{"title":"[Swelling of the salivary glands and sicca symptoms in Kimura's disease, a rare rheumatological differential diagnosis with an indicatively high IgE serum level-A current overview after literature search].","authors":"Jürgen Braun, Kirsten Karberg","doi":"10.1007/s00393-023-01411-9","DOIUrl":"10.1007/s00393-023-01411-9","url":null,"abstract":"<p><p>Rheumatic and musculoskeletal diseases (RMD) include various diseases with sometimes rather different symptoms, some of which are locally confined and others show systemic features. Autoimmune phenomena, such as those occurring in Sjögren's syndrome, often cause symptoms such as xerostomia and xerophthalmia in association with inflammation of the salivary glands. The pathogenesis of these diseases is only partly clarified. This is similar to allergic diseases, which are otherwise clearly different with respect to the symptoms and pathomechanisms but swelling of the salivary glands can also rarely occur here. As this is a possible differential diagnosis of Sjögren's syndrome or also IgG4-associated diseases, and such a case was recently described, a literature search was carried out in PubMed, the results of which are presented here and summarized in this article in a brief overview.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"650-660"},"PeriodicalIF":0.9,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10233015","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Head-to-head studies on connective tissue diseases and vasculitides].","authors":"Bernhard Hellmich, Johanna Mucke, Martin Aringer","doi":"10.1007/s00393-024-01537-4","DOIUrl":"10.1007/s00393-024-01537-4","url":null,"abstract":"<p><p>Head-to-head (H2H) studies enable the direct comparison of several alternative therapeutic approaches and thus provide the evidence-based foundation for the relative position of one treatment as compared to others for a specific indication. These trials constitute an important addition to placebo-controlled clinical trials. Among the controlled clinical trials not performed by the pharmaceutical industry, there are a relevant number of H2H trials for connective tissue diseases (CTDs) and vasculitides, particularly for systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). This article encompasses a review of the H2H trials for CTDs and vasculitides and discusses their relevance for current treatment algorithms. For SLE the H2H trials were predominantly performed for the treatment of lupus nephritis, demonstrating the impact of low-dose cyclophosphamide and mycophenolate as well as azathioprine for maintenance therapy. In recent H2H trials rituximab could be established as induction and maintenance therapy for AAV, which has now been incorporated into current treatment guidelines. Further comparative trials will be necessary in order to select the most effective and safest treatment for every patient, in the sense of personalized medicine.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"620-628"},"PeriodicalIF":0.9,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141627823","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Is lymphoma screening necessary in Sjögren`s disease, and if so, how?]","authors":"Torsten Witte","doi":"10.1007/s00393-024-01562-3","DOIUrl":"10.1007/s00393-024-01562-3","url":null,"abstract":"","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"664-665"},"PeriodicalIF":0.9,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142296729","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Head-to-head studies on radiographic progression in axial spondyloarthritis].","authors":"Uta Kiltz, Hildrun Haibel","doi":"10.1007/s00393-024-01518-7","DOIUrl":"10.1007/s00393-024-01518-7","url":null,"abstract":"<p><p>In this review article four clinical comparative studies in axial spondylarthritis (axSpA) are presented and discussed. SURPASS as the only head-to-head study investigated the effect of adalimumab biosimilar disease-modifying antirheumatic drug (bsDMARD) or secukinumab on radiographic progression over a time period of 2 years. Overall, the radiographic progression of the spine was low and no significant difference between adalimumab bsDMARD or secukinumab was noted. The three other studies were not constructed as direct head-to-head studies but compared the efficacy of non-steroidal antirheumatic drugs (NSARD) with and without simultaneous treatment with biological DMARDs (bDMARD). The CONSUL study showed no statistically significant difference in the delay of radiographic progression of the spine over 2 years in radiographic axSpA (r-axSpA) patients, who underwent either combined treatment with golimumab and celecoxib or treatment with golimumab alone over 2 years. The ESTHER study showed that patients with early axSpA active inflammatory lesions, which were detected by whole-body magnetic resonance imaging (MRI), showed a significantly greater improvement under treatment with etanercept than those treated with sulfasalazine. The INFAST study showed that patients with early active axSpA who received a combined treatment of infliximab and naproxen, achieved a clinical remission twice as frequently as those who only received naproxen. Therefore, for the endpoint of radiological progression no difference could be shown in the inhibition of radiological progression between the mechanisms of action investigated. The comparative data for the endpoint of clinical efficacy showed that patients with bDMARDs showed a clearly better response to treatment than patients with NSAR or conventional synthetic DMARDs (csDMARD).</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"629-633"},"PeriodicalIF":0.9,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141072045","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Stefanie Hirsch, K Hoeper, D Meyer-Olson, A Schwarting, K Gente, M Dreher, J Hoeper, T Witte, T Thiele
