Journal of Rheumatic Diseases最新文献

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is an autoimmune rheumatic disease consisting of three discrete diagnoses of microscopic polyangiitis, granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis. Among diseases treated in a rheumatology department, AAV has poor clinical outcomes, with high rates of mortality and progression to end-stage renal disease and frequent disease relapse. Due to the frequent negative patient outcomes, optimal therapeutic strategies are essential in the management of AAV. In the present review, four guidelines for management of AAV are summarized: British Society for Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guideline for the management of adults with AAV; European League Against Rheumatism (EULAR)/European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) recommendation for the management of AAV; 2021 American College of Rheumatology (ACR)/Vasculitis Foundation Guideline for the Management of AAV; Kidney Disease: Improving Global Outcome (KDIGO) 2021 Clinical Practice Guideline for the Management of Glomerular Diseases, which will aid in clinicians' medical decisions. Finally, the summary of the 2022 Update of the EULAR Recommendations on the Management of AAV, presented in the EULAR Congress 2022 is also introduced.

Objective: Although nonsteroidal anti-inflammatory drugs (NSAIDs) are the first-line treatment for ankylosing spondylitis (AS), their effect on kidney function remains unclear. This longitudinal study investigated the correlation between long-term NSAID use and kidney function in patients with AS using electronic medical records.

Methods: The electronic medical records of 1,280 patients with AS collected from a single center between January 2001 and December 2018 were reviewed. The Assessment of Spondyloarthritis International Society (ASAS) NSAID Intake Score was used to determine the cumulative dose of all NSAIDs prescribed for a different time intervals. Each ASAS NSAID Intake Score was obtained for intervals of 6 months, 1 year, 2 years, 3 years, 5 years, and 10 years. The correlation between the ASAS NSAID Intake Score and final estimated glomerular filtration rate (eGFR) for each interval was investigated.

Results: The mean ASAS Intake Scores for 6-month, 1-year, 2-year, 3-year, 5-year, and 10-year intervals were 55.30, 49.28, 44.84, 44.14, 44.61, and 41.17, respectively. At each interval, the pearson correlation coefficients were -0.018 (95% CI -0.031 to -0.006, p=0.004), -0.021 (95% CI -0.039 to -0.004, p=0.018), -0.045 (95% CI -0.071 to -0.019, p=0.001), -0.069 (95% CI -0.102 to -0.037, p<0.001), -0.070 (95% CI -0.114 to -0.026, p=0.002), -0.019 (95% CI -0.099 to 0.062, p=0.645), respectively. There was a very weak negative relationship between ASAS Intake Score and eGFR at each interval.

Conclusion: Long-term NSAID use did not correlate with kidney function based on real-world data in patients with AS.

Objective: The aim of the study was to investigate the association between the levels of leptin in the circulating of individuals with rheumatoid arthritis (RA) and the severity of the disease.

Methods: We looked through the databases of Embase, Medline, and the Cochrane Library. We conducted a meta-analysis on the correlations between circulating leptin and the Disease Activity Score 28-erythrocyte sedimentation rate (DAS28-ESR) and C-reactive protein (CRP) levels in RA patients, as well as a meta-analysis of circulating or circulating leptin levels in RA patients.

Results: This meta-analysis study analyzed 42 different comparisons from 37 different publications, including a total of 2,350 patients with RA and 1,815 controls. The RA group had substantially higher leptin levels than the control group (standardized mean difference [SMD]=0.507, 95% confidence interval [CI]=0.309~0.704, p<0.001). The finding that RA patients had higher leptin levels was unaffected by sample size. The correlation between circulating leptin levels and DAS28 is statistically significant (correlation coefficient=0.247, 95% CI=0.087~0.396, p=0.003). Leptin levels are also correlated with CRP levels (correlation coefficient=0.203, 95% CI=0.048~0.349, p=0.010).

Conclusion: This comprehensive meta-analysis demonstrates that the circulating leptin levels of RA patients are elevated, and provides compelling evidence of the significant relationship between leptin levels and the activity of RA. The findings of this research suggest that leptin plays a significant role in the pathophysiology of this disease.

