Pub Date : 2023-03-01DOI: 10.1016/j.berh.2023.101848
Francesca Oliviero , Brian F. Mandell
Synovial fluid analysis can provide a prompt and definite diagnosis of crystal-induced arthritis, the most common acute inflammatory arthritis and a cause of chronic arthritis that may mimic rheumatoid, psoriatic, or peripheral spondyloarthritis. In many patients the diagnosis of gout or calcium pyrophosphate arthritis cannot be made with certainty without synovial fluid analysis. Additional information from fluid analysis can assist the clinician in honing the differential diagnosis of non-crystalline arthritis.
{"title":"Synovial fluid analysis: Relevance for daily clinical practice","authors":"Francesca Oliviero , Brian F. Mandell","doi":"10.1016/j.berh.2023.101848","DOIUrl":"10.1016/j.berh.2023.101848","url":null,"abstract":"<div><p>Synovial fluid analysis can provide a prompt and definite diagnosis of crystal-induced arthritis, the most common acute inflammatory arthritis and a cause of chronic arthritis that may mimic rheumatoid, psoriatic, or peripheral spondyloarthritis. In many patients the diagnosis of gout or calcium pyrophosphate arthritis cannot be made with certainty without synovial fluid analysis. Additional information from fluid analysis can assist the clinician in honing the differential diagnosis of non-crystalline arthritis.</p></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":null,"pages":null},"PeriodicalIF":5.2,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1521694223000347/pdfft?md5=2964ac879f6eb15f26dfc59b6b388395&pid=1-s2.0-S1521694223000347-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9769765","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-03-01DOI: 10.1016/j.berh.2023.101850
Sahil Koppikar , Pamela Diaz , Gurjit S. Kaeley , Lihi Eder
Musculoskeletal ultrasonography has become an increasingly valuable tool as a complement to the physical exam in rheumatology practice. Its point-of-care access, low cost, safety, portability, and reliability in trained hands, make this technique especially useful in patients with inflammatory arthritis. Growing evidence has demonstrated the value of musculoskeletal ultrasound in the detection of inflammatory and structural changes in patients with joint pain without obvious joint swelling, in differentiating various inflammatory diagnoses, in the monitoring of inflammatory arthritis, and interventional procedures. The potential role of ultrasound guiding treat-to-target strategies or tapering treatment in inflammatory arthritis requires further research. However, musculoskeletal ultrasound can also have pitfalls and limitations that a clinician should be aware of.
{"title":"Seeing is believing: Smart use of musculoskeletal ultrasound in rheumatology practice","authors":"Sahil Koppikar , Pamela Diaz , Gurjit S. Kaeley , Lihi Eder","doi":"10.1016/j.berh.2023.101850","DOIUrl":"10.1016/j.berh.2023.101850","url":null,"abstract":"<div><p><span><span>Musculoskeletal ultrasonography has become an increasingly valuable tool as a complement to the physical exam in </span>rheumatology<span> practice. Its point-of-care access, low cost, safety, portability, and reliability in trained hands, make this technique especially useful in patients<span> with inflammatory arthritis. Growing evidence has demonstrated the value of musculoskeletal ultrasound in the detection of inflammatory and structural changes in patients with </span></span></span>joint pain<span> without obvious joint swelling, in differentiating various inflammatory diagnoses, in the monitoring of inflammatory arthritis, and interventional procedures. The potential role of ultrasound guiding treat-to-target strategies or tapering treatment in inflammatory arthritis requires further research. However, musculoskeletal ultrasound can also have pitfalls and limitations that a clinician should be aware of.</span></p></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":null,"pages":null},"PeriodicalIF":5.2,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9855691","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-03-01DOI: 10.1016/j.berh.2023.101847
Wolfgang A. Schmidt
Rheumatologists are increasingly using vascular ultrasound. Several guidelines now recommend ultrasound as the first diagnostic modality in giant cell arteritis (GCA). The German curriculum for rheumatology training has recently included ultrasound for the acute diagnosis of vasculitis.
