Intravascular NK/T-cell lymphoma: a case report and literature review

Intravascular lymphoma is characterized by an exclusively intravascular distribution of tumor cells. Intravascular natural killer/T-cell lymphoma (IVNKTL) is extremely rare, highly aggressive, commonly Epstein-Barr virus (EBV)–positive, and predominantly affects the skin and central nervous system. Here we report a case of IVNKTL diagnosed in a 67-year-old female, presenting with persistent intermittent fever and skin rashes throughout the body. Incisional biopsy of an erythematous lesion on the chest exhibited aggregation of medium to large-sized atypical lymphoid cells confined to the lumen of small vessels that were positive for CD3, granzyme B, and CD56 on immunohistochemistry and EBV-encoded RNA in situ hybridization. EBV DNA was also detected in serum after diagnosis. With a review of 26 cases of IVNKTL to date, we suggest that active biopsy based on EBV DNA detection may facilitate early diagnosis of IVNKTL.

Patient Perspectives and Preferences Regarding Gout and Gout Management:Impact on Adherence

Background@#Patient-centered management is becoming increasingly important in gout, but there are limited studies exploring patients' perspectives and preferences. We aimed to investigate patients' perspectives and preferences regarding gout and gout management, and their impacts on adherence to urate lowering therapy (ULT). @*Methods@#A paper-based survey was performed in patients with gout seen at the rheumatology outpatient clinics of 16 tertiary hospitals. The survey included questions regarding demographics, comorbidities, gout attacks, current treatment and adherence, and patients' perspectives and preferences regarding gout and gout management. Multivariate regression analysis was performed to determine the factors associated with ULT adherence. @*Results@#Of 809 surveyed patients with gout, 755 (94.5%) were using ULT. Among those using ULT, 89.1% had ≥ 80% adherence to ULT. Majority of the patients knew management strategies to some extent (94.8%), perceived gout as a life-long disease (91.2%), and were making efforts toward practicing at least one lifestyle modification (89.2%). Most patients (71.9%) obtained information about gout management during their clinic visits.Approximately half of the patients (53.6%) preferred managing their disease with both ULT and lifestyle modification, 28.4% preferred ULT only, and 17.4% preferred lifestyle modification only. Adherence was better in patients with older age (odds ratio [OR], 1.03), those with better knowledge of gout management strategies (OR, 3.56), and those who had preference for ULT (OR, 2.07). @*Conclusion@#Patients' perspectives and management preferences had high impacts on adherence to ULT in gout. Consideration of patients' perspectives and preferences is important for achieving the desired clinical outcome in gout.

Patient Perspectives and Preferences Regarding Gout and Gout Management:Impact on Adherence

Background@#Patient-centered management is becoming increasingly important in gout, but there are limited studies exploring patients' perspectives and preferences. We aimed to investigate patients' perspectives and preferences regarding gout and gout management, and their impacts on adherence to urate lowering therapy (ULT). @*Methods@#A paper-based survey was performed in patients with gout seen at the rheumatology outpatient clinics of 16 tertiary hospitals. The survey included questions regarding demographics, comorbidities, gout attacks, current treatment and adherence, and patients' perspectives and preferences regarding gout and gout management. Multivariate regression analysis was performed to determine the factors associated with ULT adherence. @*Results@#Of 809 surveyed patients with gout, 755 (94.5%) were using ULT. Among those using ULT, 89.1% had ≥ 80% adherence to ULT. Majority of the patients knew management strategies to some extent (94.8%), perceived gout as a life-long disease (91.2%), and were making efforts toward practicing at least one lifestyle modification (89.2%). Most patients (71.9%) obtained information about gout management during their clinic visits.Approximately half of the patients (53.6%) preferred managing their disease with both ULT and lifestyle modification, 28.4% preferred ULT only, and 17.4% preferred lifestyle modification only. Adherence was better in patients with older age (odds ratio [OR], 1.03), those with better knowledge of gout management strategies (OR, 3.56), and those who had preference for ULT (OR, 2.07). @*Conclusion@#Patients' perspectives and management preferences had high impacts on adherence to ULT in gout. Consideration of patients' perspectives and preferences is important for achieving the desired clinical outcome in gout.

What is the Best Choice for Urate-lowering Therapy for Korean?

