Systemic lupus erythematosus (SLE) is a multiorgan-involved autoimmune disease, and it can overlap organ-specific autoimmune diseases such as autoimmune thyroid diseases, autoimmune hepatitis and inflammatory bowel disease. There may be some association between SLE and these autoimmune diseases, such as common immunological and genetic basis, but the pathogenic mechanism is still unclear. This review focuses on current knowledge regarding the prevalence and possible pathogenesis of SLE overlapping the above three autoimmune diseases.
Autoimmune Diseases ; complications ; epidemiology ; Hepatitis, Autoimmune ; complications ; epidemiology ; Humans ; Inflammatory Bowel Diseases ; complications ; epidemiology ; Lupus Erythematosus, Systemic ; complications ; epidemiology ; Prevalence ; Research ; trends ; Thyroiditis, Autoimmune ; complications ; epidemiology

Adult ; Asymptomatic Diseases/epidemiology* ; Autoimmune Diseases/etiology* ; China/epidemiology* ; Female ; Humans ; Hypothyroidism/etiology* ; Male ; Metabolic Syndrome/epidemiology* ; Middle Aged ; Obesity/epidemiology* ; Risk Factors ; Thyroid Diseases/etiology* ; Young Adult

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: Alopecia areata is the most common cause of localized, nonscarring alopecia. Unfortunately, there are few data regarding clinical features and epidemiology of alopecia areata in Korean patients, and its clinical course and treatment response rates are unpredictable. OBJECTIVE: This study strived to investigate the differences in clinical profiles according to disease severity and to determine risk factors for severe alopecia areata. METHODS: A total of 1,137 patients from 2006 to 2015 were analyzed retrospectively. Patients were subdivided into two groups: mild-to-moderate and severe alopecia areata. The groups were compared on the basis of age of onset, duration, sex, family history, comorbid disorders including autoimmune diseases, nail changes, and laboratory test results. RESULTS: Eight hundred eighty-three patients were in the mild-to-moderate alopecia areata group and 254 patients were in the severe group. Average onset age was 30.77±17.66 years and 30.60±16.75 years in the mild-to-moderate and severe groups, respectively. Disease duration was statistically longer in the severe group. Male sex, nail changes, and thyroid diseases were more common in the severe group. Hypertension, diabetes mellitus, dyslipidemia, atopic dermatitis, and family history did not differ between groups. Of the serologic values, only alkaline phosphatase was considerably differing between groups. Male sex, presence of nail changes, and disease duration greater than one year were identified as significant risk factors for severe alopecia areata. CONCLUSION: This is the largest case analysis in Korean patients with alopecia areata. Clinical profiles stratified by disease severity warrant further study.
Age of Onset ; Alkaline Phosphatase ; Alopecia Areata* ; Alopecia* ; Autoimmune Diseases ; Dermatitis, Atopic ; Diabetes Mellitus ; Dyslipidemias ; Epidemiology ; Humans ; Hypertension ; Male ; Retrospective Studies ; Risk Factors ; Thyroid Diseases

Autoimmune liver diseases including autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) are rare diseases. The aim of this review is to examine the epidemiology and clinical characteristics of AIH and PBC in South Korea. There were 4,085 patients registered as AIH in the Rare Intractable Disease Registry of Korea between 2009-2013, with a median age of 56 years and female-to male ratio of 6.4. The age-adjusted incidence and prevalence of AIH were 1.07/100,000/year and 4.82/100,000 persons, respectively. Among the patients, 1.1% underwent liver transplantation, and case fatality was 2.18%. Liver cirrhosis at diagnosis was accompanied in 23%; liver biopsy was performed in 75.2%, and prednisolone therapy or prednisolone and azathioprine combination therapy was done in 73% with a remission rate of 86%. There were 2,824 patients with PBC (≥20 years) registered in Korea between 2009-2013 with a median age of 57 years and female-to male ratio of 6.2. The age-adjusted incidence and prevalence of PBC were 0.86/100,000/year and 4.75/100,000 persons, respectively. Among the patients, 2.5% underwent liver transplantation, and case fatality was 2.2% with a 5-year transplantation-free survival of 95.4%. Ursodeoxycholic acid (UDCA) was prescribed in 90% of the patients with a UDCA inadequate response rate of 30%. In conclusion, AIH and PBC are rare but mostly treatable diseases if diagnosed in the early stages. However, scarce data, low awareness, delayed diagnosis and non-availability of 2nd line therapeutics are important issues to be solved. Therefore, governmental support for research and drug development and nationwide cooperative studies are warranted.
Azathioprine ; Biopsy ; Cholangitis ; Delayed Diagnosis ; Diagnosis ; Epidemiology* ; Hepatitis, Autoimmune ; Humans ; Incidence ; Korea* ; Liver Cirrhosis ; Liver Diseases* ; Liver Transplantation ; Liver* ; Male ; Prednisolone ; Prevalence ; Rare Diseases ; Ursodeoxycholic Acid

