. Cyclophosphamide pulse therapy was later reintroduced, but there was subsequent CMV reactivation, and, therefore, cyclophosphamide pulse therapy was suspended again and ganciclovir and intravenous immunoglobulin were started. After control of CMV, cyclophosphamide pulse therapy was reinitiated. Prolonged immunosuppressive therapy controlled disease activity, without CMV reactivation.

Objectives: To evaluate the outcome of balloon angioplasty in the arteries of the upper extremities in patients with giant-cell arteritis (GCA) and stenosing extracranial involvement. Methods: Percutaneous transluminal angioplasty (PTA) for symptomatic upper limb artery stenoses (n = 29) and occlusions (n = 1) resistant to medical treatment was carried out in 10 patients (all women, mean age 65 years) with GCA. Vascular lesions were located in the subclavian (n = 4), axillary (n = 10) and brachial (n = 16) arteries. Interventional treatment was accompanied by immunosuppressive drugs in all patients. Follow-up included clinical and serological examination, magnetic resonance angiography and colour duplex ultrasound. Results: Initial technical success of angioplasty was achieved in the case of all vascular lesions. In five patients, marked recurrent stenoses (vascular territories; n = 10/30) were found during follow-up (mean 24 months). The cumulative primary patency rate was 65.2%. All recurrent lesions developed in the territories of the initial long-segment stenoses. Repeated PTA (vascular territories, n = 8; patients, n = 5) provided a cumulative secondary patency rate of 82.6% and a cumulative tertiary patency rate of 89.7%. Conclusions: Despite a tendency to restenoses, balloon angioplasty of the upper-extremity artery, in combination with immunosuppressive treatment, is an efficient method for the treatment of extracranial GCA.