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Even in the era of modern guidelines, the treatment of rheumatic diseases is only as good as the framework of rheumatological care within which the treatment is carried out. The access to high-quality medical treatment for all patients is therefore essentially decisive for the prognosis of the patients. This article describes the current state of outpatient treatment in rheumatology and demonstrates which quality projects, such as treatment contracts, outpatient specialized medical treatment (ASV), digitalization and training as specialized rheumatological assistant (RFA), have been created in order to ensure the treatment of our patients. Furthermore, standards are defined that can guarantee a contemporary and guideline-conform treatment in outpatient rheumatological units. As an example it is an affirmation of the Professional Association of German Rheumatologists (BDRh) for ensuring optimal care for all rheumatology patients through early or emergency rheumatology clinics, treat to target, appropriate delegation of medical duties and diversification of treatment, thus an assurance of the quality and comprehensive treatment in rheumatology. The important topic of safeguarding the next generation of rheumatologists, which is indispensable for this, is also discussed.

Digitalization in the healthcare system is a great challenge for rheumatology as for other medical disciplines. The German Society for Rheumatology (DGRh) wants to actively participate in this process and benefit from it. By founding the commission on digital rheumatology, the DGRh has created a committee that deals with the associated tasks, advises the DGRh on questions and positions associated with digital health. For the DGRh, this affects the most diverse areas of digitalization in medicine and rheumatology. This position paper presents the topics and developments currently handled by the commission and the tasks identified.

From the perspective of patients with rheumatic diseases, the reduction of inflammatory disease activity alone is not a sufficient treatment goal. In addition the functional health and participation also have to be improved. Starting with the first symptoms the empowerment for the self-management of the disease is important for the patients; therefore, the established treat to target-strategy has to be expanded by the functional dimension to treat to participation. The position paper of the German Society for Rheumatology (GSR) summarizes the relevant fields of the multiprofessional action that is frequently necessary. This includes the acquirement of function-related competencies during training, further education and advanced training as well as implementation in the everyday practice of patient care. Furthermore, the GSR acknowledges the need for research related to functional and sociomedical consequences of rheumatic diseases and to individual and combined function-related programs in outpatient and inpatient care in rheumatology.

Rheumatic diseases in childhood and adolescence differ from those of adulthood according to type, manifestation, treatment and course. A specialized therapy, starting as early as possible, improves the prognosis, can prevent long-term damage and saves the costs of long-term care. Only a specialized pediatric care system can guarantee optimum quality of the processes involved and the results for rheumatology in childhood and adolescence within a global financial system. This requires adequate structural quality of the specialized clinics and departments for pediatric rheumatology. The management of rheumatic diseases in childhood and adolescence is comprehensive and requires a multidisciplinary, specialized and engaged team which can cover the whole spectrum of rheumatic diseases with their various age-dependent aspects. In order to guarantee an adequate, cost-efficient routine, a specialized center which concentrates on inpatient care should treat at least 300 patients with pediatric rheumatic diseases per year. The diagnoses should be divided among the various disease categories with at least 70% of them involving inflammatory rheumatic diseases. For the inpatient care of small children, an accompanying person (parent) is necessary, requiring adequate structures and services. Patient rooms as well as diagnostic (radiography, sonography, etc.) and therapeutic services (physiotherapy, occupational therapy, pool, etc.) must be adequate for small children and school children as well as adolescents. Suitable mother-child units must also be provided and a school for patients is required within the clinic. A pediatric rheumatologist must be available 24 h a day, and it must be possible to reach other specialists within a short time. For painful therapeutic procedures, age-appropriate pain management is obligatory. A continuous adjustment of these recommendations to changing conditions in health politics is intended.

Background: In the next few years many general practitioners and specialists will retire. As in other disciplines the question arises in rheumatology whether sufficient training positions are available to maintain or expand the supply of care according to demand. Therefore, the German Society of Rheumatology (DGRh) has assigned its committee for education and training to review the currently available training opportunities in Germany. The aim of this work is the quantitative survey of the training capacity to become a specialist in internal medicine and rheumatology., Methods: Within the framework of this study, a survey was conducted via the homepages of the 17 state medical associations to determine the postgraduate medical officers, their place of work and the duration of their postgraduate training capabilities. Based on the data, a nationwide survey of training positions was conducted., Results: Specialized rheumatology training is established at 229 training centers in Germany, whereby data from 187 training sites were available for analysis. The training locations are distributed as followed: 52.4% clinical sector and 47.6% outpatient sector. In total, 478.4 training positions are available in Germany (clinical sector: 391.4 and outpatient sector: 87) and 17.2% of the positions (clinical sector: 11.4% and outpatient sector: 43.1%) are not occupied., Conclusion: Based on this study, it can be shown that most of the continuing education positions are available in the clinical sector. In contrast, half of the training positions in the outpatient area are not filled. In order to improve the training situation, it is essential to integrate outpatient colleagues into the training program. This presupposes that further training is supported or financed by the healthcare system. In this context, optimal rheumatological care must be permanently guaranteed throughout Germany in order to provide sufficient care for the approximately 2 million patients with inflammatory rheumatic diseases., (© 2022. The Author(s).)

