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Background: Chronic inflammatory diseases (immune-mediated inflammatory diseases, IMID) can overlap or occur simultaneously due to clinical similarities. The resulting utilization of heathcare structures has not yet been investigated across disciplines but is of potential importance for optimizing the treatment of patients with IMID., Aim of the Work: Analysis of epidemiological data including utilization of care services in patients with selected IMIDs: psoriasis, psoriatic arthritis (PsA), rheumatoid arthritis (RA), ankylosing spondylitis, ulcerative colitis, Crohn's disease and connective tissue disease., Material and Methods: In a retrospective cross-sectional analysis based on health insurances accounting data with a sample of approximately 4 million insured persons, the prevalence of the abovementioned IMID and the frequency of IMID combinations were analyzed based on documented diagnoses (ICD-10 GM). The frequency of hospitalizations and utilization of outpatient physician contacts was recorded in predefined specialist disciplines (general medicine, dermatology, gastroenterology, rheumatology) and compared with an age-adjusted and gender-adjusted reference population., Results: A total of 188,440 patients had at least 1 of the IMID diagnoses analyzed (4.7%), with an age peak of 61-70 years. The highest prevalence was observed for psoriasis (1.85%), followed by rheumatoid arthritis (1.38%). Combinations with at least one other IMID were relatively common (29%), with this being most common in patients with psoriatic arthritis (82.9%, of which 68.2% had psoriasis), followed by ankylosing spondylitis (27.5%) and Crohn's disease (21.6%). Compared to the reference population, patients with IMID were hospitalized more often and more frequently utilized the outpatient disciplines., Discussion: The study results describe that IMIDs occur in combination and that the patients make comparatively more use of care structures of different disciplines. A multidisciplinary approach could increase the efficiency of care; an evaluation is still pending., (© 2023. The Author(s).)

Background: The updates to the European recommendations and the German guidelines for the treatment of systemic sclerosis are expected shortly, which are very good evidence-based guidelines for all those treating the disease; however, there are still disease manifestations with insufficient studies and current study results that were published after the review of the literature for the guidelines and might be of interest to the reader., Objective and Methods: The aim of this work is to provide an overview of the publications in the last year that are interesting from the authors' point of view. The aim is to provide practically relevant information on the current state of knowledge that can supplement the guidelines., Results: The pathogenesis of systemic sclerosis (SSc) is becoming better understood in its interplay between environmental factors and the development of autoantibodies. There have also been overviews of the manifestation and prognosis of cardiac involvement in the last year. The American Thoracic Society issued the first guidelines for the treatment of interstitial lung disease in SSc. There are an increasing number of studies that suggest that disease-modulating combination therapies, such as rituximab and mycophenolate mofetil (MMF) are beneficial. Work addressing the involvement of joints suggests that inflammatory changes are common. Current options for the treatment of gastrointestinal involvement are presented., Conclusion: The diagnosis and treatment of systemic sclerosis is making progress and many symptoms and complications are treatable. Nevertheless, much remains to be done to improve the quality of life of the patients., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Background: Interdisciplinary medical treatment is required to care for patients with complex autoimmune diseases. Although there are an increasing number of interdisciplinary centers for autoimmune diseases in Germany, they are not yet available throughout the country and the focuses and interdisciplinary structures are not organized according to a generally agreed standard. Furthermore, they are not regularly reflected in the general care structure., The Aim of the Work: To analyze the care structure using as an example an established center and a clinical case to demonstrate the usefulness of in-house standardized procedures., Material and Methods: In order to determine the status quo regarding interdisciplinary centers for autoimmune diseases in Germany, a university hospital is exemplarily presented for a structural analysis and a case presentation from another center to demonstrate the importance of an interdisciplinary patient care., Results: At the selected center for autoimmune diseases of the university hospital, patients with autoimmune diseases receive interdisciplinary care from experts from various disciplines. The structures are anchored in an organizational chart. The case report demonstrates a standardized diagnostic and therapeutic pathway (standardized operating procedures, SOP) in a patient with systemic sclerosis and lung involvement., Discussion: The article discusses which measures are necessary across disciplines for comprehensive diagnostics and treatment of certain autoimmune diseases, which challenges arise during implementation and which advantages can arise compared to guidelines because, among other things, they can be immediately adapted. The establishment of a national consensus for the structure, necessary settings and implementation into patient care within an interdisciplinary center for autoimmune diseases is desirable., (© 2024. The Author(s).)

