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Behçet's syndrome (BS, synonym: Behçet's disease, or Adamantiades-Behçet's disease, ABD) is classified as a vasculitis of variable vessel size and can manifest itself in both arterial and venous vessels. Its extensive and at the same time interindividually very different clinical picture is not uncommon a challenge, both with regard to the diagnosis of this rheumatic systemic disease, which is rather rare in our latitudes, and its therapeutic options. In addition to the four cardinal symptoms of recurrent oral aphthae, genital aphthae, skin and eye lesions, the clinical picture offers numerous other manifestations which often require interdisciplinary cooperation. In addition to the above mentioned ocular involvement, which can still lead to blindness if inadequately treated, this is especially true for intestinal and cerebral manifestations as well as for large vessel vasculitis.A final revision of the European League Against Rheumatism recommendations for the management of Behcet's syndrome (EULAR) was made in 2018, and the recommendations are now established internationally as an important treatment guide. Therapy is based on the leading organ involvement. After adalimumab received approval for the treatment of posterior ocular involvement in 2016, another agent, apremilast, became available in 2020. The drug is recommended for the treatment of recurrent oral aphthae in adult Behçet's patients requiring systemic therapy. Nevertheless, there is a further need for new drugs.This article aims to highlight recent findings in the areas of epidemiology, immunopathogenesis & genetics, clinical findings, and therapy, with an emphasis on clinical relevance., Competing Interests: TX: Vortrags- und Beratertätigkeit von AMGEN und Reisekostenerstattung durch Abbvie. JCH: Vortragstätigkeit und Reisekostenerstattung von Abbvie., (Thieme. All rights reserved.)

Behçet's syndrome (BS, synonym: Behçet's disease, or Adamantiades-Behçet's disease, ABD) is classified as a vasculitis of variable vessel size and can manifest itself in both arterial and venous vessels. Its extensive and at the same time interindividually very different clinical picture is not uncommon a challenge, both with regard to the diagnosis of this rheumatic systemic disease, which is rather rare in our latitudes, and its therapeutic options. In addition to the four cardinal symptoms of recurrent oral aphthae, genital aphthae, skin and eye lesions, the clinical picture offers numerous other manifestations which often require interdisciplinary cooperation. In addition to the above mentioned ocular involvement, which can still lead to blindness if inadequately treated, this is especially true for intestinal and cerebral manifestations as well as for large vessel vasculitis.A final revision of the European League Against Rheumatism recommendations for the management of Behcet's syndrome (EULAR) was made in 2018, and the recommendations are now established internationally as an important treatment guide. Therapy is based on the leading organ involvement. After adalimumab received approval for the treatment of posterior ocular involvement in 2016, another agent, apremilast, became available in 2020. The drug is recommended for the treatment of recurrent oral aphthae in adult Behçet's patients requiring systemic therapy. Nevertheless, there is a further need for new drugs.This article aims to highlight recent findings in the areas of epidemiology, immunopathogenesis & genetics, clinical findings, and therapy, with an emphasis on clinical relevance., Competing Interests: TX: Vortrags- und Beratertätigkeit von AMGEN und Reisekostenerstattung durch Abbvie. JCH: Vortragstätigkeit und Reisekostenerstattung von Abbvie., (Thieme. All rights reserved.)

Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht.

Background: The purpose of this trial was to evaluate the effectiveness and safety of the IL-6 receptor antibody Tocilizumab (TCZ) in the treatment of Familial Mediterranean Fever (FMF). Methods: This was a randomized, double-blinded, placebo-controlled phase II trial in adult patients with active FMF and an inadequate response or intolerance to colchicine (crFMF). The physician’s global assessment of disease activity (PGA), based on a five-point scale for six symptoms, was used as a clinical score, which had to be >2 at screening, together with elevated c-reactive protein (CRP) or erythrocyte sedimentation rate (ESR) and serum amyloid A (SAA) levels, to be eligible for inclusion. Patients were randomized 1:1 to either receive monthly TCZ or a placebo over a period of 24 weeks. The primary endpoint was the number of patients achieving an adequate response to treatment at week 16, defined as a PGA of ≤2 and normalized ESR or CRP and normalized SAA. Results: We randomized 25 patients with a median age of 31 years. At week 16, an adequate treatment response was achieved by two patients in the TCZ and none of the patients in the placebo arm (p = 0.089). SAA levels normalized with TCZ, but not with the placebo (p = 0.015). Conclusion: In this first randomized, placebo-controlled study in patients with active crFMF, more patients in the TCZ arm experienced a response to treatment in comparison to those receiving the placebo. As the prevention of amyloidosis is a major treatment goal in FMF, the normalization of SAA in TCZ-treated patients is essential. These findings have to be confirmed in a larger trial.

Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht.