The diagnosis of systemic vasculitis (SV) is a major clinical challenge due to the very different forms of presentation and requires an interdisciplinary approach. Targeted laboratory diagnostics support making the diagnosis, differential diagnosis and classification and are also a key component in the detection of active organ manifestations and treatment complications. The basic laboratory tests include the erythrocyte sedimentation rate (ESR), C‑reactive protein (CRP), blood count, serum creatinine, urinalysis, specific autoantibodies, complement, immunoglobulins, cryoglobulins and hepatitis B and C serology. Antineutrophil cytoplasmic autoantibodies (ANCA), antiglomerular basement membrane antibodies (anti-GBM antibodies) and anti-C1q antibodies are valuable laboratory markers for the diagnosis of the various forms of small vessel vasculitis. There are no specific laboratory tests for the diagnosis of medium and large vessel vasculitis. Despite advances in our understanding of the pathogenesis of vasculitis, no biomarkers have yet been identified that can be reliably used to guide treatment or that are useful in distinguishing vasculitis from other inflammatory diseases such as infections or treatment complications., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Background: In March 2020 the SARS-CoV‑2 pandemic disseminated initially especially in Bavaria. At that time data on patients with rheumatic diseases and immunomodulatory treatment was lacking., Objective: The aim was to analyze the influence of the SARS-CoV‑2 pandemic on the clinical treatment strategy., Material and Methods: Between 16 March and 31 July 2020 all patients who consecutively presented at the rheumatology outpatient clinic of the Klinikum rechts der Isar of the Technical University of Munich were included in the study. Individual treatment adjustments were based on clinical judgment and the recommendations for action of the German Society for Rheumatology (DGRh)., Results: A total of 322 patients were included. The most frequent diagnosis was rheumatoid arthritis with 17%, ANCA-associated vasculitis (AAV) with 14% and SLE with 12%. Of the patients 262 were on DMARD treatment and 77 received oral glucocorticoids. There were 5 cases of suspected SARS-CoV‑2 infection; however, no patient verifiably became ill due to COVID-19. In 40 patients, treatment adjustments were done due to the pandemic, whereby 3 patients developed a flare of the underlying disease. In retrospect, treatment de-escalation occurred most frequently in AAV, IgG4-related disease, immunosuppressive treatment with rituximab and the simultaneous presence of malignant diseases., Conclusion: The total lack of confirmed SARS-CoV‑2 infections in an otherwise strongly affected region could indicate that the infection risk for SARS-CoV‑2 is not substantially increased for patients with inflammatory rheumatic diseases. A continuation of most immunosuppressive medications therefore seems reasonable during the ongoing pandemic.

Juvenile dermatomyositis (JDM) is by far the most frequent inflammatory myopathy in childhood and adolescence. It is clinically characterized by inflammatory changes of the skin and muscles but as a multisystemic disease can also affect the skeletal system, the gastrointestinal tract, lungs and heart. Intrinsic (multigenetic risk) and extrinsic factors (triggers) are involved in the pathogenesis resulting in endothelial damage, involvement of fascies, activation of the interferon system and autoimmune reactions including formation of myositis-specific autoantibodies (MSA). In contrast to dermatomyositis in adults, in children and adolescents there are no associations with malignant diseases. The variable expression, the rarity of the disease and the risk of long-term damage and complications necessitate pediatric rheumatological experience in the diagnostics and treatment. Recently, new approaches in drug treatment have substantially improved the outcome and prognosis but a multidisciplinary treatment (including physicians, physiotherapists, psychologists, social workers) is mandatory, especially in the first phases of the disease. Particularly important is a professionally correct treatment of the functional sequelae, which are a particular focus of this article., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Background: With the introduction of tumor necrosis factor (TNF) alpha inhibitors, the treatment of inflammatory rheumatic diseases (IRD) has undergone a fundamental change. Several of the originally high-priced biologics are now accessible as lower cost biosimilars, removing a significant impediment to prescription., Objective: The present study investigated whether the availability of biosimilars is associated with an improvement in the care of IRD. Moreover, the subjective acceptance of biosimilars by physicians and patients was investigated and compared with objectifiable parameters., Material and Methods: Pseudonymized claims data of the Bavarian Association of Statutory Health Insurance Physicians from 2014 to 2019 as well as a paper and pencil survey of patients and rheumatologists formed the data basis of the study., Results: During the observation period, the proportion of diagnosed patients receiving drug therapy increased from 38.5% to 43.2%. Also, the care changed in terms of the prescribed agents. Conventional drug therapy declined overall and, in particular, glucocorticoid prescriptions decreased from 39.3% in 2014 to 34.3% in 2019. At the same time, the proportion of targeted treatments increased from 12.3% to 20.4%. The median duration of basic treatment before first-time bDMARD use dropped from 3.15 years in 2014 to 2.17 years in 2019., Conclusion: Over the observation period, in which three biosimilars entered the market, the care of patients with IRD improved both quantitatively and qualitatively. The market share of biosimilars increased in parallel with this development. With an overall high acceptance of biosimilars, the assessment of the disease course by physicians and patients indicates a slight subjectively perceived advantage of therapy with originals compared to biosimilars, which, however, is not confirmed when standardized scores are applied. A possible explanation for this might be a nocebo effect, which could be minimized by suitable communication strategies., (© 2022. The Author(s).)

Imaging instruments, such as conventional X‑ray, ultrasound and magnetic resonance imaging (MRI) are now fully established and highly valued in the care of rheumatology patients. However, the information provided by these imaging modalities in their current form is of limited utility for the prognostic prediction of individual patient outcomes. This article illuminates an important part of the development of imaging and shows that the vision of personalized medicine is becoming increasingly more tangible due to the further development of high-resolution imaging techniques, molecular imaging and artificial intelligence., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Cocaine is a psychotropic tropane alkaloid and stimulant drug. Nasal insufflation of cocaine powder is a common route of administration. In Germany, cocaine is frequently adulterated with levamisole, an anthelminthic drug with immunomodulatory effects. Both substances are linked to various autoimmune conditions. Cocaine-induced midline destructive lesions cause a progressive destruction of osteocartilaginous structures within the upper respiratory tract and can mimic localized granulomatosis with polyangiitis. In addition, systemic vasculitis due to cocaine and levamisole has been reported. Differentiation of these conditions from primary vasculitis can be challenging because antineutrophil cytoplasmic antibodies (ANCA) are commonly detected. Early diagnosis of these conditions is crucial as clinical improvement is closely related to drug cessation., (© 2022. The Author(s).)

