To investigate the clinical and immunological features of dermatomyositis (DM) complicated with macrophage activation syndrome (MAS). The demographic and clinical characteristics of five patients diagnosed with DM complicated with MAS hospitalized in the Department of Rheumatology and Immunology, Peking University People ' s Hospital from 2011 to 2021 were collected. The results of clinical manifestations, laboratory tests, immunological features, treatments and prognosis were analyzed and summarized. In this study, five female patients in Peking University People's Hospital with an average age of 63.8 (44.0-83.0) years and an average disease duration of 16.1 (1.5-48.0) months. All the patients had typical DM rash (such as heliotrope sign, V/shawl sign or Gottron's sign/papules). They all had muscle involvement (including myalgia or muscle weakness). Two patients had positive myositis-specific antibodies (MSAs), in which case 1 had anti-TIF1-γ antibody and case 5 had anti-NXP-2 antibody. Four patients had interstitial lung disease except case 3. All of the cases developed MAS in the active stage of DM. Common manifestations of MAS in these five patients included high-grade fever, cytopenia, decreased fibrinogen, elevated ferritin and increased soluble CD25. Case 1 presented with neutropenia (0.6×10⁹ /L), thrombocytopenia (26.0×10⁹ /L), hypofibrinogenemia (0.9 g/L), markedly elevated ferritin (26 331.0 μg/L), decreased NK cell activity. Case 2 had anaemia (hemoglobin 81.0 g/L), thrombocytopenia (55.0×10⁹ /L), hypertriglyceridemia (4.7 mmol/L), hypofibrinogenemia (1.2 g/L), elevated ferritin (>100 000.0 μg/L), hemophagocytosis in bone marrow. Case 3 had anaemia (hemoglobin 88 g/L), decreased fibrinogen (1.9 g/L), increased ferritin (>27 759.0 μg/L), splenomegaly, hemophagocytosis in bone marrow. Case 4 suffered from neutropenia(0.3×10⁹ /L), anaemia(hemoglobin 78 g/L), hypertriglyceridemia (4.2 mmol/L), hypofibrinogenemia (0.9 g/L), increased ferritin (>100 000.0 μg/L), and decreased NK cell activity. Case 5 presented anaemia (hemoglobin 60.0 g/L), thrombocytopenia (67.0×10⁹ /L), hypertriglyceridemia (12.7 mmol/L), decreased fibrinogen (1.1 g/L), and elevated ferritin (>923.0 μg/L). All the patients were treated with methylprednisone pulse therapy (200-500 mg) combined with cyclosporine while case 5 received rituximab after methylprednisone pulses. In addition, case 3 also received the combination of mycophenolate mofetil. Case 1 was given etoposide while case 4 was treated with cyclophosphamide and repeated plasmapheresis at the same time. Moreover, intravenous immunoglobulin was added meantime apart from case 3. The condition of four patients improved significantly, nevertheless case 4 experienced recurred pulmonary symptoms and died of respiratory failure. As for complications about infection, case 2 had bacterial infection with high level procalcitonin (PCT) before MAS treatment and condition was improved after empiric antibacterial therapy. Case 3 had cytomegalovirus DNAemia before diagnosis of MAS and viral titer turned negative after ganciclovir therapy. After treatment of MAS, four patients developed cytomegalovirus DNAemia except case 3, in which case 5 was co-infected with bacteria. To sum, DM complicated with MAS is relatively rare, and its patients are of ten in life-threatening condition. Early detection, treatment and prevention of infection during treatment are critical to improve the prognosis.
Humans ; Female ; Middle Aged ; Dermatomyositis/complications* ; Macrophage Activation Syndrome/complications* ; Afibrinogenemia/complications* ; Autoantibodies ; Neutropenia ; Thrombocytopenia/complications* ; Ferritins/therapeutic use* ; Hypertriglyceridemia/complications* ; Fibrinogen/therapeutic use*

