[Polymyalgia rheumatica-A challenge in geriatrics : Interdisciplinary presentation of diagnostics and treatment]. / Polymyalgia rheumatica ­ eine Herausforderung in der Altersmedizin : Diagnostik und Therapie interdisziplinär dargestellt.

Polymyalgia rheumatica is the second most frequent inflammatory rheumatic disease in people aged over 50 years, after rheumatoid arthritis. It is characterized by pain and morning stiffness in the region of the shoulders, hip girdle and neck. It can be associated with giant cell arteritis (CGA). Treatment with glucocorticoids is indispensable. The duration of treatment varies and often exceeds 1 year. The additive administration of methotrexate is an option for saving glucocorticoids. The biologicals tocilizumab or secukinumab are very promising alternatives. The course of treatment should be closely monitored for inflammation parameters, glucocorticoid side effects, pain, visual acuity, depression, activities of daily living and especially related to functions of the upper extremities. The geriatric assessment plays an important role in the management of this condition.

[Castleman's disease in the rheumatological practice]. / Morbus Castleman in der rheumatologischen Praxis.

The term Castleman's disease encompasses a group of rare lymphoproliferative diseases that show histopathological similarities in lymph node biopsy. Diagnostic criteria and a specific ICD-10 code have been available for a few years. Case studies listed at the beginning illustrate that close cooperation between clinicians and pathologists is required to enable a reliable diagnosis. For an optimal histopathological assessment, the pathologist is also dependent on the removal of a complete lymph node. Before distinguishing a potentially fatal multicentric idiopathic Castleman's disease from the resectable unicentric form, which is important in terms of prognosis and treatment, early diagnosis presupposes that Castleman's disease is considered in the differential diagnosis. Various immune phenomena and overlaps with autoimmune diseases can increase the probability of misdiagnosis or undetected cases in the clinical routine of rheumatologists. The intention of the present overview is therefore to point out the similarities with autoimmune diseases that are relevant for differential diagnoses and to point out situations that justify a review of the previous diagnosis.

[Polymyalgia rheumatica-A challenge in geriatrics : Interdisciplinary presentation of diagnostics and treatment]. / Polymyalgia rheumatica ­ eine Herausforderung in der Altersmedizin : Diagnostik und Therapie interdisziplinär dargestellt.

Polymyalgia rheumatica is the second most frequent inflammatory rheumatic disease in people aged over 50 years, after rheumatoid arthritis. It is characterized by pain and morning stiffness in the region of the shoulders, hip girdle and neck. It can be associated with giant cell arteritis (CGA). Treatment with glucocorticoids is indispensable. The duration of treatment varies and often exceeds 1 year. The additive administration of methotrexate is an option for saving glucocorticoids. The biologicals tocilizumab or secukinumab are very promising alternatives. The course of treatment should be closely monitored for inflammation parameters, glucocorticoid side effects, pain, visual acuity, depression, activities of daily living and especially related to functions of the upper extremities. The geriatric assessment plays an important role in the management of this condition.

[VEXAS syndrome]. / VEXAS-Syndrom.

The VEXAS syndrome is a recently identified autoinflammatory systemic disease. The acronym VEXAS stands for Vacuoles, E1 enzyme, X­linked, Autoinflammatory, Somatic. The disease is due to an acquired somatic mutation of the UBA1 gene, which encodes for the E­1 enzyme, which in turn is responsible for the ubiquitination of proteins. Due to its location on the X chromosome, the disease predominantly affects men (in the second half of life). The patients present with a plethora of inflammatory clinical symptoms, often with overlap of hematologic, dermatologic, and rheumatologic syndromes. In particular, the presence of cytoplasmic vacuoles in the bone marrow is characteristic. In this article we report the clinical case of a VEXAS patient and give an overview of the pathophysiology, clinical symptoms and diagnostics of the disease.

[Behçet's syndrome : Current diagnostics and treatment]. / Behçet-Syndrom : Aktuelle Diagnostik und Therapie.

Behçet's syndrome (BS) in our latitude is a rare vasculitis of various vessels. It mainly consists of oral and genital aphthous ulcers, skin lesions, oligoarthritis and ocular manifestations, mostly a posterior uveitis with occlusive retinal vasculitis. This review article summarizes the diagnostic and classification criteria as well as possible differential diagnoses and current treatment recommendations.