Introduction: Sarcopenia (SP) is defined as the pathological loss of muscle mass and function. This is a clinically relevant problem, especially in geriatric patients, because SP is associated with falls, frailty, loss of function, and increased mortality. People with inflammatory and degenerative rheumatic musculoskeletal disorders (RMD) are also at risk for developing SP; however, there is little research on the prevalence of this health disorder in this patient group using currently available SP criteria., Objective: To investigate the prevalence and severity of SP in patients with RMD., Methods: A total of 141 consecutive patients over 65 years of age with rheumatoid arthritis (RA), spondylarthritis (SpA), vasculitis, and noninflammatory musculoskeletal diseases were recruited in a cross-sectional study at a tertiary care center. The European Working Group on Sarcopenia in Older People (EWGSOP 1 and 2) definitions of presarcopenia, SP, and severe SP were used to determine the prevalence. Lean mass as a parameter of muscle mass and bone density were measured by dual X‑ray absorptiometry (DXA). Handgrip strength and the short physical performance battery (SPPB) were performed in a standardized manner. Furthermore, the frequency of falls and the presence of frailty were determined. Student's T-test and the χ 2 -test were used for statistics., Results: Of the patients included 73% were female, the mean age was 73 years and 80% had an inflammatory RMD. According to EWGSOP 2, 58.9% of participants probable had SP due to low muscle function. When muscle mass was added for confirmation, the prevalence of SP was 10.6%, 5.6% of whom had severe SP. The prevalence was numerically but not statistically different between inflammatory (11.5%) and noninflammatory RMD (7.1%). The prevalence of SP was highest in patients with RA (9.5%) and vasculitis (24%), and lowest in SpA (4%). Both osteoporosis (40% vs. 18.5%) and falls (15% vs. 8.6%) occurred more frequently in patients with SP than those without SP., Discussion: This study showed a relatively high prevalence of SP, especially in patients with RA and vasculitis. In patients at risk, measures to detect SP should routinely be performed in a standardized manner in the clinical practice. The high frequency of muscle function deficits in this study population supports the importance of measuring muscle mass in addition to bone density with DXA to confirm SP., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Sarcoidosis is the most common granulomatous disease in northern Europe. A distinction is made between acute forms of sarcoidosis and chronic sarcoidosis. Chronic sarcoidosis can affect practically all organs but the lungs are affected in 90-95% of patients. The clinical appearance varies between asymptomatic and oligosymptomatic courses, which are diagnosed more by chance, to courses with acute organ failure. An extensive organ work-up is necessary at the time of the initial diagnosis in order to record the extent of organ involvement and to make appropriate treatment decisions. Asymptomatic courses with purely pulmonary sarcoidosis do not require treatment and can be observed over the course of the disease, whereas courses with extensive organ involvement or organ dysfunction require treatment. The treatment consists primarily of the administration of glucocorticoids. If the effect of the glucocorticoids is insufficient or if there are side effects, various immunosuppressive agents, including biologics can be added., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Background: Allergies are frequent and approximately 30% of the general population in Germany are affected. The specific sensitization against an allergen is asymptomatic. On renewed allergen contact the symptoms are indicative of the underlying pathomechanism. A variety of different test procedures are available to identify allergic reactions., Objective and Aim: In this review article the typical clinical symptoms of allergic reactions are assigned to mechanisms and possible test methods are presented and discussed. Current developments in recombinant serum diagnostics and cellular testing methods are presented., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

For the continued existence of the specialty of internal medicine and rheumatology and the assurance of a qualitative patient care, attractive further education for motivated resident physicians is of central importance. Continuing training in rheumatology takes place primarily in the inpatient setting, although reliable figures on outpatient and inpatient further education positions are not yet available. Further training in rheumatology is predefined by the model further training regulations (Musterweiterbildungsverordnung; MWBO) 2018, which have now been implemented by most state medical associations, in some cases with state-specific changes. Based on the MWBO of 2018, a model curriculum was developed by the German Society of Rheumatology (DGRh) for further training in the specialty of internal medicine and rheumatology. This model curriculum is intended to provide orientation for trainees and trainers as well as to facilitate structured rheumatology training in inpatient and outpatient settings., (© 2022. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Osteopathy in rheumatology can either be primary a condition as a consequence of inflammatory rheumatic diseases but can also be drug induced. The most severe clinical manifestations are insufficiency fractures and osteonecrosis. The risk of fractures is highest for patients treated with glucocorticoids depending on the daily intake, the cumulative glucocorticoid dosage and the duration of administration. An incidence rate of nearly 13% was reported after administration of glucocorticoids lasting > 1 year. Cases of osteonecrosis under glucocorticoids are, in contrast, less frequent and not associated with glucocorticoid-induced osteoporosis. The antiresorptive substances bisphosphonates and denosumab, as well as romosumab are effective and important in treating osteoporosis; however, they can also cause atypical fractures, particularly of the femur as well as osteonecrosis of the jawbone. According to the most recent guidelines the benefits of bisphosphonate treatment have only been verified for 3-5 years and for denosumab for 3 years. There are clear preventive recommendations to avoid osteonecrosis of the jaw. Ultimately, the disease-modifying antirheumatic drugs (DMARD) methotrexate and leflunomide also affect the metabolism of bones. There is a rare but very characteristic form of osteopathy associated with methotrexate, mainly occurring in cases of long-term treatment. The typical manifestations are insufficiency fractures, particularly of the distal tibia, which persist for many years under continuous methotrexate administration. The treatment is the discontinuation of methotrexate and in most cases the fractures will heal within 3-4 months. Leflunomide has been associated with cases of persisting pseudarthrosis that only disappeared after a wash-out of the active metabolite., (© 2022. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Only the correct diagnosis enables an effective treatment of rheumatic diseases. Digitalization has already significantly accelerated and simplified our everyday life. An increasing number of digital options are available to patients and medical personnel in rheumatology to accelerate and improve the diagnosis. This work gives an overview of current developments and tools for patients and rheumatologists, regarding digital diagnostic support in rheumatology., (© 2021. Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Cyclophosphamide is still clinically used in rheumatic diseases with severe disease courses. Cyclophosphamide has a pronounced gonadotoxic effect largely depending on the cumulative dose. The risk of amenorrhea is reported to be in the range of 12-54% and is dependent on the age of the patient at initiation of treatment. Every patient of reproductive age should therefore be offered counseling on options for fertility protection. There are 3 options for fertility protection: oocyte harvesting and cryopreservation after a hormonal stimulation of 10-14 days, ovarian wedge resection and cryopreservation and administration of a gonadotropin-releasing hormone (GnRH) agonist. The decision whether and, if so, which treatment should be performed is made in close consultation between the patient, rheumatologists and reproductive physicians and depends on the available treatment time window, the age of the patient and the severity of the underlying disease., (© 2021. Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

This article reports the case of a 34-year-old female patient with multiple thrombotic events (cerebral venous sinus thrombosis, ischemic duodenitis) with simultaneous thrombocytopenia (ca. 70/nl). A paroxysmal nocturnal hemoglobinuria without detectable hemolysis and with simultaneous myelodysplastic syndrome was causative for these symptoms. This rare disease is important to consider in the differential diagnosis of chronic inflammatory rheumatic diseases, such as systemic lupus erythematosus, antiphospholipid antibody syndrome and vasculitis.

