Pub Date : 2024-06-01Epub Date: 2024-05-27DOI: 10.1007/s00393-024-01522-x
Anne Pankow, Martin Krusche
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, X‑linked, 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.
{"title":"[The most frequent febrile syndromes and autoinflammatory diseases in adulthood].","authors":"Anne Pankow, Martin Krusche","doi":"10.1007/s00393-024-01522-x","DOIUrl":"10.1007/s00393-024-01522-x","url":null,"abstract":"<p><p>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, X‑linked, 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.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141158993","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}
Pub Date : 2024-05-28DOI: 10.1007/s00393-024-01520-z
Vlora Ibishi, Uta Kiltz, Styliani Tsiami, Michael Wessels, Xenofon Baraliakos
Background: The adaptation of structures and processes in treatment procedures can contribute to increasing patient satisfaction and is the focus of patient-oriented quality assurance.
Objective: To identify patient satisfaction as well as needs, expectations and preferences with respect to care and, based on this, to formulate recommendations for action to optimize the quality of care at a large tertiary rheumatology center.
Material and methods: As part of a qualitative research approach, semi-structured patient interviews and a focus group interview consisting of physicians in rheumatology training in outpatient specialist care were conducted. The quality dimensions of Donabedian were recorded. The data material was evaluated and analyzed using the content-structuring qualitative content analysis according to Kuckartz with the MAXQDA evaluation software.
Results: Using 12 patient interviews and a focus group of 3 future rheumatologists, recommendations for action to optimize the quality of care were derived on the basis of the structural, process and outcome quality. There was a need for optimization in the areas of personnel management, internal practice processes, practice equipment and treatment processes in the outpatient clinic.
Conclusion: The results from the patient interviews and the focus group revealed the aspects in need of optimization. The methodology and results of this study can serve as a reference point for analyses of other rheumatology clinics in order to improve the quality of care within the framework of patient-oriented quality management and continuous further development.
{"title":"[Patient-oriented optimization of the quality of care in a specialized outpatient clinic in a tertiary rheumatology center : A qualitative study].","authors":"Vlora Ibishi, Uta Kiltz, Styliani Tsiami, Michael Wessels, Xenofon Baraliakos","doi":"10.1007/s00393-024-01520-z","DOIUrl":"https://doi.org/10.1007/s00393-024-01520-z","url":null,"abstract":"<p><strong>Background: </strong>The adaptation of structures and processes in treatment procedures can contribute to increasing patient satisfaction and is the focus of patient-oriented quality assurance.</p><p><strong>Objective: </strong>To identify patient satisfaction as well as needs, expectations and preferences with respect to care and, based on this, to formulate recommendations for action to optimize the quality of care at a large tertiary rheumatology center.</p><p><strong>Material and methods: </strong>As part of a qualitative research approach, semi-structured patient interviews and a focus group interview consisting of physicians in rheumatology training in outpatient specialist care were conducted. The quality dimensions of Donabedian were recorded. The data material was evaluated and analyzed using the content-structuring qualitative content analysis according to Kuckartz with the MAXQDA evaluation software.</p><p><strong>Results: </strong>Using 12 patient interviews and a focus group of 3 future rheumatologists, recommendations for action to optimize the quality of care were derived on the basis of the structural, process and outcome quality. There was a need for optimization in the areas of personnel management, internal practice processes, practice equipment and treatment processes in the outpatient clinic.</p><p><strong>Conclusion: </strong>The results from the patient interviews and the focus group revealed the aspects in need of optimization. The methodology and results of this study can serve as a reference point for analyses of other rheumatology clinics in order to improve the quality of care within the framework of patient-oriented quality management and continuous further development.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-05-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141161470","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}
Pub Date : 2024-05-21DOI: 10.1007/s00393-024-01518-7
Uta Kiltz, Hildrun Haibel
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).
