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Introduction
Autoinflammation, AoSD, FMF, Gout
Transition
Additional affiliations
August 1998 - July 2020
August 1998 - present
Publications
Publications (356)
Background/Objectives: Adult-onset Still’s disease (AOSD) is an autoinflammatory disorder that can be challenging to diagnose and manage. The aim of this study was to analyze retrospective data to provide insights into the clinical presentation, disease activity, and treatment patterns and outcomes of AOSD during routine clinical care prior to the...
Interim analysis of the long-term safety and effectiveness of canakinumab, at a patient level, in the mevalonate kinase deficiency/hyperimmunoglobulin-D syndrome (MKD/HIDS) cohort of the RELIANCE registry.
From June 2018, the RELIANCE registry enrolled paediatric (aged ≥ 2 years) and adult patients (aged ≥ 18 years) with MKD/HIDS who were receiving...
Background/Objectives: Adult-onset Still’s disease (AOSD) is an autoinflammatory disorder that can be challenging to diagnose and manage. The goal of this study was to analyze retrospective data to provide insights into the clinical presentation, disease activity, and treatment outcomes of AOSD during routine clinical care. Methods: This retrospect...
Objective
Adult‐onset Still disease (AOSD) is a systemic autoinflammatory disorder (AID) of unknown etiology. Genetic studies have been limited. Here, we conducted detailed genetic and inflammatory biomarker analysis of a large cohort with AOSD to investigate the underlying pathology and identify novel targets for potential treatment.
Methods
We i...
Background: As recent data suggest an involvement of GPCR-fAAb in PCS pathogenesis, neutralisation of such GPCR-fAAbs by BC007 could improve PCS symptoms. The aim of the reCOVer trial was to investigate safety, tolerability and clinical effects of BC007 on fatigue, its severity and quality of life in PCS patients.
Methods: reCOVer is a prospective,...
Adult-onset Still’s disease (AOSD) is a rare autoinflammatory disease. Since it can lead to variable organ involvement, including life-threatening complications, and due to newly available therapeutic approaches, the German Society for Rheumatology and Clinical Immunology (Deutsche Gesellschaft für Rheumatologie und klinische Immunologie; DGRh) iss...
Background
Adult-onset Still’s disease (AOSD) is a systemic autoinflammatory condition of unknown aetiology. AOSD is presumed to have a polygenic basis but there is genetic and clinical overlap with monogenic autoinflammatory disorders[1]. Genetic studies thus far have been limited and although a role for NLRP3 inflammasome in disease pathogenesis...
Background:
Autoinflammatory diseases (AIDs) are rare, mostly genetic diseases that affect the innate immune system and are associated with inflammatory symptoms. Both paediatric and adult patients face daily challenges related to their disease, diagnosis and subsequent treatment. For this reason, a survey was developed in collaboration between th...
Objective
Interim analysis of the RELIANCE registry, an on-going, non-interventional, open-label, multicentre, prospective study evaluating the long-term safety, dosing regimens and effectiveness of canakinumab in patients with cryopyrin-associated periodic syndromes (CAPS), familial Mediterranean fever (FMF), tumour-necrosis factor receptor-associ...
Objective
To formulate evidence-based recommendations and overarching principles on the use of imaging in the clinical management of crystal-induced arthropathies (CiAs).
Methods
An international task force of 25 rheumatologists, radiologists, methodologists, healthcare professionals and patient research partners from 11 countries was formed accor...
These authors contributed equally to this work. Abstract: Patients with systemic autoinflammatory diseases (sAIDs) are a section of the population at high risk of severe COVID outcomes, but evidence on the efficacy of SARS-CoV-2 vaccination in this group of patients is scarce. To investigate the efficacy of SARS-CoV-2 vaccination in patients with s...
Patients with sAID are a population at high risk of severe COVID outcomes but evidence on the efficacy of SARS-CoV-2 vaccination in this group of patients is scarce. To investigate the efficacy of SARS-CoV-2 vaccination in patients with systemic autoinflammatory diseases (sAID) receiving interleukin-1 inhibition is important. Vaccination responses...