Background: The initial presentation of patients with symptoms indicative of a rheumatic disease is in most cases not directly to a rheumatologist. This study evaluated the following questions: I. Which medical specialists refer patients to a department of rheumatology? II. Evaluation of the accordance of the presumptive referral diagnosis and the final diagnosis by a rheumatologist., Methods: A total of 947 patients (279 men and 668 women) who initially presented to a university hospital for rheumatological diagnostics were included in the study. The referring medical specialist fields were identified. Furthermore, a kappa analysis was performed to evaluate the accordance of the presumptive referral diagnosis and the final diagnosis generated after a rheumatological evaluation of the patients., Results: Of the referrals 73% were initiated by general practitioners or internists functioning as general practitioners. The other referrers were 5% specialists in internal medicine (excluding rheumatology), 4% orthopedic/trauma surgeons, 1% other surgeons and 4% other specialist fields. A rheumatological diagnosis was made in 58% of the patients and rheumatological inflammatory joint diseases (26%), collagenosis (14%) and vasculitides (5%) were the most frequently diagnoses. The accordance of the presumptive diagnosis of the general practitioners and the final diagnosis after rheumatological evaluation was a kappa coefficient of κ = 0.304. Lower kappa values were evaluated for orthopedic surgeons (κ = 0.277) and other specialists (κ = 0.200)., Conclusion: The referrals to a rheumatology institution were frequently initiated by general practitioners and internists functioning as general practitioners. In this context the presumptive diagnosis of general practitioners showed a low accordance with the final rheumatological diagnosis. In contrast, a detailed presumptive diagnosis is desirable for optimal use of the limited resources for rheumatological care.

Background and Objective: The work environment for rheumatologists has significantly changed over the last years. The healthcare of patients with rheumatic diseases is at risk due to the age structure of specialized rheumatologists in middle Germany and the limited availability of training positions for rheumatologists. In this context, it is important to have detailed information on the resident physicians in rheumatology concerning their own visions regarding their future professional career., Material and Methods: A questionnaire was sent to resident physicians in 25 rheumatology training hospitals in the middle of Germany (Saxony, Saxony-Anhalt and Thuringia). The questionnaire was completed and returned by 27 participants (17 women and 10 men)., Results: Most of the participants (60%) aimed to qualify as a specialist in internal medicine followed by a specialization in rheumatology (altogether training for a minimum of 8 years). After finishing training 44% would prefer to work in an outpatient setting while 30% planned to work in a combined outpatient and clinical setting. Of the participants 48% would prefer to work as part-time rheumatologists and 74% (women 94% and men 40%) were interested in employment in an outpatient medical healthcare center. The compatibility of family and work as well as the work-life balance was considered to be highly relevant for the future professional life., Conclusion: Less than half of the participants intended to work exclusively in an outpatient setting after completing the training in rheumatology. In addition, the participants preferred a part-time employment with compatibility of professional and private life. Consequently, alternative models of employment should be created in rheumatology to be attractive for future physicians. On the other hand, the study revealed that the independent rheumatological practice has a lower priority for the young rheumatologists taking part in this survey.

Background: The tenosynovial giant cell tumor (pigmented villonodular synovitis) is a proliferative, mainly benign soft tissue tumor of the tendon sheaths, bursae and joints arising from the synovia. It can be divided into circumscribed localized and destructive diffuse types. Approximately 1% of all joint diseases are due to this entity. The tumor is considered as a rarity. Mostly case studies exist. For this study the focus was set on the localized type (L-TSRZT), which accounts for 90% of the diagnoses of this tumor. Given its rarity, data are limited. Therefore, the research aim was to provide data on prevalence, primary location and sensitivity of clinical versus histopathological diagnosis in a German sample., Methods: Based on the Histopathological Arthritis Register of the German Society for Orthopedic Rheumatology, the data of the L‑TSRZT were retrospectively analyzed (time frame 1 January 2018-28 December 2020)., Results: This database contained N = 7595 cases of arthropathy. A total of n = 45 patients with the diagnosis L‑TSRZT were identified. The prevalence of the tumor was 0.6%, 95% CI [0.4%, 0.8%], or 5.9 cases per 1000. The primary location involved the finger (48.9%). In 14 of 45 cases the diagnosis was correctly determined from the clinical side, corresponding to a sensitivity of 31.1%, 95% CI [18.2%, 46.7%]., Conclusion: For the first time, this paper was able to provide data on a large sample for Germany. Notably, the low sensitivity of the clinical diagnosis confirms the importance of histopathology for diagnosing L‑TSRZT., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

A growing number of health insurance data analyses show an increase in the prevalence of rheumatoid arthritis (RA) in Germany. The studies refer to the claims diagnosis of RA, which is more frequent in recent years compared to earlier periods. Depending on the case definition, the numbers vary between 0.6% and 1.4% of the adult population. In this paper, the different studies are reviewed with regard to their data sources, the case definitions of RA and the frequency of the diagnosis. Due to the lack of clinical validation, the prevalence cannot be precisely determined from claims data., (© 2022. The Author(s).)