Gout is one of the most common forms of acute inflammatory arthritis caused by long-standing hyperuricemia. Various clinical and epidemiological studies have demonstrated that uric acid, which is strongly associated with the pathogenesis of gout, is closely related with increased cardiovascular (CV) risk. Thus, properly controlling uric acid levels within its physiological level using urate-lowering therapy has been hypothesized to improve CV outcomes. Recently, however, on the basis of the results of the largest prospective, the double-blind, randomized controlled trial, entitled “the Cardiovascular Safety of Febuxostat or Allopurinol in Patients with Gout (CARES),” has aroused the possibility of increased CV-related and all-cause mortality in patients receiving febuxostat. Largely on the basis of this unpredicted result, the US and Korea Food and Drug Administration issued a public safety alert concerning the high risk of CV death with the use of febuxostat in February 2019. This unexpected announcement left many rheumatologists confused when they decide the first-line urate-lowering drug in Korea. In this review, we searched for previous studies on uric acid and increased risk of CV disease. In addition, we will introduce various interpretations of the results of the CARES trial and discuss the best choice of urate-lowering therapy for Korean.

Immunoglobulin G4-Related Aortitis of the Abdominal Aorta / 대한흉부외과학회지

Noninfectious aortitis, inflammatory abdominal periaortitis, and idiopathic retroperitoneal fibrosis are chronic inflammatory diseases with unclear causes. Recent studies have shown that some cases of aortitis are associated with immunoglobulin G4 (IgG4)-related systemic disease. Herein, we report a case of IgG4-related aortitis (IgG4-RA) that was diagnosed after surgery. Our patient was a 46-year-old man who had experienced abdominal pain for several weeks. Preoperative evaluations revealed an area of aortitis on the infrarenal aorta. He underwent surgery, and histological examination resulted in a diagnosis of IgG4-RA.

Immunoglobulin G4-Related Aortitis of the Abdominal Aorta

Noninfectious aortitis, inflammatory abdominal periaortitis, and idiopathic retroperitoneal fibrosis are chronic inflammatory diseases with unclear causes. Recent studies have shown that some cases of aortitis are associated with immunoglobulin G4 (IgG4)-related systemic disease. Herein, we report a case of IgG4-related aortitis (IgG4-RA) that was diagnosed after surgery. Our patient was a 46-year-old man who had experienced abdominal pain for several weeks. Preoperative evaluations revealed an area of aortitis on the infrarenal aorta. He underwent surgery, and histological examination resulted in a diagnosis of IgG4-RA.

Response to comment on “Medication nonadherence in Korean patients with rheumatoid arthritis: the importance of belief about medication and illness perception”

No abstract available.

Medication nonadherence in Korean patients with rheumatoid arthritis: the importance of belief about medication and illness perception

BACKGROUND/AIMS@#To investigate medication nonadherence in Korean patients with rheumatoid arthritis (RA) and analyze related factors.@*METHODS@#A total of 292 patients with RA participated in this study. Medication nonadherence, intentional or unintentional, was gauged via self-reported questionnaire. Patient perceptions of illness, treatment beliefs, and moods were measured via Brief Illness Perception Questionnaire, Beliefs about Medicines Questionnaire, and Patient Health Questionnaire-2, respectively. Demographic and clinical data were also collected. Multinomial regression analysis was used to assess the impact of demographic, clinical, and psychological factors on medication nonadherence.@*RESULTS@#The medication nonadherence rate was 54.1% (intentional, 21.6%; unintentional, 32.5%). Intentional nonadherence was reported most often in patients treated daily drugs (nonsteroidal anti-inflammatory drugs and/or disease-modifying antirheumatic drugs) (24.2%), and unintentional nonadherence was highest in patients receiving methotrexate (33.3%) (p = 0.872). In univariate analysis, beliefs in necessity and concerns of medication differed significantly in adherent and nonadherent patients (intentional or unintentional). When controlling for other factors that may impact medication nonadherence, less belief in necessity of medication (odds ratio [OR], 0.81; 95% confidence interval [CI], 0.68 to 0.95) and greater emotional response to disease (OR, 1.19; 95% CI, 1.01 to 1.40) were important predictors of intentional nonadherence.@*CONCLUSIONS@#Medication nonadherence is common in Korean patients with RA. Less belief in necessity of medication and greater emotional response to disease were identified as key factors prompting intentional nonadherence. These factors may be strategically targeted to improve medication adherence rates and subsequent clinical outcomes.

Ankylosing spondylitis associated with primary aldosteronism in a middle-aged woman

No abstract available.

Impact of early diagnosis on functional preservation in patients with rheumatoid arthritis: the early bird catches the worm

No abstract available.

Development of autoimmune hepatitis in a psoriasis patient without immunosuppressive therapy

We present a case of a 65-year-old man with psoriasis who developed autoimmune hepatitis (AIH) without receiving immunosuppressive therapy with either anti-tumor necrosis factor-α or methotrexate. The AIH had completely resolved at 2 months after prednisolone and azathioprine therapy. This case confirms the need to consider AIH in psoriasis patients who experience new elevations in liver enzymes. To our knowledge, this is first description of the development of AIH in an immunosuppressant-naïve patient with psoriasis.