A German expert committee recommends defining fast-track clinics (FTC) for the acute diagnosis of giant cell arteritis (GCA) as follows: easy and prompt reachability at least on weekdays, scheduling appointments ideally within 24 h, examination by a specialist with GCA expertise, ≥ 2 experts per FTC, ≥ 50 patients with suspected GCA per year, sonologists with ≥ 300 (≥ 50) temporal and axillary artery examinations, adherence to standard operating procedures, availability of an ≥ 18 (≥ 15) MHz and a lower frequency linear ultrasound probe, and collaboration with partners for neurology and ophthalmology consultations, magnetic resonance imaging (MRI), positron emission tomography-computed tomography (PET-CT, possibly CT), and for temporal artery biopsy., (© 2024. The Author(s).)

An expert committee recommends defining fast-track clinics (FTC) for the acute diagnostics of giant cell arteritis (GCA) as follows: low-threshold, easy and prompt reachability at least on weekdays, scheduling appointments ideally within 24 h, examination by a specialist with GCA expertise, ≥ 2 experts per FTC, ≥ 50 patients with suspected GCA per year, sonologists with ≥ 300 (≥ 50) temporal and axillary artery examinations, adherence to standard operating procedures, availability of an ≥ 18 (≥ 15) MHz and a lower frequency linear ultrasound probe and collaboration with partners for fast performance of neurological and ophthalmological examinations, magnetic resonance imaging (MRI), positron emission tomography-computed tomography (PET-CT, possibly CT) and for temporal artery biopsy., (© 2024. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Background: Immune-mediated inflammatory diseases (IMID) can lead to a substantial disease burden for those affected, in particular by the concomitant occurrence of other IMIDs or in the presence of comorbidities. The care of patients with IMIDs is complex and involves various medical disciplines., Objective: To describe the burden of disease and the current routine drug treatment of patients with IMID., Material and Methods: The retrospective cross-sectional analysis was based on statutory health insurance claims data from the InGef database. Prevalent patients with psoriasis (Pso), psoriatic arthritis (PsA), spondylarthritis (SpA), rheumatoid arthritis (RA), Crohn's disease (MC), ulcerative colitis (CU), or connective tissue disease were identified among 3,988,695 insured patients in 2018. The concomitant occurrence of different IMIDs and the extent to which patients with IMID are affected by other comorbidities compared to a reference population were investigated. The current routine drug treatment was described based on the use of predefined forms of treatment., Results: In the database 188,440 patients with IMID (4.7%) were identified. Compared to the reference population the prevalence of comorbidities, such as depressive episodes and cardiovascular risk factors was higher in patients with IMID. For MC, CU, RA, and PsA disease-modifying antirheumatic drugs (DMARD) and classical systemic forms of treatment were used most commonly. In Pso, SpA, and connective tissue disease nonsteroidal anti-inflammatory drugs (NSAID) were the most frequently used treatment often in combination with other drugs., Conclusion: A considerable number of patients with IMIDs (16.9-27.5%) suffer from different diseases of the IMID group. They are frequently affected by accompanying illnesses and require interdisciplinary medical treatment., (© 2022. The Author(s).)

This article describes a hantavirus-associated pronounced myositis as a rare differential diagnosis to polymyositis. The literature on the pathogenesis of hantavirus disease discusses less a direct viral cytopathology but more a secondary immune dysregulation with induction of a capillary leak. This article describes for the first time a case of successful treatment of protracted hantavirus myositis using high-dose glucocorticoids and cyclophosphamide, followed by ciclosporin and MTX., (© 2022. The Author(s).)

In the last few years, several studies emerged for the treatment of interstitial lung disease (ILD) associated with systemic sclerosis (SSc). Some of the studied drugs showed efficacy compared to placebo or to a control group. Since early 2020, nintedanib has been approved for the treatment of SSc-ILD. Although several drugs are used to treat SSc-LD, it is now the first approved drug for SSc-ILD. Here, we aim to provide an overview about our current therapy algorithm and strategy as well as our interpretation of study results as an experienced SSc-ILD center.