Treatment of systemic lupus erythematosus (SLE) without permanent glucocorticoid therapy is inconceivable for most patients and their physicians. Although we have significantly improved the prognosis of SLE, management remains constrained by a lack of effective, targeted therapies and the lack of evidence-based approaches to the use of existing compounds. For example, for glucocorticoids (GC), which are used continuously in a majority of patients, there are no evidence-based recommendations for initiation, tapering, and cessation in the treatment of SLE. Even today, GC are without alternatives in acute situations, especially organ- or life-threatening ones. However, due to the known long-term adverse effects, the role of GC is viewed increasingly critically. Long-term data from cohorts show that the use of GC actually contributes to morbidity and mortality in SLE. Strategies to reduce the use of GC in SLE are therefore urgently needed and are proposed in this paper.

Background: The often slow onset of ankylosing spondylitis (AS), the initially partially unspecific symptoms (back pain) and the scarcity of resources in rheumatological care are important factors leading to delayed diagnosis and treatment of these mostly young patients in Germany. Qualified nurses specialized in rheumatology might improve quality of care by providing medical services delegated by the rheumatologists., Objective: The aim was to investigate whether qualified nurses specialized in rheumatology can interpret anamnestic and clinical findings such as rheumatologists in patients with chronic low back pain and still unclear diagnosis using a structured questionnaire., Material and Methods: In the multicenter PredAS study a structured anamnestic questionnaire was applied independently by qualified nurses specialized in rheumatology and rheumatologists to patients referred to rheumatology practices with the leading symptom of low back pain. The questionnaire covered basic demographic data, medical history and patient reported outcomes. Additionally, measurements of physical function using the Bath ankylosing spondylitis functional index (BASFI) and spinal mobility using the Bath ankylosing spondylitis metrology index (BASMI) were standardized. In order to test the possible facilitation by using digital media, the results of two patient groups were separately documented on paper-based report forms and on an i‑pad. Concordance between documentation by qualified nurses specialized in rheumatology and rheumatologists was studied by calculating Cohen's kappa, intraclass correlation coefficients (ICC) and percentage agreement on an individual patient level., Results: Nearly 75% of the 141 patients with chronic low back pain were identified as having the characteristics of inflammatory back pain. The concordance of the documentation for the anamnesis of back pain by qualified nurses specialized in rheumatology and physicians was higher than for the localization of the back pain. The results for the BASMI showed no differences between qualified nurses specialized in rheumatology and physicians (ICC 0.925, 95 % confidence interval, CI 0.879-0.953). The time taken for the structured documentation was 20 ± 6.7 min for physicians and 28.5 ± 13 min for qualified nurses specialized in rheumatology., Conclusion: The results indicate that well-trained qualified nurses specialized in rheumatology have a high potential to take over some of the workload from rheumatologists during documentation of the anamnesis and the initial physical examination in the diagnosis of ankylosing spondylitis.

Background: Systemic sclerosis (SSc) is a fibrosing autoimmune disease of the connective tissue. In addition to skin fibrosis, pulmonary involvement and interstitial lung disease (ILD) in particular are the most common and severe manifestations of SSc. The disease is associated with a substantial risk of morbidity and mortality, especially in progressive ILD. In the last 5 years new treatment concepts for SSc-ILD have been investigated in numerous clinical studies., Material and Methods: This review is based on a literature search in PubMed, focusing on the most relevant papers published up to the end of 2018 with the keywords "SSc" and "treatment"., Results: The treatment of SSc-ILD has changed over the last few years due to the results of many clinical studies. The updated guidelines of the European League Against Rheumatism (EULAR) recommend the use of cyclophosphamide or hematopoietic stem cell transplantation. Data for a positive influence on SSc-ILD are also available for mycophenolate, tocilizumab and anabasum. Because of the pathophysiological similarities to idiopathic pulmonary fibrosis, the use of the antifibrotic agents nintedanib and pirfenidone is currently being investigated in randomized, multicenter clinical trials and could be a novel and promising therapeutic strategy., Conclusion: Current drug studies may provide innovative therapeutic perspectives for SSc-ILD and could significantly improve the prognosis of affected patients in the future.

Background: Infections are one of the most common clinical problems in patients with rheumatic diseases who need to be treated with glucocorticoids in an intensive care unit. To date, there are no recommendations for the standardized control of glucocorticoid treatment in such situations., Objective: Based on a literature search this paper provides an overview of evidence-based and eminence-based recommendations for the control of glucocorticoid treatment under intensive care conditions using the example of systemic lupus erythematosus., Methods: A systematic literature search was carried out using a MeSH term search in the PubMed database., Results: Infections are one of the most common causes for the treatment of patients with rheumatic diseases in intensive care units. In the case of systemic lupus erythematosus it is particularly challenging to distinguish the infection from increased disease activity or to treat the parallel occurrence. Patients in an intensive care unit are exposed to an increased level of physical stress due to the severity of the disease, which is why special attention should be paid to symptoms of adrenocortical insufficiency. Evidence-based recommendations for prophylaxis of an adrenal crisis only exist in relation to perioperative procedures and not for the situation of severe infections., Conclusion: The use of glucocorticoids in systemic lupus erythematosus is often chronic and there is an increased risk of infections. In the case of infections (or simultaneous disease flare) adequate anti-infective treatment should be administered, the treatment with glucocorticoids should be adjusted accordingly and symptoms of adrenocortical insufficiency should simultaneously be looked for.

Background: In May 2018 a revision of the training regulations for German doctors (MWBO) was passed which will significantly impact the medical training for German rheumatologists. The goal of this paper was to create an overview of the status quo in advanced medical training in this field., Methods: A quantitative and qualitative analysis was conducted to identify the strengths and weaknesses of the current continuous education in the field of rheumatology in Germany. The number of newly trained specialists in rheumatology in the years 2015 to 2017 was determined from the regional medical associations. Furthermore, an online survey assessing the training conditions of assistant physicians was conducted. 71 physicians participated in the nationwide survey., Results: In 2015 (53 specialists), in 2016 (43 specialists) and in 2017 (43 specialists) completed their training in the field of rheumatology. The online survey of assistant physicians found that the medical training was graded 3.1 on a scale of 1 to 6 (SD ± 1.1). 19.4% of respondents reported that they had regular mentoring, and 41.7% had on-the-job evaluations. Deficits were evident concerning practical diagnostic skills: 22.8% of the physicians were not practicing joint ultrasonography and another 20% performed it only once a month. As points of criticism 46.6% mentioned a missing structure in training programme and 33.3% noted a lack of supervision. The majority of respondents wanted more training options, supervision and better practical training in ultrasound diagnostics., Conclusion: The nationwide number of newly acquired specialist qualifications (between 2015 and 2017) showed a decreasing trend. It is to be feared that the numbers will continue to stagnate, which increases the importance to invest further in medical education. The online survey also showed that the current medical training in Germany has deficits. Here, the new MWBO could create better guidelines for a structured training, which should be guided by the principle of "Teach to Target".

