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1.
Muscle Nerve ; 69(3): 334-339, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38197490

RESUMO

INTRODUCTION/AIMS: The performance of magnetic resonance imaging (MRI) for diagnosing suspected idiopathic inflammatory myopathy (IIM) remains controversial. Furthermore, the role of contrast-enhanced magnetic resonance imaging (CE-MRI) sequences is unclear. The aim of this study was to evaluate the sensitivity and specificity of a non-enhanced magnetic resonance imaging (NE-MRI) protocol compared to a CE-MRI protocol in adult patients with confirmed IIM. METHODS: This study retrospectively enrolled patients with suspected IIM who underwent MRI of the upper thigh between 2008 and 2020. The protocol consisted of a T1-weighted (T1w) sequence, a turbo inversion recovery magnitude (TIRM) sequence and a contrast-enhanced T1-weighted sequence (CE-T1w). After randomly stratifying patients into a group with only the T1w and TIRM sequences available and another group with additional availability of CE-T1w, three blinded readers assessed the presence of IIM based on characteristic imaging features. Confirmation of the diagnosis was determined based on the 2017 ACR/EULAR criteria. RESULTS: Of the 80 patients (mean age 49.0 ± 21.1 years; 42 female, 38 male) included, 54 (67.5%) had a positive diagnosis of IIM. Cumulated sensitivity and specificity for MRI to detect IIM was 87.1% and 83.3% in the NE-MRI group versus 87.0% and 63.0% in the CE-MRI group. The group differences for sensitivity and specificity were non-significant for each of the three readers, respectively (p ≥ .081). DISCUSSION: NE-MRI detects suspected IIM with high diagnostic accuracy and performs equivalently to CE-MRI. Therefore, it may be appropriate to omit the use of contrast agents in MRI scans performed for suspected IIM.


Assuntos
Imageamento por Ressonância Magnética , Miosite , Humanos , Adulto , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Estudos Retrospectivos , Imageamento por Ressonância Magnética/métodos , Miosite/diagnóstico por imagem , Sensibilidade e Especificidade , Coxa da Perna , Meios de Contraste
2.
Clin Exp Rheumatol ; 42(4): 852-858, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38607682

RESUMO

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.


Assuntos
Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos , Sistema de Registros , Humanos , Feminino , Pessoa de Meia-Idade , Masculino , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/epidemiologia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/terapia , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/tratamento farmacológico , Vasculite Associada a Anticorpo Anticitoplasma de Neutrófilos/diagnóstico , Idoso , Estudos Prospectivos , Alemanha/epidemiologia , Imunossupressores/uso terapêutico , Resultado do Tratamento , Granulomatose com Poliangiite/tratamento farmacológico , Granulomatose com Poliangiite/epidemiologia , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/imunologia , Granulomatose com Poliangiite/terapia , Recidiva , Poliangiite Microscópica/epidemiologia , Poliangiite Microscópica/tratamento farmacológico , Poliangiite Microscópica/diagnóstico , Poliangiite Microscópica/terapia , Poliangiite Microscópica/imunologia , Síndrome de Churg-Strauss/epidemiologia , Síndrome de Churg-Strauss/tratamento farmacológico , Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/imunologia , Progressão da Doença , Fatores de Tempo , Rituximab/uso terapêutico
3.
Clin Exp Rheumatol ; 42(4): 895-904, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38683207

RESUMO

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.


Assuntos
Arterite de Células Gigantes , Imunossupressores , Sistema de Registros , Humanos , Arterite de Células Gigantes/tratamento farmacológico , Arterite de Células Gigantes/epidemiologia , Arterite de Células Gigantes/diagnóstico , Masculino , Feminino , Idoso , Idoso de 80 Anos ou mais , Estudos Prospectivos , Imunossupressores/uso terapêutico , Alemanha/epidemiologia , Resultado do Tratamento , Fatores de Tempo , Recidiva
4.
Rheumatol Int ; 44(2): 303-306, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37742280