Recent studies have shown that ultrasound of temporal, axillary, subclavian, and vertebral arteries has sensitivities and specificities of >90%.
Vascular ultrasound detects subclinical GCA in approximately 20% of patients with “pure” polymyalgia rheumatica. GCA fast-track clinics might regularly include these patients.
A new score based on the intima-media thickness of the temporal and axillary arteries allows the monitoring of structural changes with treatment. The score decreases faster for the temporal arteries than it does for the axillary arteries.
Measuring the diameter of the ascending aorta and the aortic arch might become a fast and cost-effective tool for the long-term monitoring of aortic aneurysms in extracranial GCA.
Vascular ultrasound also has a role for Takayasu arteritis, thrombosis, Behçet's syndrome, and Raynaud's phenomenon.
{"title":"Vascular ultrasound in rheumatology practice","authors":"Wolfgang A. Schmidt","doi":"10.1016/j.berh.2023.101847","DOIUrl":"10.1016/j.berh.2023.101847","url":null,"abstract":"<div><p>Rheumatologists are increasingly using vascular ultrasound<span><span>. Several guidelines now recommend ultrasound as the first diagnostic modality in giant cell arteritis (GCA). The German curriculum for </span>rheumatology training has recently included ultrasound for the acute diagnosis of vasculitis.</span></p><p>Recent studies have shown that ultrasound of temporal, axillary, subclavian, and vertebral arteries has sensitivities and specificities of >90%.</p><p>Vascular ultrasound detects subclinical GCA in approximately 20% of patients with “pure” polymyalgia rheumatica. GCA fast-track clinics might regularly include these patients.</p><p>A new score based on the intima-media thickness of the temporal and axillary arteries<span><span> allows the monitoring of structural changes with treatment. The score decreases faster for the </span>temporal arteries than it does for the axillary arteries.</span></p><p><span>Measuring the diameter of the ascending aorta<span> and the aortic arch might become a fast and cost-effective tool for the long-term monitoring of </span></span>aortic aneurysms in extracranial GCA.</p><p><span><span>Vascular ultrasound also has a role for Takayasu arteritis, thrombosis, </span>Behçet's syndrome, and </span>Raynaud's phenomenon.</p></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":null,"pages":null},"PeriodicalIF":5.2,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10137738","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-03-01DOI: 10.1016/j.berh.2023.101853
Michael Toprover, Nicole Leung, Michael H. Pillinger
Accessing a joint with a needle (arthrocentesis) to extract synovial fluid is a skill intrinsic to the rheumatologist's praxis. Joint aspirations are essential for diagnosing or excluding septic joints, are the gold standard for diagnosing acute crystal arthritis, and can provide valuable information about the nature of other forms of arthritis. In appropriate settings, injecting medications into joints can provide rapid, temporary, or even prolonged relief of pain and swelling and can provide a window of relief until other treatment modalities (anti-inflammatories, immunomodulators, and physical therapy) can enforce durable responses. Soft tissue aspirations (e.g., of bursae) and soft tissue injections (of bursae, tendons, trigger points, and areas of nerve compression) can provide similar relief, earning the practitioner the gratitude of the patient. Here, we provide a primary on joint and soft tissue aspiration and injection, including indications for and against procedures, preparing for procedures, and approaches to specific musculoskeletal structures.
