Journal of Rheumatic Diseases最新文献

{"title":"Modified Body Mass Index at Diagnosis is a Useful Predictor of Mortality in Patients With Antineutrophil Cytoplasmic Antibody-associated Vasculitis.","authors":"Jung Yoon Pyo,&nbsp;Sung Soo Ahn,&nbsp;Jason Jungsik Song,&nbsp;Yong-Beom Park,&nbsp;Sang-Won Lee","doi":"10.4078/jrd.2022.29.3.154","DOIUrl":"10.4078/jrd.2022.29.3.154","url":null,"abstract":"<p><strong>Objective: </strong>We investigated whether modified body mass index (mBMI) at diagnosis could predict all-cause mortality during follow-up in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).</p><p><strong>Methods: </strong>The medical records of 203 AAV patients with BMI ≥18.5 kg/m² were reviewed. mBMI was calculated using an equation mBMI=BMI (kg/m²)×serum albumin (g/L). All-cause mortality was considered as a poor outcome, and the follow-up duration based on all-cause mortality was defined as the period from AAV diagnosis to death for deceased patients, and the period from AAV diagnosis to the last visit for surviving patients.</p><p><strong>Results: </strong>The median age was 59.0 years (35.5% were male). The median BMI and mBMI were 22.8 kg/m² and 813.2 kg · g/m² · L. Twenty-five patients (12.3%) died. mBMI was well correlated with age, BVAS, FFS, erythrocyte sedimentation rate and C-reactive protein at diagnosis. Deceased patients exhibited significantly lower mBMI at diagnosis compared to surviving patients. AAV patients mBMI ≤570.1 kg · g/m² · L showed a significantly higher frequency of all-cause mortality (38.5% vs. 8.5%), and furthermore, exhibited a significantly higher risk for all-cause mortality than those with mBMI >570.1 kg · g/m² · L (RR 6.750). mBMI ≤570.1 kg · g/m² · L showed a significantly lower cumulative patients' survival rate than those with mBMI >570.1 kg · g/m² · L. In the multivariable Cox hazards model analysis, either serum albumin or mBMI was significantly associated with all-cause mortality in AAV patients.</p><p><strong>Conclusion: </strong>In conclusion, mBMI ≤570.1 kg · g/m² · L at diagnosis may be a useful predictor of all-cause mortality during follow-up additionally to serum albumin in AAV patients.</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/6b/d4/jrd-29-3-154.PMC10324925.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9904349","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Mechanism of the NLRP3 Inflammasome Activation and Pathogenic Implication in the Pathogenesis of Gout.","authors":"Seong-Kyu Kim","doi":"10.4078/jrd.2022.29.3.140","DOIUrl":"10.4078/jrd.2022.29.3.140","url":null,"abstract":"The NACHT, LRR, and PYD-domains-containing protein 3 (NLRP3) inflammasome is an intracellular multi-protein signaling platform that is activated by cytosolic pattern-recognition receptors such as NLRs against endogenous and exogenous pathogens. Once it is activated by a variety of danger signals, recruitment and assembly of NLRP3, ASC, and pro-caspase-1 trigger the processing and release of pro-inflammatory cytokines including interleukin-1β (IL-1β) and IL-18. Multiple intracellular and extracellular structures and molecular mechanisms are involved in NLRP3 inflammasome activation. Gout is an autoinflammatory disease induced by inflammatory response through production of NLRP3 inflammasome-mediated proinflammatory cytokines such as IL-1β by deposition of monosodium urate (MSU) crystals in the articular joints and periarticular structures. NLRP3 inflammasome is considered a main therapeutic target in MSU crystal-induced inflammation in gout. Novel therapeutic strategies have been proposed to control acute flares of gouty arthritis and prophylaxis for gout flares through modulation of the NLRP3/IL-1 axis pathway. This review discusses the basic mechanism of NLRP3 inflammasome activation and the IL-1-induced inflammatory cascade and explains the NLRP3 inflammasome-induced pathogenic role in the pathogenesis of gout.","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/ad/6a/jrd-29-3-140.PMC10324924.