RESUMO

Pre-clinical studies suggest that large language models (i.e., ChatGPT) could be used in the diagnostic process to distinguish inflammatory rheumatic (IRD) from other diseases. We therefore aimed to assess the diagnostic accuracy of ChatGPT-4 in comparison to rheumatologists. For the analysis, the data set of Gräf et al. (2022) was used. Previous patient assessments were analyzed using ChatGPT-4 and compared to rheumatologists' assessments. ChatGPT-4 listed the correct diagnosis comparable often to rheumatologists as the top diagnosis 35% vs 39% (p = 0.30); as well as among the top 3 diagnoses, 60% vs 55%, (p = 0.38). In IRD-positive cases, ChatGPT-4 provided the top diagnosis in 71% vs 62% in the rheumatologists' analysis. Correct diagnosis was among the top 3 in 86% (ChatGPT-4) vs 74% (rheumatologists). In non-IRD cases, ChatGPT-4 provided the correct top diagnosis in 15% vs 27% in the rheumatologists' analysis. Correct diagnosis was among the top 3 in non-IRD cases in 46% of the ChatGPT-4 group vs 45% in the rheumatologists group. If only the first suggestion for diagnosis was considered, ChatGPT-4 correctly classified 58% of cases as IRD compared to 56% of the rheumatologists (p = 0.52). ChatGPT-4 showed a slightly higher accuracy for the top 3 overall diagnoses compared to rheumatologist's assessment. ChatGPT-4 was able to provide the correct differential diagnosis in a relevant number of cases and achieved better sensitivity to detect IRDs than rheumatologist, at the cost of lower specificity. The pilot results highlight the potential of this new technology as a triage tool for the diagnosis of IRD.


Assuntos
Reumatologia , Humanos , Reumatologistas
5.
Rheumatol Int ; 44(2): 263-271, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37747561

RESUMO

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 comprised 152 patients with familial Mediterranean fever (FMF; n = 71), cryopyrin-associated periodic syndromes (CAPS; n = 43), TNF-receptor associated periodic syndrome (TRAPS; n = 19), mevalonate kinase deficiency (MKD; n = 3) and unclassified AID (uAID; n = 16). Inflammatory attacks started in 61.2% of the patients before the age of 18 years. The delay between the first AID attack and anti-IL-1 therapy was 17.8 years. Monogenetic AIDs were diagnosed by clinical symptoms. Genetic analyses confirmed the diagnosis in 87.3% of patients with FMF, 65.2% with CAPS and 94.8% with TRAPS. Among this group, heterozygous MEFV variants and variants of unknown significance (VUS) were detected in 22.5% of patients with FMF, 51.2% with CAPS and 47.4% with TRAPS. Patients with VUS were older at disease onset which is consistent with a milder phenotype. Twenty-four patients had secondary AA amyloidosis (AA) at initiation of anti-IL-1 therapy. The mean age of these patients was 16.4 years at their first attack and 44.9 years at the time of AA diagnosis. Turkish-Armenian ancestry correlated with MEFV variants and higher FMF disease activity compared to German ancestry. Molecular genetic analyses should substantiate the clinical diagnosis of a monogenetic AID. Our data support the concept of variable penetrance of VUS which can be associated with late-onset AID.


Assuntos
Amiloidose , Febre Familiar do Mediterrâneo , Doenças Hereditárias Autoinflamatórias , Humanos , Adolescente , Estudos Retrospectivos , Doenças Hereditárias Autoinflamatórias/diagnóstico , Doenças Hereditárias Autoinflamatórias/tratamento farmacológico , Doenças Hereditárias Autoinflamatórias/genética , Febre/diagnóstico , Febre Familiar do Mediterrâneo/diagnóstico , Febre Familiar do Mediterrâneo/tratamento farmacológico , Febre Familiar do Mediterrâneo/genética , Sistema de Registros , Pirina/genética , Proteína Amiloide A Sérica
6.
Z Rheumatol ; 83(3): 234-241, 2024 Apr.
Artigo em Alemão | MEDLINE | ID: mdl-37289217

RESUMO

As a result of digitalization in medicine wearable computing devices (wearables) are becoming increasingly more important. Wearables are small portable electronic devices with which the user can record data relevant to health, such as number of steps, activity profile, electrocardiogram (ECG), heart and breathing frequency or oxygen saturation. Initial studies on the use of wearables in patients with rheumatological diseases show the opening up of new possibilities for prevention, disease monitoring and treatment. This study provides the current data situation and the implementation of wearables in the discipline of rheumatology. Additionally, future potential fields of application as well as challenges and limits of the implementation of wearables are illustrated.