{"title":"Arthrocentesis and soft tissue aspiration and injection","authors":"Michael Toprover, Nicole Leung, Michael H. Pillinger","doi":"10.1016/j.berh.2023.101853","DOIUrl":"10.1016/j.berh.2023.101853","url":null,"abstract":"<div><p>Accessing a joint with a needle (arthrocentesis) to extract synovial fluid<span><span> is a skill intrinsic to the rheumatologist's praxis. Joint aspirations<span> are essential for diagnosing or excluding septic joints, are the gold standard for diagnosing acute crystal arthritis<span>, and can provide valuable information about the nature of other forms of arthritis. In appropriate settings, injecting medications into joints can provide rapid, temporary, or even prolonged relief of pain and swelling and can provide a window of relief until other treatment modalities (anti-inflammatories, </span></span></span>immunomodulators<span>, and physical therapy) can enforce durable responses. Soft tissue aspirations (e.g., of bursae) and soft tissue injections (of bursae, tendons, trigger points, and areas of nerve compression) can provide similar relief, earning the practitioner the gratitude of the patient. Here, we provide a primary on joint and soft tissue aspiration and injection, including indications for and against procedures, preparing for procedures, and approaches to specific musculoskeletal structures.</span></span></p></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":null,"pages":null},"PeriodicalIF":5.2,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9888439","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
This review overviews the challenges in the assessment of disease activity, damage, and therapy of Takayasu arteritis (TAK). Recently developed disease activity scores for TAK are more useful for follow-up visits and require validation of cut-offs for active disease. A validated damage score for TAK is lacking. Computed tomography angiography (CTA), magnetic resonance angiography (MRA), and ultrasound enable the evaluation of vascular anatomy and arterial wall characteristics of TAK. 18-fluorodeoxyglucose (18-FDG) positron emission tomography (PET) visualizes arterial wall metabolic activity and complements the information provided by circulating C-reactive protein (CRP) levels. ESR and CRP alone moderately reflect TAK disease activity. TAK is corticosteroid-responsive but relapses upon tapering corticosteroids. Conventional synthetic disease-modifying anti-rheumatic drugs (DMARDs) are the first-line maintenance agents, and tumor necrosis factor-alpha inhibitors, tocilizumab, or tofacitinib are second-line agents for TAK. Revascularization procedures for TAK should be used judiciously during periods of inactive disease.
本综述概述了评估高安动脉炎(TAK)的疾病活动性、损害和治疗所面临的挑战。最近开发的TAK疾病活动性评分对随访更有用,但需要对活动性疾病的临界值进行验证。目前还没有经过验证的 TAK 损伤评分。计算机断层扫描血管造影术(CTA)、磁共振血管造影术(MRA)和超声波检查可评估TAK的血管解剖和动脉壁特征。18-氟脱氧葡萄糖(18-FDG)正电子发射断层扫描(PET)可观察动脉壁的代谢活动,并补充循环C反应蛋白(CRP)水平提供的信息。仅血沉和 CRP 可适度反映 TAK 的疾病活动。TAK 对皮质类固醇有反应,但皮质类固醇减量后会复发。传统合成的改善病情抗风湿药(DMARDs)是一线维持治疗药物,肿瘤坏死因子-α抑制剂、托西珠单抗或托法替尼是治疗TAK的二线药物。在疾病处于非活动期时,应慎用TAK的血管重建手术。
{"title":"Management of Takayasu arteritis","authors":"Durga Prasanna Misra , Kritika Singh , Upendra Rathore , Chengappa G. Kavadichanda , Manish Ora , Neeraj Jain , Vikas Agarwal","doi":"10.1016/j.berh.2023.101826","DOIUrl":"10.1016/j.berh.2023.101826","url":null,"abstract":"<div><p><span><span><span>This review overviews the challenges in the assessment of disease activity, damage, and therapy of Takayasu arteritis<span> (TAK). Recently developed disease activity scores<span> for TAK are more useful for follow-up visits and require validation of cut-offs for active disease. A validated damage score for TAK is lacking. Computed tomography angiography<span> (CTA), magnetic resonance angiography<span> (MRA), and ultrasound enable the evaluation of vascular anatomy and arterial wall characteristics of TAK. 18-fluorodeoxyglucose (18-FDG) </span></span></span></span></span>positron emission tomography<span> (PET) visualizes arterial wall metabolic activity and complements the information provided by circulating C-reactive protein (CRP) levels. ESR and CRP alone moderately reflect TAK disease activity. TAK is corticosteroid-responsive but relapses upon tapering corticosteroids. Conventional synthetic disease-modifying anti-rheumatic drugs (DMARDs) are the first-line maintenance agents, and tumor necrosis factor-alpha inhibitors, tocilizumab, or </span></span>tofacitinib are second-line agents for TAK. </span>Revascularization procedures for TAK should be used judiciously during periods of inactive disease.</p></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":null,"pages":null},"PeriodicalIF":5.2,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9904514","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-03-01DOI: 10.1016/j.berh.2023.101830
