Cancer risk in Korean patients with gout

Background/Aims@#Using a nationwide cohort, we investigated the cancer risk in Korean patients with gout. @*Methods@#Data were obtained from the Korean National Health Insurance Service Database. Patients with gout were defined as those aged ≥ 20 years who were diagnosed with gout and received anti-gout medication (allopurinol, colchicine, and benzbromarone) between 2008 and 2010. Patients with nail disorders were randomly assigned to a control group (1:1 ratio) after frequency matching for age and sex. Cancer incidence was then investigated between 2012 and 2018. Cox proportional hazard regression analysis was used to investigate the association between gout and cancer after adjusting for concomitant diseases. @*Results@#This study included 179,930 patients with gout and an equal number of matched controls. The incidence of overall cancer was higher in patients with gout than in controls (incidence rate ratio, 1.08). Cox proportional hazards regression analysis showed that gout was associated with a hazard ratio of 1.053 (95% confidence interval ,1.031 to 1.077) after adjusting for concomitant diseases. @*Conclusions@#Gout was associated with a significantly high risk of cancer, especially esophageal, stomach, colon, liver, pancreatic, lung, ovarian, renal, and bladder cancers.

A Patient With Late-onset Limb-girdle Muscular Dystrophy Type 2B Mimicking Dermatomyositis: A Case Report and Review

Limb-Girdle Muscular Dystrophy 2B (LGMD2B) presents with proximal and/or distal muscle weakness and markedly high creatine kinase level. It is caused by the loss of dysferlin due to mutations in the DYSF gene. Due to its similar clinical features as inflammatory myopathy, it is often difficult to distinguish between the two. We present a case of a 48-year-old male who developed progressive proximal muscle weakness, papulosquamous lesions on the knuckles, elevated levels of muscle enzymes, and electromyogram abnormalities. Based on the clinical presentation, the initial impression was dermatomyositis, yet it was refractory to immunosuppressive therapy. Subsequently, dysferlin immunostaining and genetic analysis led to the final diagnosis of LGMD2B. This case shows that LGMD2B can present with extramuscular symptoms mimicking inflammatory myopathy in later stages of life. Dysferlin immunostaining and/or genetic analysis of the DYSF gene are essential for its diagnosis.

Mutation of ten-eleven translocation-2 is associated with increased risk of autoimmune disease in patients with myelodysplastic syndrome

Background/Aims@#Myelodysplastic syndrome (MDS) is caused by genetic and epigenetic alteration of hematopoietic precursors and immune dysregulation. Approximately 20% of patients with MDS develop an autoimmune disease (AID). Here, we investigated whether particular genetic mutations are associated with AID in patients with MDS. @*Methods@#Eighty-eight genetic mutations associated with myeloid malignancy were sequenced in 73 MDS patients. The association between these mutations and AID was then analyzed. @*Results@#The median age of the 73 MDS patients was 70 years (interquartile range, 56 to 75), and 49 (67.1%) were male. AID was observed in 16 of 73 patients (21.9%). Mutations were detected in 57 (78.1%) patients. The percentage (68.8% vs. 80.7%, p = 0.32) and the mean number of mutations (1.8 ± 1.6 vs. 2.2 ± 1.8, p = 0.34) in MDS patients with or without AID were similar. However, the ten-eleven translocation- 2 (TET2) mutation rate was significantly higher in patients with AID than in those without (31.3% vs. 5.3%, respectively; p = 0.001). All TET2 mutations were variants of strong clinical significance. @*Conclusions@#Mutation of TET2 in patients with MDS may be associated with increased risk of developing AID.

Practical Utility of D-dimer Test for Venous Thromboembolism in Systemic Lupus Erythematosus Depends on Disease Activity: a Retrospective Cohort Study