Behçet's disease (BD) can affect the genital system and is more common in Middle Eastern countries and Asia but also occurs in Caucasian people. Aim of this study was to evaluate the prevalence of sexual dysfunction (SD) and depression in patients with BD compared to a healthy control group (HCG). In addition, differences with regard to depression and patients' origin were evaluated. This prospective, monocentric study included 106 consecutive patients from our specialized BD outpatient clinic. Patients were asked to fill out the paper based standardized and validated questionnaires International Index of Erectile Function (IIEF), the Female Sexual Function Index (FSFI) and the Beck Depression Inventory (BDI). In addition, 206 healthy controls were asked to fill out the questionnaires. 106 patients with BD were evaluated and 206 participants in the HCG. The mean age in BD group was 40.5 years as compared to 44.4 years in the HCG. Half of the patients had Middle Eastern and half Caucasian origin. SD was found in 24.5% of all subjects. Only 6.9% of male patients showed signs of SD, while half of the women's group was suffering from SD. The prevalence for SD was significantly higher in women with Middle Eastern ethnic origin compared to women with Caucasian origin (75 vs. 33.3%, p = 0.024). Erectile Dysfunction occurred in 55% of all male patients which was not statistical different from the HCG. Genital ulcers affected 73.6% of all patients. Depression was found in 36.7% of all subjects as compared to 6.25% in the HCG (p < 0.001). Both, SD and depression correlated positively in males (p = 0.017) and females (p = 0.013). SD and depression are very common problems in BD and should be addressed by the treating physician. Both manifestations are intensifying each other. Depression especially is more prevalent compared to the healthy population., (© 2021. The Author(s).)

Rationale and Objectives: To assess muscular abnormalities related to systemic sclerosis (SSc) using shear wave elastography and correlate results with those of clinical tests., Materials and Methods: We evaluated 55 patients (mean age: 50.4 years; range: 18-88; 34 female) with SSc before treatment and choose muscle groups based on cutaneous involvement and functional impairment [forearms (9); thighs (41); thenar/hypothenar (5)]. We performed shear wave elastography in two orientations to access heterogeneity using virtual touch IQ and mean shear wave velocity values (SWV) and measured skin and fascia thickness. We compared SWVs to the modified Rodnan skin score (mRSS). Twenty-two healthy controls (mean age: 52.0; range: 19-88; 7 female) underwent the same protocol., Results: SWV pattern analysis (homogeneous vs heterogeneous) detected SSc with sensitivity/specificity/negative predictive value/positive predictive value0.79/0.81/0.91/0.62 in transverse and 0.75/0.18/0.91/0.56 in longitudinal orientation. In patients we found poor correlation between SWVs and mRSS but a cutoff of mRSSE = 20 separated them significantly (p < 0.01). Skin and muscle fascia were significantly thicker in patients vs. controls (p < 0.001)., Conclusion: SSc involves more than increased skin and fascial thickness which is not fully represented by mRSS. Elasticity differs in muscles with and without SSc. The former shows higher SWV and increased heterogeneity in transversal planes of muscular fibers., Competing Interests: Funding Jan Fritz received institutional research support from Siemens Healthcare USA, DePuy, Zimmer. Micorsoft, and BTG International; is a scientific advisor of Siemens Healthcare USA, Alexion Pharmaceuticals, and BTG International; received speaker's honorarium from Siemens Healthcare USA' and has shared patents with Siemens Healthcare and Johns Hopkins University. Horger Marius received institutional research support from Siemens Healthineers Germany; he is a scientific advisor of Siemens Healthineers Germany and received speaker's honorarium from Siemens Healthineers Germany., (Copyright © 2020 The Association of University Radiologists. Published by Elsevier Inc. All rights reserved.)

Objectives: Women are more frequently affected by connective tissue diseases like systemic sclerosis (SSc). Therefore, few studies exist on male-specific complaints. This study aimed to investigate the prevalence and associated factors of erectile dysfunction (ED) in SSc compared with other connective tissue diseases (CTD) and healthy controls., Methods: 64 patients were analysed and compared with 123 age-matched HC. The 15-item International Index for Erectile Function (IIEF) questionnaire was used to assess sexual function. The prevalence of depression was quantified by using the validated Beck Depression Inventory (BDI)., Results: Mean age was 52.3 years (SD 10.75) for SSc, 52.9 years (SD 11.01) for patients with other CTD and 52.6 (SD 12.37) for HC. Mean IIEF-15 score was 13.6 for SSc, 11.7 for other CTD and 23.6 for HC. ED was significantly more frequent in patients with SSc (55.0%) and other CTD (54.4%) than in HC (12.7%) (p<0.001) and correlated with diseases severity. The mean BDI score was 10.8 for SSc/CTD and 5.4 for HC (p<0.001). With 36.6%, SSc patients suffered more often from a depression than patients with other CTD (17.4%) and HC (6.3%). We found a significant correlation between the IIEF-15 and depression classified by BDI (r= -0.527; p<0.001)., Conclusions: This is the first study to show increased prevalence of ED, especially severe ED, in men with SSc compared to other CTD and age-matched HC. Physicians should be aware of this influence on sexual health and its correlation to depression and disease severity.