Objective: The sleep quality is worse in rheumatoid arthritis (RA) patients than in healthy controls and it is more difficult to achieve a satisfactory quality of life after treatment with age. Our aim is to assess the quality of life and sleep in elderly onset RA patients and to analyze the effect of disease-modifying agents on sleep and quality of life.

Methods: Thirty-four older patients with RA patients and 30 healthy controls are included in the study. Sleep quality was evaluated with the Pittsburg sleep quality index and quality of life with Short Form-36. Parametric/non-parametric tests and Spearman/Pearson correlation analysis were applied for the data according to the distribution.

Results: While the rate of poor sleep quality before treatment was 67.6%, the rate was 26.5% after treatment. There was a statistically significant difference before and after treatment in terms of subjective sleep quality, sleep latency, sleep duration, sleep efficiency, and scores for sleep disturbance. The mean steroid dose and Disease Activity Score-28 were higher in patients with poor sleep quality than in patients with good sleep quality. Patients with poor sleep quality had lower mean physical function, pain, general health, social function, emotional role difficulties, and energy/vitality values than patients with good sleep quality.

Conclusion: Both sleep and quality of life improved after treatment in older patients with RA patients. In older patients, it should be regularly evaluated in terms of sleep and quality of life and appropriate treatment should be provided.

Gout is the most common form of inflammatory arthritis that affects mainly middle-aged men, and there is clear evidence of an association between hyperuricemia and the risk for gout. Increasing prevalence of gout and hyperuricemia has been reported in many countries. The prevalence of gout and hyperuricemia are constantly increasing in Korea with the patients at risk for developing a variety of comorbidities. Although there have been studies on the association between gout or serum uric acid level and several neurodegenerative diseases, cancer, and cardiovascular mortality, the causal relationship between gout and these comorbidities are still unclear. The associations of substantial economic burden with hyperuricemia, gout attack, and suboptimal treatment are well known. Gout is a disease that requires lifelong management including lifestyle modification. However, gout is poorly managed worldwide although effective urate-lowering drugs exist. In this review, we addressed epidemiological studies and treatment-related problems in the Korean population with gout or hyperuricemia to obtain the best clinical outcomes and reduce their medical burden.

Genetic defects of SLC29A3 result in a wide range of syndromic histiocytosis that encompasses H syndrome. Patients with SLC29A3 variants typically have hyperpigmentation, hypertrichosis, hepatosplenomegaly, sensorineural hearing loss, diabetes mellitus, and hypogonadism. Herein, we identify a novel phenotype in a girl presenting with clinical and laboratory findings similar to systemic juvenile arthritis and hyperferritinemia. Exome sequencing identified a homozygous variant in SLC29A3 (NM_018344.5: c.707C>T [p.T236M]). Our patient did not show the cardinal features of the broad spectrum of SLC29A3-related disorders. She demonstrated remarkable improvement in her clinical and laboratory manifestations after starting interleukin-1 blockade (Anakinra). Recent research suggests that SLC29A3-related disorders are accompanied with autoinflammation and autoimmunity due to an overactive inflammasome pathway, which is most likely induced by mitochondrial and lysosomal dysfunction. Hence, our findings may expand the phenotypic features of the SLC29A3 variant. Patients with the SLC29A3 variant and systemic inflammation may benefit from interleukin-1 blockade as a therapeutic option.

Objective: This study investigated the incidence and patterns of the acute coronary syndrome (ACS) after AAV diagnosis and searched for the predictors of ACS in a single-centre cohort of Korean patients diagnosed with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).

Methods: A total of 262 patients with AAV were included in this study. ST-segment elevation myocardial infarction (STEMI), non-STEMI (NSTEMI), and unstable angina (UA) were defined as ACS in this study. Only ACS that occurred during or after AAV diagnosis was counted.

Results: The incidence of ACS in patients with AAV was 2.7% (7 patients), and the most common type of ACS was NSTEMI regardless of the affected site or the number of coronary arteries. Five patients with ACS were diagnosed with microscopic polyangiitis (MPA) and all of them had myeloperoxidase (MPO)-ANCA (or perinuclear [P]-ANCA), whereas the remaining two patients were diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA). Of the seven patients, 2 patients experienced ACS within the first year after AAV diagnosis, and 2 experienced ACS 5 years after AAV diagnosis. Among clinical variables, only the male sex was a predictor of ACS during the follow-up period in patients diagnosed with AAV.