Background: A number of designations for diseases, medicines and human body structures derive from classical mythology. To date, these eponyms have not been systematically investigated., Objectives: This paper provides an overview of this fringe component of medical vocabulary, looks at the history of several terms and formulates hypotheses as to why such creative etymologies have come into being., Materials and Methods: In addition to relevant texts on ancient mythology, a variety of medical textbooks from the early modern period were analyzed., Results: Between the 16th and the 20th centuries some 30 figures from Greek and Roman literature made their way into the terminology of medical sciences. A few of these expressions can be encountered in clinical use (e. g., Caput Medusae, Proteus, Oedipus complex) and remain official anatomical (atlas, Achilles tendon) or pharmaceutical nomenclature (atropine, morphine). The choice of these designations has often been similarity of form or analogies in function. Classical eponyms have gained acceptance on account of their succinctness, conciseness and scholarly veneer. Finally, this vocabulary shares its origin with other relevant terminology., Conclusions: In clinical classes, mythological designations can serve as a point of departure for digressions into literary, art and medical history in order to provide an understanding of cultural traditions and enhance education.

Background: Systemic amyloidoses are rare protein deposition disorders, which are often diagnosed in an advanced stage of the disease due to non-specific symptoms. Any chronic inflammatory disease can lead to an AA-type amyloidosis., Aim: This paper summarizes the current state of the art of diagnosis and treatment of AA amyloidosis and presents data from the past 10 years of our amyloidosis center., Material and Methods: Our data represents an analysis of our cohort of patients with amyloidosis and a selective research in the PubMed database for AA amyloidosis., Results: The underlying diseases comprise autoinflammatory syndromes, polyarthritis, and chronic inflammatory bowel and lung diseases. Renal organ involvement is the most prevalent in AA amyloidosis. It can be detected early through the evaluation of proteinuria. The treatment depends on the individual underlying disease. Patients without an associated inflammatory disease are considered to have idiopathic AA amyloidosis and empiric treatment is mandatory., Discussion: Survival of this fatal disease has recently improved due to the new diagnostic tools and treatment options; however, early diagnosis plays a crucial role in the prevention of end-stage renal failure. New therapeutic strategies aim to remove existing amyloid deposits.

Background: The primary vasculitides are rare conditions in childhood. The most common disease subtypes are Schönlein-Henoch purpura and Kawasaki's syndrome, which frequently have a self-limiting course. In the majority of vasculitides, the etiology remains unknown. Environmental exposure, including infections, is suspected to trigger an autoinflammatory response in predisposed individuals., Goal: The aim of this review is to present the various aspects of childhood vasculitis., Materials and Methods: Reviews and special original papers on childhood vasculitis, published classification criteria and current therapy guidelines were reviewed and summarized., Results: The classification of vasculitides in childhood has been modified from the previous adult Chapel Hill classification for vasculitides in 2008. Most therapy recommendations for children are adapted from results of studies in adults. This review covers the current classifications, pathogenesis, clinical manifestations and therapy recommendations for children., Discussion: Although etiology and pathogenesis of many vasculitides in childhood are still unknown, clarifying diagnostic methods and effective therapeutic options are available. The knowledge about various forms of disease manifestation may contribute to an early diagnosis and timely initiation of treatment, which may prevent devastating irreversible impairment.

Background: Gout is the most common inflammatory arthropathy in the Western world. This is mainly due to the high socioeconomic status, sufficient even superfluous nutrition, overweight and alcohol consumption. Despite adequate medication, information and advice on nutrition and lifestyle are one of the cornerstones in the management of these patients., Objective: The aim was to provide recommendations on nutrition and lifestyle in cases of gout and hyperuricemia by a group of rheumatologists, based on a review of the most recent literature., Materials and Methods: The study group for osteoarthritis and crystal arthropathies of the Austrian Society for Rheumatology and Rehabilitation (ÖGR) carried out a literature search on this topic. The selected papers were listed according to the level of evidence., Results: Based on this literature search nine recommendations were generated and modified via a Delphi approach: four red "don'ts" concerning nutrition and beverages to be avoided, three green "do's" concerning favorable food as well as two blue general lifestyle recommendations. The format of the recommendations is a two-page leaflet with the list of recommendations, level of evidence, strength of recommendation and literature citations on the front page and a colored icon presentation of food and beverages in a circle, matching the colors of the written recommendations, on the reverse., Conclusion: For the first time in Austria, nine recommendations on nutrition, beverages and lifestyle for patients with gout and hyperuricemia were defined for everyday practice, as education material for patients and updated information for physicians.

Background: Children and adolescents with inflammatory rheumatic diseases have a disease and treatment-related increased risk of infections. This risk includes vaccine-preventable diseases; therefore, vaccinations represent an important preventive measure against infection in these patients. However, approximately one in three patients with a juvenile rheumatic disease is nowadays still inadequately vaccinated, mostly due to uncertainty regarding the efficacy and safety of vaccination in these patients., Objectives: This paper summarizes the available evidence regarding the efficacy and safety of vaccinations in children and adolescents with rheumatic diseases and gives recommendations for the clinical practice., Results and Perspectives: Almost 2000 children and adolescents with rheumatic diseases were examined in the more than 30 previously published vaccination studies, comprising nearly all standard vaccinations in the immunization schedule. The immunogenicity was usually sufficient and there was no evidence of a relevant aggravation of the underlying disease. Recommendations for the clinical practice are given also considering data beyond pediatric rheumatology; however, a final benefit-risk assessment is not yet possible.