Assuntos
Reumatologia , Telemedicina , Dispositivos Eletrônicos Vestíveis , Humanos , Eletrocardiografia
7.
Z Rheumatol ; 83(5): 363-375, 2024 Jun.
Artigo em Alemão | MEDLINE | ID: mdl-38802504

RESUMO

Autoinflammatory diseases are characterized by inflammatory manifestations in various organ systems, whereby recurrent febrile episodes, musculoskeletal complaints, gastrointestinal and cutaneous symptoms frequently occur accompanied by serological signs of inflammation. Autoinflammatory diseases include rare monogenic entities and multifactorial or polygenic diseases, which can manifest as a variety of symptoms in the course of time. Examples of monogenic autoinflammatory diseases are familial Mediterranean fever (FMF), cryopyrin-associated periodic syndrome (CAPS), tumor necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS) and the recently described VEXAS (vacuoles, E1 enzyme, X­linked, autoinflammatory and somatic) syndrome. For non-monogenically determined autoinflammatory diseases, the most important representatives in adulthood are adult-onset Still's disease (AOSD) and the Schnitzler syndrome, in which a polygenic susceptibility and epigenetic factors are more likely to play a role.


Assuntos
Doenças Hereditárias Autoinflamatórias , Humanos , Doenças Hereditárias Autoinflamatórias/genética , Doenças Hereditárias Autoinflamatórias/diagnóstico , Adulto , Síndrome , Febre de Causa Desconhecida/etiologia , Febre de Causa Desconhecida/genética
8.
Z Rheumatol ; 83(5): 341-353, 2024 Jun.
Artigo em Alemão | MEDLINE | ID: mdl-38634905

RESUMO

Fever is a frequent and important symptom in patients with rheumatological diseases and can be an expression of activity of the underlying rheumatological disease. There is great variability in the incidence of fever as a symptom of the disease between individual diseases. The growing understanding of the molecular signatures of the diseases can help to explain these discrepancies: A genetic overactivation of potently pyrogenic cytokines is the reason why fever is nearly always present in autoinflammatory syndromes. In contrast, fever is less common in polyarthritis and myositis and mostly limited to severe courses of disease. In the diagnostic work-up of fever, frequent differential diagnoses, such as infections, malignancies, side effects of drugs and hypersensitivity reactions should be considered. This article provides an overview of the physiology of the development of fever, describes the relevance of fever in individual rheumatological diseases and proposes a workflow for the clinical clarification of rheumatological patients who present with fever.


Assuntos
Febre , Doenças Reumáticas , Humanos , Doenças Reumáticas/complicações , Doenças Reumáticas/diagnóstico , Febre/diagnóstico , Febre/etiologia , Diagnóstico Diferencial , Febre de Causa Desconhecida/etiologia , Febre de Causa Desconhecida/diagnóstico
9.
Z Rheumatol ; 83(5): 376-386, 2024 Jun.
Artigo em Alemão | MEDLINE | ID: mdl-38267764

RESUMO

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome that is characterized by hyperferritinemia, cytopenia, disseminated intravascular coagulopathy and functional disorders of the liver and the central nervous system. The term macrophage activation syndrome is predominantly used for secondary HLH in the context of autoimmune diseases (e.g., systemic juvenile idiopathic arthritis). In addition, malignancies and genetic inborn errors of immunity can predispose to the development of HLH. Infections (e.g., Epstein-Barr virus) in turn represent possible triggers of an acute episode. Due to the unspecific manifestation of the disease, a systematic evaluation of the organ systems is recommended in the clinical and laboratory analytical clarification of hyperinflammatory syndromes. In general, the treatment should be carried out by a multidisciplinary team with expertise in rheumatology, hematological oncology, infectious diseases and intensive care medicine. The primary treatment of HLH usually consists of glucocorticoids and in cases of a rapid deterioration of the condition anakinra (interleukin 1 block) and intravenous immunoglobulins can be employed. Treatment of the underlying disease should be consequently carried out in parallel, together with antimicrobial treatment.