[Autologous hematopoietic stem cell transplantation for systemic sclerosis : Position statement of the stem cell therapy working party of the German Society of Rheumatology]. / Autologe hämatopoetische Stammzelltransplantation bei systemischer Sklerose : Positionspapier des Arbeitskreises Stammzelltherapie der Deutschen Gesellschaft für Rheumatologie.

There have been three randomized controlled trials on autologous hematopoietic stem cell transplantation (AHSCT) in systemic sclerosis (SSc) that demonstrated significant superiority with respect to survival, improvement of cutaneous fibrosis, lung function and quality of life compared to standard treatment; however, these advantages must be carefully weighed against the transplantation-related risks. For this reason, an expert group from the stem cell therapy working party of the German Society for Rheumatology (DGRh) has now developed recommendations for the use of AHSCT in SSc. Based on the high-quality evidence, AHSCT is considered as the standard option for the treatment of selected SSc patients. Potential candidates for AHSCT are those with early, rapidly progressive, diffuse cutaneous SSc with visceral manifestations who have not yet developed severe damage to internal organs. A close cooperation between rheumatologists and transplantation centers is crucial for optimizing patient selection and treatment outcomes.

[Reality of inpatient vasoactive treatment with prostacyclin derivatives in patients with acral circulation disorders due to systemic sclerosis in Germany]. / Versorgungsrealität der stationären vasoaktiven Therapie mit Prostazyklinderivaten bei Patienten mit akralen Durchblutungsstörungen bei systemischer Sklerose in Deutschland.

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.

[Under the skin-A special manifestation of a systemic granulomatous disease]. / Unter die Haut ­ Eine besondere Manifestation einer granulomatösen Systemerkrankung.

Based on the case of a 33-year-old female patient with Löfgren's syndrome and skin changes in the region of a tattoo, this article describes tattoo sarcoidosis as a special manifestation of cutaneous sarcoidosis. Papulonodular changes that are strictly confined to a tattoo are highly suspicious for tattoo sarcoidosis. Interestingly, tattoo sarcoidosis is commonly associated with the involvement of other organs.

[Rare erosive arthritis and dermatitis syndrome in Whipple's disease]. / Seltenes erosives Arthritis- und Dermatitissyndrom bei Morbus Whipple.

Whipple's disease is a rare infectious disease, which can affect various organ systems. Arthritis is a common symptom and therefore the infection is often misdiagnosed as seronegative rheumatoid arthritis. In rare cases an infection with Tropheryma whipplei can also cause skin lesions, such as subcutaneous nodules, erythema nodosum or vasculitis. This article reports the case of a 77-year-old female patient with erosive joint changes, persistently elevated serological inflammation markers and recurrent ulcerative lesions of the lower extremities, which were initially misdiagnosed as rheumatoid vasculitis. In cases of a clinically suspected infection with Tropheryma whipplei an early biopsy of the affected organ system is essential for the diagnosis.

[Muscular polyarteritis nodosa-a case-based review]. / Muskuläre Polyarteriitis nodosa ­ eine fallbasierte Übersichtsarbeit.

BACKGROUND: Myalgia is a common but unspecific set of symptoms that may be caused by orthopedic, neurological and internal medical conditions, often resulting in a diagnostic challenge. Muscular polyarteritis nodosa (PAN) is a rare differential diagnosis of myalgia with elevated serological inflammatory markers. OBJECTIVE: Based on three clinical cases and the literature this review describes the essential clinical and diagnostic features of muscular PAN. RESULTS: Muscular PAN typically presents with immobilizing myalgia confined to the lower limbs and elevated serological inflammatory markers but often normal creatine kinase (CK) levels. Contrast-enhanced magnetic resonance imaging of the affected muscles, which can often mimic myositis, and muscle biopsy provide the relevant histological findings that lead to the diagnosis of a vasculitis. CONCLUSION: With respect to own experiences and the reviewed literature, muscular PAN should be considered as a possible diagnosis in cases of myalgia with elevated inflammatory markers but normal CK levels and a lack of further symptoms typical for vasculitis.

[Vasculitis mimics]. / Vaskulitis-Mimics.

Vasculitis mimics need to be differentiated from primary and secondary vasculitides as described in the Chapel Hill nomenclature. The clinical symptomatology resembles that of vasculitis of small and medium, rarely also large vessels and hence imitates the classical vasculitic disorders. Pathogenetically, the causes are partly genetic mutations, embolization syndromes, infections and substance abuse. Also, B­cell lymphomas can mimic vasculitis. The present manuscript summarizes the vasculitis mimics.