Protection against severe acute respiratory syndrome Coronavirus 2 (SARS-CoV-2) infection of inactivated vaccines is not well characterized in people with comorbidities, who are at high risk of severe infection. We compared the risk of SARS-CoV-2 infection after complete vaccination with Sinopharm/BBIBP in people with comorbidities (e.g., autoimmune diseases, cardiovascular disease, chronic lung disease, and diabetes) with healthy individuals using a Cox-proportional hazard model. In July-September 2021, a total of 10 548 people (comorbidities, 2143; healthy, 8405) receiving the complete primary series of vaccination with Sinopharm/BBIBP in Bangkok, Thailand were prospectively followed for SARS-CoV-2 infection through text messaging and telephone interviewing for 6 months. A total of 295 infections from 284 participants were found. HRs (95% CI) of individuals with any comorbidities did not increase (unadjusted, 1.02 (0.77-1.36), P = 0.89; adjusted, 1.04 (0.78-1.38), P = 0.81). HRs significantly increased in the subgroup of autoimmune diseases (unadjusted, 2.64 (1.09-6.38), P = 0.032; adjusted, 4.45 (1.83-10.83), P = 0.001) but not in cardiovascular disease, chronic lung disease, or diabetes. The protection against SARS-CoV-2 infection of the Sinopharm vaccine was similar in participants with any comorbidities vs. healthy individuals. However, the protection appeared lower in the subgroup of autoimmune diseases, which may reflect suboptimal immune responses among these people.
Humans ; COVID-19/prevention & control* ; Vaccines, Inactivated ; COVID-19 Vaccines ; SARS-CoV-2 ; Cardiovascular Diseases ; Prospective Studies ; Thailand ; Autoimmune Diseases ; Diabetes Mellitus/epidemiology*

The purpose of this study was to investigate the etiology, pathological characteristics, management and prognosis of chronic pancreatitis in the Chinese population. The clinical data of 142 patients with chronic pancreatitis were retrospectively studied. All patients were of Chinese nationality and hospitalized from January 2008 to December 2011. Their ages ranged from 14 to 76 years, with a mean of 43 years. Of 142 patients, there were 72 cases of obstructive chronic pancreatitis (50.70%), 19 cases of alcoholic chronic pancreatitis (13.38%), 14 cases of autoimmune pancreatitis (9.86%) and 37 cases of undetermined etiology (26.06%). Pathologically, the average inflammatory mass diameter was 3.8 ± 3.3 cm, biliary obstruction occurred in 36 cases, gall stones in 70 cases, calcification in 88 cases, ductal dilatation in 61 cases, side branch dilatation in 32 cases, ductal irregularity in 10 cases, lymphocytic inflammation in 23 cases, obliterative phlebitis in 14 cases, extra pancreatic lesion in 19 cases and fibrosis in 142 cases. Location of pancreatic lesion in the region of head (n=97), neck (n=16), body (n=12), tail (n=15) and whole pancreas (n=2) influenced the choice of surgical procedures. Ninety-four patients (66.20%) received surgical treatment and 33.80% received other treatments. After operation, 80.85% of 94 patients experienced decreased pain, and 8.51% of 94 showed recovery of endocrine function but with a complication rate of 12.77%. All the operations were performed successfully. According to the pain scale of European Organization for Research and Treatment of Cancer (QLQ-C30) a decrease from 76 ± 22 to 14 ± 18 was observed. Etiology, pathological characteristics, management and prognosis of chronic pancreatitis in the Chinese population vary from others.
Adolescent ; Adult ; Aged ; Autoimmune Diseases ; epidemiology ; therapy ; China ; epidemiology ; Cholestasis ; epidemiology ; therapy ; Female ; Humans ; Male ; Middle Aged ; Pancreatitis, Alcoholic ; epidemiology ; therapy ; Pancreatitis, Chronic ; etiology ; pathology ; therapy ; Prognosis ; Retrospective Studies ; Treatment Outcome ; Young Adult