Behçet's syndrome (BS) is classified as a variable vessel vasculitis. The clinical picture is very diverse and usually requires interdisciplinary collaboration. Pathogenetically, BS seems to take a middle position between a polygenic autoinflammatory disease and an autoimmune disease. New EULAR recommendations were issued in 2018. The therapy depends on which organs are most affected. Since 2016, adalimumab has been approved for the treatment of posterior ocular involvement. Infliximab, interferon a2a, interleukin-1 antagonists and apremilast may be alternative therapies., Competing Interests: Th. Xenitidis:1) Abbvie: Vortragstätigkeit, Unterstützung bezüglich Kongressteilnahme2) Celgene: Unterstützung bezüglich KongressteilnahmeJ. C. Henes:1) Celgene: Unterstützung bezüglich Kongressteilnahme, (© Georg Thieme Verlag KG Stuttgart · New York.)

Metatarsalgia defined as pain at the plantar aspect of the forefoot. Intermetatarsal bursitis is considered one potential soft-tissue cause of metatarsalgia that is presumably under-estimated, under-investigated, and, consequently, often misdiagnosed. To assess the role of MRI in the elucidation of the cause of metatarsalgia in patients with different autoimmune disorders presenting primarily with this symptom and to present the accompanying clinical and radiological findings of intermetatarsal bursitis. Retrospective evaluation of the medical records of patients with different rheumatological conditions claiming primarily of pedal pains suggests metatarsalgia and who underwent, therefore, all magnetic resonance imaging between March 2010 and April 2018. Of them, six patients fulfilled these criteria and were diagnosed subsequently with intermetatarsal bursitis. Several underlying autoimmune conditions were diagnosed. All patients were clinically assessed by the squeeze test and radiologically investigated with MRI; three patients underwent additional sonography. All patients presented intermetatarsal bursitis as first disease manifestation. The number of involved bursae ranged from one to three on one side. The main MR findings were distension of the intermetatarsal bursa with increased signal intensity on T2-weighted and post-contrast fat saturation T1-weighted images. Most frequent locations were the second and third intermetatarsal spaces. The size of the intermetatarsal bursitis and its plantar extension were correlated in all patients. Intermetatarsal bursitis can potentially be the first manifestation of different rheumatological diseases. Awareness of this potential association as well as cognizance of its imaging findings can help for making a more accurate and prompt earlier diagnosis of the underlying disease changing also the therapeutic approach.

Competing Interests: Disclosure The authors report no conflicts of interest in this work.

Purpose: To analyze the efficacy of tocilizumab in uveitic macular edema (ME) resistant to various immunomodulatory drugs., Methods: Patients received tocilizumab every 4 weeks intravenously. Central foveal thickness (CFT) was assessed by optical coherence tomography (OCT)., Results: Five patients (8 eyes) who were ineffectively pretreated with systemic prednisolone, at least one immunosuppressive drug, and at least one biologic drug for uveitic macular edema were included in the study. At 3 months, a response of ME (≥25% reduction in CFT) was observed in 6 eyes (75.0%) of 5 patients. During follow-up, complete resolution of ME was achieved in 5 eyes (62.5%) of 4 patients. Improvement of BCVA was observed in 3 eyes of 3 patients, and stabilization in 3 eyes of 3 patients. Tocilizumab was well tolerated, and no severe side effects occurred., Conclusions: Treatment with tocilizumab can be considered in chronic uveitic macular edema even if previous immunomodulatory therapy has failed.

Rationale and Objectives: To evaluate the role of perfusion CT for monitoring inflammatory activity in patients with aortitis and chronic periaortitis undergoing immunosuppressive therapy., Materials and Methods: Seventeen symptomatic patients (median age 68.5 years) who underwent perfusion-based computed tomography (CT) monitoring after diagnostic contrast-enhanced CT were retrospectively included in this study. Blood flow (BF), blood volume (BV), volume transfer constant (k-trans), time to peak, and mean transit time were determined by setting circular regions of interest in prominently thickened parts of the vessel wall or perfused surrounding tissue at sites where the perfusion CT color maps showed a maximum BF value. Differences in CT perfusion and, morphological parameters, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) were tested for significance during therapy., Results: In all patients BF and BV dropped at second perfusion CT (P < 0.05). In aortitis patients, CRP dropped from 3.86 ± 5.31 mg/dL to 0.9 ± 1.37 mg/dL and in periaortitis patients from 1.78 ± 2.25 mg/dL to 0.79 ± 1.55 mg/dL, whereas ESR dropped from 45.71 ± 37.59 seconds to 8.57 ± 3.1 seconds and 36.78 ± 34.67 seconds to 17.22 ± 21.82 seconds in aortitis and in periaortitis, respectively., Conclusions: The course of perfusion CT parameters in aortitis and chronic periaortitis undergoing immunosuppressive therapy dropped at different extent after therapy., (Copyright © 2017 The Association of University Radiologists. Published by Elsevier Inc. All rights reserved.)