Background: Raynaud's phenomenon and the frequently ensuing digital ulcerations represent an early and very distressing symptom in patients with systemic sclerosis (scleroderma, SSc) causing significant limitations in the ability to work and quality of life. The use of vasoactive drugs (especially intravenous prostacyclin derivatives) is recommended to reduce the risk of hypoxic tissue damage up to the loss of fingers., Methods: In order to obtain information about the current state of treatment of patients with prostacyclin derivatives in routine clinical life in Germany, a survey was conducted among the centers affiliated to the German Network for Systemic Scleroderma (DNSS). In addition, a separate patient survey was conducted by the schleroderma self-help group (Sklerodermie Selbsthilfe e. V.), which only covered the symptoms Raynaud's syndrome, digital ulcers and the use of intravenous prostacyclin derivatives., Results: Of the 433 patients surveyed 56% stated that they had already been treated with prostacyclin derivatives (iloprost/alprostadil) because of their illness and symptoms. A total of 61% received the treatment for severe Raynaud's phenomenon and 39% for digital ulcerations. Most respondents not only experienced an improvement in Raynaud's phenomenon and digital ulcers but also a significant improvement of limitations in everyday life. They also needed significantly less outside help and absenteeism from work was much lower., Conclusion: Patients consistently reported a positive effect of treatment with prostacyclin derivatives on Raynaud's phenomenon, acral ulcerations, pain and daily restrictions and felt well and safely cared for during inpatient treatment. These positive effects in the patients' perceptions provide crucial information supporting and confirming the current European and international treatment recommendations.

Introduction: DeSScipher is the first European multicentre study on management of systemic sclerosis (SSc), and its observational trial 1 (OT1) evaluated the efficacy of different drugs for digital ulcer (DU) prevention and healing. The aim of this study was to assess current use of vasoactive/vasodilating agents for SSc-related DU in the expert centres by analysing the baseline data of the DeSScipher OT1., Method: Baseline characteristics of patients enrolled in the OT1 and data regarding DU were analysed., Results: The most commonly used drugs, in both patients with and without DU, were calcium channel blockers (CCBs) (71.6%), followed by intravenous iloprost (20.8%), endothelin receptor antagonists (ERAs) (20.4%) and phosphodiesterase 5 (PDE-5) inhibitors (16.5%). Of patients, 32.6% with DU and 12.8% without DU received two drugs (p < 0.001), while 11.5% with DU and 1.9% without DU were treated with a combination of three or more agents (p < 0.001). Sixty-five percent of the patients with recurrent DU were treated with bosentan and/or sildenafil. However, 64 out of 277 patients with current DU (23.1%) and 101 (23.6%) patients with recurrent DU were on CCBs alone., Conclusions: Our study shows that CCBs are still the most commonly used agents for DU management in SSc. The proportion of patients on combination therapy was low, even in patients with recurrent DU: almost one out of four patients with current and recurrent DU was on CCBs alone. Prospective analysis is planned to investigate the efficacy of different drugs/drug combinations on DU healing and prevention. Key Points • The analysis of DeSScipher, the first European multicentre study on management of SSc, has shown that the most commonly used vasoactive/vasodilating drugs for DU were CCBs, followed by intravenous Iloprost, ERAs and PDE-5 inhibitors. • More than half of the patients with recurrent DU received bosentan and/or sildenafil. • However, the proportion of patients on combination therapy of more than one vasoactive/vasodilating drug was low and almost one out of four patients with current and recurrent DU was on CCBs alone.

Background: Systemic sclerosis (SSc) is characterized by heterogeneous clinical symptoms. Peripheral skin fibrosis can be a common symptom. Nevertheless, a variety of diseases with different etiologies are associated with a thickening of the skin and make the initial diagnosis of systemic sclerosis more difficult., Objective: The different disease entities that can lead to dermal fibrosis should be differentiated. An earlier diagnosis of SSc would therefore be facilitated., Methods: A literature search was carried out for clinical pictures that can be associated with skin fibrosis. The clinical picture, the etiology and the treatment of the individual diseases are described., Results: Diseases that can mimic the cutaneous symptoms of SSc include morphea, scleroderma, diabetic cheirarthritis, scleromyxedema, nephrogenic systemic fibrosis and eosinophilic fasciitis. The characteristic pronounced skin involvement, an accompanying Raynaud's phenomenon, capillary microscopy, histopathology and antinuclear antibodies help to enable a differentiation of SSc from its mimics., Conclusion: An early differential diagnostic distinction between SSc and other sclerosing diseases is important due to SSc-associated and potentially life-threatening systemic organ involvement. If a diagnosis of SSc has been made, a critical and organ-specific evaluation with respect to pulmonary, gastrointestinal, renal and cardiac involvement is mandatory and should be repeated at regular intervals.

