BACKGROUND: Antiphospholipid antibody syndrome (APS), an important cause of acquired thrombophilia, is diagnosed when vascular thrombosis or pregnancy morbidity occurs with persistently positive antiphospholipid antibodies (aPL). APS is a risk factor for unprovoked recurrence of pulmonary embolism (PE). Performing laboratory testing for aPL after a first unprovoked acute PE is controversial. We investigated if a specific phenotype existed in patients with unprovoked with acute PE, suggesting the need to evaluate them for APS. METHODS: We retrospectively reviewed patients with PE and APS (n=24) and those with unprovoked PE with aPL negative (n=44), evaluated 2006–2016 at the Asan Medical Center. We compared patient demographics, clinical manifestations, laboratory findings, and radiological findings between the groups. RESULTS: On multivariate logistic regression analysis, two models of independent risk factors for APS-PE were suggested. Model I included hemoptysis (odds ratio [OR], 12.897; 95% confidence interval [CI], 1.025–162.343), low PE severity index (OR, 0.948; 95% CI, 0.917–0.979), and activated partial thromboplastin time (aPTT; OR, 1.166; 95% CI, 1.040–1.307). Model II included age (OR, 0.930; 95% CI, 0.893–0.969) and aPTT (OR, 1.104; 95% CI, 1.000–1.217). CONCLUSION: We conclude that patients with first unprovoked PE with hemoptysis and are age <40; have a low pulmonary embolism severity index, especially in risk class I–II; and/or prolonged aPTT (above 75th percentile of the reference interval), should be suspected of having APS, and undergo laboratory testing for aPL.
Antibodies, Antiphospholipid* ; Antiphospholipid Syndrome* ; Chungcheongnam-do ; Demography ; Hemoptysis ; Humans ; Logistic Models ; Partial Thromboplastin Time ; Phenotype* ; Pregnancy ; Pulmonary Embolism* ; Recurrence ; Retrospective Studies ; Risk Factors ; Thrombophilia ; Thrombosis

Polymyalgia rheumatica (PMR) is a chronic inflammatory disorder that usually affects adults over 50 years of age. It is characterized by pain and stiffness of the neck, shoulders and hips. Laboratory tests have shown marked elevation of acute phase reactants (ESR and CRP), but there is no specific test to diagnose PMR. Recently, the European League Against Rheumatism and the American College of Rheumatology jointly proposed a new set of classification criteria for PMR. The mainstay of PMR treatment is low-dose glucocorticoids (prednisolone 10-20 mg/day). patients with PMR usually respond very well to steroid therapy within a few days to one week, but will often relapse despite treatment.
Acute-Phase Proteins ; Adult ; Classification ; Diagnosis* ; Glucocorticoids ; Hip ; Humans ; Neck ; Polymyalgia Rheumatica* ; Recurrence ; Rheumatic Diseases ; Rheumatology ; Shoulder

Tumor necrosis factor (TNF) inhibitors are increasingly used in treatment of rheumatoid arthritis (RA), ankylosing spondylitis, psoriatic arthritis, and inflammatory bowel diseases including Crohn's disease and ulcerative colitis. Rarely, anti-TNF therapy is associated with neurological complications, including both central and peripheral nervous system disorders. To the best of our knowledge, only one case of infliximab-associated multifocal motor neuropathy with conduction block in a patient with spondyloarthropathy has been reported to date in Korea. Here, we report on the case of a 58-year-old Korean woman affected by RA who developed multifocal motor neuropathy after infliximab treatment.
Arthritis, Psoriatic ; Arthritis, Rheumatoid* ; Colitis, Ulcerative ; Crohn Disease ; Female ; Humans ; Inflammatory Bowel Diseases ; Infliximab ; Korea ; Middle Aged ; Peripheral Nervous System Diseases ; Spondylarthropathies ; Spondylitis, Ankylosing ; Tumor Necrosis Factor-alpha

