IgG4-related disease (IgG4-RD) is a potentially multiorgan disorder. In this study, clinical and serological features from 132 IgG4-RD patients were compared about organ correlations. Underlying pathologies comprised autoimmune pancreatitis (AIP) in 85 cases, IgG4-related sclerosing cholangitis (IgG4-SC) in 12, IgG4-related sialadenitis (IgG4-SIA) in 56, IgG4-related dacryoadenitis (IgG4-DAC) in 38, IgG4-related lymphadenopathy (IgG4-LYM) in 20, IgG4-related retroperitoneal fibrosis (IgG4-RF) in 19, IgG4-related kidney disease (IgG4-KD) in 6, IgG4-related pseudotumor (IgG4-PT) in 3. Sixty-five patients (49%) had multiple IgG4-RD (two affected organs in 36 patients, three in 19, four in 8, five in 1, and six in 1). Serum IgG4 levels were significantly higher with multiple lesions than with a single lesion (P<0.001). The proportion of association with other IgG4-RD was 42% in AIP, the lowest of all IgG4-RDs. Serum IgG4 level was lower in AIP than in other IgG4-RDs. Frequently associated IgG4-RDs were SIA (25%) and DAC (12%) for AIP; AIP (75%) for IgG4-SC; DAC (57%), AIP (38%) and LYM (27%) for IgG4-SIA; AIP (26%) and LYM (26%) for IgG4-DAC; SIA (75%), DAC (50%) and AIP (45%) for IgG4-LYM; SIA (58%), AIP (42%) and LYM (32%) for IgG4-RF; AIP (100%) and SIA (67%) for IgG4-KID; and DAC (67%) and SIA (67%) for IgG4-PT. Most associated IgG4-RD lesions were diagnosed simultaneously, but IgG4-SIA and IgG4-DAC were sometimes identified before other lesions. About half of IgG4-RD patients had multiple IgG4-RD lesions, and some associations were seen between specific organs.
Adult ; Aged ; Autoimmune Diseases/*epidemiology/*immunology ; Comorbidity ; Female ; Humans ; Immunoglobulin G/*immunology ; Japan/epidemiology ; Male ; Middle Aged ; Multiple Organ Failure/*epidemiology/*immunology ; Organ Specificity/immunology ; Prevalence ; Risk Factors ; Statistics as Topic

PURPOSE: Vitiligo prevalence and its associated comorbidities rate have been reported variably among different populations. We aimed to determine the prevalence of vitiligo in Korea along with the baseline rate of comorbidities and compared the risks to the general population using hospital visit information of the total population in Korea. MATERIALS AND METHODS: We assessed demographic characteristics of vitiligo patients in Korean population from 2009 to 2011 in a nationwide data from Health Insurance Review Assessment Service. Patients who had at least one visit to Korea's primary, secondary, or tertiary referral hospitals with International Classification of Diseases, 10th Revision, Clinical Modification diagnosis code for vitiligo were identified. As a supplementary study, comorbidities associated with vitiligo were selected for further review to calculate relative risks compared to the general population. RESULTS: The annual prevalence of vitiligo determined by hospital-visiting rate in Korea was 0.12% to 0.13% over a three year period. In sync with other previous epidemiological studies, there was bimodal distribution among the age groups and no difference between genders. Also, vitiligo in Korean population was associated with various autoimmune/non-autoimmune diseases such as thyroiditis, atopic dermatitis, and psoriasis. CONCLUSION: This study was by far the most comprehensive review on prevalence of vitiligo using a data of total population in Korea. The prevalence is within a range of those reported in previous literatures, and increased risk of comorbidities such as thyroid diseases and psoriasis in vitiligo might aid clinicians in the initial work up of vitiligo patients and concurrent follow ups.
Adult ; Aged ; Autoimmune Diseases/*epidemiology/immunology ; Comorbidity ; Diabetes Mellitus, Type 1/epidemiology ; Female ; Humans ; Male ; Middle Aged ; Population Surveillance ; Prevalence ; Republic of Korea/epidemiology ; Socioeconomic Factors ; Thyroid Diseases/epidemiology ; Vitiligo/*epidemiology