Background: Digital ulcers (DU) affect up to 60 % of patients with systemic sclerosis (SSc) and have a considerable impact on quality of life and morbidity. It is unclear to what extent authorised medicines are used, and if therapy guidelines are implemented in everyday practice., Method: This retrospective health care study examined current standards of treatment for therapy and prevention of SSc-associated DU in an online survey with 83 physicians. Additionally, data from 161 case studies of SSc patients with DU were analysed, and the effect of DU treatment on the course of the disease determined., Results: For treatment and prevention of active DU, physicians predominantly indicated topical therapies, calcium channel blockers, iloprost and endothelin receptor antagonists. According to the case studies, 90 % of episodes with acute DU were treated with bosentan and iloprost in mono- or combination therapy. Preventive treatment was only administered during 50 % of episodes without DU, even after three or more phases with active DU. For the prevention of new DU, bosentan was used in mono- or combination therapy in 57 % of episodes without DU. Bosentan therapy during prevention shortened the following acute phase by 32 %. Additionally, continuous treatment with bosentan in acute and prevention phases reduced the duration of the following acute phase and increased the time to onset of new DU by 16 %. Moreover, bosentan stabilised the number of new DU., Conclusion: In summary, these data confirm the efficacy of bosentan in preventing new DU when used in DU-free episodes and possibly also in phases of acute DU. Therapy recommendations for the treatment of DU are currently not fully implemented. In the future, even more attention should be paid to DU therapy.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) require a differentiated therapeutic approach depending on the degree of organ dysfunction and disease activity. In organ dysfunction and life-threatening AAV cyclophosphamide and rituximab are recommended for the induction of remission. For remission induction with a lack of organ dysfunction and non-life-threatening AAV, methotrexate or mycophenolate mofetil are recommended. For remission maintenance therapy azathioprine or methotrexate are used. In the case of contraindications, intolerance or previous failure of azathioprine and methotrexate treatment, rituximab, leflunomide or mycophenolate mofetil may be used as alternatives. Maintenance therapy is usually continued for at least 2 years. De-escalation of therapy requires continuous clinical monitoring while the glucocorticoid medication and immunosuppressive therapy is tapered; however, every de-escalation of therapy carries a risk of relapse.

Published data suggest that coexisting interstitial lung disease (ILD) has an impact on mortality in patients with systemic sclerosis (SSc) and pulmonary arterial hypertension (PAH), but there is scarce knowledge if this is reflected by hemodynamics, exercise capacity, autoantibody profile, or pulmonary function. In this partially retrospective study, 27 SSc-PAH patients were compared to 24 SSc-PAH patients with coexisting ILD respecting to survival, pulmonary function, hemodynamics, exercise capacity, and laboratory parameters. Survival was significantly worse in SSc-PAH-ILD patients than in SSc patients with isolated PAH (1, 5, and 10-year survival rates 86, 54, and 54% versus 96, 92, and 82%, p = 0.013). Compared to isolated SSc-PAH patients, patients with SSc-PAH-ILD revealed lower forced expiratory volume after 1 s (FEV1) values at the time of PAH diagnosis as well as 1 and 2 years later (p = 0.002) without significant decrease in the PAH course in both groups. At PAH diagnosis, diffusion capacity for carbon monoxide (DLCO) values were lower in the ILD-PAH group. Coexisting ILD was not associated with lower exercise capacity, different FEV1/forced vital capacity (FVC) ratio, higher WHO functional class, or reduced hemodynamics. Higher levels of antibodies against angiotensin and endothelin receptors predict mortality in all SSc-PAH patients but could not differentiate between PAH patients with and without ILD. Our study confirmed an impact of ILD on mortality in SSc-PAH patients. Pulmonary function parameters can be used to distinguish PAH from PAH-ILD. The higher mortality rate cannot be explained by differences in hemodynamics, exercise capacity, or autoantibody levels. Mechanisms of mortality remain to be studied.