Hemophagocytic lymphohistiocytosis (HLH) was a life-threatening syndrome due to the uncontrolled immune activation of cytotoxic T lymphocytes, natural killer (NK) cells, and macrophages. HLH is characterized by primary and secondary causes, the early diagnosis and treatment of patients are closely related to the prognosis and clinical outcome of patients. The clinical presentation is variable but mostly includes prolonged fever, splenomegaly, coagulopathy, hypertriglyceridemia, and hemophagocytosis, none of them is specific and particular for HLH. Tuberculosis (TB) infection is one of the causes of HLH. HLH caused by TB is very rare clinically, but it has a high mortality. For patients with fever of unknown origin, HLH-related clinical manifestations sometimes present before the final diagnosis of TB, and HLH is associated with the most significant mortality rate. This article is mainly about a 28-year-old patient with HLH who suffered from severe TB infection. The patient attended a hospital with a history of 2 months of prolonged fever, 10 days booger and subcutaneous hemorrhage in lower limbs. Before this, he was in good health and denied any history of tuberculosis exposure. Combined with relevant laboratory test results (such as splenomegaly, hemoglobin, platelet count, and hypertriglyceridemia) and clinical manifestations (e.g. fever), the patient was diagnosed with hemophagocytic lymphohistiocytosis, but the etiology of HLH remained to be determined. To confirm the etiology, the patient was asked about the relevant medical history (intermittent low back pain) and was performed chest CT scan, bone marrow biopsy, and fundus photography. Finally, he was diagnosed with hemophagocytic lymphohistiocytosis caused by hematogenous disseminated pulmonary tuberculosis. In response to this, intravenous methylprednisolone and anti-tuberculosis treatment (isoniazid, pyrazinamide, moxifloxacin, and amikacin) were administered to the patient. After more than a month of treatment, the patient recovered from HLH caused by severe TB infection. Therefore, this case suggests that we should be vigilant to the patient who admitted to the hospital with fever for unknown reasons, to diagnose HLH as early as possible and clarify its cause, then perform interventions and treatment, especially HLH secondary to tuberculosis. Also, cases of atypical TB and severe TB should be carefully monitored to achieve early diagnosis and early intervention.
Male ; Humans ; Adult ; Lymphohistiocytosis, Hemophagocytic/diagnosis* ; Splenomegaly ; Tuberculosis, Pulmonary/diagnosis* ; Bone Marrow/pathology* ; Fever/etiology* ; Hypertriglyceridemia/complications*

BACKGROUND/AIMS: Nonalcoholic fatty liver disease (NAFLD) is an emerging problem in Asia, but little is known about the disease in the nonobese population. The aims of this study were to investigate the prevalence of NAFLD and the factors associated with it in a nonobese Korean population and to compare the clinical characteristics of nonobese and obese subjects with NAFLD. METHODS: This cross-sectional study used data from 2,058 subjects who participated in a medical checkup program. RESULTS: The prevalence of NAFLD was 12.4% (213/1,711) in the nonobese population. A higher body mass index (BMI), higher homeostasis model assessment of insulin resistance (HOMA-IR) values, higher alanine aminotransferase (ALT) levels, triglyceride concentrations 150 mg/dL, and hyperuricemia were independently associated with the presence of NAFLD in the nonobese subjects. Compared with the obese subjects with NAFLD, the nonobese subjects with NAFLD were composed of a higher proportion of females and had lower BMIs, smaller waist circumferences, lower HOMA-IR values, and fewer metabolic irregularities. CONCLUSIONS: Higher BMIs, HOMA-IR values, ALT levels, hypertriglyceridemia, and hyperuricemia were associated with NAFLD in the nonobese subjects. Clinicians should be particularly aware of the possibility of NAFLD in nonobese Asian people.
Adult ; Alanine Transaminase/blood ; Anthropometry ; Asian Continental Ancestry Group/*statistics & numerical data ; Body Mass Index ; Cross-Sectional Studies ; Female ; Homeostasis ; Humans ; Hypertriglyceridemia/complications ; Hyperuricemia/complications ; Insulin Resistance ; Male ; Middle Aged ; Non-alcoholic Fatty Liver Disease/blood/*epidemiology/*etiology ; Obesity/complications ; Prevalence ; Republic of Korea/epidemiology ; Risk Factors ; Sex Factors ; Triglycerides/blood ; Waist Circumference

