No abstract available.
Lupus Erythematosus, Systemic*

Systemic lupus erythematosus is a connective tissue disease which can affect every organ system. Neurologic abnormalities are common, occuring in approximately half of all patients at some time during the course of their illness. But symptoms of nervous system as the sole presenting symptoms occur in less than 1% of lupus patients. In patients initially presenting with neurologic symptoms and signs, differential diagnosis is difficult and sometimes it may be misdiagnosed. Therefore extensive laboratory investigations should be carried out in all patients with unusual neurological symptoms, since early diagnosis of lupus can help in providing effective treatment. We report a patient with systemic lupus erythematosus who presented with dysarthria and dysphagia resembling multiple sclerosis.
Connective Tissue Diseases ; Deglutition Disorders ; Diagnosis, Differential ; Dysarthria ; Early Diagnosis ; Humans ; Lupus Erythematosus, Systemic* ; Multiple Sclerosis* ; Nervous System ; Neurologic Manifestations

Rheumatoid arthritis is a chronic inflammatory disease that primarily affects joint synovium. Although its etiology has yet to be identified, the underlying mechanism of joint inflammation is understood as autoimmune process. The inflamed synovium is thickened due to synovial hyperplasia and infiltrating mononuclear cells such as T and B lymphocytes, macrophages, and plasma cells. Key inflammatory cytokines TNF-alpha and IL-1, as shown by the significant therapeutic effect of their blockade, are mainly secreted by macrophages whereas IL-17 is secreted by a newly recognized subset of T cells (Th17 cells) and induces TNF-alpha and IL-1 production by adjacent macrophages, synoviocytes, and chondrocytes. IL-17 has also been shown to induce RANKL from osteoblasts, thereby indicating that this cytokine plays as an upstream molecule that regulates inflammation and osteoclastogenesis. More importantly, IL-17 has been shown to convert acute inflammation into chronic inflammation and when combined with already important cytokines, more marked inflammation occurs. The role of B cells as antigen presenting cells are now being recognized based on the therapeutic effect of rituximab, a B cell inhibitor, in rheumatoid arthritis. Great attention has been turned to anti-citrullinated peptide antibodies because they form immune complex and contribute to inflammation by activating complement system. Recently, clinical trials showed therapeutic efficacy of tocilizumab, monoclonal antibody against IL-6 receptor, suggesting relevant involvement of IL-6 in disease process of rheumatoid arthritis. Thus, various cellular and molecular players seem to interact within rheumatoid synovium to perpetuate inflammation. Further studies are needed to explore the exact mechanisms of development and maintenance of inflammation in rheumatoid arthritis.
Antibodies ; Antibodies, Monoclonal, Humanized ; Antibodies, Monoclonal, Murine-Derived ; Antigen-Antibody Complex ; Antigen-Presenting Cells ; Arthritis, Rheumatoid ; B-Lymphocytes ; Chondrocytes ; Complement System Proteins ; Cytokines ; Hyperplasia ; Inflammation ; Interleukin-1 ; Interleukin-17 ; Interleukin-6 ; Joints ; Macrophages ; Osteoblasts ; Plasma Cells ; Receptors, Interleukin-6 ; Synovial Membrane ; T-Lymphocytes ; Tumor Necrosis Factor-alpha ; Rituximab