Jason M. Springer , Tanaz A. Kermani
Giant cell arteritis (GCA) is a systemic, granulomatous, large-vessel vasculitis that affects individuals over the age of 50 years. Morbidity from disease includes cranial manifestations which can cause irreversible blindness, while extra-cranial manifestations can cause vascular damage with large-artery stenosis, occlusions, aortitis, aneurysms, and dissections. Glucocorticoids while efficacious are associated with significant adverse effects. Furthermore, despite treatment with glucocorticoids, relapses are common. An understanding of the pathogenesis of GCA has led to the discovery of tocilizumab as an efficacious steroid-sparing therapy while additional therapeutic targets affecting different inflammatory pathways are under investigation. Surgical treatment may be indicated in cases of refractory ischemia or aortic complications but data on surgical outcomes are limited. Despite the recent advances, many unmet needs exist, including the identification of patients or subsets of GCA who would benefit from earlier initiation of adjunctive therapies, patients who may warrant long-term immunosuppression and medications that sustain permanent remission. The impact of medications like tocilizumab on long-term outcomes, including the development of aortic aneurysms and vascular damage also warrants investigation.
{"title":"Recent advances in the treatment of giant cell arteritis","authors":"Jason M. Springer , Tanaz A. Kermani","doi":"10.1016/j.berh.2023.101830","DOIUrl":"10.1016/j.berh.2023.101830","url":null,"abstract":"<div><p><span><span>Giant cell arteritis<span> (GCA) is a systemic, granulomatous, large-vessel vasculitis that affects individuals over the age of 50 years. Morbidity from disease includes cranial manifestations which can cause irreversible </span></span>blindness<span><span>, while extra-cranial manifestations can cause vascular damage with large-artery stenosis, occlusions, </span>aortitis, aneurysms, and dissections. </span></span>Glucocorticoids<span><span><span> while efficacious are associated with significant adverse effects. Furthermore, despite treatment with glucocorticoids, relapses are common. An understanding of the pathogenesis of GCA has led to the discovery of </span>tocilizumab<span><span> as an efficacious steroid-sparing therapy while additional therapeutic targets affecting different inflammatory pathways are under investigation. Surgical treatment may be indicated in cases of refractory ischemia or aortic complications but data on surgical outcomes are limited. Despite the recent advances, many unmet needs exist, including the identification of patients or subsets of GCA who would benefit from earlier initiation of adjunctive therapies, patients who may warrant long-term </span>immunosuppression and medications that sustain permanent remission. The impact of medications like tocilizumab on long-term outcomes, including the development of </span></span>aortic aneurysms and vascular damage also warrants investigation.</span></p></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":null,"pages":null},"PeriodicalIF":5.2,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9999596","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-03-01DOI: 10.1016/j.berh.2023.101849
Vanessa Smith , Claudia Ickinger , Elvis Hysa , Marcus Snow , Tracy Frech , Alberto Sulli , Maurizio Cutolo
Nailfold capillaroscopy is a safe and well-established method for the assessment of structural alterations of the microcirculation. It is a crucial tool in the investigation and monitoring of patients presenting with Raynaud's phenomenon. Detection of the characteristic “scleroderma pattern” on capillaroscopy may indicate an underlying rheumatic disease, particularly systemic sclerosis (SSc). Herein, we highlight the practical aspects of videocapillaroscopy, including image acquisition and analysis, with mention of dermoscopy. Special emphasis is placed on standardized use of terminology to describe capillary characteristics. Systematic evaluation of images in discerning the normal from the abnormal using the validated European Alliance of Associations for Rheumatology (EULAR) Study Group consensus reporting framework is paramount. In addition to the relevance of capillaroscopy in the (very) early diagnosis of SSc, its emerging predictive value (especially capillary loss) for new organ involvement and disease progression is underscored. We further provide capillaroscopic findings in selected other rheumatic diseases.