Myeloid deletion of SIRT1 suppresses collagen-induced arthritis in mice by modulating dendritic cell maturation

The type III histone deacetylase silent information regulator 1 (SIRT1) is an enzyme that is critical for the modulation of immune and inflammatory responses. However, the data on its role in rheumatoid arthritis (RA) are limited and controversial. To better understand how SIRT1 regulates adaptive immune responses in RA, we evaluated collagen-induced arthritis (CIA) in myeloid cell-specific SIRT1 knockout (mSIRT1 KO) and wild-type (WT) mice. Arthritis severity was gauged on the basis of clinical, radiographic and pathologic scores. Compared with their WT counterparts, the mSIRT1 KO mice exhibited less severe arthritis, which was less destructive to the joints. The expression levels of inflammatory cytokines, matrix metalloproteinases and ROR-γT were also reduced in the mSIRT1 KO mice compared with the WT mice and were paralleled by reductions in the numbers of Th1 and Th17 cells and CD80- or CD86-positive dendritic cells (DCs). In addition, impaired DC maturation and decreases in the Th1/Th17 immune response were observed in the mSIRT1 KO mice. T-cell proliferation was also investigated in co-cultures with antigen-pulsed DCs. In the co-cultures, the DCs from the mSIRT1 KO mice showed decreases in T-cell proliferation and the Th1/Th17 immune response. In this study, myeloid cell-specific deletion of SIRT1 appeared to suppress CIA by modulating DC maturation. Thus, a careful investigation of DC-specific SIRT1 downregulation is needed to gauge the therapeutic utility of agents targeting SIRT1 in RA.

Contrast Media-induced Sweet's Syndrome / 대한피부과학회지

No abstract available.

Prevalence and Risk Factors of Upper Extremity Musculoskeletal Diseases among Farmers in Gyeongnam

OBJECTIVE: To evaluate the prevalence and risk factors of upper extremity musculoskeletal diseases (MSDs) among Korean farmers. METHODS: The study was carried out from June 2013 to August 2015 on 850 farmers and 203 non-farmers (controls) in Gyeongnam Province. Physical examinations were performed by rheumatologists, orthopedists, and rehabilitation specialists. Plain radiography, a nerve conduction examination, and magnetic resonance imaging were performed, and the Disabilities of the Arm, Shoulder, and Hand (DASH) questionnaire was used to assess upper extremity function. RESULTS: Thirty-four different types of upper extremity MSDs were detected in the 1,053 study subjects. The prevalence of any MSD in farmers was 8.96-fold higher than in control (p<0.001). The most obvious difference in prevalence between farmers and non-farmers was hand osteoarthritis (48.2% vs. 4.9%). Mean total DASH score was higher for farmers than non-farmers (14.29+/-13.66 vs. 10.03+/-10.85, p<0.001). Among farmers, myofascial pain syndrome, rotator cuff tear, and epicondylitis were more prevalent among overhead workers (growing persimmons, pears, and grapes) than in non-overhead workers (growing rice and upland crops). The following factors were associated with a rotator cuff tear; older age, overhead work, high waist circumference, and lower level of education. Hand osteoarthritis was found to be associated with older age, a female gender, high waist circumference, and longer total work time. CONCLUSION: The prevalence of upper extremity MSDs is much higher in farmers than non-farmers and greater still for farmers doing overhead work. Various factors contribute to the occurrence of upper extremity MSDs, and thus, the authors suggest an efficient preventive strategy, which involves consideration of type of work and risk factors, be established for farmers to reduce upper extremity MSDs.

Homonymous hemianopia in a patient with Behcet's disease

No abstract available.

Inhibitory Effects for Rheumatoid Arthritis of Dietary Supplementation with Resveratrol in Collagen-induced Arthritis