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9851145","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Use of Disease-modifying Antirheumatic Drugs After Cancer Diagnosis in Rheumatoid Arthritis Patients.","authors":"Young Bin Joo,&nbsp;Seung Min Jeong,&nbsp;Yune-Jung Park,&nbsp;Ki-Jo Kim,&nbsp;Kyung-Su Park","doi":"10.4078/jrd.2022.29.3.162","DOIUrl":"https://doi.org/10.4078/jrd.2022.29.3.162","url":null,"abstract":"<p><strong>Objective: </strong>There is no recommendation for the use of disease-modifying antirheumatic drugs (DMARDs) in patients with rheumatoid arthritis (RA) who developed cancer. We examined changes in the DMARDs prescription patterns associated with cancer diagnosis in RA patients.</p><p><strong>Methods: </strong>We reviewed the medical records of 2,161 RA patients who visited rheumatology clinic between January 2008 and February 2017 and found 40 patients who developed cancer during RA treatment. In these patients, we examined DMARDs prescription patterns before and right after cancer diagnosis and at recent outpatient clinic visits.</p><p><strong>Results: </strong>Before cancer diagnosis, methotrexate (MTX)-combined conventional synthetic DMARDs (csDMARDs) were most commonly prescribed (22, 55.0%) and biological DMARDs (biologics) in nine patients (22.5%). For cancer treatment, 19 patients received chemotherapy (including adjuvant chemotherapy) and 21 patients had surgery only. Right after cancer diagnosis, changes in the DMARDs prescription patterns were similar in discontinuation (13, 32.5%), switching (14, 35.0%), and maintenance (13, 32.5%). DMARDs were discontinued more frequently in the chemotherapy group (9/19, 47.4%) than the surgery only group (4/2, 19.0%) (p<0.05). Among the 13 patients who discontinued DMARDs, nine (69.2%) resumed DMARDs after a median of 5.5 months (interquartile range [IQR] 2.9, 18.3) due to arthritis flare. At a median of 4.6 years (IQR 3.3, 6.7) after cancer diagnosis, 25 patients were evaluated at recent outpatient clinic visits. Four patients received no DMARD, three MTX monotherapies, 11 csDMARDs combination therapies, and seven biologics.</p><p><strong>Conclusion: </strong>A significant number of RA patients who developed cancer during RA treatment were still receiving DMARDs including biologics after cancer diagnosis.</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/8f/b5/jrd-29-3-162.PMC10324922.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10208259","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical Implications of Shared Epitope and Anti-citrullinated Peptide Antibody in Patients With Rheumatoid Arthritis.","authors":"Seung Min Jung,&nbsp;Yune-Jung Park,&nbsp;Kyung-Su Park,&nbsp;Ki-Jo Kim","doi":"10.4078/jrd.2022.29.3.171","DOIUrl":"https://doi.org/10.4078/jrd.2022.29.3.171","url":null,"abstract":"<p><strong>Objective: </strong>The shared epitope (SE) and anti-citrullinated peptide antibody (ACPA) are involved in the pathogenesis of rheumatoid arthritis (RA). This study evaluated the clinical implications of SE and ACPA in terms of disease manifestation and response to biologic disease modifying anti-rheumatic drugs (DMARDs).</p><p><strong>Methods: </strong>Patients with identified human leukocyte antigen (HLA)-DRB1 alleles were included to compare the clinical characteristics and drug survival rate of tumor necrosis factor (TNF) inhibitors or abatacept based on the presence of SE and ACPA.</p><p><strong>Results: </strong>Of the 533 patients with identified HLA-DRB1 alleles, 329 patients (61.7%) with SE alleles showed higher disease activity and erosive changes compared to patients without SE alleles. SE-positive patients were more likely to start biologic (b-) or targeted synthetic DMARDs (tsDMARDs) within the first 5 years (p=0.020). The presence of SE, smoking, dyslipidemia, and higher erythrocyte sedimentation rate were independently associated with the initiation of b- or tsDMARDs (p=0.016, 0.028, 0.031, and 0.001, respectively). The presence of SE and ACPA did not affect the drug survival rate of TNF inhibitors, whereas the abatacept retention rate was higher in ACPA-positive patients (p=0.024).</p><p><strong>Conclusion: </strong>The presence of SE affected disease characteristics and prognosis in Korean patients with RA without a significant impact on drug survival rate of TNF inhibitors and abatacept. ACPA positivity was associated with abatacept drug retention, suggesting that abatacept may be helpful in ACPA-positive patients than in ACPA-negative patients.