{"title":"[Head-to-head studies on radiographic progression in axial spondyloarthritis].","authors":"Uta Kiltz, Hildrun Haibel","doi":"10.1007/s00393-024-01518-7","DOIUrl":"https://doi.org/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":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-05-21","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}
Pub Date : 2024-05-08DOI: 10.1007/s00393-024-01508-9
David Kiefer, Kristina Vaupel, Uta Kiltz, Ludwig Hammel, Yvonne M van der Kraan, Suzanne Arends, Xenofon Baraliakos
Background: Patients with axial spondyloarthritis (axSpA) often experience chronic pain and inflammation, resulting in physical impairments, reduced mobility and decreased physical activity. The modified short questionnaire to assess health-enhancing physical activity (mSQUASH) was developed to assess daily physical activity in patients with axSpA.
Objective: To translate, cross-culturally adapt and linguistically validate the original mSQUASH into German for patients with axSpA.
Methods: The original mSQUASH was translated from Dutch into German using a multistep process (Beaton method) with forward-backward translations into German by bilingual Dutch-German lay people and experts. Any remaining discrepancies were resolved by a scientific committee, resulting in a prefinal German version. Field testing with cognitive debriefing interviews with patients with axSpA from diverse backgrounds led to a final German version.
Results: Minor discrepancies, primarily related to formalities, semantic errors and syntax were found during translations. These were addressed, resulting in slight wording modifications. The prefinal German version was validated through cognitive debriefing by 10 patients with axSpA, confirming its linguistic validity and equivalence to the Dutch version.
Conclusion: Overall, this study confirmed the final German mSQUASH as a comprehensive measurement instrument for daily physical activity. It can now be used as a patient-reported outcome by German patients with axSpA. This can enable cross-linguistic comparisons and expanding its utility across language barriers.
{"title":"[German translation and linguistic validation of the modified short questionnaire to assess health-enhancing physical activity (mSQUASH) for patients with axial spondyloarthritis (axSpA)].","authors":"David Kiefer, Kristina Vaupel, Uta Kiltz, Ludwig Hammel, Yvonne M van der Kraan, Suzanne Arends, Xenofon Baraliakos","doi":"10.1007/s00393-024-01508-9","DOIUrl":"https://doi.org/10.1007/s00393-024-01508-9","url":null,"abstract":"<p><strong>Background: </strong>Patients with axial spondyloarthritis (axSpA) often experience chronic pain and inflammation, resulting in physical impairments, reduced mobility and decreased physical activity. The modified short questionnaire to assess health-enhancing physical activity (mSQUASH) was developed to assess daily physical activity in patients with axSpA.</p><p><strong>Objective: </strong>To translate, cross-culturally adapt and linguistically validate the original mSQUASH into German for patients with axSpA.</p><p><strong>Methods: </strong>The original mSQUASH was translated from Dutch into German using a multistep process (Beaton method) with forward-backward translations into German by bilingual Dutch-German lay people and experts. Any remaining discrepancies were resolved by a scientific committee, resulting in a prefinal German version. Field testing with cognitive debriefing interviews with patients with axSpA from diverse backgrounds led to a final German version.</p><p><strong>Results: </strong>Minor discrepancies, primarily related to formalities, semantic errors and syntax were found during translations. These were addressed, resulting in slight wording modifications. The prefinal German version was validated through cognitive debriefing by 10 patients with axSpA, confirming its linguistic validity and equivalence to the Dutch version.</p><p><strong>Conclusion: </strong>Overall, this study confirmed the final German mSQUASH as a comprehensive measurement instrument for daily physical activity. It can now be used as a patient-reported outcome by German patients with axSpA. This can enable cross-linguistic comparisons and expanding its utility across language barriers.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140877506","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}
Pub Date : 2024-05-08DOI: 10.1007/s00393-024-01519-6
Wolfgang A Schmidt, Michael Czihal, Michael Gernert, Wolfgang Hartung, Bernhard Hellmich, Sarah Ohrndorf, Gabriela Riemekasten, Valentin S Schäfer, Johannes Strunk, Nils Venhoff
An expert committee recommends defining fast-track clinics (FTC) for the acute diagnostics of giant cell arteritis (GCA) as follows: low-threshold, easy and prompt reachability at least on weekdays, scheduling appointments ideally within 24 h, examination by a specialist with GCA expertise, ≥ 2 experts per FTC, ≥ 50 patients with suspected GCA per year, sonologists with ≥ 300 (≥ 50) temporal and axillary artery examinations, adherence to standard operating procedures, availability of an ≥ 18 (≥ 15) MHz and a lower frequency linear ultrasound probe and collaboration with partners for fast performance of neurological and ophthalmological examinations, magnetic resonance imaging (MRI), positron emission tomography-computed tomography (PET-CT, possibly CT) and for temporal artery biopsy.