[Safety and efficacy of off-label use of biologic therapies in patients with inflammatory rheumatic diseases refractory to standard of care therapy : Data from a nationwide German registry (GRAID2)]. / Sicherheit und Wirksamkeitshinweise zum Off-label-Einsatz von Biologikatherapien nach Versagen konventioneller Therapien bei Patienten mit entzündlich rheumatischen Erkrankungen : Ergebnisse eines nationalen Registers (GRAID2).

BACKGROUND: The German Registry of Autoimmune Diseases 2 (GRAID2) is a retrospective, non-interventional, multicenter registry study collecting data from patients with inflammatory, mainly rheumatic diseases refractory to standard of care therapy and treated with an off-label biologic therapy. The retrospective documentation comprised case history, diagnosis, course of disease (including safety and global efficacy). The objective was to evaluate the global clinical outcome and safety of off-label biologic therapy in clinical practice. RESULTS: Data from 311 patients with an overall observation period of 338.5 patient-years were collected. The mean patients age was 47.8 years with 56.9% females. The most frequently documented diagnoses comprised rejection prophylaxis/therapy after renal transplantation (NTX, 18.3%), ANCA-vasculitides (17.4%), systemic lupus erythematosus (SLE, 10.3%), autoinflammatory fever syndromes (8.4%), autoimmune myositis (7.4%) and pemphigus (5.8%). Documented biologic therapies included rituximab (RTX, 70.1%), tocilizumab (TCZ, 9.3%), infliximab (IFX, 7.1%), anakinra (ANK, 5.5%), adalimumab (ADA, 3.5%), etanercept (ETA, 2.3%) and certolizumab (CTZ, 0.6%). After initiation of off-label biologic treatment, tolerability was assessed by the physicians as "very good"/"good" in 95.5%. Altogether, 275 adverse events were documented and of these, 104 were classified as serious adverse events and occurred in 62 patients. In 19 of these patients severe infections (30.6%) were documented, resulting in a rate of 5.6 severe infections per 100 patient years. A total of six deaths were documented, while five of these cases were rated as not related to the biologics treatment. Notably, the use of RTX in patients with small vessel vasculitides and of TCZ in patients with large vessel vasculitides prior to their approval support their relevance in clinical management of patients with severe diseases. CONCLUSION: The results of this registry together with data of GRAID1 provide evidence that use of off-label biologic therapies in patients with inflammatory rheumatic diseases refractory to conventional treatment did not result in any new safety signal already known for these compounds or subsequently shown by clinical trials in certain entities.

[Ocular involvement in rheumatoid arthritis, connective tissue diseases and vasculitis]. / Augenbeteiligung bei rheumatoider Arthritis, Kollagenosen und Vaskulitiden.

There are many interfaces between ophthalmologists and rheumatologists. On the one hand ophthalmologists face the question if an inflammation of the eye is caused by systemic inflammatory rheumatic diseases and on the other hand rheumatologists have to consider that ocular manifestations are relatively common in some inflammatory rheumatic diseases. Furthermore, these ocular manifestations may influence therapeutic decisions of the rheumatologist. This article summarizes which ocular inflammations can be associated with rheumatoid arthritis, connective tissue diseases and vasculitides. The description of acute anterior uveitis in spondyloarthritis and in juvenile idiopathic arthritis is omitted in this article but will be dealt with elsewhere in this issue.

[Autologous stem cell transplantation in systemic sclerosis]. / Autologe Stammzelltransplantation bei systemischer Sklerose.

Autologous hematopoietic stem cell transplantation (HSCT) is a very effective treatment option for patients with severe systemic sclerosis (SSc). In addition to various case series two randomized controlled trials could prove its superiority over intense cyclophosphamide pulse therapy. Nevertheless, HSCT is associated with a treatment-related mortality of approximately 10 %; therefore, further studies should be carried out to reduce the toxicity of HSCT by adaptation of the therapy regimen and the option of HSCT should be made available earlier to patients with a high risk of mortality. The mechanism of action of HSCT is still poorly understood. While profibrotic cytokines or even autoantibodies hardly appear to be influenced by the treatment, alterations to regulatory T­cells may play a role. Further improvement of transplantation regimens as well as a better understanding of the underlying pathogenetic principles and mechanisms of action should be the aim of further studies on HSCT.