Dry eye syndrome (DES) is recognized as a public health concern. One of the pathophysiologies in the development of DES is inflammation, and metabolic syndrome (MetS), which is highly prevalent in the general population, is a well-known chronic and systemic inflammatory condition. Despite the increasing interest regarding a relationship between DES and MetS, information is lacking on the association between DES and MetS and its individual components. We investigated the association between DES symptoms and MetS and its components among adults aged ≥ 19 years using population-based data from the Korea National Health and Nutrition Examination Survey V. A sample group of 15,294 adults (42.67% men and 57.33% women) completed household interviews in which they provided blood (for high-density lipoprotein cholesterol, triglyceride, and glucose) and anthropometric measurements (including waist circumference, weight, and height) to define MetS. We also collected information regarding sociodemographic and behavioral risk factors. The survey results showed that 11.50% of men and 22.35% of women experienced DES and 5.30% of patients had both DES and diagnosis of MetS, including 204 men and 606 women. Thus, no significant difference was observed between DES and the diagnosis of MetS according to sex (P = 0.4008 in men; P = 0.0804 in women); however, a significant association was observed between DES and hypertriglyceridemia in women (OR, 1.13; 95% CI, 1.01-1.29). Therefore, hypertriglyceridemia might be an important factor in the association between DES and MetS. Further longitudinal research is needed to evaluate this relationship.
Adult ; Aged ; Blood Glucose/analysis ; Body Height ; Body Weight ; Cholesterol, HDL/blood ; Cross-Sectional Studies ; Dry Eye Syndromes/complications/*diagnosis/epidemiology ; Female ; Humans ; Hypertriglyceridemia/complications/diagnosis ; Interviews as Topic ; Male ; Metabolic Syndrome X/complications/*diagnosis/epidemiology ; Middle Aged ; Nutrition Surveys ; Prevalence ; Republic of Korea/epidemiology ; Risk Factors ; Sex Factors ; Triglycerides/blood ; Waist Circumference ; Young Adult

BACKGROUND: We aimed to determine the major contributing component of metabolic syndrome (MetS) that results in an elevated small dense LDL cholesterol (sdLDL-C) concentration and sdLDL-C/LDL-C ratio. METHODS: Four hundred and forty-seven subjects (225 men; 222 women) with MetS were randomly selected from the Korean Metabolic Syndrome Research Initiatives-Seoul cohort study. Age- and sex-matched healthy controls (181 men; 179 women) were also randomly selected from the same cohort. RESULTS: A comparison of the median values of the sdLDL-C concentration between subgroups, divided according to whether subjects met or did not meet the criteria for each MetS component in patients with MetS, revealed a significant difference in the sdLDL-C concentration only between subgroups divided according to whether subjects met or did not meet the triglyceride (TG) criteria (P<0.05 for each gender). The TG level showed a good correlation with sdLDL-C concentration (correlation coefficients [r]=0.543 for men; 0.653 for women) and the sdLDL-C/LDL-C ratio (r=0.789 for men; 0.745 for women). Multiple linear regression analyses conducted for the MetS group concordantly identified TG as one of the most significant contributors to sdLDL-C concentration (beta=0.1747+/-0.0105, P<0.0001) and the sdLDL-C/LDL-C ratio (beta=6.9518+/-0.3011, P<0.0001). CONCLUSIONS: Among five MetS components, only the abnormal TG level was a differentiating factor for sdLDL-C concentration and sdLDL-C/LDL-C ratio. These results were reproducible in both genders, with or without MetS.
Adult ; Case-Control Studies ; Cholesterol, LDL/*blood ; Cohort Studies ; Female ; Humans ; Hypertriglyceridemia/*complications/diagnosis ; Linear Models ; Male ; Metabolic Syndrome X/*complications/diagnosis ; Middle Aged ; Risk Factors ; Triglycerides/blood

