A 45-year-old woman was admitted to our hospital because of a high fever, dyspnea, and myalgia. At the time of admission, a diagnosis of systemic lupus erythematosus(SLE) was made by fulfilling four of the 1982 American College of Rheumatology criteria with increasing levels of anti-nuclear antibody titer(speckled pattern). Prednisolone given orally in an initial dosage of 30mg/day was not effective and she was expired by respiratory failure due to disseminated intravascular coagulation. A diagnosis of hemophagocytic syndrome was made because of the increased number of unusual hemophagocytic cells in the bone marrow. High levels of serum ferritin which are known to reflect macrophage activition, supported the diagnosis of hemophagocytic syndrome. Hemophagocytic syndrome is characterized by activated phagocytosis presumably induced by hypersecretion of cytokines. Malignant lymphoma and infection are the two representative diseases which may cause hemophagocytic syndrome. Recently several acute lupus hemophagocytic syndromes were reported in patients with SLE. Here we report a case of acute lupus hemophagocytic syndrome observed in a patient with SLE with brief review of literatures.
Bone Marrow ; Cytokines ; Diagnosis ; Disseminated Intravascular Coagulation ; Dyspnea ; Female ; Ferritins ; Fever ; Humans ; Lymphohistiocytosis, Hemophagocytic* ; Lymphoma ; Macrophages ; Middle Aged ; Myalgia ; Phagocytosis ; Prednisolone ; Respiratory Insufficiency ; Rheumatology

Patients with NIDDM are at increased risk for the development of coronary atherosclerosis and experience more silent myocardial infarction than non-diabetic subjects. The screening tools for early detection of coronary artery disease without significant narrowing has been requested in diabetic patients. Coronary artery calcification (CAC) score, quantified by electron beam computed tomography (EBT), have been reported to correlate with the amount of atherosclerotic plaque and vascular luminal narrowing. We investigated the distribution of CAC score and associated risk factors in asymptomatic NIDDM patients and patients with ischemic heart disease to estimate the usefulness of CAC as a screenig tool for ischemic heart disease in asymptomatic diabetes. METHOD: 136 NIDDM patients without any symptom of coronary artery disease and 37 patients with significant coronary artery stenosis were included. CAC were measured by electron bean tomography (ultrafast CT). Forty contiguous 3-mm thickness transverse two-dimensional sections were obtained through root of aorta and heart. Coronary calcification were defined as the presense of at least two adjacent pixel within the border of visualized coronary artery with CT number of at least 130 HU. Body mass index, waist-hip ratio were measured and body fat components were counted by impedence method. Visceral fat versus subcutaneous fat ratio were calculated by abdominal computed tomography. Plasma lipid profile, fasting insulin, C-peptide level, HbA1c concentration were measured. Correlations between natural log of CAC score and clinical parameters were evaluated and multiple regression analysis with natural log of CAC score as a independent variable was performed. Coronary angiography were performed in 17 asymptomatic NIDDM patients.. RESULT: CAC score was significantly higher in male than female subjects and increased significantly with aging (p<0.01). In patients with hypertension, previous history of cerebrovascular or peripheral vascular disease (p<0.05), CAC score was significantly increased. The CAC score showed significant positive correlations with smoking amount, duration of diabetes and a negative correlation with HDL-cholesterol (p<0.05). There were no association between CAC score and total cholesterol, LDL-cholesterol, waist to hip circumference ratio, or fasting insulin levels. After adjustment of compounding variables (age, sex), duration of diabetes, amount of smoking and previous history of atherosclerotic vascular disease were shown to be associated with CAC score. In multiple logistic regression analysis with natural log of CAC score as dependent variable, age, HDL-cholesterol, duration of diabetes, male gender were found to be significant independent variables. Seventeen diabetic patients with high CAC score, were taken coronary angiography and significant luminal narrowings (more than 50%) of coronary artery were documented in 16 patients. But, In 7 out of 17 patients with coronary one-vessel disease, coronary calcification were not detected by EBT. CONCLUSION: coronary artery calcium score quantified by electron beam computed tomography may be useful for screening of preclinical or asymptomatic coronary artery disease in asymptomatic NIDDM patients.
Adipose Tissue ; Aging ; Aorta ; Body Mass Index ; C-Peptide ; Calcium ; Cholesterol ; Coronary Angiography ; Coronary Artery Disease* ; Coronary Disease ; Coronary Stenosis ; Coronary Vessels* ; Diabetes Mellitus ; Diabetes Mellitus, Type 2 ; Fasting ; Female ; Heart ; Hip ; Humans ; Hypertension ; Insulin ; Intra-Abdominal Fat ; Logistic Models ; Male ; Mass Screening* ; Myocardial Infarction ; Myocardial Ischemia ; Peripheral Vascular Diseases ; Phenobarbital ; Plaque, Atherosclerotic ; Plasma ; Risk Factors ; Smoke ; Smoking ; Subcutaneous Fat ; Tomography, X-Ray Computed* ; Vascular Diseases ; Waist-Hip Ratio

Catastrophic antiphospholipid syndrome is a rare clinical syndrome characterized by acute multi-organ failure occurring in patients with antiphospholipid antibodies. It is associated with involvement of several end-organs particularly kidneys, lungs, gastrointestinal tracts and adrenal glands and presents catastrophic clinical pictures such as acute renal failure with thrombotic microangiopathy, myocardial failure, adult respiratory distress syndrome, convulsion and disseminated intravascular coagulation. Conventional treatments(e.g. intravenous heparin, steroid, immunosuppressants) were not effective, while plasmapheresis seems to be a useful therapy. We experienced a case of catastrophic primary antiphospholipid syndrome in 41-year-old woman proved by renal biopsy and immuno-serological tests. She developed acute renal failure, multiple esophageal and oral ulcers, adult respiratory distress syndrome, abnormal elevation of hepatic and pancreatic enzymes and signs of disseminated intravascular coagulation. Evidences of any other connective tissue diseases were not found. Renal biopsy revealed features of thrombotic microangiopathic nephropathy and serum antiphospholipid antibody level was elevated(34GPL). In spite of steroid, cyclophosphamide and supportive therapies, her respiratory distress was not improved.
Acute Kidney Injury ; Adrenal Glands ; Adult ; Antibodies, Antiphospholipid ; Antiphospholipid Syndrome* ; Biopsy ; Connective Tissue Diseases ; Cyclophosphamide ; Disseminated Intravascular Coagulation ; Female ; Gastrointestinal Tract ; Heart Failure ; Heparin ; Humans ; Kidney ; Lung ; Oral Ulcer ; Plasmapheresis ; Respiratory Distress Syndrome, Adult ; Seizures ; Thrombotic Microangiopathies