No abstract available.
Aneurysm

Giant cell arteritis (GCA) is a systemic vasculitis predominantly found in individuals of Northern European ancestry over 50 years of age. Typically it presents with new-onset persistent headache, claudication of jaws, and existence of an abnormal temporal artery. However, the diagnosis of GCA and the assessment of its activity remain challenging, especially in patients presenting with a variety of non-specific symptoms and laboratory tests. In those cases, 18F-fluorodeoxy-glucose positron emission tomography (18F-FDG-PET) is useful for the diagnosis of GCA. Recently, the number of foreign patients who visit domestic hospitals for medical care is increasing in Korea. Here-in, we report a Russian patient who was admitted to our hospital with fever of unknown origin (FUO). FUO study was performed to search for infection or malignancy and GCA was suspected by 18F-FDG-PET. The patient was eventually diagnosed with GCA by random temporal artery biopsy.
Biopsy ; Fever ; Fever of Unknown Origin ; Giant Cell Arteritis ; Giant Cells ; Headache ; Humans ; Jaw ; Korea ; Positron-Emission Tomography ; Systemic Vasculitis ; Temporal Arteries

Recently the incidence of Group B streptococcus (Streptococcus agalactiae) infection has been increased in nonpregnant adults, especially including the elderly and those with underlying diseases. One year ago, a 72-year-old diabetic woman underwent both total knee replacement and then received intermittently acupuncture due to both knee pain. Five days ago, she developed painful swelling on right knee joint. The synovial fluid showed leukocytosis (WBC 8,200/mm3), she was diagnosed as prosthetic joint infection, and treated with cefazolin. Her condition was rapidly aggravated despite of antibiotics therapy, open debridement and drainage was performed. But she expired due to sepsis. Both blood and synovial fluid culture yielded S. agalactiae. We reported a case of prosthetic knee joint infection caused by S. agalactiae.
Acupuncture ; Adult ; Aged ; Anti-Bacterial Agents ; Arthroplasty, Replacement, Knee ; Cefazolin ; Debridement ; Drainage ; Female ; Humans ; Incidence ; Joints ; Knee ; Knee Joint ; Leukocytosis ; Sepsis ; Streptococcus ; Streptococcus agalactiae ; Synovial Fluid

There are numerous studies about the transformation of renal pathology during lupus nephritis progression. A number of researchers suggest that patients with previous proliferative glomerulonephritis may not need to repeat renal biopsy in relation to treatment strategies. However, the pathology of renal biopsy could offer important information to clinicians about the progression of disease. Here, we report a rare case of the convertion of ISN/RPS classification from a proliferative lesion to a wholly non-proliferative lesion. A 40-year-old female was admitted complaining of generalized edema for 1 month. At the age of 33 she had been diagnosed as SLE with proliferative lupus nephritis. The renal remission was induced with corticosteroid pulse therapy and 12 cycles of intravenous cyclophosphamide treatment. The repeated renal biopsy revealed class V lupus nephritis compared with referential biopsy of class IV-G. A better prognosis is expected with lower activity and a lower chronicity index. Repeat renal biopsy may give useful information relating to the prognosis of nephritis.
Adult ; Biopsy ; Cyclophosphamide ; Edema ; Female ; Glomerulonephritis ; Humans ; Lupus Nephritis ; Nephritis ; Prognosis

Macrophagic myofasciitis (MMF) is a rare disease, often associated with the pathological persistence of aluminum hydroxide used in some vaccines, and is characterized by macrophage infiltration of the muscle. We report a case of MMF, initially thought to be a metastatic infection. A 38-year-old woman presented with fever, as well as pain and weakness in both thighs. On physical examination both thighs were swollen and lower-extremity motor-power was decreased to grade III. Laboratory tests showed leukocytosis and elevation of acute phase reactants, but all muscle enzymes except lactate dehydrogenase (LDH) were within normal range. Initially metastatic infection was suspected but she was diagnosed with MMF by muscle biopsy showing heavy CD68 positive macrophage infiltration. Her myalgia and muscle weakness improved after systemic steroid treatment. This case suggests that MMF might be considered for a patient with unexplained inflammatory myopathy with or without a history of vaccination.
Acute-Phase Proteins ; Adult ; Aluminum Hydroxide ; Biopsy ; Fasciitis ; Female ; Fever ; Humans ; Hydroxides ; L-Lactate Dehydrogenase ; Leukocytosis ; Macrophages ; Muscle Weakness ; Muscles ; Myositis ; Physical Examination ; Rare Diseases ; Reference Values ; Thigh ; Vaccination ; Vaccines

Secondary amyloidosis is one of the most serious complications in chronic inflammatory diseases such as rheumatoid arthritis. The extracellular deposit of aggregates of amyloid leads to target organ dysfunction. The mainstay treatment of secondary amyloidosis is the control of underlying disease activity. Many reports have reported that TNF-alpha inhibitors improve clinical outcomes. Here, we encountered a 34-year-old patient with juvenile rheumatoid arthritis who developed secondary amyloidosis despite treatment with TNF-alpha inhibitors. We present this case and include a review of the literature.
Adult ; Amyloid ; Amyloidosis ; Arthritis, Juvenile Rheumatoid ; Arthritis, Rheumatoid ; Humans ; Tumor Necrosis Factor-alpha