Background@#The D-dimer test is a screening tool for venous thromboembolism (VTE);however, its utility for patients with systemic lupus erythematosus (SLE) remains unclear.Here, we examined the utility of the D-dimer test as a screening tool for VTE in SLE patients. @*Methods@#SLE patients (n = 276) and age- and sex-matched patients with non-rheumatic disease (n = 1,104), all of whom underwent D-dimer testing to screen for VTE, were enrolled.The sensitivity and specificity and receiver operating characteristics curve of the D-dimer test were compared in both groups. Then, subgroup of SLE patients in whom the D-dimer test can be useful was sought. @*Results@#The incidence of VTE was more common in SLE patients than controls (10.9% vs.4.0%). Although the sensitivity of the D-dimer test was comparable between SLE patients and controls (93.3% vs. 90.9%), the specificity of the test was profoundly lower in SLE patients compared to controls (28.4% vs. 84.4%). The area under the curve (AUC) of the D-dimer for VTE was 0.669 in SLE patients and 0.90 in control group. Multiple linear regression analysis demonstrated that SLE disease activity index-2000 (SLEDAI-2K) was significantly associated with D-dimer levels in SLE patients (β = 0.155; P = 0.022). Subgroup analysis showed that the AUC is moderate (0.768) with low disease activity, while it is low (0.518) with high SLEDAI-2K. @*Conclusion@#The D-dimer test may not be a useful screening tool for VTE in patients with active SLE. D-dimer test for predicting VTE in SLE patients should be differentially applied according to disease activity of SLE.

Effect of Drug Adherence on Treatment Outcome in Rheumatoid Arthritis

OBJECTIVE: The present study aimed to evaluate the effect of drug adherence on treatment outcome in Korean patients with rheumatoid arthritis (RA). METHODS: A total of 2,694 RA patients who had complete data from annual follow-ups over three years in the Korean Observational Study Network for Arthritis were included in this study. Patients were divided into adherent and non-adherent groups according to data for drug adherence over three years. The European League against Rheumatism response and rate of disease flare were compared between two groups over three years. We also compared continuous variables representing treatment outcomes between the two groups. RESULTS: After propensity score matching using a ratio of 1:3, patients were allocated into non-adherent (n=522) and adherent (n=1,447) groups. The rate of non-response was higher in the non-adherent group over three years; however, there were no significant differences between continuous variables related to treatment outcome between the two groups. To evaluate the difference according to disease duration, patients were classified into early and late RA based on 48-month disease duration. In patients with early RA, the adherent group had lower patient's global health visual analog scale and lower disease activity 28 scores at three years compared with the non-adherence group. In patients with late RA, the non-adherent group had a higher rate of disease flare. CONCLUSION: The adherent group tended to show lower disease activity, especially in early RA, whereas the non-adherence group was associated with non-response and higher risk of disease flare.

A Case of Polymyositis Associated with Immunoglobulin A Nephropathy

Polymyositis (PM) is a chronic inflammatory disease that predominantly affects muscles. Systemic organ involvement, including the respiratory and gastrointestinal tracts, is frequently observed in PM, but renal involvement is rare. Herein, we report the case of a 56-year-old woman presenting with weight gain, edema, and generalized myalgia. Laboratory tests revealed elevated creatinine kinase level, hypoalbuminemia, and proteinuria. Histopathological examination of muscle biopsy revealed inflammatory myositis, and a renal biopsy confirmed immunoglobulin A (IgA) nephropathy. Based on the clinico-pathological results, the patient was diagnosed with PM with IgA nephropathy. This is a report of a rare occurrence of IgA nephropathy in a patient with PM presenting with chronic glomerulonephritis.

Progressive Occipitocervical Subluxation Despite Biologic Agent Therapy in a Patient with Advanced Rheumatoid Arthritis

No abstract available.

Case of Moyamoya Disease Aggravated during the Treatment of Behçet’s Disease

Behçet’s disease (BD) is a multi-organ involved inflammatory disorder described by recurrent oral ulcers and other systemic manifestations. Almost all the clinical manifestations of BD are believed to be due to vasculitis. On the other hand, the cerebral arteries are rarely involved. Moyamoya disease (MMD) is an unusual chronic cerebrovascular disorder that is described by bilateral progressive stenosis or occlusion of the internal carotid artery and an abnormal collateral vascular network. A 32-year-old woman with MMD was referred for fever, oral pain, and diarrhea, and was diagnosed with BD. Her MMD was aggravated during treatment with high dose steroids to control the intestinal BD and a reduction in the MMD medication due to gastrointestinal bleeding. This is the first reported case of intestinal BD in a patient previously diagnosed with MMD, who experienced aggravation of her MMD after the cessation of MMD medication due to aggravated intestinal BD.