</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/c8/2a/jrd-29-3-171.PMC10324929.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9851148","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Idiopathic Inflammatory Arthritis in the Auditory Canal in a Patient With Hearing Impairment: A Case Report and Literature Review.","authors":"So Hye Nam,&nbsp;Gi Hwan Kim,&nbsp;Hong Ju Park,&nbsp;Yong-Gil Kim","doi":"10.4078/jrd.2022.29.2.116","DOIUrl":"10.4078/jrd.2022.29.2.116","url":null,"abstract":"<p><p>Inflammatory arthritis can affect the auditory system during the disease course. Although most cases show asymptomatic hearing impairment, it can result in hearing loss. Here we describe the case of a 70-year-old female with hearing impairment associated with idiopathic inflammatory arthritis in her auditory system. She had suffered from hearing difficulties for decades; however, the causes of her hearing impairment had not been evaluated. Pure tone audiometry showed severe sensorineural hearing loss requiring a cochlear implant. The workup for the cochlear implant revealed erosive changes in the incudomalleolar and incudostapedial joints with soft tissue swelling on temporal bone computed tomography. Bone pathology revealed plasmacytic infiltration and granulomatous inflammation. Laboratory examinations showed elevated levels of inflammatory markers; otherwise, she had negative results for all autoantibodies. In patients with idiopathic hearing loss, inflammatory arthritis of the middle ear without peripheral arthritis can provide a clue regarding the cause of the hearing loss.</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/49/43/jrd-29-2-116.PMC10327615.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9850267","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Korean College of Rheumatology: Forty Years of Excellence.","authors":"Jung-Yoon Choe,&nbsp;Tae-Hwan Kim","doi":"10.4078/jrd.2022.29.2.71","DOIUrl":"https://doi.org/10.4078/jrd.2022.29.2.71","url":null,"abstract":"Rheumatology in Korea began as an independent field in October 1979, when senior professors of orthopedics, internal medicine, and pediatrics held a preparatory meeting at the Scandinavian Club in the National Medical Center [1]. In November 1980, 21 promoters held a general meeting of the Korean Rheumatism Association, and in January 1981, the inaugural general meeting and a commemorative symposium were held in Seoul National University Hospital. Professor Moon-Sik Han served as the president of the KCR for 10 years from the launching of the society. Since then, the Association has held two academic conferences every year. In November 1990, the first Asian Pacific League Against Rheumatism (APLAR) Seoul Symposium was held in Seoul, and 400 people from more than 20 countries attended. The Korean Rheumatism Association joined the Korean Medical Association Branch Science Council (currently the Korean Medical Association) as an associate member in 1983 and was approved as a full member in 2000. It was renamed the Korean College of Rheumatology in 2010 and adopted a new logo, which remains in use.","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/d7/90/jrd-29-2-71.PMC10327619.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9850263","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Differential Diagnosis of Inflammatory Arthropathy Accompanying Active Tuberculosis Infection.","authors":"Youjin Jung,&nbsp;Byoong Yong Choi","doi":"10.4078/jrd.2022.29.2.108","DOIUrl":"https://doi.org/10.4078/jrd.2022.29.2.108","url":null,"abstract":"<p><strong>Objective: </strong>The study aimed to ascertain the clinical manifestations of inflammatory arthritis accompanying tuberculosis (TB) for the differential diagnosis.</p><p><strong>Methods: </strong>We retrospectively reviewed patients with active TB who presented with inflammatory arthropathy at Seoul Medical Center. Among 2,872 patients with active TB infection, 47 had inflammatory arthropathy 14 had crystal-induced arthropathy; 12, TB arthritis; 12, Poncet's disease (PD); 8, Rheumatoid arthritis (RA); and 1, septic arthritis. The clinical characteristics and laboratory and radiographic findings of each group were analyzed.