{"title":"[Recommendations for defining giant cell arteritis fast-track clinics].","authors":"Wolfgang A Schmidt, Michael Czihal, Michael Gernert, Wolfgang Hartung, Bernhard Hellmich, Sarah Ohrndorf, Gabriela Riemekasten, Valentin S Schäfer, Johannes Strunk, Nils Venhoff","doi":"10.1007/s00393-024-01519-6","DOIUrl":"10.1007/s00393-024-01519-6","url":null,"abstract":"<p><p>An expert committee recommends defining fast-track clinics (FTC) for the acute diagnostics of giant cell arteritis (GCA) as follows: low-threshold, easy and prompt reachability at least on weekdays, scheduling appointments ideally within 24 h, examination by a specialist with GCA expertise, ≥ 2 experts per FTC, ≥ 50 patients with suspected GCA per year, sonologists with ≥ 300 (≥ 50) temporal and axillary artery examinations, adherence to standard operating procedures, availability of an ≥ 18 (≥ 15) MHz and a lower frequency linear ultrasound probe and collaboration with partners for fast performance of neurological and ophthalmological examinations, magnetic resonance imaging (MRI), positron emission tomography-computed tomography (PET-CT, possibly CT) and for temporal artery biopsy.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140877507","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}
Pub Date : 2024-05-01Epub Date: 2023-08-11DOI: 10.1007/s00393-023-01395-6
Fabian Proft, Diana Vossen, Xenofon Baraliakos, Michael N Berliner, Martin Fleck, Gernot Keyßer, Andreas Krause, Hanns-Martin Lorenz, Bernhard Manger, Florian Schuch, Christof Specker, Jürgen Wollenhaupt, Anna Voormann, Matthias Raspe, Martin Krusche, Alexander Pfeil
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.
{"title":"[Survey on the working, training, and research conditions of resident physicians in internistic and rheumatological continuing education-BEWUSST].","authors":"Fabian Proft, Diana Vossen, Xenofon Baraliakos, Michael N Berliner, Martin Fleck, Gernot Keyßer, Andreas Krause, Hanns-Martin Lorenz, Bernhard Manger, Florian Schuch, Christof Specker, Jürgen Wollenhaupt, Anna Voormann, Matthias Raspe, Martin Krusche, Alexander Pfeil","doi":"10.1007/s00393-023-01395-6","DOIUrl":"10.1007/s00393-023-01395-6","url":null,"abstract":"<p><strong>Background: </strong>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.</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11058970/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9974884","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}
Pub Date : 2024-05-01Epub Date: 2023-06-06DOI: 10.1007/s00393-023-01371-0
Nikolas Ruffer, Martin Krusche, Konstanze Holl-Ulrich, Fabian Lötscher, Ina Kötter
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.
{"title":"[Hughes-Stovin syndrome: a life-threatening manifestation of Behçet's syndrome].","authors":"Nikolas Ruffer, Martin Krusche, Konstanze Holl-Ulrich, Fabian Lötscher, Ina Kötter","doi":"10.1007/s00393-023-01371-0","DOIUrl":"10.1007/s00393-023-01371-0","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11058631/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9940995","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}
Pub Date : 2024-05-01Epub Date: 2023-08-24DOI: 10.1007/s00393-023-01402-w
M Liebisch, N El Hamrawi, M Dufour, F Nöllner, V Krenn
Background: The tenosynovial giant cell tumor (pigmented villonodular synovitis) is a proliferative, mainly benign soft tissue tumor of the tendon sheaths, bursae and joints arising from the synovia. It can be divided into circumscribed localized and destructive diffuse types. Approximately 1% of all joint diseases are due to this entity. The tumor is considered as a rarity. Mostly case studies exist. For this study the focus was set on the localized type (L-TSRZT), which accounts for 90% of the diagnoses of this tumor. Given its rarity, data are limited. Therefore, the research aim was to provide data on prevalence, primary location and sensitivity of clinical versus histopathological diagnosis in a German sample.