Assuntos
Linfo-Histiocitose Hemofagocítica , Síndrome de Ativação Macrofágica , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfo-Histiocitose Hemofagocítica/terapia , Linfo-Histiocitose Hemofagocítica/imunologia , Humanos , Síndrome de Ativação Macrofágica/diagnóstico , Síndrome de Ativação Macrofágica/terapia , Síndrome de Ativação Macrofágica/imunologia , Síndrome de Ativação Macrofágica/etiologia , Equipe de Assistência ao Paciente , Glucocorticoides/uso terapêutico , Medicina Baseada em Evidências , Diagnóstico Diferencial , Resultado do Tratamento , Imunoglobulinas Intravenosas/uso terapêutico , Reumatologia , Proteína Antagonista do Receptor de Interleucina 1/uso terapêutico
10.
Z Rheumatol ; 83(Suppl 1): 31-39, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37847298

RESUMO

BACKGROUND: Data on the training and continuing education situation of residents in the field of internal medicine and rheumatology are not available for Germany. For this reason, the Commission for Education and Training of the German Society of Rheumatology (DGRh) initiated the BEWUSST survey on the working, training and research conditions of residents in rheumatology. METHODS: A total of 102 questions on the topics of working conditions in everyday professional life, continuing medical education and training, compatibility of career and family, compatibility of work and research, perspectives as a rheumatologist and practical activities were included in an online questionnaire. RESULTS: A total of 102 participants took part in the survey. Of the respondents 48.1% were satisfied with their professional situation, 40.2% of the participants were supervised by a specialist mentor and 54.9% were working as scientists during their work as a physician. A compatibility of family and career was possible for 34.7%. After completion of the residency 52.9% of the respondents aspired to a combined clinical and outpatient activity. CONCLUSION: Half of the trainee rheumatologists are satisfied with their professional activities, although mentoring of the assistants in training should be further improved. With respect to the desired combined clinical and outpatient activity, the existing options should be expanded or new professional fields of activity should be established, so that the specialty remains attractive for the upcoming generations.


Assuntos
Internato e Residência , Médicos , Doenças Reumáticas , Reumatologia , Humanos , Reumatologia/educação , Inquéritos e Questionários , Educação Continuada , Doenças Reumáticas/diagnóstico , Doenças Reumáticas/terapia
11.
Rheumatology (Oxford) ; 62(11): 3518-3525, 2023 11 02.
Artigo em Inglês | MEDLINE | ID: mdl-37233149

RESUMO

OBJECTIVES: To evaluate the effectiveness and safety of current treatment strategies for the vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome. METHODS: A protocolized systematic review according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines was performed. Three databases were searched for reports on treatment strategies for VEXAS. Data from the included publications was extracted and a narrative synthesis was performed. Treatment response was recorded as complete (CR), partial (PR) or none (NR) depending on changes in clinical symptoms and laboratory parameters. Patient characteristics, safety data and previous treatments were analysed. RESULTS: We identified 36 publications with a total of 116 patients; 113 (98.3%) were male. The identified reports included azacytidine (CR 9/36, 25%; PR 14/36, 38.9%), Janus kinase inhibitors (JAKi) (CR 11/33, 33%; PR 9/33, 27.3%), tocilizumab (CR 3/15, 20%; PR 6/15, 40%), allogeneic stem cell transplantation (CR 6/7, 85.7%; one patient died), anakinra (CR 4/5, 80%; NR 1/5, 20%), canakinumab (CR 1/2, 50%; PR 1/2, 50%) and glucocorticoid monotherapy (CR 1/6, 16.7%; PR 4/6, 66.7%). Individual reports were available for TNF inhibitors, rituximab and MTX. Data on adverse events were available for 67 patients (67/116, 57.8%) and included: pneumonia (12/67, 17.9%), other infections (9/67, 13.4%), venous thromboembolisms (6/67, 8.9%), cytopenias (4/67, 5.9%), and acute (4/67, 5.9%) and chronic graft-vs-host-disease (2/67, 2.9%). CONCLUSION: Current data on VEXAS treatment are limited and inhomogeneous. Treatment decisions should be individualized. For the devolvement of treatment algorithms clinical trials are needed. Adverse events remain a challenge, especially an elevated risk for venous thromboembolism associated to JAKi treatment should be carefully considered.