Psoriasis is a common, debilitating immune-mediated skin disease. Genetic studies have identified biological mechanisms of psoriasis risk, including those targeted by effective therapies. However, the genetic liability to psoriasis is not fully explained by variation at robustly identified risk loci. To move towards a saturation map of psoriasis su...
To investigate clinical symptoms and genetic variants in patients from the German anti-IL-1 registry for autoinflammatory orphan diseases (GARROD) between 2013 and 2022. Multicentre, retrospective analysis of demographic, clinical and genetic data of patients with autoinflammatory diseases (AID) who received anti-IL-1 targeted therapy. The cohort c...
Citation: Muth, K.N.; Rech, J.; Losch, F.O.; Hoerning, A. Reversing the Inflammatory Process-25 Years of Tumor Necrosis Factor-α Inhibitors. Abstract: Immune-mediated inflammatory diseases, such as rheumatoid arthritis, psoriatic arthritis, peripheral and/or axial spondyloarthritis, Crohn's disease, and ulcerative colitis, are characterized by mole...
Objective:
To provide real-world evidence on patient-individual tapering patterns of disease-modifying antirheumatic drugs (DMARDs) in rheumatoid arthritis (RA) patients in daily clinical practice.
Methods:
Data obtained through a controlled prospective cohort study in Germany conducted from July 2018 to March 2021 were analyzed. Participants co...
Background
CAPS (Cryopyrin-associated periodic fever syndromes) are monogenic autoinflammatory diseases causing great burden to patients due to severe systemic and organ inflammation caused by increased production of interleukin-1β (IL-1β). In clinical trials as well as in real-life, the monoclonal antibody canakinumab (CAN) effectively inhibits IL...
Background
The NLRP3 inflammasome is a critical multi-molecular platform involved in mediating innate immune responses, and could play a role in Adult onset Still’s disease (AOSD). Upon NLRP3 inflammasome activation, a large protein complex assembles, resulting in the re-localisation of apoptosis-associated speck-like protein containing a caspase r...
Background
The multifaceted clinical presentation in crystal-induced arthropathies (CiA) poses challenges to imaging.
Objectives
To formulate evidence-based recommendations on the use of imaging in the diagnosis and management of CiA.
Methods
Following EULAR standard operating procedures a task force of 25 stakeholders from 11 countries was creat...
Background
Hyper-IgD Syndrome/Mevalonate Kinase Deficiency (HIDS/MKD) is a rare autoinflammatory disease caused by a defect in the gene encoding mevalonate kinase. Treatment with the interleukin-1β inhibitor canakinumab (CAN, approved since 2017 for HIDS/MKD) resulted in rapid remission in most patients, both in controlled trials and in clinical pr...
Background
Autoinflammatory periodic diseases (AID) can be treated with the interleukin-1β inhibitor canakinumab (CAN). CAN has been shown to be safe and effective in controlled trials and routine clinical practice.
Objectives
In this study general safety parameters in patients with canakinumab in cryopyrin-associated periodic syndromes (CAPS), fa...
Background
Familial Mediterranean Fever (FMF) is characterized by recurrent attacks of fever, serositis, and elevated laboratory parameters (CRP, SAA). According to EULAR recommendations, treatment goals are the control of acute attacks and subclinical inflammation as well as the improvement of patient´s quality of life. In a phase 3 pivotal study...
Several studies have shown that tapering or stopping disease-modifying anti-rheumatic drugs (DMARDs) in rheumatoid arthritis (RA) patients in sustained remission is feasible. However, tapering/stopping bears the risk of decline in physical function as some patients may relapse and face increased disease activity. Here, we analyzed the impact of tap...
Background The treatment of giant cell arteritis with glucocorticoid-sparing agents is an unmet medical need. We evaluated the efficacy and safety of secukinumab, an anti-interleukin-17A monoclonal antibody, in patients with giant cell arteritis.
Several studies have shown that tapering or stopping disease-modifying anti-rheumatic drugs (DMARDs) in rheumatoid arthritis (RA) patients in sustained remission is feasible. However, tapering/stopping bears the risk of decline in physical function as some patients may relapse and face increased disease activity. Here we analysed the impact of tape...
Dear Editor, We report on an 81-year-old male Caucasian patient, who initially presented with oral ulcerations and disseminated erythematous papules on the upper back, chest, arms and legs with histopathological evidence of lymphocytic infiltration and small vessel vasculitis matching subacute lupus erythematous (Fig. 1A and B). His skin manifestat...