The aim of this study was to describe the incidence of metabolic syndrome and to identify five components as metabolic syndrome predictors. The final study included 1,095 subjects enrolled in a rural part of Daegu Metropolitan City, Korea for a cohort study in 2003. Of these, 762 (69.6%) subjects had participated in the repeat survey. During the five-year follow-up, incidence density was significantly higher for women than for men (men, 30.0/1,000 person-years; women, 46.4/1,000 person-years). In both men and women, incidence of metabolic syndrome showed a significant increase with increasing number of metabolic syndrome components at baseline. Compared with individuals presenting none of components at baseline, relative risks were increased 1.22 (men; 95% CI, 0.43-3.51), 2.21 (women; 95% CI, 0.98-4.97) times more for individuals with one component of metabolic syndrome and 5.30 (men; 95% CI, 2.31-12.13), 5.53 (women; 95% CI, 2.78-11.01) times more for those who had two components. In multivariate analysis, the most powerful risk factor for metabolic syndrome was abdominal obesity in men and low HDL-cholesterol in women (adjusted relative risk, 3.28, 2.53, respectively). Consequently, finding a high risk group for metabolic syndrome according to gender and prevention of metabolic syndrome through lifestyle modification are essential.
Adult ; Aged ; Cholesterol, HDL/blood ; Cohort Studies ; Diabetes Mellitus, Type 2/complications ; Female ; Follow-Up Studies ; Humans ; Hypertension/complications ; Hypertriglyceridemia/complications ; Incidence ; Male ; Metabolic Syndrome X/complications/*epidemiology ; Middle Aged ; Multivariate Analysis ; Obesity, Abdominal/complications ; Republic of Korea/epidemiology ; Risk Factors

BACKGROUND/AIMS: Current international guidelines recommend colorectal cancer screening for average-risk people over 50 years of age. Accordingly, we aimed to estimate the prevalence of colorectal neoplasms in all age groups and evaluate associated risk factors. METHODS: Data of 14,932 subjects who underwent colonoscopy from July 2006 to January 2012 at Health Promotion Center, Gangnam Severance Hospital (Seoul, Korea) as part of a health check-up were reviewed retrospectively. RESULTS: The overall prevalence of colorectal neoplasms and adenoma were 34.6% and 25.3%, respectively. Colorectal adenoma was found in 3.2%, 13.0%, 21.7%, 33.8%, 44.0%, 50.5%, and 54.2% of subjects under 30 years, 30-39 years, 40-49 years, 50-59 years, 60-69 years, 70-79 years, and over 80 years of age (trend p<0.0001). Independent predictors of colorectal adenoma included male gender (OR 2.38, 95% CI 2.084-2.718), positive occult blood (2.266, 1.761-2.917), positive serology of Helicobacter pylori (1.253, 1.114-1.409) and hypertriglyceremia (1.267, 1.065-1.508). Compared to the 30-39 years of age reference group, the ORs for each age group were 0.195 (under 30 years), 1.634 (40-49 years), 2.954 (50-59 years), 5.159 (60-69 years), 5.640 (70-79 years), 11.020 (over 80 years), while the 95% CIs were 0.071-0.536 (under 30 years), 1.340-1.992 (40-49 years), 2.421-3.604 (50-59 years), 4.109-6.476 (60-69 years), 3.822-8.322 (70-79 years), and 2.809-42.234 (over 80 years). CONCLUSIONS: Colorectal adenoma prevalence increased proportionally with age. Only subjects under the age of 30 years had a definitely lower prevalence of colorectal adenoma. Male gender, positive occult blood, positive serology of H. pylori, and hypertriglyceremia were associated risk factors of colorectal adenoma.
Adenoma/*epidemiology/etiology ; Adult ; Age Factors ; Aged ; Aged, 80 and over ; Asian Continental Ancestry Group ; Colonoscopy ; Colorectal Neoplasms/*epidemiology/etiology ; Female ; Helicobacter Infections/complications ; Helicobacter pylori ; Humans ; Hypertriglyceridemia/complications ; Logistic Models ; Male ; Middle Aged ; Odds Ratio ; Republic of Korea/epidemiology ; Retrospective Studies ; Sex Factors

