OBJECTIVES: Prospective long-term observational data on the disease course of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) were missing in Germany to date. Therefore, the Joint Vasculitis Registry in German-speaking countries (GeVas) has been established to follow the course of patients with AAV. The aim of this study is to present baseline data of patients with newly diagnosed and relapsing AAV enrolled in the GeVas registry. METHODS: GeVas is a prospective, web-based, multicentre, clinician-driven registry for the documentation of organ manifestations, damage, long-term outcomes, and therapy regimens in various types of vasculitis. Recruitment started in June 2019. RESULTS: Between June 2019 and October 2022, 266 patients with AAV were included in the GeVas registry: 173 (65%) with new-onset and 93 (35%) with relapsing AAV. One hundred and sixty-two (61%) patients were classified as granulomatosis with polyangiitis (GPA), 66 (25%) as microscopic polyangiitis (MPA), 36 (13%) as eosinophilic granulomatosis with polyangiitis (EGPA), and 2 (1%) as renal limited AAV. The median age was 59 years (51-70 years, IQR), 130 (51%) patients were female. Most patients were ANCA positive (177; 67%) and affected by general symptoms, pulmonary, ear nose throat (ENT), renal and neurological involvement. For induction of remission, the majority of patients received glucocorticoids (247, 93%) in combination with either rituximab (118, 45%) or cyclophosphamide (112, 42%). CONCLUSIONS: Demographic characteristics are comparable to those in other European countries. Differences were found regarding ANCA status, frequencies of organ manifestations, and therapeutic regimens. The GeVas registry will allow longitudinal observations and prospective outcome measures in AAV.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , Registries , Humans , Female , Middle Aged , Male , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/epidemiology , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/therapy , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis , Aged , Prospective Studies , Germany/epidemiology , Immunosuppressive Agents/therapeutic use , Treatment Outcome , Granulomatosis with Polyangiitis/drug therapy , Granulomatosis with Polyangiitis/epidemiology , Granulomatosis with Polyangiitis/diagnosis , Granulomatosis with Polyangiitis/immunology , Granulomatosis with Polyangiitis/therapy , Recurrence , Microscopic Polyangiitis/epidemiology , Microscopic Polyangiitis/drug therapy , Microscopic Polyangiitis/diagnosis , Microscopic Polyangiitis/therapy , Microscopic Polyangiitis/immunology , Churg-Strauss Syndrome/epidemiology , Churg-Strauss Syndrome/drug therapy , Churg-Strauss Syndrome/diagnosis , Churg-Strauss Syndrome/immunology , Disease Progression , Time Factors , Rituximab/therapeutic use
OBJECTIVES: Giant cell arteritis (GCA) is one of the most common forms of vasculitis. There is an abundance of studies which are conducted in a randomised controlled trial setting but limited with respect to cohort size and follow-up time. GeVas is the first large-scale registry for vasculitides in German-speaking countries that enables to evaluate this rare disease. Herein we focus on the subgroup of GCA patients including follow-up data up to one year. METHODS: GeVas is a prospective, web-based, multicentre registry for the documentation of organ manifestations, outcomes, and therapy regimens in vasculitides. Recruitment started in June 2019. By April 2023, 15 centres were initiated and have started to enrol patients. RESULTS: After 4 years, 195 GCA-patients were included in the registry, of which 64% were female and 36% were male. The average age was 76 years at the time of recruitment (IQR=69-82). Seventy-nine percent were included in the registry because of a newly diagnosed GCA and 21% because of a relapse. At the first assessment most of the patients (89%) described general symptoms. Thirty-one percent stated ocular symptoms. Cranial symptoms were documented in 78% of the cases. All patients were documented with immunosuppressive treatment at start, of whom 95% received prednisolone, 16% cyclophosphamide, 20% methotrexate, and 48% tocilizumab. After three months 62% and after one year 91% of the patients achieved remission. CONCLUSIONS: Regarding demographics, clinical manifestations and diagnostics, our study showed a similar composition compared to other studies. However, our data differed in terms of treatment regimens.
Giant Cell Arteritis , Immunosuppressive Agents , Registries , Humans , Giant Cell Arteritis/drug therapy , Giant Cell Arteritis/epidemiology , Giant Cell Arteritis/diagnosis , Male , Female , Aged , Aged, 80 and over , Prospective Studies , Immunosuppressive Agents/therapeutic use , Germany/epidemiology , Treatment Outcome , Time Factors , Recurrence
This report presents a case of co-occurrence of Takayasu arteritis (TA) and multiorgan tuberculosis (TB) in a 20-year-old female and provides a review of 18 previously reported cases of co-occurring TA and TB. All patients were between 9 and 24 years of age. Most reports describe a concomitant diagnosis of active TB and TA. TB lymphadenitis was described in 11 cases (57.9%), and microbiologically confirmed in 4 of these. All patients received antituberculous therapy and most received corticosteroids (89.5%). In our and two other cases, TA relapses necessitating additional immunosuppressive therapy were observed.
Takayasu Arteritis/drug therapy , Tuberculosis/drug therapy , Adrenal Cortex Hormones/therapeutic use , Adult , Antitubercular Agents/therapeutic use , Female , Humans , Takayasu Arteritis/complications , Takayasu Arteritis/diagnostic imaging , Tuberculosis/complications , Tuberculosis/diagnostic imaging