Methods: Based on the Histopathological Arthritis Register of the German Society for Orthopedic Rheumatology, the data of the L‑TSRZT were retrospectively analyzed (time frame 1 January 2018-28 December 2020).
Results: This database contained N = 7595 cases of arthropathy. A total of n = 45 patients with the diagnosis L‑TSRZT were identified. The prevalence of the tumor was 0.6%, 95% CI [0.4%, 0.8%], or 5.9 cases per 1000. The primary location involved the finger (48.9%). In 14 of 45 cases the diagnosis was correctly determined from the clinical side, corresponding to a sensitivity of 31.1%, 95% CI [18.2%, 46.7%].
Conclusion: For the first time, this paper was able to provide data on a large sample for Germany. Notably, the low sensitivity of the clinical diagnosis confirms the importance of histopathology for diagnosing L‑TSRZT.
背景:腱鞘巨细胞瘤(色素性绒毛滑膜炎腱鞘巨细胞瘤(色素性绒毛滑膜炎)是腱鞘、滑囊和关节滑膜上的一种增生性软组织肿瘤,主要为良性。它可分为环状局部型和破坏性弥漫型。在所有关节疾病中,约有 1%是由这种肿瘤引起的。这种肿瘤非常罕见。大多数情况下都是病例研究。本研究的重点是局部型(L-TSRZT),它占该肿瘤诊断的 90%。鉴于其罕见性,相关数据十分有限。因此,研究的目的是提供德国样本的发病率、原发部位以及临床诊断与组织病理学诊断的敏感性方面的数据:基于德国骨科风湿病学会组织病理学关节炎登记册,对L-TSRZT的数据进行了回顾性分析(时间范围为2018年1月1日至2020年12月28日):该数据库包含N = 7595例关节病病例。共发现 n = 45 例诊断为 L-TSRZT 的患者。肿瘤发病率为 0.6%,95% CI [0.4%,0.8%],即每 1000 人中有 5.9 例。原发部位涉及手指(48.9%)。在 45 个病例中,有 14 个病例的临床诊断正确,灵敏度为 31.1%,95% CI [18.2%,46.7%]:本文首次为德国提供了大量样本数据。值得注意的是,临床诊断的低敏感性证实了组织病理学对诊断 L-TSRZT 的重要性。
{"title":"[Localized tenosynovial giant cell tumor : Results from the Histopathological Arthritis Register of the German Society for Orthopedic Rheumatology].","authors":"M Liebisch, N El Hamrawi, M Dufour, F Nöllner, V Krenn","doi":"10.1007/s00393-023-01402-w","DOIUrl":"10.1007/s00393-023-01402-w","url":null,"abstract":"<p><strong>Background: </strong>The tenosynovial giant cell tumor (pigmented villonodular synovitis) is a proliferative, mainly benign soft tissue tumor of the tendon sheaths, bursae and joints arising from the synovia. It can be divided into circumscribed localized and destructive diffuse types. Approximately 1% of all joint diseases are due to this entity. The tumor is considered as a rarity. Mostly case studies exist. For this study the focus was set on the localized type (L-TSRZT), which accounts for 90% of the diagnoses of this tumor. Given its rarity, data are limited. Therefore, the research aim was to provide data on prevalence, primary location and sensitivity of clinical versus histopathological diagnosis in a German sample.</p><p><strong>Methods: </strong>Based on the Histopathological Arthritis Register of the German Society for Orthopedic Rheumatology, the data of the L‑TSRZT were retrospectively analyzed (time frame 1 January 2018-28 December 2020).</p><p><strong>Results: </strong>This database contained N = 7595 cases of arthropathy. A total of n = 45 patients with the diagnosis L‑TSRZT were identified. The prevalence of the tumor was 0.6%, 95% CI [0.4%, 0.8%], or 5.9 cases per 1000. The primary location involved the finger (48.9%). In 14 of 45 cases the diagnosis was correctly determined from the clinical side, corresponding to a sensitivity of 31.1%, 95% CI [18.2%, 46.7%].</p><p><strong>Conclusion: </strong>For the first time, this paper was able to provide data on a large sample for Germany. Notably, the low sensitivity of the clinical diagnosis confirms the importance of histopathology for diagnosing L‑TSRZT.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10068820","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}
Pub Date : 2024-05-01Epub Date: 2024-02-21DOI: 10.1007/s00393-024-01482-2
Jürgen Braun, Martin Krekeler, Uta Kiltz
Aim: For diseases caused by calcium pyrophosphate deposition (CPPD), validated classification criteria were previously lacking. In this article the recently developed and validated classification criteria are translated, explained, and assessed.