No abstract available.
Behcet Syndrome*

No abstract available.
Behcet Syndrome*

BACKGROUND: Infantile Hypertrophic Pyloric Stenosis (IHPS) is one of the most common surgical problems of early infancy and one for which an eminently successful surgical treatment has been available since the work of Ramstedt in 1912. A clinical study was begun to access further the accuracy of ultrasonography in identifying hypertrophic pylorus. METHODS: This study is a retrospective clinical analysis of 31 cases of IHPS treated at the Department of Surgery of Pohang St. Mary's Hospital from Jan. 1990 to Dec. 1997. RESULTS: (1) The most prevalent age group was between 3 weeks and 8 weeks in 24 cases (77.4%), and the ratio of males to females was 5.2:1. (2) Among the 31 cases, new born babies were 21 cases (67.7%). (3) In 30 cases (96.8%), the gestational age was between 37 weeks and 42 weeks, and the birth weight was more than 3.5 kg in 21 cases (67.7%). The body weight percentile at admission was lower than the 50 percentile in 31 cases. (4) Among the 31 cases, breast-fed infants were 15 cases (48.4%), milk-fed 13 cases (41.9%), and mixed-fed 3 cases (9.7%). B type blood group was 23 cases (74.2%), and O type was 4 cases (12.9%). (5) In 4 cases (12.9%), an inguinal hernia was noted as an associated anomaly. (6) The onset of symptoms was neonatal (1 week-12 weeks) in all 31 cases, and the duration of the symptoms was between 1 week and 2 weeks in 18 cases (58.1%). (7) Non-bile stained, projectile vomiting was noted in all 31 cases (100.0%), an olive-shaped mass in right upper quadrant of the abdomen was felt in 27 cases (87.1%), and visible peristalsis on the epigastrium was noted in 25 cases (80.6%). (8) On laboratory tests, 17 patients had leukocytosis, and anemia was observed in 2 cases. Hypokalemia was observed in 9 cases (29.0%), hypochloremia in 4 cases (12.9%), and moderate to severe alkalosis (CO2 content > 25 mEq) in 7 cases. (9) Among the 25 cases, for which an the ultrasonographic evaluation was performed, the length of the stenotic canal was from 16 mm to 20 mm in 23 cases (92.0%), and the thickness of the stenotic portion was from 5 mm to 6 mm in 21 cases (84.0%). (10) All 31 cases were surgically treated by using a Fredet-Ramstedt pyloromyotomy, and the mortality was nil. The average hospitalization was 9.4 days. (11) There was 1 case of duodenal perforation and 1 case of intermittent non-projectile vomiting after the operation. CONCLUSIONS: We conclude that early accurate diagnosis, adequate preoperative preparation of the fluid & electrolyte imbalance, immediate surgical correction, and scheduled careful oral feeding are important in treatment of IHPS. Ultrasonographic determination of pyloric muscle length and thickness is the most accurate of the currently available techniques. A Fredet-Ramstedt pyloromyotomy is a safe and successful surgical procedure.
Abdomen ; Alkalosis ; Anemia ; Birth Weight ; Body Weight ; Diagnosis ; Female ; Gestational Age ; Gyeongsangbuk-do ; Hernia, Inguinal ; Hospitalization ; Humans ; Hypokalemia ; Infant ; Leukocytosis ; Male ; Mortality ; Peristalsis ; Pyloric Stenosis, Hypertrophic* ; Pylorus ; Retrospective Studies ; Ultrasonography ; Vomiting

BACKGROUND: Antineutrophil cytoplasmic antibodies (ANCA) are autoantibodies that are specific for proteins in the cytoplasm of neutrophils and monocytes. In the 10 years since their discovery, ANCA have become widely used serological markers for various systemic necrotizing vasculitides, including Wegener's granulomatosis, polyarteritis nodosa, crescentic glomerulonephritis, and Churg-Strauss syndrome. Rheumatic manifestations (such as arthralgia, myalgia, even frank arthritis) are observed frequently in the group of primary vasculitides. In the group of collagen vascular diseases and the various forms of chronic inflammatory arthritis, vasculitis may severely complicate the course of the disease. Since atypical vasculitic diseases are indistingushable from other rheumatic disorders in the initial period of disease, immunological studies must be performed. We conducted this study for obtaining the seroprevalence of ANCA in rheumatic disorders including vasculitides which are common in Korea. METHOD: ANCA was detected with indirect immunofluorescent microscopy of alcohol-fixed granulocytes. RESULTS: Total 185 patients were enrolled in this study. There was no patient having C-ANCA except one patient with Wegener's granulomatosis. Total 5 patients were positive for P-ANCA; 2 of 41 SLE patients, 1 of 22 dermatomyositis/polymyositis patients, 2 of 50 Behcet's disease patients. All 11 patients with Takayasu's meritis were ANCA-negative. These results were similar to those of others. CONCLUSION: ANCA, as a adjunct to other autoantibodies, will be helpful for differential diagnosis of various vasculitides and rheumatic disorders.
Antibodies, Antineutrophil Cytoplasmic* ; Arthralgia ; Arthritis ; Autoantibodies ; Churg-Strauss Syndrome ; Collagen ; Cytoplasm ; Diagnosis, Differential ; Glomerulonephritis ; Granulocytes ; Humans ; Immune System Diseases ; Korea ; Microscopy ; Monocytes ; Myalgia ; Neutrophils ; Polyarteritis Nodosa ; Prevalence* ; Rheumatic Diseases* ; Seroepidemiologic Studies ; Vascular Diseases ; Vasculitis* ; Wegener Granulomatosis

