Tocilizumab for the Treatment of Familial Mediterranean Fever-A Randomized, Double-Blind, Placebo-Controlled Phase II Study.

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.

Sexual dysfunction and depression in Behçet's disease in comparison to healthy controls.

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.

Erectile function and connective tissue diseases. Prevalence of erectile dysfunction in German men with systemic sclerosis compared to other connective tissue diseases and healthy subjects.

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.

[Behçet's Syndrome].

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.

Shear Wave Elastography for Assessment of Muscular Abnormalities Related to Systemic Sclerosis.

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)].

Intermetatarsal bursitis as first disease manifestation in different rheumatological disorders and related MR-imaging findings.

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.

Chest CT texture analysis for response assessment in systemic sclerosis.

Purpose: To evaluate the role of CT-textural features for monitoring lung involvement in subjects with systemic sclerosis(SSc) undergoing autologous stem cell transplantation(SCT) by comparison with semi-quantitative chest-CT, pulmonary function tests(PFT) and clinical parameters (modified Rodnan skin score[mRSS]).

Methods: In a retrospective single centre analysis, we identified 23 consecutive subjects(11male) with SSc between 07/2005 and 11/2016 that underwent chest CTs before, 6 and 12 months post-SCT. Response to therapy was defined at 6 months after transplantation as stabilisation or improvement in FVC > 10% and DLCO > 10%.

[Varicella gastritis under immunosuppression : Case report of a woman after lung transplantation due to granulomatosis with polyangiitis].

A 35-year-old woman who had previously undergone a lung transplantation presented with severe abdominal pain and vomiting. The gastroscopy showed diffuse ulcerative gastric lesions. Tests for varicella zoster virus and Epstein-Barr virus via polymerase chain reactions (PCR) on endoscopically obtained gastric biopsies were found to be positive and confirmed varicella gastritis.

Monitoring Disease Activity in Patients with Aortitis and Chronic Periaortitis Undergoing Immunosuppressive Therapy by Perfusion CT.

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.

Tocilizumab in Uveitic Macular Edema Refractory to Previous Immunomodulatory Treatment.

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).

Whole Exome Sequencing Identifies Rare Protein-Coding Variants in Behçet's Disease.

Objective: Behçet's disease (BD) is a systemic inflammatory disease with an incompletely understood etiology. Despite the identification of multiple common genetic variants associated with BD, rare genetic variants have been less explored. We undertook this study to investigate the role of rare variants in BD by performing whole exome sequencing in BD patients of European descent.

MRI parametric monitoring of biological therapies in primary large vessel vasculitides: a pilot study.

Objective: To evaluate the development of characteristic MRI changes in patients with primary large-vessel vasculitis (LVV) when treated with biological therapies.

Methods: 12 patients with primary LVV (8 patients with Takayasu arteritis and 4 patients with giant-cell arteritis) received biological therapy with tumour necrosis factor-α blockers (n = 9) or an interleukin-6 inhibitor (n = 3). MRI investigations were performed at baseline (pre-treatment) and follow-up.

Imaging diagnosis in relapsing polychondritis and correlation with clinical and serological data.

Objective: We hypothesize that imaging findings from CT and MRI correlate better with clinical markers for assessment of disease activity in patients with the rare relapsing polychondritis (RPC) than with serological inflammatory markers.

Materials And Methods: Retrospective database search at our institution identified 28 patients (13 females; age 49.0 years±15.

Perfusion-based assessment of disease activity in untreated and treated patients with aortitis and chronic periaortitis: correlation with CT morphological, clinical and serological data.

Objective: To evaluate the role of perfusion-based assessment of inflammatory activity in patients with treated and untreated aortitis and chronic periaortitis as compared with clinical and serological markers.

Methods: 35 patients (20 females; median age 66 years) with (peri) aortitis were retrospectively evaluated. All patients had clinical symptoms prompting at the time of imaging.

Ovarian reserve alterations in premenopausal women with chronic inflammatory rheumatic diseases: impact of rheumatoid arthritis, Behçet's disease and spondyloarthritis on anti-Müllerian hormone levels.

Objective: Recent publications have shown a negative influence of SLE on female ovarian reserve. Other authors have not found a significant impact of Crohn's disease or early RA on anti-Müllerian hormone (AMH) levels. This study aimed to investigate the potential effect of Behçet's disease (BD), RA and SpA on ovarian reserve as reflected by serum AMH levels.

[Relapsing polychondritis. Primary and follow-up diagnostics with (18)F-FDG-PET/CT].

Case Report: This article presents an overview of the typical clinical and imaging features of relapsing polychondritis (RP), a rare autoimmune disease, based on a case report

Conclusion: Although the diagnosis is mainly established clinically, the use of (18)F fluorodeoxyglucose positron emission tomography-computed tomography ((18)F-FDG-PET/CT) has been proven to be a useful diagnostic tool to accurately determine the extent of inflammation throughout the body [corrected]. Furthermore (18)F-FDG-PET/CT provides a high sensitivity for detection of a relapse, especially when clinical and laboratory results are inconclusive. Additionally, (18)F-FDG-PET/CT helps to assess disease activity during the course of therapy.