Introduction: Peripheral arthritis is the most common presenting complaint in clinical rheumatology. Unequivocal identification of the underlying entity can be difficult, particularly at an early stage. Such cases are commonly referred to as undifferentiated peripheral inflammatory arthritis (UPIA). Since evidence-based recommendations for the clinical management of UPIA are lacking, this international 3e initiative convened 697 rheumatologists from 17 countries to develop appropriate recommendations., Methods: Based on a systematic literature research in Medline, EMBASE, Cochrane Library, and the ACR/EULAR abstracts of 2007/2008, 10 multinational recommendations were developed by 3 rounds of a Delphi process. In Germany, a national group of experts worked on 3 additional recommendations using the same method. The recommendations were discussed among the members of the 3e initiative and the degree of consensus was analyzed as well as the potential impact of the recommendations on clinical practice., Results: A total of 39,756 references were identified, of which 250 were systematically reviewed for the development of 10 multinational recommendations concerning differential diagnosis, diagnostic and prognostic value of clinical assessments, laboratory tests and imaging techniques, and monitoring of UPIA. In addition, 3 national recommendations on the diagnostic and prognostic value of a response to anti-inflammatory therapy on the analysis of synovial fluid and on enthesitis were developed by the German experts based on 35 out of 5542 references., Conclusions: The article translates the 2011 published original paper of the international 3e initiative (Machado et al., Ann Rheum Dis 70:15-24, 2011) and reports the methods and results of the national vote and the additional 3 national recommendations.

Psoriasis arthritis (PsA) encompasses many diverse clinical symptoms. Epidemiological data about the prevalence in general, predisposed age groups, prevalence of joint, spine, enthesis, and extra-articular manifestations are very heterogeneous. Even for the PsA specialist the clinical picture is not always easy to differentiate from other overlapping or clinically similar disease entities. This paper tries to give some guidance on how to screen for and how to detect PsA early in the population of psoriasis patients on the basis of the Mainz PsA screening and early diagnosis algorithm.

In psoriatic arthritis (PsA) the heterogeneous spectrum of the disease with arthritis/synovitis, axial manifestation, enthesitis, dactylitis, psoriatic skin disease and nail psoriasis has to be considered. Moreover, PsA activity and severity as well as comorbidities are of importance for making therapeutic decisions. Measurement instruments developed for therapeutic studies of rheumatoid arthritis or ankylosing spondylitis are often not appropriate for application in PsA investigations. In this paper established therapies with nonsteroidal antirheumatic drugs, disease modifying antirheumatic drugs (DMARDs) and TNF-alpha inhibitors and the current EULAR guidelines from 2012 are reviewed. However, there is a need for new therapeutic agents for those patients who do not respond to or do not tolerate the current therapies. Other biologic agents have also been tested for PsA with moderate effects only. New therapeutic options could result from the anti-IL12 and anti-IL23 receptor monoclonal antibody ustekinumab and from small molecules such as the oral PDE-4 inhibitor apremilast.

For decades, G. Thibierge and R.J. Weissenbach worked at the Hôpital St. Louis in Paris, one of the first and largest dermatological centers.In 1911 they described the occurrence of subcutaneous calcification in scleroderma patients, emphasizing that this was not a coincidental finding, but rather a causal link. Since then this syndrome has been given their names worldwide.Thibierge gave his attention solely to dermatology and venereology. Weissenbach initially concentrated on the overlapping areas between dermatology and rheumatology, such as gonococcal arthritis, joint disease in Lues and psoriatic arthritis. He later dealt with all areas of rheumatology, set up a large rheuma clinic at the hospital, wrote a series of authoritative books and a considerable number of individual papers. Thus he became a rheumatologist even before the specialty rheumatology existed.

The growing burden of chronic disease and the current nature of healthcare systems which are structurally ill-equipped to cater to the complex needs of patients with chronic conditions has led to governments and healthcare providers seeking alternative ways to improve patients own capacity to actively self-manage their chronic condition. In Australia, there has been a focus on patient education and self-management programs within the healthcare system to achieve this. These programs aim to empower patients through providing information and teaching skills and techniques to improve self-care and doctor-patient interaction with the ultimate goal of improving quality of life. Patient education and self-management programs have been supported through several national government initiatives and implemented within the healthcare setting. This paper describes the current position of patient education and self-management within the Australian healthcare system. It further describes a new collaboration project between an Australian and a German research team which aims at translating an assessment questionnaire used in Australia for the evaluation of self-management programs, the "Health Education Impact Questionnaire" ("heiQ"); this instrument is expected to be of significant use in the German rehabilitative system.

Systemic sclerosis is a challenging disease in terms of early risk assessment and the implications for therapy. The current article draws on a literature analysis and on personal experience of over 450 patients with systemic sclerosis and related illnesses, describing an attempt at a risk-adapted treatment strategy for patients with systemic sclerosis, and is intended to be used as a discussion paper. A major point of emphasis is on novel therapeutic options for patients who are refractory to current treatment strategies and who are at a high risk of serious complications or a limited prognosis. The paper also highlights possible future treatment options, including vasoactive, cellular and immune suppressant treatments.

Aim: To gather information on current organizational structures in rheumatologic ambulatory health care in Germany. Based on the results recommendations on future structures will be discussed., Methods: This study involved data collection and statistical analysis via a structured 10-page questionnaire among the members of the German Association of Rheumatologists. The questions concerned a variety of topics including information on office structures, patient structure, structure of services offered, co-operation with colleagues and hospitals, quality assurance measures, economic factors, and a subjective assessment of the health care structures in rheumatology by the participants., Results: Data obtained from 197 rheumatologists who participate in health care were analyzed. In this paper results concerning the organizational as well as the medical ambulatory health care structure will be presented. Data on economic factors will be presented in part 2 of this study., Conclusions: The organization of ambulatory treatment regarding processes and treatment differences between office-based physicians and rheumatologic outpatient departments in hospitals was very homogeneous. However, physicians in the eastern regions treated significantly more patients compared with the western parts of Germany. This difference was also observed between the north and south. Differences in patient groups (e.g. underlying diseases) were reported between different sub-groups of rheumatologists (e.g. internal specialists vs. GP vs. orthopedic rheumatologists). Integrated health care, as promoted by German social law, did not play a major role. Overall there was a high level of self-initiated training of physicians and participation in education of patients and other physicians.