Assuntos
Síndrome de Bronquiolite Obliterante , Inibidores de Janus Quinases , Humanos , Masculino , Feminino , Algoritmos , Azacitidina , Bases de Dados Factuais , Mutação
12.
Rheumatology (Oxford) ; 62(11): 3654-3661, 2023 11 02.
Artigo em Inglês | MEDLINE | ID: mdl-36864623

RESUMO

OBJECTIVE: To investigate the differential diagnostic spectrum in patients with suspected Behçet's syndrome (BS) in low prevalence regions. In addition, the number of patients fulfilling the ICBD criteria despite not having BS was evaluated. METHODS: This retrospective analysis was performed in two referral centres for BS. Patients with confirmed BS (clinical diagnosis with fulfilment of ISG criteria or a score of ≥5 points in the ICBD criteria) were excluded. The remaining patients were divided into 11 differential diagnosis categories. If no definitive alternative diagnosis could be established, patients were termed 'probable BS' in case of (i) relapsing orogenital aphthosis in the absence of other causes and either HLA-B51 positivity, or origin from an endemic area or presence of an additional typical BS symptom that is not part of the classification criteria, or (ii) with 3-4 points scored in the ICBD criteria. RESULTS: In total 202 patients were included and categorized as follows: 58 patients (28.7%) as 'probable BS', 57 (28.2%) skin disease, 26 (12.9%) chronic pain syndrome, 14 (6.9%) eye disease, 11 (5.4%) spondyloarthropathy, 9 (4.5%) gastrointestinal disease, 7 (3.5%) neurological disease, 4 (2%) arthritis, 3 (1.5%) auto-inflammation, 3 (1.5%) connective tissue disease and 10 (5.0%) miscellaneous disease. HLA-B51 was positive in 55/132 (41.7%); 75/202 (37.1%) of the patients fulfilled the ICBD criteria. CONCLUSION: In a low disease prevalence setting, the straightforward application of the ICBD criteria may lead to overdiagnosis of BS. The differential diagnosis of BS is enormously broad. Clinicians should be aware that HLA-B51 positivity is still not considered as a diagnostic feature in BS.


Assuntos
Síndrome de Behçet , Estomatite Aftosa , Humanos , Síndrome de Behçet/diagnóstico , Síndrome de Behçet/epidemiologia , Estudos Retrospectivos , Antígeno HLA-B51 , Diagnóstico Diferencial
13.
Rheumatol Int ; 43(3): 551-557, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36576539

RESUMO

Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a potentially fatal acquired thrombotic microangiopathy syndrome that frequently develops in the context of infectious diseases or systemic autoimmune conditions including connective tissue diseases. We report the case of a 42-year-old female suffering from severe iTTP associated with anti-Jo-1 positive antisynthetase syndrome, which was successfully treated with combination therapy of intravenous immune globulin, rituximab and plasma exchange. Based on a systematic review of the literature, two additional cases of idiopathic inflammatory myopathy-associated iTTP (secondary iTTP) were identified. In conclusion, iTTP may be a rare complication of IIM that clinicians should consider in cases of marked thrombocytopenia. Further work-up of this finding should include a peripheral blood smear (schistocyte count) and ADAMTS13 activity. The concomitant manifestation of these autoimmune conditions may require intensive immunosuppressive therapy.


Assuntos
Doenças Autoimunes , Miosite , Púrpura Trombocitopênica Trombótica , Feminino , Humanos , Adulto , Púrpura Trombocitopênica Trombótica/complicações , Púrpura Trombocitopênica Trombótica/terapia , Rituximab , Doenças Autoimunes/complicações , Miosite/complicações
14.
Rheumatol Int ; 43(1): 163-171, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-36374326

RESUMO

Getting access to specialists for autoinflammatory diseases (AID) can be challenging. Therefore, an increasing number of patients and healthcare professionals are seeking information on AID via the Internet, using the video platform YouTube, for example. However, the quality of such videos has not yet been evaluated. A YouTube search was conducted to assess videos about AID to evaluate the quality and usefulness from both the patient's and healthcare professional´s perspectives. Video duration, number of views, likes, dislikes, comments, and uploading source on various AID were extracted. Video quality was evaluated by the modified global quality scale (GQS). The reliability was assessed by the modified five-point DISCERN score. In total, 140 videos were screened of which 105 videos met the inclusion criteria for further analysis. Based on the GQS, the overall quality of videos for patients was found to be low in 64.8%, intermediate in 27.6%, and high in 7.6% of videos. The quality of videos for professionals was similar (54.3% low, 23.8% intermediate, and 21.9% of high quality). Videos were more often targeting medical professionals (65.7%) and less often patients (34.3%). This analysis demonstrates that the majority of videos regarding AIDs are of limited quality. Available videos more often address users with a professional medical background. Only a small proportion of existing videos provide understandable and useful information for AID patients. Thus, there is a strong need to develop high-quality and audience-oriented videos in the context of educational campaigns for these rare disease groups.