Conclusion: The incidence of ACS was 2.7%, and the most common type of ACS was NSTEMI in Korean patients with AAV.

Objective: To evaluate the perspective of healthcare professionals towards the 2019 European Alliance of Associations for Rheumatology (EULAR) vaccination guideline in patients with autoimmune inflammatory rheumatic diseases (AIIRD).

Methods: Healthcare professionals who care for patients with AIIRD were invited to participate in an online survey regarding their perspective on the 2019 update of the EULAR recommendations for vaccination in adult patients with AIIRD. Level of agreement and implementation of the 6 overarching principles and 9 recommendations were rated on a 5-point Likert scale (1~5).

Results: Survey responses of 371 healthcare professionals from Asia (42.2%) and North America (41.6%), Europe (13.8%), and other countries were analyzed. Only 16.3% of participants rated their familiarity with the 2019 EULAR guideline as 5/5 ("very well"). There was a high agreement (≥4/5 rating) with the overarching principles, except for the principles applying to live-attenuated vaccines. There was a high level of agreement with the recommendations regarding influenza and pneumococcal vaccinations; implementation of these recommendations was also high. Participants also reported a high level of agreement with the remaining recommendations but did not routinely implement these recommendations.

Conclusion: The 2019 update of EULAR recommendations for the vaccination of adult patients with AIIRD is generally thought to be important by healthcare professionals, although implementation of adequate vaccination is often lacking. Better education of healthcare providers may be important to optimize the vaccination coverage for patients with AIIRD.

Objective: This study investigated the clinical implications of serious infections in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV) who received the first cycle of rituximab (RTX) during the first 6 months of follow-up.

Methods: The medical records of 36 AAV patients treated with RTX were reviewed. A weekly dose of 375 mg/m2 RTX was administered for 4 weeks to all patients along with glucocorticoids. Serious infections were defined as those requiring hospitalization. All-cause mortality during the first 6 months of follow-up was counted. The follow-up duration was defined as the period from the first RTX infusion to 6 months after the first RTX infusion.

Results: The median age was 60.5 years, and 16 patients were male. Seven of 36 patients (19.4%) died and three AAV patients had five cases of serious infection such as enterocolitis, pulmonary aspergillosis, atypical pneumonia, cytomegalovirus pneumonia, and cellulitis. AAV patients with serious infections during the first 6 months of follow-up exhibited a significantly lower cumulative survival rate than those without serious infections (p<0.001). However, we found no independent predictor of serious infections using the Cox hazard model analysis.

Conclusion: Serious infection is an important predictor of all-cause mortality in Korean patients with AAV who received their first cycle of RTX but there were no significant variables to predict the occurrence of serious infections at the first RTX. Thus, in cases refractory to other induction therapies, RTX should be strongly considered, despite an increase in mortality rate.

Objective: The aim of the present study is to assess carpal tunnel syndrome's (CTS's) ultrasonography (US) and magnetic resonance imaging (MRI) findings in patients with psoriatic arthritis (PsA) and compare them with healthy controls.

Methods: Thirty-nine PsA and twenty-eight healthy volunteers were examined in this study. Demographic and clinical features were recorded. CTS-6, a diagnostic algorithm, was used to estimate the probability of CTS. Electrodiagnostic study (EDS) was applied to all wrists included in the report, where the diagnosis of CTS was made by EDS. The cross-sectional area (CSA) of the median nerve was measured at pisiform bone level by US and MRI.

Results: Regarding to the demographic characteristics, no statistically significant difference was found between the groups. Twelve of 39 (30.76%) PsA patients had CTS, whereas CTS was not detected in the control group (p=0.001). US and MRI showed increased median nerve CSA in PsA patients compared to healthy controls (p=0.005, p<0.001; respectively). Also, US and MRI showed increased median nerve CSA in CTS patients compared to others (p=0.002, p<0.001; respectively). The Pearson correlation coefficient between MRI and US measurements of the CSA was 0.85 (p<0.001).

Conclusion: CTS frequency in PsA patients is found higher than healthy controls. The relationship between CTS diagnosed by EDS and CSA measured by both US and MRI was observed in PsA patients.