</p><p><strong>Results: </strong>In TB arthritis, weight-bearing joints were more commonly affected than the elbow and wrist joints. When compared to TB arthritis, PD demonstrated a significantly higher proportion of polyarthritis and involved both large and small-to-medium-sized joints. The duration of arthritis symptoms after anti-TB treatment was significantly shorter in patients with PD (56 days vs. 90 days, p=0.028). When compared to PD, RA flares during active TB infection involved only small-to-medium-sized joints rather than a mixed distribution (62.5% vs. 16.7%, p=0.035). Patients with PD more commonly had fever at onset and showed a good response to nonsteroidal anti-inflammatory drugs alone or were in remission within 3 months after anti-TB treatment. The presence of rheumatoid factor or anti-cyclic citrullinated peptide and radiographic progression after 12 months was frequently observed in patients with RA flares.</p><p><strong>Conclusion: </strong>The differential diagnosis of inflammatory arthritis accompanying active tuberculosis infection is challenging. Comprehensive history taking and physical examination, synovial fluid analysis, and a high level of clinical suspicion are essential to avoid delayed diagnosis and to reduce the significant morbidity involved.</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/f6/ed/jrd-29-2-108.PMC10327614.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9850266","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Recent Advances in Basic and Clinical Aspects of Rheumatoid Arthritis-associated Interstitial Lung Diseases.","authors":"Hanna Lee,&nbsp;Sang-Il Lee,&nbsp;Hyun-Ok Kim","doi":"10.4078/jrd.2022.29.2.61","DOIUrl":"https://doi.org/10.4078/jrd.2022.29.2.61","url":null,"abstract":"<p><p>Rheumatoid arthritis (RA) is a common autoimmune disease that mainly affects the joints and systemic organs, such as the skin, eyes, heart, gastrointestinal tract, and lungs. In particular, among various pulmonary involvements, interstitial lung disease (ILD) is closely related to the selection of anti-rheumatic drugs and the long-term prognosis of patients with RA. Although the exact pathogenesis of RA-ILD is not well defined, several mechanistic pathways, similar to those of idiopathic pulmonary fibrosis, have been elucidated recently. Conversely, RA-related autoantibodies, including anti-cyclic citrullinated peptide antibody, are detectable in circulation and in the lungs, even in the absence of articular symptoms. RA-ILD can also predate years before the occurrence of joint symptoms. This evidence supports the fact that local dysregulated mucosal immunity in the lung causes systemic autoimmunity, resulting in clinically evident polyarthritis of RA. Because the early diagnosis of RA-ILD is important, imaging tests, such as computed tomography and pulmonary function tests, are being used for early diagnosis, but there is no clear guideline for the early diagnosis of RA-ILD and selection of optimal disease-modifying anti-rheumatic drugs for the treatment of patients with RA with ILD. In addition, the efficacy of nintedanib, a new anti-fibrotic agent, for RA-ILD treatment, has been investigated recently. This review collectively discusses the basic and clinical aspects, such as pathogenesis, animal models, diagnosis, and treatment, of RA-ILD.</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/a9/ef/jrd-29-2-61.PMC10327618.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9928413","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Rituximab can Decrease Proteinuria in Refractory Lupus Nephritis.","authors":"Chang-Hee Suh","doi":"10.4078/jrd.2022.29.2.59","DOIUrl":"https://doi.org/10.4078/jrd.2022.29.2.59","url":null,"abstract":"www.jrd.or.kr Recently, there is good news for the patients with lupus nephritis (LN), which the US Food and Drug Administration approved 2 medications (belimumab and voclosporin) for the treatment of active LN [1]. Two new drugs showed better renal response than placebo in the LN patients in addition to standard therapy (steroids and such as mycophenolate mofetil (MMF) and low-dose cyclophosphamide) with favorable safety profiles [2,3]. However, the primary efficacy renal response (a ratio of urinary protein to creatinine (UPCR) of ≤0.7, preserved estimated glomerular filtration, and no use of rescue therapy) is