Objective MicroRNAs (miRNAs) are small non-coding RNAs that control gene expression. Specific intra-and extracellular miRNA signatures have been identified in various diseases. Whether certain miRNA signatures are associated with psoriasis (PsO) and psoriatic arthritis (PsA) is currently unknown. We aimed to search for circulating miRNA signatures...
Objective:
MicroRNAs (miRNAs) are small non-coding RNAs that control gene expression. Specific intra- and extracellular miRNA signatures have been identified in various diseases. Whether certain miRNA signatures are associated with psoriasis (PsO) and psoriatic arthritis (PsA) is currently unknown. We aimed to search for circulating miRNA signatur...
Background
The role of methotrexate in combination with biological agents in patients with psoriatic arthritis remains unclear. The MUST phase 3b trial aimed to compare the efficacy of ustekinumab plus placebo with ustekinumab plus methotrexate in patients with active psoriatic arthritis.
Methods
In this investigator-initiated, randomised, multicen...
Dear Editor, Adult-onset Still’s disease (AOSD) is an autoinflammatory disease with to-date unexplained aetiology. Secondary hemophagocytic lymphohistiocytosis (HLH), here referred to as macrophage activation syndrome HLH (MAS-HLH) is the most devastating complication of AOSD [1]. Signs of evolution include antibiotic-resistant fever, hepatosplenom...
Introduction: Methotrexate (MTX) is used as first-line DMARD-therapy in active psoriatic arthritis (PsA) according to local guidelines, but its value for a combination therapy with bDMARDs has remained unclear to date. Here we report the results of an investigator-initiated, randomized, placebo-controlled trial (IIT) in active PsA to clarify whethe...
Objective
We aim to provide real-world evidence on the effectiveness of patient-individual tapering of DMARDs for patients with rheumatoid arthritis (RA) in daily clinical practice using medical records and claims data.
Methods
We utilize data obtained through a controlled prospective cohort study in Germany conducted from July 2018 to March 2021....
Background
Familial Mediterranean Fever (FMF) is a chronic disease characterized by recurrent attacks of fever as well as serositis and bears the risk of serious complications (e. g. amyloidosis). Treatment of FMF according to EULAR aims to control acute attacks and subclinical inflammation as well as to improve patient´s quality of life¹. Clinical...
Background
Little is known about glucocorticoid-sparing agents in giant cell arteritis (GCA) except for IL-6 inhibition. Secukinumab (SEC) has shown significant improvements in the signs and symptoms of IL-17A driven medical conditions such as plaque psoriasis, psoriatic arthritis, and axial spondyloarthritis. 1,2 It has a favourable long-term safe...
Background
The use of bDMARDs treatments in patients with psoriatic arthritis (PsA) usually requires treatment failure or intolerance of csDMARD/MTX before initiation. The value of MTX in combination with bDMARDs is still unclear. We designed an investigator-initiated, randomized, placebo-controlled trial (IIT) in active PsA to examine if outcomes...
Background
Autoinflammatory diseases (AID) are characterized by severe systemic and organ inflammation as well as high burden of disease for patients and their families. Treatment with the monoclonal antibody canakinumab (CAN), an interleukin-1β inhibitor, has been proven to be safe and effective in clinical trials and real-life.
Objectives
The pr...
Background
The development of RA is described by a preclinical phase of autoimmunity, that precedes clinical disease. This autoimmune phase is characterized by the presence of anti-modified protein antibodies that recognize citrullinated proteins (ACPA). A subset of individuals with ACPA develops RA, i.e. those with imaging signs of subclinical inf...
Background
Hyper-IgD syndrome/mevalonate kinase deficiency (HIDS/MKD) is a rare autoinflammatory condition caused by a defect in the gene coding for mevalonate kinase. This periodic fever syndrome is characterized by severe systemic and organ inflammation. Treatment with interleukin-1β inhibitor canakinumab (CAN), approved and applied for treatment...
Background
Monitoring disease activity in patients with inflammatory arthritis is essential for effective treatment. While the health assessment questionnaire (HAQ) is commonly used to assess physical function, additional functional tests, such as isometric grip strength and the Moberg Pick-Up-Test (MPUT), provide objective measures for hand functi...