In the present study, we investigated if priming of autoreactive CD8+ T cells would be inhibited by competitive peptides for major histocompatibility complex (MHC) class I binding. We used a mouse model of vitiligo which is induced by immunization of Kb-binding tyrosinase-related protein 2 (TRP2)-180 peptide. Competitive peptides for Kb binding inhibited IFN-gamma production and proliferation of TRP2-180-specific CD8+ T cells upon ex vivo peptide restimulation, while other MHC class I-binding peptides did not. In mice, the capability of inhibition was influenced by T-cell immunogenicity of the competitive peptides. The competitive peptide with a high T-cell immunogenicity efficiently inhibited priming of TRP2-180-specific CD8+ T cells in vivo, whereas the competitive peptide with a low T-cell immunogenicity did not. Taken together, the inhibition of priming of autoreactive CD8+ T cells depends on not only competition of peptides for MHC class I binding but also competitive peptide-specific CD8+ T cells, suggesting that clonal expansion of autoreactive T cells would be affected by expansion of competitive peptide-specific T cells. This result provides new insights into the development of competitive peptides-based therapy for the treatment of autoimmune diseases.
Animals ; Autoimmune Diseases ; Immunization ; Major Histocompatibility Complex ; Mice ; Peptides ; T-Lymphocytes ; Vitiligo

Objective: . Hydronephrosis, a common complication of idiopathic retroperitoneal fibrosis (iRPF), may lead to poor renal outcomes unless resolved in a timely manner. IgG4-related diseases characterized by elevated serum IgG4 levels are responsible for a few iRPF cases. However, the underlying immunologic features of most iRPF cases have not been clearly defined, and these cases exhibit varied responses to medical treatment. Thus, we investigated the predictive factors for hydronephrosis-associated outcomes among iRPF patients. Methods: . We retrospectively included 18 iRPF patients with hydronephrosis in a tertiary referral hospital from 2012 to 2019. Hydronephrosis improvement was assessed on images taken 6 months after diagnosis. Categorical variables were compared using chi-square or Fisher’s exact test. Continuous variables were compared using Mann–Whitney U-test. Results: . On follow-up images, 8 patients (44.4%) showed an improvement in hydronephrosis. Patients with improvement more frequently had reverse serum IgG4/IgG3 ratio (87.5% vs. 30%, p=0.025), abdominal aorta involvement (87.5% vs. 30%, p=0.025) and glucocorticoid treatment administration (87.5% vs. 30%, p=0.025) than those without improvement. The proportion of elevated serum IgG4 level did not differ between the two groups.Even in the 14 cases with normal serum IgG4 levels, reverse serum IgG4/IgG3 ratio was more frequently observed in patients with improvement than in those without improvement (83.3% vs. 12.5%, p=0.026). Conclusion . The reverse serum IgG4/IgG3ratio was associated with hydronephrosis improvement in iRPF patients, suggesting it to be a suitable serologic marker for predicting favourable responses to glucocorticoid treatment.

Behçet's disease is a systemic vasculitis of unknown etiology characterized by recurrent oral and genital ulcers and uveitis. The vascular involvement of Behçet's disease affects arteries, veins, and blood vessels of all sizes, and it can include venous or arterial thrombosis and arterial aneurysms. There are only a few reports of an aortic aneurysm invading a vertebral body in a patient with Behçet's disease. Here, we report the case of a 45-year-old man who was initially diagnosed with vertebral invasion of a mycotic aneurysm. He underwent vascular surgery and received empirical antibiotics, but all cultures were negative. However, he had persistent, recurrent deep vein thrombosis and elevated inflammatory markers. After reviewing the pathology, a final diagnosis of Behçet's disease was made. He was successfully treated with corticosteroids. This report presents a rare case of Behçet's disease mimicking vertebral invasion of a mycotic aneurysm.
Adrenal Cortex Hormones ; Aneurysm ; Aneurysm, Infected* ; Anti-Bacterial Agents ; Aortic Aneurysm ; Arteries ; Behcet Syndrome ; Blood Vessels ; Diagnosis ; Humans ; Middle Aged ; Osteomyelitis ; Pathology ; Systemic Vasculitis ; Thrombosis ; Ulcer ; Uveitis ; Veins ; Venous Thrombosis