{"title":"[The subanalysis of Rheuma-VOR demonstrates a considerable need for rheumatological care].","authors":"Stefanie Hirsch, K Hoeper, D Meyer-Olson, A Schwarting, K Gente, M Dreher, J Hoeper, T Witte, T Thiele","doi":"10.1007/s00393-024-01490-2","DOIUrl":"10.1007/s00393-024-01490-2","url":null,"abstract":"<p><strong>Background: </strong>Early diagnosis and treatment of inflammatory rheumatic diseases can prevent consequential damage such as permanently limited mobility and joint or organ damage. Simultaneously, there is an increasing deficit in medical care owing to the lack of rheumatological capacity. Rural regions are particularly affected.</p><p><strong>Objectives: </strong>The available unconfirmed diagnoses of the study Rheuma-VOR were analysed regarding another definitive inflammatory rheumatic disease.</p><p><strong>Materials and methods: </strong>The returned questionnaires of the rheumatologists participating in Rheuma-VOR were screened for definitive inflammatory rheumatic diseases other than the required diagnosis of rheumatoid arthritis, psoriatic arthritis or spondyloarthritis.</p><p><strong>Results: </strong>Of 910 unconfirmed diagnoses, in 245 patients another definitive diagnosis could be confirmed. A total of 29.8% of the diagnoses corresponded to degenerative joint changes or chronic pain syndrome, whereas 26.1% involved different forms of inflammatory arthritis. The majority of diagnoses (40.5%) were collagenosis or vasculitis, DISCUSSION: The available data show that a rheumatological presentation was indicated for the majority of patients. Owing to the increasing deficits in medical care a prior selection of the patients is crucial to make optimal use of restricted rheumatological capacities.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"679-685"},"PeriodicalIF":0.9,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11485139/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140060609","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Update on Behçet syndrome].","authors":"Ina Kötter, Nikolas Ruffer, Martin Krusche","doi":"10.1007/s00393-024-01576-x","DOIUrl":"https://doi.org/10.1007/s00393-024-01576-x","url":null,"abstract":"<p><strong>Background: </strong>Behçet syndrome (BS) is a vasculitis of variable vessels with multiple organ manifestations.</p><p><strong>Objective: </strong>This article gives an overview of innovations in the last 2 years.</p><p><strong>Material and methods: </strong>A literature search was carried out using the keyword \"Behcet\" in 2022-2024 in PubMed. The selection of suitable articles was based on the relevance.</p><p><strong>Results and conclusion: </strong>With respect to the pathophysiology it is now clear that BS occupies an intermediate position between autoinflammatory and autoimmune clinical pictures. It is now classified as MHC-I-opathy, i.e., a disease that has a strong association with HLA class I antigens, which also play a prominent role in the pathogenesis. The diagnostic international criteria for Behcet's disease (ICBD) from 2014 with a score of 4 points or more that makes the diagnosis of BS probable have become established; however, in countries with a low prevalence of BS, the differential diagnosis of BS from other diseases is difficult and a higher point limit in the diagnostic score seems to make sense in order to avoid incorrect diagnoses. Clusters or phenotypes of the disease have now been described in various countries in which different symptom complexes frequently occur together; however, the clusters differ between the different countries of origin and depending on the age of the patients. Sonography of the common femoral vein with specific wall thickening in BS patients has been established as an additional tool for the differential diagnosis. Typical characteristics of oral aphthae in BS were also described and the frequency of positivity in the pathergy test could be significantly increased using pneumococcal antigens as the reagent. The treatment recommendations of the EULAR from 2018 still apply; in treatment-refractory cases, tocilizumab, secukinumab, Janus kinase inhibitors (JAKi) and ustekinumab have now also been successfully used. The new EULAR treatment recommendations are expected in 2025.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142362145","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Lupus nephritis and associated thrombotic microangiopathy].","authors":"Antonia Schuster, Bernhard Banas, Tobias Bergler","doi":"10.1007/s00393-024-01489-9","DOIUrl":"10.1007/s00393-024-01489-9","url":null,"abstract":"<p><p>Lupus nephritis represents the most common manifestation of lupus of the solid organs and is associated with an increased risk of chronic kidney disease. The co-occurrence of lupus nephritis and thrombotic microangiopathy is described to be rare but implies the risk of fatal organ dysfunction. We report three patients in whom these two disease entities occurred in parallel, necessitating intensive immunosuppressive therapy, including complement blockade.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"675-678"},"PeriodicalIF":0.9,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11485079/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140029128","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Viktoria Weber, Konstantin Weimann, Isabel Kolm, Barbara Meier-Schiesser
{"title":"[Erythema nodosum].","authors":"Viktoria Weber, Konstantin Weimann, Isabel Kolm, Barbara Meier-Schiesser","doi":"10.1007/s00393-024-01529-4","DOIUrl":"10.1007/s00393-024-01529-4","url":null,"abstract":"<p><p>Erythema nodosum (EN) is the most frequently occurring form of acute panniculitis. It is characterized by painful red to livid raised nodules or bumps that typically occur symmetrically in the shin area. The cause of EN is often a reaction of the immune system to various triggers including infections, inflammatory diseases or medications. In approximately half of the cases no trigger can be identified. After treatment of the underlying pathology EN is typically self-limiting.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"641-649"},"PeriodicalIF":0.9,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11485184/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141331921","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}