OBJECTIVE: Resveratrol is well-known for its anti-inflammatory, anti-oxidant effects on several diseases. We investigated whether dietary supplementation with resveratrol may suppress joint inflammation and destruction in a mouse model of collagen-induced arthritis (CIA). METHODS: Mice were randomly divided into two groups; CIA mice with normal diet-fed and CIA mice fed a 0.05% resveratrol diet. The effect of resveratrol on arthritis was assessed by clinical scoring system. The plain radiographs of paws were obtained to evaluate the effects on preventing bone destruction. Joint inflammation, cartilage damage, and osteoclastic bone resorption were checked by staining with H&E, Safranin-O, and tartrate resistant acid phosphatase (TRAP). Levels of pro-inflammatory cytokines were checked by enzyme-linked immunosorbent assay. The level of expression of nuclear factor (NF)-kappaB was measured by electrophoretic mobility shift assay (EMSA). RESULTS: Dietary supplementation with resveratrol led to mitigated severity of arthritis compared to the normal diet group (6.7+/-0.8 vs. 2.7+/-0.6, p<0.01). Resveratrol-fed mice showed decreased bone destruction on radiograph (3.4+/-0.3 vs. 2.0+/-0.2, p<0.01), and showed significantly inhibited pathological changes (inflammation 2.0+/-0.3 vs.3.2+/-0.2, p<0.01; cartilage damage 1.5+/-0.3 vs. 3.2+/-0.2, p<0.01; pannus formation 1.4+/-0.3 vs. 3.0+/-0.3, p<0.01; erosion; 1.4+/-0.2 vs. 3.3+/-0.3, p<0.01). Generation of TRAP-positive osteoclasts was inhibited in the resveratrol-fed mice (55.3+/-12.7 vs. 3.27+/-0.8, p<0.01). Resveratrol-fed mice showed decreased levels of tumor necrosis factor-alpha, interleukin (IL)-1beta, IL-6,monocyte chemoattractant protein 1, and the soluble receptor activator of NF-kappaB ligand in joint tissues and sera. Expression of NF-kappaB, measured by EMSA, was decreased in resveratrol-fed mice. CONCLUSION: Dietary supplementation with resveratrol mitigates inflammation and bone destruction in CIA mice.

A Case of Antineutrophil Cytoplasmic Antibody-negative Granulomatosis with Polyangiitis Presenting as Breast Mass

Granulomatosis with polyangiitis (GPA) is a disease characterized by a granulomatous necrotizing vasculitis of the small vessels, along with the presence of antineutrophil cytoplasmic antibody (ANCA), serologically. GPA is a multisystem disease, in which the diagnosis is frequently based on respiratory and renal manifestations, with rare breast invasion. To date, several cases of breast invasion by GPA have been published, and most cases have been positive for ANCA. However, ANCA-negative forms of breast invasion by GPA are extremely rare and have not been reported in Korea thus far. Therefore, we report a case of ANCA-negative GPA in a 70-year-old woman, who was initially presented with a localized palpable mass in the left breast.

Conservative Care was Successfully Used to Manage Acute Hepatitis A Infection with Blindness / 대한내과학회지

Hepatitis A is usually a mild, self-limiting illness, but in certain cases it can develop into a severe or fatal disease. The most common symptoms of acute hepatitis A virus (HAV) infection are fever, myalgia, nausea, vomiting, jaundice, and abdominal pain. Extrahepatic symptoms are rare, especially neurologic manifestations. We investigated a 34-year-old male who presented after suffering two days of fever, chills and general myalgia. His condition gradually deteriorated and he developed sudden blindness on day nine. This blindness lasted for 20 min and at this point abnormal brain images were observed. Initial tests for immunoglobulin M anti HAV were negative. These tests were repeated, later confirming acute HAV infection. After conservative management, the individual fully recovered. We report this extremely rare acute HAV infection case with metabolic encephalopathy exhibiting transient blindness.

A Case of Steroid and Methotrexate-Resistant Eosinophilic Fasciitis Treated with Adalimumab

Eosinophilic fasciitis (EF) is a rare fibrosing disorder characterized by painful swelling and induration of the limbs and trunk, characteristic histology with sclerosis and lymphocytic inflammation affecting the fascia. The cause and pathogenesis of EF are still unknown and current therapies include glucocorticoids with or without use of immunosuppressive agents. Recently, there have been several case reports documenting the efficacy of a TNF alpha inEosinophilic fasciitis (EF) is a rare fibrosing disorder characterized by painful swelling and induration of the limbs and trunk, characteristic histology with sclerosis and lymphocytic inflammation affecting the fascia. The cause and pathogenesis of EF are still unknown and current therapies include glucocorticoids with or without use of immunosuppressive agents. Recently, there have been several case reports documenting the efficacy of a TNF alpha inhibitor in EF following a steroid-resistant disease course. However, there has been no report on the experience in treatment of EF with a TNF alpha inhibitor in Korea. Hence, we report a case of steroid and methotrexate-resistant EF which was successfully treated with adalimumab, along with a review of the relevant articles.

Delayed Ventricular Septal Rupture Following Blunt Chest Trauma

Cardiac injury is a common but occasionally serious complication of blunt chest trauma. A ventricular septal rupture (VSR) is a rare complication and is variable in its presentation, temporal course and severity. Here, we report a rare case of 75-year-old man who developed delayed VSR following blunt chest trauma.