Antiphospholipid syndrome (APS) is characterized by recurrent arterial or venous thromboembolism or pregnancy loss in association with antibodies directed against anionic phospholipids or plasma proteins bound to anionic phospholipids. A common cause of the huge variety of clinical manifestations is vaso-occlusive disease and not vasculitis in venous or arterial blood vessels of different sizes and sites (i.e. deep vein thrombosis, pulmonary embolism, cerebrovascular disease). In accordance with this, fetal abortion, typically beyond the tenth week of gestation, is also caused by infarctions of blood vessels in the placenta. Establishing the correct diagnosis of APS is not easy. To estimate the risk of thrombotic complications is challenging, as well as the questions of, which, how long and in what strength anticoagulation is recommended. This paper should enable the reader to apply international consensus classification criteria correctly, to interpret the different laboratory tests for anti-phospholipid antibodies and to gain an awareness of the different forms of anticoagulation in order to stratify therapeutic decisions.

Background: Methotrexate is considered to have the best ratio of efficacy to toxicity of the disease modifying antirheumatic drugs. Recently it has been shown to enhance the life expectancy of patients with rheumatoid arthritis. Some 30-60% of RA patients discontinue MTX treatment within 1 year because of side-effects. In this review, the current data about supplementation with folate or folinic acid and their effect on the toxicity and efficacy of low-dose methotrexate therapy are analysed., Methods: A Medline search was performed using "folate", "folic acid", "folinic acid", "homocysteine", "methotrexate", "cardiovascular", "heart infarction" and "rheumatoid arthritis" as search terms. The relevant literature was reviewed and other papers referred to as references were explored., Conclusion: Both folate and folinic acid reduce methotrexate toxicity and the discontinuation rate, and decrease methotrexate-induced hyperhomocysteinemia. Folate is less expensive, more secure and easier to handle than folinic acid. The efficacy of methotrexate probably decreases slightly, but the benefit outweighs the risk. Folate supplementation should, therefore, be routinely prescribed to every patient taking low-dose methotrexate.

The therapeutic specialties of osteoporosis in inflammatory rheumatic diseases has gained mounting interest in the last year. The paper describes special aspects of osteoporosis in rheumatoid arthritis, ankylosing spondylitis and systemic lupus erythematodes. The problems of glucocorticoid therapy are discussed intensively with regard to the recently published recommendations for glucocorticoid-induced osteoporosis. Risk factors of osteoporosis and the therapeutic implications are demonstrated intensively as well as the modifications of the recommendations.

Aim: The aim of this study is to review the data of skeletal manifestations of Behçet's disease (BD, Morbus Adamantiades- Behçet, silk road disease), which is clinically diagnosed by dermatological, neurological and ophthalmological symptoms. This paper demonstrates the diagnostic and therapeutic difficulties in the management of osteonecrosis and recurrent arthritis associated with BD. Arthrogenic symptoms are a well-recognized feature of the syndrome but low in incidence., Method: A literature review served as the database to show the characteristics of osseous and articular manifestations in Behçet's disease. Furthermore, we present a 25-year clinical follow up of a patient with BD and multiple osteonecrosis., Results/conclusions: Chronic or intermittent joint pain should lead the physician to include BD into the differential diagnosis especially if there are findings of cutaneous ulcerations, ophthalmological inflammations or neurological symptoms. For detection of osteonecrosis at an early stage, which would allow for successful treatment, MRI scans of at least the symptomatic joints are recommended.

The spondyloarthritides (SpA) are common inflammatory rheumatic diseases with an overall prevalence of 0.6-1.9%. Ankylosing spondylitis (AS) and undifferentiated spondyloarthritis (uSpA) are the most common subsets of SpA. Women are almost as frequently affected as men, but the total burden of disease may be similar. At least one third of AS patients are severely affected with impaired function and a poor quality of life similar to patients with rheumatoid arthritis. However, AS starts 20-30 years earlier in life. Thus, AS has a relevant socioeconomic impact on society. After decades of no progress concerning treatment options in SpA, there is now accumulating evidence that the new anti-TNF alpha agents do not only reduce signs and symptoms of AS caused by inflammation but they may also diminish structural damage. Very recent magnetic resonance imaging (MRI) data of a follow-up study support this assumption. The following paper reviews the currently available literature on anti-TNF alpha therapy in AS and uSpA. Efficacy, side effects and experiences with different doses are discussed. In expectation of the approval of infliximab and etanercept for the treatment of active AS international guidelines for initiation, monitoring and discontinuation of these agents have been recently proposed.

In the diffuse form of pigmented villonodular synovitis, the transformed synovia invades local bone and cartilage, leading to destruction of the osseous and cartilage tissue. Secondary osteoarthritis and ultimately joint destruction is a result. Malignant development is under discussion. The knee is the most frequently involved joint, followed by involvement of the hip, which is well documented in several studies. This paper introduces a case of pigmented villonodular synovitis involving the hip. Its general diagnostic features and treatment options are introduced and discussed, including the role of reconstructive joint surgery in advanced cases of PVS. The key function of early diagnosis and treatment resulting in joint-salvaging procedures is emphasized.

The 2002 ATS/ERS consensus classification of idiopathic interstitial pneumonias standardizes definitions and criteria for classification and diagnosis of idiopathic interstitial pneumonias and replaces previous classifications. Based on clinico-radiologic-pathologic criteria seven entities were defined: idiopathic pulmonary fibrosis, nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia and lymphoid interstitial pneumonia. The following paper includes a brief overview of the histopathological diagnosis of these entities as compared to other diffuse interstitial pulmonary diseases and pulmonary manifestations of collagenvascular diseases.