OBJECTIVE: To investigate the clinical manifestations of adult onset Still's disease in Korea. METHODS: 22 patients who were diagnosed at Seoul National University Hospital during 10 years from March, 1984 to June, 1994 were reviewed. RESULTS: Age of onset was evenly distributed between 16 and 45 years in most of the patients. Fever and arthralgia were present in all cases. Arthritis and skin rash were developed in 91% and 77% of the patients respectively. Laboratory tests showed accelerated ESR in 95~o, increased serum ferritin in 79%, leukocytosis in 68%, anemia in 32%, hypoalbuminemia in 32%, and abnormal liver function test in 36% of the patients. Commonly affected joints were knees, wrists, proximal interphalangeal joints, ankles, elbows and shoulders in order. Bone marrow biopsy in 7, lymph node biopy in 2 cases revealed reactive hyperplasia. Nonsteroidal anti-inflammatory drugs (NSAID) were effective in 73% of the patients, corticosteroid with or without NSAID in 50% of the patients. Toxic hepatitis was developed in 2, acute renal failure in 1 and drug rash in 1 case during NSAID treatment, which resolved after discontinuance or switching to another NSAID. CONCLUSIONS: The clinical manifestations of AOSD in Korea generally resemble those previously reported in other countries except that lymphadenopathy, splenomegaly, joint deformity, pleurisy, leukocytosis and anemia were significantly less frequent in our cases.
Acute Kidney Injury ; Adult* ; Age of Onset ; Anemia ; Ankle ; Arthralgia ; Arthritis ; Biopsy ; Bone Marrow ; Congenital Abnormalities ; Drug-Induced Liver Injury ; Elbow ; Exanthema ; Ferritins ; Fever ; Humans ; Hyperplasia ; Hypoalbuminemia ; Joints ; Knee ; Korea* ; Leukocytosis ; Liver Function Tests ; Lymph Nodes ; Lymphatic Diseases ; Pleurisy ; Seoul ; Shoulder ; Splenomegaly ; Still's Disease, Adult-Onset* ; Wrist

Osteoarthdtis (OA) is a common, chronic, and painful disorder characterized by cartilage loss. It is the most common among all rheumatic disorders and is a major cause of disability. OA can be managed with a variety of pharmacological therapies, including acetaminophen, traditional nonsteroidal antiinflammatory drugs, cyclooxygenase2 inhibitors, intraarticular steroids, viscosupplements, glucosamine, chondroitin sulfate, and capsaicin. In this review, I describe the clinical efficacy and side effects of these pharmaceuticals and review diseasemodifying osteoarthritis drugs (DMOAD), such as glucosamine, diacerein, and avocado/soybean unsaponifiables, of which the clinical efficacy still remains to be determined.
Acetaminophen ; Capsaicin ; Cartilage ; Chondroitin Sulfates ; Glucosamine ; Osteoarthritis* ; Steroids ; Viscosupplements

OBJECTIVES: Avascular necrosis of bone has been frequently documented in association with systemic lupus erythematosus and it has been suggested by many investigators that systemic factors may be implicated in its pathogenesis. In order to define the incidence, clinical feature and related risk factors of avascular necrosis in corticosteroid- treated rheumatic disease patients, we conducted this retrospective study. METHODS: Medical records of 278 patients with diagnoses of systemic lupus erythematosus (SLE), polymyositis/dermatomyositis, overlap syndrome comprising either of SLE, polymyositis, or dermatomyositis, and mixed connective tissue disease were reviewed with regards to the following: 1) duration of disease, risk factors of avascular necrosis, such as the presence of Raynaud phenomenon, small vessel vasculitis, alcoholism. 2) history of steroid treatment, including duration, initial dose, cumulative dose and mean daily dose during follow-up, cumulative dose and mean daily dose during the first year of disease, history of steroid pulse therapy, and history of cytotoxic drug therapy. 3) laboratory findings including false positive VDRL, lupus anticoagulant, anti-phospholipid antibody, and activated partial thromboplastin time. 4) Development of avascular necrosis, duration of disease, activity of disease at the time of diagnosis of avascular necrosis, and the site. RESULTS: Nineteen patients developed avascular necrosis leading to the incidence rate of 18.5/1,000 patient-year. Sites of involvement were hip in 16 cases(84.2%), talus in 2 cases(10.5% ), and phalanx, scaphoid, and humerus in 1 case(5.3% ), respectively. Fifty-eight percent of patients had involvement in more than one site. Presence of Raynaud phenomenon, small vessel vasculitis, history of cytotoxic therapy, history of steroid pulse therapy, cumulative dose and mean daily dose of steroid during follow-up and 1st year of diagnosis were not significantly different between the 2 groups. CONCLUSIONS: The incidence of avascular necrosis in our patient population was similar to that reported in SLE patients previously, but other risk factor including steroid dosage could not be identified.
Alcoholism ; Dermatomyositis ; Diagnosis ; Drug Therapy ; Follow-Up Studies ; Hip ; Humans ; Humerus ; Incidence ; Lupus Coagulation Inhibitor ; Lupus Erythematosus, Systemic ; Medical Records ; Mixed Connective Tissue Disease ; Necrosis* ; Osteonecrosis ; Partial Thromboplastin Time ; Polymyositis ; Raynaud Disease ; Research Personnel ; Retrospective Studies ; Rheumatic Diseases* ; Risk Factors ; Talus ; Vasculitis