Background
The cryopyrin-associated periodic fever syndromes (CAPS) are hereditary monogenic autoinflammatory diseases with severe systemic and organ inflammation due to increased production of Interleukin-1β (IL-1β). The subcutaneously administered monoclonal antibody canakinumab (CAN) effectively inhibits IL-1β and results in rapid remission of C...
Background
MicroRNAs (miRNAs) are small non-coding RNAs that control gene expression. Specific miRNA signatures have been identified in numerous diseases and may serve as potential biomarkers or new drug targets. Whether certain miRNA signatures are associated with psoriatic joint disease is currently unknown.
Objectives
To search for circulating...
Background
Autoinflammatory diseases (also referred to as hereditary periodic fever syndromes) are caused by defects in the innate immune system. Many autoinflammatory syndromes arise from inherited genetic mutations which begin in childhood and persist throughout adult life. These diseases are often present in several members and generations withi...
Background
Enthesitis represents a hallmark feature of spondyloarthritis, including psoriatic arthritis (PsA). ¹ So far, most of the data on enthesitis in PsA are based on clinical assessment of tenderness as well as MRI or ultrasound examinations. ² These approaches, however, do not allow molecular analysis of entheses, which will require acquisit...
Background
Giant cell arteritis (GCA) has the tendency to relapse once treatment is tapered or stopped. Such relapses represent a potential threat to GCA patients as they can lead to severe symptoms and organ damage.
Objectives
To assess the frequency and type of relapses in patients with GCA
Methods
The Real-Life Treatment and Safety (REATS)-GCA...
Background
The clinical course of adult-onset Still’s disease (AoSD) is highly variable, ranging from subtle constitutional symptoms to life-threatening complications such as macrophage activation syndrome. Therefore, it is of interest whether baseline disease activity in AoSD predicts the clinical course, i.e. clinical and serological remission....
Objectives:
To establish a minimally invasive biopsy technique for the analysis of entheseal tissue in patients with psoriatic arthritis (PsA).
Methods:
Human cadavers were used for establishing the technique to retrieve tissue from the lateral humeral epicondyle enthesis (cadaveric biopsies). After biopsy, the entire enthesis was surgically res...
Objective
To assess if microstructural bone lesions in individuals at risk of developing rheumatoid arthritis (RA) are related to the spectrum of anti–modified protein antibodies (AMPAs) and affect the risk of developing RA.
Methods
Cortical microchannels as well as cortical and trabecular bone mineral density (BMD) volumes (expressed as mg hydrox...
Objective
To test whether the presence of structural entheseal lesions in psoriasis patients influences the risk of progression to psoriatic arthritis (PsA).
Methods
We conducted a prospective cohort study of psoriasis patients without clinical evidence of musculoskeletal involvement who underwent baseline assessment of structural entheseal lesion...
Background
Granulomatosis with polyangiitis (GPA) is a systemic autoimmune disease characterized by small and medium vessel vasculitis. The use of biological therapies such as rituximab and infliximab has improved the treatment of ocular manifestations in GPA.
Case report
We report a case of a 45-year-old Caucasian male suffering with rhinitis, si...
Objectives
Patients with Rheumatoid Arthritis (RA) are increasingly achieving stable disease remission, yet the mechanisms that govern ongoing clinical disease and subsequent risk of future flare are not well understood. We sought to identify serum proteomic alterations that dictate clinically important features of stable RA, and couple broad-based...
It is an extension of the GiACTA trial demonstrating that weekly tocilizumab delays relapse in newly diagnosed and relapsing patients, more efectively than every 2 weeks.
Objective
Tocilizumab plus prednisone induces sustained glucocorticoid-free remission in patients with giant cell arteritis (GCA). However, its long-term benefits in new-onset vs relapsing disease are uncertain and the value of weekly vs every-other-week dosing has not been evaluated.
Methods
In GiACTA part 1, patients with new-onset or relapsing...
Background
Owing to increasing remission rates, the management of patients with rheumatoid arthritis in sustained remission is of growing interest. The Rheumatoid Arthritis in Ongoing Remission (RETRO) study investigated tapering and withdrawal of disease-modifying antirheumatic drugs (DMARDs) in patients with rheumatoid arthritis in stable remissi...