BACKGROUND: Type 1 diabetes mellitus is frequently associated with other autoimmune diseases. The occurrence of common features of autoimmune diseases and the coassociation of multiple autoimmune diseases in the same individual or family supports the notion that there may be common genetic factors. METHODS: To investigate potential clustering of autoimmune thyroid disease (ATD) among type 1 diabetes patients and the contribution of common susceptibility genes to this, HLA DR/DQ alleles as well as antithyroid autoantibodies were measured in 115 Korean patients with type 1 diabetes and their 96 first-degree family members. RESULTS: Twenty-five percent of the patients had ATD, whereas 3 of 36 (8%) age-matched normal controls had ATD (RR = 3.7, p < 0.05). Twenty-six of ninty-six (27%) type 1 diabetes family members had ATD. No differences in the distribution of HLA alleles/haplotypes and genotypes between the patients with and without ATD were found. CONCLUSION: From this finding, we could assess that individuals with type 1 diabetes and their relatives frequently develop ATD, perhaps due to common susceptibility genes that are shared among first degree relatives.
Adult ; Alleles ; Autoantibodies/blood ; Autoimmune Diseases/epidemiology* ; Child ; Child, Preschool ; Diabetes Mellitus, Insulin-Dependent/genetics ; Diabetes Mellitus, Insulin-Dependent/complications* ; Female ; Glutamate Decarboxylase/immunology ; HLA-DQ Antigens/genetics ; HLA-DR Antigens/genetics ; Human ; Male ; Prevalence ; Thyroid Diseases/epidemiology*

Since heparin is an anticoagulant commonly used in hemodialysis and the patients on hemodialysis are repeatedly exposed to heparin, heparin may be the cause of the development of heparin-dependent antibodies and thrombotic complications in patients on hemodialysis. The purpose of this study was to determine the prevalence and the clinical significance of the antibodies against heparin-platelet factor 4 complexes as determined by enzyme immunoassay in patients on maintenance hemodialysis. The prevalence of anti-heparin-platelet factor 4 antibodies was higher in hemodialysis patients than in normal subjects (8.8 vs 0.0%, p<0.05). The number of past episodes of vascular access obstruction per year was significantly higher in the anti-heparin-platelet factor 4 antibody positive group than antibody negative group. Anti-heparin-platelet factor 4 antibody positive patients experienced more frequent vascular access obstructions than control subjects. In conclusion, anti-heparin-platelet factor 4 antibody might be a risk factor for vascular access obstructions in patients with end-stage renal disease on maintenance hemodialysis.
Adult ; Autoantibodies/immunology* ; Autoimmune Diseases/immunology* ; Catheters, Indwelling* ; Enzyme-Linked Immunosorbent Assay ; Female ; Heparin/immunology* ; Human ; Kidney Failure, Chronic/blood ; Kidney Failure, Chronic/immunology ; Kidney Failure, Chronic/therapy* ; Male ; Middle Aged ; Platelet Factor 4/immunology* ; Recurrence ; Renal Dialysis* ; Risk Factors ; Thrombophilia/immunology* ; Thrombosis/epidemiology* ; Thrombosis/immunology ; Thrombosis/prevention & control

To determine the approximate incidence and clinical features of pernicious anemia in a Korean population, we retrospectively analyzed clinical data for patients with pernicious anemia who were diagnosed between 1995 and 2010 at five hospitals in Chungnam province. Ninety-seven patients were enrolled, who accounted for 24% of patients with vitamin B12 deficiency anemia. The approximate annual incidence of pernicious anemia was 0.3 per 100,000. The median age was 66 (range, 32-98) yr, and the male/female ratio was 1.25. Anemia-associated discomfort was the most common symptom (79.4%), followed by gastrointestinal and neurological symptoms (78.4% and 38.1%, respectively). Pancytopenia was found in 36 patients (37.1%), and autoimmune disorders were found in 15 patients (15.5%). Antibody to intrinsic factor was detected in 62 (77.5%) of 80 patients examined, and antibody to parietal cells was detected in 35 (43.2%) of 81 patients examined. Of the 34 patients who underwent tests for Helicobacter pylori, 7 (12.5%) were positive. The anemia-associated and gastrointestinal symptoms resolved completely in all patients after intramuscular injection of cobalamin, whereas neurological symptoms remained in some. In conclusion, pernicious anemia is less frequent in Koreans than in Western populations; however, the clinical features of this disorder in Koreans do not differ from those of Western cases.
Adult ; Aged ; Anemia, Pernicious/complications/*diagnosis/epidemiology ; Asian Continental Ancestry Group ; Autoimmune Diseases/complications/epidemiology ; Female ; Gastrointestinal Diseases/complications/drug therapy/epidemiology ; Helicobacter Infections/diagnosis ; Helicobacter pylori ; Humans ; Isoantibodies/blood ; Male ; Middle Aged ; Nervous System Diseases/complications/epidemiology ; Parietal Cells, Gastric/immunology ; Republic of Korea/epidemiology ; Retrospective Studies ; Vitamin B 12/blood/therapeutic use