Methods: In recent years a multinational research group developed classification criteria for CPPD disease with the support by the European Alliance of Associations for Rheumatology (EULAR) and the American College of Rheumatology (ACR), following an established method. The developed criteria were finally validated in an independent cohort. The translation and annotation of the new first classification criteria were carried out in an iterative procedure in consensus with the authors.
Results: The presence of a crowned dens syndrome or calcium pyrophosphate crystals in the synovial fluid in patients with pain, swelling or sensitivity of the joints (entry criterion) is sufficient for the classification as CPPD disease, where the symptoms cannot be completely explained by another rheumatic disease (exclusion criterion). If these symptoms are not present, a count of more than 56 points based on weighted criteria comprised of clinical features and the results of laboratory and imaging investigations can be included for classification as a CPPD disease. These criteria had a sensitivity of 92.2% and a specificity of 87.9% in the derivation cohorts (190 CPPD cases and 148 mimics), whereas the sensitivity was 99.2% and the specificity 92.5% in the validation cohorts (251 CPPD cases and 162 mimics).
Conclusion: The ACR/EULAR classification criteria 2023 of a CPPD disease will facilitate clinical research in this field. The use in the clinical routine will show how practical the criteria are.
{"title":"[First classification criteria for diseases caused by calcium pyrophosphate deposition (CPPD)-Translation, explanation and assessment].","authors":"Jürgen Braun, Martin Krekeler, Uta Kiltz","doi":"10.1007/s00393-024-01482-2","DOIUrl":"10.1007/s00393-024-01482-2","url":null,"abstract":"<p><strong>Aim: </strong>For diseases caused by calcium pyrophosphate deposition (CPPD), validated classification criteria were previously lacking. In this article the recently developed and validated classification criteria are translated, explained, and assessed.</p><p><strong>Methods: </strong>In recent years a multinational research group developed classification criteria for CPPD disease with the support by the European Alliance of Associations for Rheumatology (EULAR) and the American College of Rheumatology (ACR), following an established method. The developed criteria were finally validated in an independent cohort. The translation and annotation of the new first classification criteria were carried out in an iterative procedure in consensus with the authors.</p><p><strong>Results: </strong>The presence of a crowned dens syndrome or calcium pyrophosphate crystals in the synovial fluid in patients with pain, swelling or sensitivity of the joints (entry criterion) is sufficient for the classification as CPPD disease, where the symptoms cannot be completely explained by another rheumatic disease (exclusion criterion). If these symptoms are not present, a count of more than 56 points based on weighted criteria comprised of clinical features and the results of laboratory and imaging investigations can be included for classification as a CPPD disease. These criteria had a sensitivity of 92.2% and a specificity of 87.9% in the derivation cohorts (190 CPPD cases and 148 mimics), whereas the sensitivity was 99.2% and the specificity 92.5% in the validation cohorts (251 CPPD cases and 162 mimics).</p><p><strong>Conclusion: </strong>The ACR/EULAR classification criteria 2023 of a CPPD disease will facilitate clinical research in this field. The use in the clinical routine will show how practical the criteria are.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":null,"pages":null},"PeriodicalIF":1.0,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11058609/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139913590","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}