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

In a joint initiative by the boards of the German Society for Rheumatology (DGRh) and the Association of Rheumatology Clinics (VRA) the European "standards of care" for rheumatoid arthritis, recently suggested by the European Musculoskeletal Conditions Surveillance and Information Network (eumusc.net) and supported by the European League Against Rheumatism (EULAR), were translated and annotated. The recommendations include aspects of the management of the disease, actual medical care, and access to information - this includes all types of support people with RA need, and, last but not least communication of the necessary knowledge. Furthermore, health care structures such as the availability of medical staff with relevant expertise are also important.

Functional autoantibodies are an emerging field of research that focuses on the effects of these immunoglobulins when they bind to their target molecules. Accumulating information now exists about the molecular targets and precise binding mechanisms of functional autoantibodies as well as about their downstream effects. These data raise the need to distinguish functional autoantibodies from non-functional autoantibodies with regard to their ability to stimulate or to inhibit their target protein via binding. The presence of autoantibodies has been documented in autoimmune disorders decades ago, but meanwhile, more and more autoantibodies have been identified as functional, acting as pathogenic drivers involved in the induction of organ-specific damage in systemic sclerosis as well as in other autoimmune disorders. These findings offer new opportunities for the development of novel therapeutic strategies.

Large data bases and the projects arising from them have led to a much improved understanding of systemic sclerosis over the last decade. Serology has developed further so that more autoantibodies are available for routine testing. Capillary microscopy has become standard and relevant progress has also been made in therapy. Many diagnostic terms found in medical documentation do not adequately reflect this progress. The nomenclature is inconsistent and, therefore, confusing. The international classification of diseases (ICD) nomenclature is, from our point of view, also in need of improvement. This article aims to reestablish a common German language standard for systemic sclerosis, which reflects current knowledge and is suitable for implementation in the clinical routine.

Background: Regulatory T cells (Treg) are crucial for the maintenance of immunological peripheral tolerance by controlling the activation and expansion of autoreactive cells; therefore, they make a decisive contribution to the prevention and control of autoimmune diseases., Objectives: The aims of this article are to summarize the history and role of Treg in science and medicine, to provide a brief introduction to the development and function, to explain how failures in Treg biology contribute to the development of autoimmune disease, to explain their specific role in particular rheumatic diseases and to provide an introduction to the therapeutic use of Treg in autoimmune diseases., Methods: Relevant original literature and review articles were analyzed and the results are summarized in this article., Results: Disorders in Treg biology can contribute to the development of rheumatic diseases in various ways. In addition, their capability to suppress autoimmunity renders Treg an attractive target for the treatment of rheumatic diseases., Conclusions: The concept of Treg-mediated immunoregulation has evolved into an independent field of research in immunology and medicine. First translational approaches and clinical studies confirmed the therapeutic efficacy of Treg in the treatment of autoimmune syndromes.

Background: Sex-specific differences in the prevalence and severity of immune disorders are well-known phenomena; however, it is only recently that we have begun to understand the possible causes of such differences., Material and Methods: A literature search on this topic was carried out and the results are summarized., Results: In the last few years research has been guided by technological advances in gene sequencing and new insights into the microbiome of the gut, as well as an awareness of sex- and gender-specific risk factors for infections and autoimmunity., Conclusion: The knowledge acquired in recent years will not only improve diagnostics and early identification of these disorders but also influence future research, prevention and therapy of infections and autoimmune diseases.

Vascular complications are common in systemic sclerosis (SSc). Critical limb ischemia leading to gangrene or amputation occurs in more than 10% of these patients and hence is a common emergency. This report highlights the different pathogenetic mechanisms leading to critical ischemic events and provides guidance for the diagnosis and therapy. Apart from SSc-associated vasculopathy and peripheral arterial disease, thromboembolic events and rarely also vasculitis may cause critical limb ischemia. An interdisciplinary approach to the diagnosis and therapy of these lesions is mandatory. Therapy goals are the prevention of further ischemia and, if possible, revascularization as well as optimal pain management.