Systemic lupus erythematosus (SLE) is an autoimmune disease with various manifestations, while its autoantibodies and immune reactions involve multiple organs. Neuropsychiatric involvement in SLE is known to be common, however, peripheral neuropathy is relatively rare. Guillain-Barre syndrome is clinically defined as an acute demyelinating peripheral neuropathy causing weakness and numbness in the legs and arms. We describe a case of Guillain-Barre syndrome with antiphospholipid syndrome and lupus nephritis. The patient was admitted with fever and diarrhea. He developed progressive weakness of the upper and lower extremities and dysarthria with characteristic nerve conduction patterns compatible with Guillain-Barre syndrome. He also had proteinuria and gangrene of the hand and toe with antiphospholipid antibody. He received intravenous immunoglobulin and plasmapheresis for progressive neuropathy, intravenous high dose steroid to control activity of SLE, and anticoagulation for antiphospholipid syndrome. Neuropsychiatric manifestation of SLE is related to lupus activity closely, so it is important to control lupus activity.
Antibodies, Antiphospholipid ; Antiphospholipid Syndrome ; Arm ; Autoantibodies ; Autoimmune Diseases ; Diarrhea ; Dysarthria ; Fever ; Gangrene ; Guillain-Barre Syndrome ; Hand ; Humans ; Hypesthesia ; Immunoglobulins ; Leg ; Lower Extremity ; Lupus Erythematosus, Systemic ; Lupus Nephritis ; Neural Conduction ; Peripheral Nervous System Diseases ; Plasmapheresis ; Proteinuria ; Toes

Wegener's granulomatosis (WG) is a multisystemic granulomatous inflammatory disorder which is presumably caused by an autoimmune response. It typically targets the upper and lower respiratory tract and the kidney. Ocular involvement occurs in 50 to 60% of WG patients. However, orbital myositis and diplopia as a presenting symptom is a rare ocular manifestation. We present the case of a 49-year-old man who was admitted with bilateral diplopia due to orbital myositis under a diagnosis of WG. He was successfully treated with high dose corticosteroid therapy.
Autoimmunity ; Diplopia ; Humans ; Kidney ; Middle Aged ; Myositis ; Orbital Myositis ; Respiratory System ; Wegener Granulomatosis

OBJECTIVE: A higher prevalence of metabolic syndrome (MetS) has been described in various rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus, but no direct studies have investigated the prevalence of MetS in systemic sclerosis (SSc) patients compared with the general population. We compared the frequency of MetS and insulin resistance, as measured by Homeostatic assessment model-insulin resistance (HOMA-IR) between female SSc patients and healthy subjects. METHODS: In a cross-sectional setting, 35 female SSc patients and 84 age and sex-matched healthy subjects were consecutively recruited at a single center of a tertiary hospital between 2009 and 2010. MetS was defined according to the National Cholesterol Education Program's Adult Treatment Panel (NCEP-ATP III) 2004 criteria, using the Asian criteria for central obesity. RESULTS: The frequency of MetS was not significantly higher in SSc patients (20.0%) than in healthy subjects (14.3%, p=0.425), but SSc patients had an increased insulin resistance measured by HOMA-IR (p=0.001). Significantly more patients with SSc had increased triglyceride concentrations (p=0.004) and fewer SSc patients tended to meet the waist circumference criterion (p=0.051) according to the individual MetS features. Additionally, there were no significant differences in age, high-sensitivity C-reactive protein, body mass index, HOMA-IR, disease duration, cumulative glucocorticoids dose, modified Rodnan skin score or the proportion of limited SSc cases according to the absence or presence of MetS in SSc patients. CONCLUSION: Unlike other rheumatic diseases, our preliminary report did not show a significant difference in the prevalence of MetS between female SSc patients and healthy subjects.
Adult ; Arthritis, Rheumatoid ; Asian Continental Ancestry Group ; Body Mass Index ; C-Reactive Protein ; Cholesterol ; Female ; Glucocorticoids ; Humans ; Insulin Resistance ; Lupus Erythematosus, Systemic ; Prevalence ; Rheumatic Diseases ; Scleroderma, Systemic ; Skin ; Tertiary Care Centers ; Waist Circumference

OBJECTIVE: Henoch-Schonlein purpura (HSP) is a systemic vasculitis, characterized by small-vessel leukocytoclastic vasculitis with the deposition of immune complexes containing IgA. It is the most common acute vasculitic disorder affecting children but is relatively uncommon in adults. We investigated the clinical features and factors affecting the prognosis of adult HSP in Korea. METHODS: From 1996 to 2011 seventy patients over 15 years of age with HSP were retrospectively analyzed. RESULTS: Thirty eight patients (54.3%) were female and the age at disease onset ranged from 15 to 75 years (35.0+/-15.8 years). Purpuric skin rash was observed in all patients and arthralgia was present in 34 patients (48.6%). GI symptoms and kidney involvements were observed in 28 patients (40.0%) and 34 patients (48.6%), respectively. Complete remission was achieved in 46 patients (65.7%). The remission group showed a lower incidence of hematochezia (p=0.044), hematuria (p=0.008), and proteinuria (p=0.011) at diagnosis than the no remission group. About 10% of adult HSP patient progressed to chronic kidney disease (CKD), which showed higher a incidence of nephrotic range proteinuria. Only nephrotic range proteinuria at diagnosis was a significant risk factor for CKD (OR=16.7, p=0.008, 95% CI=2.1~133.1). CONCLUSION: Hematochezia, hematuria and proteinuria at the diagnosis of HSP are important prognostic factors in predicting remission. In addition, HSP patients with nephrotic range proteinuria at diagnosis have an increased risk of renal failure.
Adult ; Antigen-Antibody Complex ; Arthralgia ; Child ; Exanthema ; Female ; Gastrointestinal Hemorrhage ; Hematuria ; Humans ; Immunoglobulin A ; Incidence ; Kidney ; Korea ; Prognosis ; Proteinuria ; Purpura, Schoenlein-Henoch ; Renal Insufficiency ; Renal Insufficiency, Chronic ; Retrospective Studies ; Risk Factors ; Systemic Vasculitis ; Vasculitis ; Vasculitis, Leukocytoclastic, Cutaneous