The purpose of current study was to investigate associations of serum 25-hydroxyvitamin D (OHVD) levels with markers for metabolic syndrome in elderly Koreans. We conducted a panel study on 301 individuals over 60 yr old in Seoul, Korea, and repeatedly measured serum OHVD, glucose, insulin, and lipid levels. Mixed effect model and generalized estimating equations were used to investigate relationships between serum OHVD levels with marker levels for metabolic syndrome and each of its categories. Of all subjects, 76.6% were vitamin D deficient (< 50 nM) and 16.9% were insufficient (< 75 nM). Inverse association was demonstrated between serum OHVD levels and insulin (P = 0.004), triglyceride (P = 0.023) and blood pressure (systolic blood pressure: P = 0.002; diastolic blood pressure: P < 0.001). Vitamin D deficiency was found to increase risk of 'hypertriglyceridemia' category of metabolic syndrome (odds ratio: 1.73, 95% confidence interval: 1.13-2.66). In conclusion, we found from our repeated measure analysis that decreasing serum OHVD levels are associated with increasing insulin resistance, increasing serum triglyceride levels and increasing blood pressure in elderly Koreans, and confirmed on the risk of 'hypertriglyceridemia' in vitamin D deficient subjects.
Aged ; Aged, 80 and over ; Biological Markers/blood ; Blood Pressure ; Female ; Humans ; Hypertriglyceridemia/diagnosis/etiology ; Insulin/blood ; Insulin Resistance ; Male ; Metabolic Syndrome X/*diagnosis/etiology ; Middle Aged ; Odds Ratio ; Risk Factors ; Triglycerides/blood ; Vitamin D/*analogs & derivatives/blood ; Vitamin D Deficiency/complications

Obesity is defined as BMI (calculated as weight in kg divided by height in m2) more than 30, and overweight is defined as BMI of 25-29.9. Obesity has been considered as a risk factor for pancreatic diseases, including pancreatitis and pancreatic cancer. Severe acute pancreatitis is significantly more frequent in obese patients. Furthermore, obese patients develop systemic and local complications of acute pancreatitis more frequently. The underlying mechanisms are increased inflammation and necrosis from increased amount of intra- and peri-pancreatic fat. In addition, obesity is a poor prognostic factor in acute pancreatitis, and overweight before disease onset appears to be a risk factor for chronic pancreatitis. Overweight and/or obesity are associated with greater risk of pancreatic cancer and younger age of onset. Physical activity appears to decrease the risk of pancreatic cancer, especially among those who are overweight. Long-standing diabetes increases the risk of pancreatic cancer. The pathogenic mechanism is that obesity and physical inactivity increase insulin resistance. In a state of hypersinulinemia, increased circulating level of insulin-like growth factor-1 induces cellular proliferation of pancreatic cancer. Obesity is associated with negative prognostic factor and increased mortality in pancreatic cancer. However, there are controversies regarding the effects of obesity on long-term post-operative results in the patient with pancreatic cancer.
Body Mass Index ; Humans ; Hypertriglyceridemia/complications ; Obesity/*complications ; Overweight ; Oxidative Stress ; Pancreatic Diseases/*etiology ; Pancreatic Neoplasms/etiology ; Somatomedins/metabolism/physiology

We report a case of diabetic ketoacidosis (DKA) and hypertriglyceridemia (severely elevated to 15,240 mg/dL) complicated by acute pancreatitis, which was treated successfully with insulin therapy and conservative management. A 20-yr-old woman with a history of type 1 diabetes came to the emergency department 7 months after discontinuing insulin therapy. DKA, severe hypertriglyceridemia and acute pancreatitis were diagnosed, with DKA suspected of contributing to the development of the other conditions. In Korea, two cases of DKA-induced hypertriglyceridemia and 13 cases of hypertriglyceridemia-induced acute pancreatitis have been previously reported separately.
Acute Disease ; Diabetes Mellitus, Type 1/complications/diagnosis/therapy ; Diabetic Ketoacidosis/complications/*diagnosis/therapy ; Female ; Humans ; Hypertriglyceridemia/complications/*diagnosis/therapy ; Insulin/therapeutic use ; Pancreatitis/complications/*diagnosis ; Tomography, X-Ray Computed ; Young Adult