The clinical manifestations of systemic sclerosis (scleroderma, SSc) are characterized by three prominent features: autoimmunity/inflammation, vascular lesions (vasculopathy) and (organ) fibrosis. Drugs and other therapies are now available for each of these features. However, due to the low prevalence and high variation in clinical signs and symptoms of the disease, there are only few high quality clinical trials. The EULAR Scleroderma Trials and Research Group (EUSTAR, a subgroup of the European League Against Rheumatism, EULAR) has therefore started to assess the therapies available today and make recommendations. The present article discusses treatment options in systemic sclerosis beyond these recommendations. It is to be expected that the establishment of national and international networks for systemic sclerosis research (e.g. the German Network for Systemic Sclerosis, DNSS, and EUSTAR) will raise the standards of evidence-based therapy for systemic sclerosis in the future by analyzing large data sets and performing clinical trials.

Pulmonary arterial hypertension is a rare disease of small pulmonary arteries of unknown origin characterised by endothelial dysfunction and cellular proliferation throughout all vessel layers, resulting in progressively elevated pulmonary arterial resistance with increasing right heart strain and finally right heart failure. The condition may develop in connective tissue diseases with variable frequency leading to a substantial worsening of prognosis. However, the spectrum of therapeutic options has broadened significantly in recent years. Several compounds have gained approval that act mainly as pulmonary vasodilators. Further drugs are under investigation, some of which target pulmonary vascular remodeling. Echocardiography remains the primary examination for disease detection. To classify pulmonary hypertension definite hemodynamic evaluation by means of right heart catheterisation and a thorough differential diagnosis are essential to provide the basis for further treatment. For differential therapy and assessment of follow-up profound knowledge is required, pointing to the need for close cooperation with specialised centres.

The diagnosis and therapy of systemic sclerosis (SSc) is demanding due to its nature as a multisystem disease and its chronic, severe course. To date, there are no generally accepted recommendations for diagnostic work-up either for the time of initial disease diagnosis or for the regular follow-up clinical examinations and diagnostic procedures required. However, due to recent advances, e.g. in the therapy of pulmonary arterial hypertension, regular examinations may contribute to early recognition and treatment of developing organ involvement. This manuscript describes the recommendations for initial and follow-up organ-specific clinical examinations and diagnostic work-up as compiled and carried out by the German Network for Systemic Scleroderma [Deutsche Netzwerk für systemische Sklerodermie (DNSS)].

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.

The SmD1(83-119) peptide is a main target of autoantibodies and T cells in human and murine lupus, but its role in autoimmunity induction remains elusive. Therefore, female Balb/c mice and (NZW x Balb/c)F1 [CWF1] mice with identical MHC haplotype as lupus prone NZB/W mice were immunized with SmD1(83-119). Immunizations of CWF1 mice with SmD1(83-119), but not with the controls (irrelevant peptide, HEL peptide, or saline), induced anti-SmD1(83-119) and anti-dsDNA antibodies and proteinuria not present in Balb/c mice. DsDNA-specific plasma cell induction after SmD1(83-119) immunizations was confirmed by ELISPOT assays showing that the generation of dsDNA-specific antibody forming cells (AFC) was mainly driven by increased T-cell help. T-cell help for the generation of dsDNA-specific AFC was also present in saline-treated CWF1 mice but was controlled on the levels of B cells preventing autoimmunity.

Regulatory T-cells play a key-role in maintaining immunological tolerance to self and may have a key role in the development of new therapeutic principles. We present an overview of the current concepts of regulatory T-cells. A proportion of these cells develops during normal T-cell maturation in the thymus, while other subtypes are induced in the periphery. They have similar functional properties: they proliferate poorly, inhibit the activation of other T-cells and are protective in models of autoimmunity. The underlying mechanisms are not completely understood, probably cell-cell-contact dependent mechanisms and cytokines such as IL-10 and TGF beta play a role. Besides CD25 several other surface markers are known to identify regulatory T-cells, however, none of these markers seems to be exclusive for regulatory T-cells. Finally, we give an outlook over possible therapeutic applications of these cells.

A 57-years old female patient with systemic sclerosis underwent a prolonged intravenous therapy during 21 consecutive days with iloprost, a stable analogue of prostacyclin. Beginning with 0.5 ng/ kg/minute the dose was increased every 2 days up to 2 ng/kg/minute. At the end of follow up, Iloprost was shown to enhance the perfusion of the finger and to improve pulmonary-function tests including the diffusion-capacity. Furthermore, the Erythrocyte Sedimentation Rate and the C-reactive protein decreased. The case report shows the necessity of a controlled study of prolonged iloprost therapy.