{"title":"Nailfold capillaroscopy","authors":"Vanessa Smith , Claudia Ickinger , Elvis Hysa , Marcus Snow , Tracy Frech , Alberto Sulli , Maurizio Cutolo","doi":"10.1016/j.berh.2023.101849","DOIUrl":"10.1016/j.berh.2023.101849","url":null,"abstract":"<div><p>Nailfold capillaroscopy is a safe and well-established method for the assessment of structural alterations of the microcirculation. It is a crucial tool in the investigation and monitoring of patients presenting with Raynaud's phenomenon. Detection of the characteristic “scleroderma pattern” on capillaroscopy may indicate an underlying rheumatic disease, particularly systemic sclerosis (SSc). Herein, we highlight the practical aspects of videocapillaroscopy, including image acquisition and analysis, with mention of dermoscopy. Special emphasis is placed on standardized use of terminology to describe capillary characteristics. Systematic evaluation of images in discerning the normal from the abnormal using the validated European Alliance of Associations for Rheumatology (EULAR) Study Group consensus reporting framework is paramount. In addition to the relevance of capillaroscopy in the (very) early diagnosis of SSc, its emerging predictive value (especially capillary loss) for new organ involvement and disease progression is underscored. We further provide capillaroscopic findings in selected other rheumatic diseases.</p></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":null,"pages":null},"PeriodicalIF":5.2,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1521694223000359/pdfft?md5=f48f713e2065154c41476c9acce52822&pid=1-s2.0-S1521694223000359-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10137742","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-03-01DOI: 10.1016/j.berh.2023.101856
Mark A. Ahlman , Peter C. Grayson
The use of fluorodeoxyglucose–positron emission tomography (FDG-PET) imaging to detect vascular inflammation is increasingly common in the clinical management of patients with large-vessel vasculitis (LVV). In this review, the role of FDG-PET imaging to diagnose and monitor vascular disease activity will be detailed. Suggestions on incorporation of FDG-PET imaging into a clinical workflow will be provided with emphasis on patient preparation, image acquisition, and image interpretation. If FDG-PET imaging is obtained, multimodal imaging assessment, whereby FDG-PET imaging and non-invasive angiography are obtained concurrently, and correlation of imaging findings with clinical assessment is generally advisable. Considering the clinical scenario and treatment status of the patient is important when interpreting vascular FDG-PET image findings.