OBJECTIVE: Urolithiasis is one of the manifestations of gout and the risk is higher in gouty patients. On the other hand, an independent association between the urinary stone and serum uric acid (UA) level has not been established. This study examined whether the risk of urolithiasis increases with increasing serum UA level. METHODS: Among the people who visited a tertiary hospital from 2010 to 2013, 13,964 individuals who underwent both ultrasonography and a laboratory test were recruited in the study. The risk of urolithiasis on ultrasonography was analyzed in association with the serum UA level by multiple logistic regression analysis with an adjustment for age, sex, body mass index, estimated glomerular filtration rate, and known underlying diseases, including diabetes mellitus and hypertension. RESULTS: Among the 6,743 men (48.3%) and 7,221 women (51.7%), the age was 51.3±13.5 and the serum UA level was 4.5±2.1 mg/dL. Hyperuricemia (>7 mg/dL) was observed in 1,381 cases (9.9%). Urolithiasis was detected by ultrasonography in 608 cases (4.4%). The detection rates of urolithiasis in individuals with hyperuricemia and normouricemia were 5.9% and 4.1%, respectively (p=0.001). Multiple logistic regression analysis showed that individuals with hyperuricemia had a significantly higher risk of urolithiasis (adjusted odds ratio [OR]=1.54; 95% confidence interval [CI], 1.20~1.96; p=0.001). A comparison of the highest with the lowest quartile of serum UA revealed a multivariable-adjusted OR of 3.17 (95% CI, 1.98~5.11) for men and 1.79 (1.08~2.93) for women. CONCLUSION: These results suggest that individuals with a higher serum UA level have a higher risk of subclinical and clinical urolithiasis.
Body Mass Index ; Diabetes Mellitus ; Female ; Glomerular Filtration Rate ; Gout ; Hand ; Humans ; Hypertension ; Hyperuricemia ; Logistic Models ; Male ; Odds Ratio ; Tertiary Care Centers ; Ultrasonography ; Uric Acid* ; Urinary Calculi ; Urolithiasis*

IL-32 acts as a pro-inflammatory cytokine by inducing the synthesis of inflammatory molecules as well as promoting the morphological changes involved in the transformation of monocytes into osteoclasts (OCs). Evaluation of the functions of IL-32 has mainly focused on its inflammatory properties, such as involvement in the pathogenesis of various autoimmune diseases. Recently, IL-32 was shown to be involved in bone metabolism, in which it promotes the differentiation and activation of OCs and plays a key role in bone resorption in inflammatory conditions. IL-32γ also regulates bone formation in conditions such as ankylosing spondylitis and osteoporosis. In this review, we summarize the results of recent studies on the role of IL-32γ in bone metabolism in inflammatory arthritis.
Arthritis* ; Arthritis, Rheumatoid ; Autoimmune Diseases ; Bone Resorption ; Inflammation ; Metabolism* ; Monocytes ; Osteoblasts ; Osteoclasts ; Osteogenesis ; Osteoporosis ; Spondylitis, Ankylosing

Calcium pyrophosphate (CPP) crystals can present as acute inflammatory arthritis which is known as an acute CPP crystal arthritis. Although monocytes/macrophages have been shown to play a role in the initiation of crystal-mediated inflammatory responses, differences in their phenotypes between acute CPP crystal arthritis and acute gouty arthritis have not yet been investigated. We examined the immunological characteristics of synovial monocytes/macrophages in patients with acute CPP crystal and acute gouty arthritis. CD14⁺CD3⁻CD19⁻CD56⁻ cell frequencies in synovial fluid mononuclear cells (SFMCs) were measured. Expression of pro- and anti-inflammatory cytokines and markers was determined. The SFMCs were dominated by a population of monocytes/macrophages in acute CPP crystal arthritis similar to that in acute gout. Synovial monocytes/macrophages showed the phenotypes of infiltrated monocytes as shown by expression of CD88, C-C chemokine receptor type 2, myeloid-related protein (MRP)8 and MRP14 but not proto-oncogene tyrosine-protein kinase MER. Comparatively, the CD14⁺ cells from patients with acute CPP crystal arthritis had similar high levels of IL-1β and TNF-α production but significantly lower expression of IL-10 and M2 marker (CD163). The monocytes/macrophages had the capacity to produce IL-8 in response to CPP crystals. Proinflammatory features were more dominant in monocytes/macrophages during acute CPP crystal arthritis than those during acute gouty arthritis.
Arthritis ; Arthritis, Gouty ; Calcium Pyrophosphate ; Calcium ; Cytokines ; Gout ; Humans ; Interleukin-10 ; Interleukin-8 ; Macrophages ; Monocytes ; Phenotype ; Phosphotransferases ; Proto-Oncogenes ; Synovial Fluid