Assuntos
Doenças Hereditárias Autoinflamatórias , Mídias Sociais , Humanos , Reprodutibilidade dos Testes , Gravação em Vídeo , Disseminação de Informação
15.
Z Rheumatol ; 82(7): 606-614, 2023 Sep.
Artigo em Alemão | MEDLINE | ID: mdl-35612660

RESUMO

Cocaine is a psychotropic tropane alkaloid and stimulant drug. Nasal insufflation of cocaine powder is a common route of administration. In Germany, cocaine is frequently adulterated with levamisole, an anthelminthic drug with immunomodulatory effects. Both substances are linked to various autoimmune conditions. Cocaine-induced midline destructive lesions cause a progressive destruction of osteocartilaginous structures within the upper respiratory tract and can mimic localized granulomatosis with polyangiitis. In addition, systemic vasculitis due to cocaine and levamisole has been reported. Differentiation of these conditions from primary vasculitis can be challenging because antineutrophil cytoplasmic antibodies (ANCA) are commonly detected. Early diagnosis of these conditions is crucial as clinical improvement is closely related to drug cessation.


Assuntos
Doenças Autoimunes , Transtornos Relacionados ao Uso de Cocaína , Cocaína , Vasculite , Humanos , Cocaína/efeitos adversos , Levamisol/efeitos adversos , Transtornos Relacionados ao Uso de Cocaína/diagnóstico , Vasculite/diagnóstico , Anticorpos Anticitoplasma de Neutrófilos
16.
Z Rheumatol ; 82(8): 678-687, 2023 Oct.
Artigo em Alemão | MEDLINE | ID: mdl-37672064

RESUMO

The concept of autoinflammation includes a heterogeneous group of monogenic and polygenic diseases. These are characterized by excessive activation of the innate immune system without antigen-specific T cells or autoantibodies. The diseases are characterized by periodic episodes of fever and increased inflammation parameters. Monogenic diseases include familial Mediterranean fever (FMF) and the newly described VEXAS (vacuoles, E1 enzyme, X­linked, autoinflammatory, somatic) syndrome. Heterogeneous diseases include adult-onset Still's disease and Schnitzler syndrome. Treatment is aimed at preventing the excessive inflammatory reaction in order to avoid long-term damage, such as amyloid A (AA) amyloidosis.

17.
Z Rheumatol ; 2023 Jun 06.
Artigo em Alemão | MEDLINE | ID: mdl-37280333

RESUMO

Hughes-Stovin syndrome (HSS) is a systemic inflammatory condition of unknown origin that is considered to be part of the Behçet's syndrome (BS) spectrum. Recurrent venous thrombosis and superficial thrombophlebitis in combination with bilateral pulmonary artery aneurysms (PAA) represent the hallmark of HSS. The diagnostic evaluation includes computed tomography pulmonary angiography to detect signs of pulmonary vasculitis. The management of HSS is based on the European Alliance of Associations for Rheumatology (EULAR) recommendations for BS and mainly comprises immunosuppressive therapy with glucocorticoids and cyclophosphamide. In addition to drug therapy, PAA should be evaluated for interventional treatment. Spontaneous PAA rupture due to fragile vessel architecture can occur even in cases of remission and/or PAA regression.