{"title":"Advanced molecular imaging in large-vessel vasculitis: Adopting FDG-PET into a clinical workflow","authors":"Mark A. Ahlman , Peter C. Grayson","doi":"10.1016/j.berh.2023.101856","DOIUrl":"10.1016/j.berh.2023.101856","url":null,"abstract":"<div><p><span>The use of fluorodeoxyglucose–positron emission tomography (FDG-PET) imaging to detect vascular inflammation is increasingly common in the clinical management of patients with large-vessel vasculitis (LVV). In this review, the role of FDG-PET imaging to diagnose and monitor vascular disease activity will be detailed. Suggestions on incorporation of FDG-PET imaging into a clinical workflow will be provided with emphasis on patient preparation, image acquisition, and image interpretation. If FDG-PET imaging is obtained, multimodal imaging assessment, whereby FDG-PET imaging and non-invasive </span>angiography<span> are obtained concurrently, and correlation of imaging findings with clinical assessment is generally advisable. Considering the clinical scenario and treatment status of the patient is important when interpreting vascular FDG-PET image findings.</span></p></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":null,"pages":null},"PeriodicalIF":5.2,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10265848","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2023-03-01DOI: 10.1016/j.berh.2023.101854
Kenneth S. O'Rourke, Mitsumasa Kishimoto
{"title":"Preface to practical procedures how to do","authors":"Kenneth S. O'Rourke, Mitsumasa Kishimoto","doi":"10.1016/j.berh.2023.101854","DOIUrl":"10.1016/j.berh.2023.101854","url":null,"abstract":"","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":null,"pages":null},"PeriodicalIF":5.2,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10362190","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Relapsing polychondritis (RP) is an uncommon inflammatory disorder that predominantly targets cartilaginous structures. The disease frequently affects the nose, ears, airways, and joints, but it can also impact organs that aren't primarily cartilage-based, such as blood vessels, skin, inner ear, and eyes. Given its infrequent occurrence and recurrent symptoms, patients often experience delays in proper diagnosis. Lately, based on the organs involved, the disease's diverse manifestations have been categorized into specific clinical groups, based on the most likely organ involvement including auricular, nasal, pulmonary, and musculoskeletal. More recently the discovery of a new disease, called (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) VEXAS syndrome, due to mutations in UBA1 gene, identified the cause of 8 % of the patients with a clinical diagnosis of RP. VEXAS is likely the cause of a previously described “hematologic subgroup” in RP. This discovery is proof of concept that RP is likely more than one disease (Beck et al., Dec 31 2020; Ferrada et al., 2021).
People diagnosed with RP face numerous hurdles, with the quality of their lives and overall prognosis being affected. Diagnosing the condition is particularly challenging due to its fluctuating symptoms, the absence of specific markers, and the lack of universally recognized classification criteria. For a correct diagnosis, it's imperative for healthcare professionals to identify its unique clinical patterns. Moreover, there are no approved metrics to gauge the disease's severity, complicating patient management.
This review seeks to equip clinicians with pertinent insights to better diagnose and attend to these complex patients.
{"title":"Relapsing polychondritis: Best Practice & Clinical Rheumatology","authors":"Phillip Mertz , Joshua Sparks , Dale Kobrin , Sandra Amara Ogbonnaya , Ecem Sevim , Clement Michet , Laurent Arnaud , Marcela Ferrada","doi":"10.1016/j.berh.2023.101867","DOIUrl":"10.1016/j.berh.2023.101867","url":null,"abstract":"<div><p><span>Relapsing polychondritis<span> (RP) is an uncommon inflammatory disorder that predominantly targets cartilaginous structures. The disease frequently affects the nose, ears, airways, and joints, but it can also impact organs that aren't primarily cartilage-based, such as blood vessels, skin, inner ear, and eyes. Given its infrequent occurrence and recurrent symptoms, patients often experience delays in proper diagnosis. Lately, based on the organs involved, the disease's diverse manifestations have been categorized into specific clinical groups, based on the most likely organ involvement including auricular, nasal, pulmonary, and musculoskeletal. More recently the discovery of a new disease, called (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) VEXAS syndrome, due to mutations in </span></span><em>UBA1</em> gene, identified the cause of 8 % of the patients with a clinical diagnosis of RP. VEXAS is likely the cause of a previously described “hematologic subgroup” in RP. This discovery is proof of concept that RP is likely more than one disease (Beck et al., Dec 31 2020; Ferrada et al., 2021).</p><p>People diagnosed with RP face numerous hurdles, with the quality of their lives and overall prognosis being affected. Diagnosing the condition is particularly challenging due to its fluctuating symptoms, the absence of specific markers, and the lack of universally recognized classification criteria. For a correct diagnosis, it's imperative for healthcare professionals to identify its unique clinical patterns. Moreover, there are no approved metrics to gauge the disease's severity, complicating patient management.</p><p>This review seeks to equip clinicians with pertinent insights to better diagnose and attend to these complex patients.</p></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":null,"pages":null},"PeriodicalIF":5.2,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41240699","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}