only 43% in 104 weeks of the belimumab treatment with standard therapy [2] and the complete renal response (a UPCR ≤0.5 mg/mg, stable renal function, and no administration of rescue medication) is 41% in 52 weeks of the voclosporin treatment with standard therapy [3]. Therefore, there is still a need of medication for the treatment of patients with LN. Considering the key role that B lymphocytes play in the pathogenesis of the LN, the chimeric monoclonal anti-CD20 antibody (rituximab) has been studied in many observational studies and one randomized controlled trial (RCT) of patients with LN. Generally, the metanalysis of observational studies showed consistent clinical benefit with good response rate (40% complete response and 34% partial response) in refractory LN [4]. However, the only large RCT failed to meet primary endpoint [5]. In spite of this discouraging result, rituximab has been recommended for the patients with refractory LN in the guidelines of the joint European League Against Rheumatism and European Renal Association-European Dialysis and Transplant Association [6]. In addition, recent meta-analysis revealed clear beneficial effects of rituximab in patients with refractory LN [7]. A recent article by Choi et al. [8] published in the Journal of Rheumatic Disease report the experience of rituximab treatment for the patients with refractory or relapsing LN in a single tertiary referral hospital in Korea. Among 22 patients with LN treated with rituximab, 10 patients (45.5%) and 12 patients (54.5%) achieved renal response (complete or partial) at 6 months and 12 months, respectively. When the patients with LN were compared according to the glomerular filtration rate (GFR), the renal response rate at 12 months was higher in patients with normal GFR (≥90 mL/min/1.73 m) than those with decreased GFR (90.9% vs. 18.2%, p<0.001). It is interesting and understandable, however there are several unbalanced findings. First, the refractory LN is more frequent in patients with decreased GFR. Second, although the use of immunosuppressant is not different statistically, cyclophosphamide was used only in patients with decrease GFR, and MMF and Tacrolimus were more commonly used in patients with normal GFR before the start of rituximab. Third, the UPCR is much higher in the patients with decreased GFR than those with normal GFR (3,110 mg/mg v","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/06/c4/jrd-29-2-59.PMC10327617.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10232907","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Case of Macrophage Activation Syndrome During the Treatment of Adult-onset Still's Disease With Tocilizumab.","authors":"Ju Ho Lee,&nbsp;You-Jung Ha,&nbsp;Eun Ha Kang,&nbsp;Sung Hae Chang,&nbsp;Yun Jong Lee,&nbsp;Sup,&nbsp;Sup","doi":"10.4078/jrd.2022.29.2.123","DOIUrl":"10.4078/jrd.2022.29.2.123","url":null,"abstract":"<p><p>Macrophage activation syndrome (MAS) is a fatal complication of adult-onset Still's disease (AOSD). Although anti-cytokine agents have been recommended for refractory AOSD or complicated with MAS, MAS cases have been rarely reported during anti-cytokine treatment. Herein, we describe the first AOSD case complicated with MAS during the treatment with tocilizumab in Korea. Two years after tocilizumab maintenance therapy, high fever and hypertransaminasemia recurred. MAS was diagnosed based on hyperferritinemia, elevated soluble IL-2 receptor levels, and the presence of hemophagocytic histiocytes in the bone marrow. However, she had normal white blood cell counts and acute phase reactant levels. High-dose glucocorticoid and anakinra therapies were not effective, but her disease improved with etoposide. This case shows that tocilizumab may not prevent MAS development and can modify clinical features making it challenging to diagnose. Cytotoxic therapy such as etoposide may be required in MAS cases that develop during anti-cytokine therapy.</p>","PeriodicalId":56161,"journal":{"name":"Journal of Rheumatic Diseases","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2022-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/df/34/jrd-29-2-123.PMC10327616.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9850268","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}