Background
Adult-onset Still’s disease (AOSD) should be considered in the differential diagnosis of patients with endocarditis, with or without a cardiac decompensation.
Case presentation
We report the case of a 68-year-old Caucasian male diagnosed with AOSD after an initial acute manifestation of endocarditis with severe aortic acute manifestatio...
Objectives
A substantial proportion of rheumatoid arthritis (RA) patients flare upon withdrawal of disease modifying anti-rheumatic drugs (DMARDs), thus the definition of prognostic markers is crucial. Anti-citrullinated protein antibody (ACPA)-positivity has been identified as a risk factor for flare. However, only the role of IgG is established i...
Background
Vasculitides comprise a group of rare diseases which affect less than 5 in 10.000 individuals. Most types of vasculitis can become organ- and life-threatening and are characterized by chronicity, high morbidity and relapses, altogether resulting in significant morbidity and mortality. Previous studies have been either monocentric or main...
Background
Rheumatoid Arthritis (RA) is preceded by a clinically silent pre-phase characterized by autoimmunity against anti-modified protein antibodies including anti-citrullinated protein antibodies (ACPA). At this pre-stage patients already experience significant loss of volumetric peripheral bone mineral density (vBMD) compared to healthy contr...
Background
In clinical trials as well as in real-life, the IL-1ß inhibitor canakinumab leads to rapid remission of symptoms in the treatment of CAPS, a monogenic autoinflammatory disease with severe systemic and organ inflammation.
Objectives
The RELIANCE registry is designed to explore long-term safety and effectiveness of canakinumab under routi...
Background
Serum concentration of cartilage oligomeric matrix protein (COMP) is related to the degree of cartilage destruction in patients with rheumatoid arthritis (RA) and shows a mechanosensitive response to ambulatory loads. We showed previously, that individuals with positive for anti-citrullinated protein antibody (ACPA+) status already show...
Background
Due to better treatment strategies and higher remission rates the management of rheumatoid arthritis (RA) patients in sustained remission is of increasing interest (1). The Rheumatoid Arthritis in Ongoing Remission (RETRO) study investigated the possibility to taper and stop disease modifying anti-rheumatic drugs (DMARDs).
Objectives
To...
Background
Understanding key mechanisms of flare development and sustained remission is one of the acute goals in modern rheumatology. Anti-citrullinated protein antibodies (ACPA) are the most abundant and specific autoantibodies in rheumatoid arthritis (RA) patients. However, the impact of ACPA of IgA isotype is poorly defined. IgA ACPA were previ...
Background
Tumor necrosis factor inhibitors (TNFi) signify a major advance in the treatment of rheumatoid arthritis (RA). However, treatment success initially remains uncertain as half of the patients do not respond adequately to TNFi. Thus, an unmet need exists to better predict therapeutic outcome of biologicals.
Objectives
We investigated wheth...
Background: Adult onset Still’s disease (AOSD) should be considered in the differential diagnosis of patients with endocarditis, with or without a cardiac decompensation.
Case presentation: We report the case of a 68-year-old Caucasian male diagnosed with AOSD after an initial acute manifestation of endocarditis with severe aortic acute manifestati...
Objectives:
Efficacy evaluation of giant cell arteritis (GCA) treatment is primarily based on non-specific symptoms and laboratory markers. We aimed to assess the change in vascular inflammation in patients with large vessel (LV)-GCA under different treatments using [18F]FDG PET/CT.
Methods:
Observational study on patients with new-onset, active...
Background: To investigate the clinical presentation and genetic variants in patients of the German anti-IL1 registry for autoinflammatory orphan diseases (GARROD) between 2013-2019.
Methods: Multicenter, retrospective analysis of demographic, clinical and genetic data of 231 patients with autoinflammatory diseases (AID) who received anti-IL1 targe...
Background
The combination of tocilizumab plus a glucocorticoid taper is effective in maintaining clinical remission without requiring additional glucocorticoid therapy in patients with giant cell arteritis, as shown in part one of the Giant Cell Arteritis Actemra (GiACTA) trial. However, the duration of the tocilizumab effect after discontinuation...