18.
Z Rheumatol ; 82(10): 885-891, 2023 Dec.
Artigo em Alemão | MEDLINE | ID: mdl-35384513

RESUMO

BACKGROUND: Refractory arthritis is a common problem in routine rheumatology practice, and can be a diagnostic challenge. In these cases, chronic Tropheryma whipplei (T. whipplei) infection is an important differential diagnosis that should be considered. OBJECTIVE: Based on five clinical cases, this case-based review describes the diagnostic and therapeutic principles in the management of chronic T. whipplei infection. RESULTS: Whipple's disease is a multisystemic infectious disease caused by the bacterium T. whipplei. The disease typically manifests with arthralgia, weight loss and diarrhoea. Joint involvement often develops years before gastrointestinal symptoms occur. In addition to systemic manifestations ("classic Whipple's disease"), T. whipplei can also lead to localized joint infections without gastrointestinal involvement. Articular manifestations of systemic and localized T. whipplei infections are commonly misdiagnosed as a sign of various forms of autoimmmune arthritis. DISCUSSION: Whipple's disease and localized T. whipplei joint infection should be considered in the diagnostic work-up of refractory arthritis. Synovial fluid analysis by means of specific polymerase chain reaction-based testing for T. whipplei is diagnostically ground-breaking.


Assuntos
Artrite Infecciosa , Doença de Whipple , Humanos , Tropheryma/genética , Doença de Whipple/diagnóstico , Diagnóstico Diferencial , Antibacterianos/uso terapêutico , Artrite Infecciosa/terapia , Artrite Infecciosa/tratamento farmacológico
19.
Z Rheumatol ; 82(2): 108-113, 2023 Mar.
Artigo em Alemão | MEDLINE | ID: mdl-34448915

RESUMO

INTRODUCTION: In order to successfully integrate telemedicine into the daily routine of rheumatology, both the patient's and the physician's perspective are important. For this purpose, a detailed study by means of a web-based survey was conducted by the Working Group Young Rheumatology (AGJR) of the German Society for Rheumatology (DGRh) and the German Rheumatism League National Association. By means of subgroup analysis of the data regarding video consultation, the aim was now to find out which requirements and wishes patients and physicians have for video consultations. METHODS: The prospective survey was distributed via social media, QR code and email. Descriptive statistics and regression analysis related to video consultation were performed and correlations were shown. RESULTS: The data indicated positive attitudes toward video consultation on the part of both patients (n = 299) and rheumatologists (n = 129). A correlation between age and positive opinion of the video consultation was found among the patients (r = 0.161, p = 0.006), especially among female patients a positive approval of the video consultation was found with increasing age (r = 0.244, p < 0.001 to male patients: r = -0.190, p = 0.145). Regarding the travelling time to the treating rheumatologist, male patients found the video consultation more attractive with increasing travelling time (r = 0.229, p = 0.078). With respect to the wishes of patients and physicians, video consultation should be used primarily for follow-up or emergency appointments. Video consultation for initial appointments, on the other hand, was very rarely mentioned. CONCLUSION: During the COVID 19 pandemic, video consultation was increasingly popular among rheumatology patients as well as among rheumatologists.


Assuntos
COVID-19 , Doenças Reumáticas , Febre Reumática , Reumatologia , Telemedicina , Humanos , Masculino , Feminino , Estudos Prospectivos , Reumatologia/métodos , Doenças Reumáticas/diagnóstico , Doenças Reumáticas/terapia , Reumatologistas
20.
Z Rheumatol ; 2023 Aug 11.
Artigo em Alemão | MEDLINE | ID: mdl-37566120

RESUMO

BACKGROUND: Data on the training and continuing education situation of residents in the field of internal medicine and rheumatology are not available for Germany. For this reason, the Commission for Education and Training of the German Society of Rheumatology (DGRh) initiated the BEWUSST survey on the working, training and research conditions of residents in rheumatology. METHODS: A total of 102 questions on the topics of working conditions in everyday professional life, continuing medical education and training, compatibility of career and family, compatibility of work and research, perspectives as a rheumatologist and practical activities were included in an online questionnaire. RESULTS: A total of 102 participants took part in the survey. Of the respondents 48.1% were satisfied with their professional situation, 40.2% of the participants were supervised by a specialist mentor and 54.9% were working as scientists during their work as a physician. A compatibility of family and career was possible for 34.7%. After completion of the residency 52.9% of the respondents aspired to a combined clinical and outpatient activity. CONCLUSION: Half of the trainee rheumatologists are satisfied with their professional activities, although mentoring of the assistants in training should be further improved. With respect to the desired combined clinical and outpatient activity, the existing options should be expanded or new professional fields of activity should be established, so that the specialty remains attractive for the upcoming generations.

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