PURPOSE: Autoimmunity is an alternative etiology of gastric inflammation, the initiating event in the gastric carcinogenic cascade. This mechanism may be an increasingly important cause of gastric cancer with the waning prevalence of its primary etiologic factor, chronic Helicobacter pylori infection. MATERIALS AND METHODS: PubMed and EMBASE were searched up to September 2018. Autoimmunity and 96 specific manifestations were considered for associations with gastric cancer risk. Random effects analysis was used to calculate pooled relative risk estimates (RR) and 95% confidence intervals (CI). RESULTS: We found a total of 52 observational studies representing 30 different autoimmune diseases. Overall, the presence of an autoimmune condition was associated with a gastric cancer pooled RR of 1.37 (95% CI, 1.24 to 1.52). Among the 24 autoimmune conditions with two or more independent reports, nine were significantly associated with increased gastric cancer risk: dermatomyositis (RR, 3.69; 95% CI, 1.74 to 7.79), pernicious anemia (RR, 2.84; 95% CI, 2.30 to 3.50), Addison disease (RR, 2.11; 95% CI, 1.26 to 3.53), dermatitis herpetiformis (RR, 1.74; 95% CI, 1.02 to 2.97; n=3), IgG4-related disease (RR, 1.69; 95% CI, 1.00 to 2.87), primary biliary cirrhosis (RR, 1.64; 95% CI, 1.13 to 2.37), diabetes mellitus type 1 (RR, 1.41; 95% CI, 1.20 to 1.67), systemic lupus erythematosus (RR, 1.37; 95% CI, 1.01 to 1.84), and Graves disease (RR, 1.27; 95% CI, 1.06 to 1.52). CONCLUSION: Our analysis documents the wide range of autoimmune diseases associated with gastric cancer. These associations may reflect unreported links between these conditions and autoimmune gastritis. Further studies are warranted to investigate potential causal mechanisms.
Addison Disease ; Anemia, Pernicious ; Autoimmune Diseases ; Autoimmunity ; Dermatitis Herpetiformis ; Dermatomyositis ; Diabetes Mellitus ; Epidemiology ; Gastritis ; Graves Disease ; Helicobacter pylori ; Inflammation ; Liver Cirrhosis, Biliary ; Lupus Erythematosus, Systemic ; Prevalence ; Stomach Neoplasms

BACKGROUND: Herpes zoster (HZ) is generally thought to occur once in a lifetime and recurrence is considered to be limited to immunocompromised individuals. Although HZ recurrence rates seem to be increasing, there have been few studies exploring these rates in the general population. We investigated the recurrence rate and associated risk factors in the general population. METHODS: We used the population-based samples of the National Health Insurance Service database to identify cases of initial HZ episodes from January 1, 2002 to December 31, 2013. We also followed up on these cases through December 31, 2013 to identify recurrence. RESULTS: Overall, the incidence rate of HZ is 5.1 per 1,000 person years and the recurrence rate is 12.0 per 1,000 person years. There were 2,100 recurrent cases out of 39,441 initial episodes with 4.4 years of the mean follow-up period. We identified significant risk factors for recurrence such as old age (51–70 years) (hazard ratio [HR], 1.447; 95% confidence interval [CI], 1.311–1.598), women (1.476; 1.345–1.619), zoster-related pain (ZRP) longer than 30 days (cases of ZRP lasting 31–90 days [1.200; 1.042–1.383], and ZRP lasting longer than 90 days [2.293; 1.990–2.643]). Concurrent hematologic malignancies (2.864; 1.929–4.251), autoimmune diseases (1.466; 1.252–1.715), dyslipidemia (1.390; 1.263–1.530), and hypertension (1.222; 1.107–1.350) were also significant risk factors. CONCLUSION: Our results suggest that the recurrence of HZ is much more common than generally expected, and that the associated risk factors can play an important role in predicting recurrence.
Autoimmune Diseases ; Cohort Studies* ; Dyslipidemias ; Epidemiology ; Female ; Follow-Up Studies ; Hematologic Neoplasms ; Herpes Zoster* ; Humans ; Hypertension ; Incidence ; National Health Programs ; Recurrence* ; Risk Factors*

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