'Rhupus syndrome', Coexistence of Rheumatoid Arthritis and Systemic Lupus Erythematosus / 대한류마티스학회지

OBJECTIVE: This study was designed to evaluate clinical features of 20 patients with rhupus syndrome and compare its characteristics with systemic lupus erythematosus(SLE) and rheumatoid artiritis(RA). METHODS: Patients considered to have rhupus who met the American College of Rheumatology(ACR) 1997 and 1987 revised criteria for SLE and RA, respectively and age, sex, and disease duration matched 64 patients with RA and 56 patients with SLE were selected for comparison. RESULTS: Twenty patients were all female and their mean age was 43.7+/-9.6 years(range 25~68). They had 5.5 ACR criteria for RA and 5 criteria for SLE. The mean age at onset of RA was 35.2+/-10.5 years(19~63) and that of SLE was 38.2+/-10.0 years(20~63), giving a mean interval between the diagnoses of the two diseases of 3.0+/-5.7 years (14~(-6)). There were 2 patients(10%) with rheumatoid nodule, and 18 patients(90%) with rheumatoid factor, and 16 patients(80%) with bone erosions on hand or wrist joints. The criterial for SLE included malar rash(20%), discoid rash(0%), photosensitivity(30%), oral ulcer(45%), arthritis(100%), serositis(35%), renal disorder(15%), neurologic disorder(0%), hematologic disorder(100%), immunologic disorder(90%), and positive antinuclear antibody(100%). Anti-dsDNA was more than 7.0U/ml in 15 patients(75%). The patients with rhupus syndrome showed lower amount of 24 hour urine protein, more severe radiologic involvement, younger age at onset of arthritis, higher titer of rheumatoid factor, lower frequency of low C3, and less complicated clinical course when compared with the patients with SLE(p<0.05). More frequent anemia, Rayanud's phenomenon, and more complicated clinical course in rhupus when compared with the patientw with RA(p<0.05), but the radiologic stage of hand and wrist was similar between rhupus and RA. CONCLUSION: Rupus syndrome showed somethisg different clinical characteristics and clinical course when compared with SLE and RA.

A case of hemolytic uremic syndrome associated with Epstein-Barr virus infection

The precise etiology of hemolytic uremic syndrome (HUS) is unknown. However, it has been associated with bacterial (Shigella, Salmonella, E. coli, S. pneumoniae), Bartonella, and viral (coxsackie, ECHO, influenza, varicella. Epstein-Barr) infections and with endotoxemia. Recently, we experienced a case of HUS in a 16-year-old boy who was in the acute phase of an Epstein-Barr virus (EBV) infection. He had typical manifestations of HUS and EBV infection. He also transiently presented disseminated intravascular coagulation. His renal dysfunction recovered by supportive care, including hemodialysis, plasmapheresis, antihypertensive medication and aspirin. We present this case with a review of the literature as the second report of HUS associated with EBV infection.

Initial Transient Neurologic Recovery Followed by Delayed Deterioration of Osmotic Demyelination Syndrome: A Case Report / 대한신장학회잡지

In literatures, most of the studies of severe hyponatremia during or following its treatment has been concentrated with special references to the rate of correction and its neurologic outcomes. But, there is relatively few ones analyzing the diverse clinical manifestations of neurologic symptorns or complications during the course of treating severe hyponatremia. We experienced a catastrophic course related to hyponatremia in a 51 year woman with severe rheumatoid arthritis, who underwent knee joint replacement, and this case revealed the initial transient neurologic recovery for 3 days by the initial rapid correction of hyponatremia, then followed by delayed deterioration of osmotic demyelination syndrome leading to locked-in syndrome. Reported cases with similar clinical course (biphasic course) in the world lituratures were reviewed with special interests in the initial maximum rate of correction of hyponatremia and radiologic findings. This review suggests that clinicians treating the patients with severe symptomatic hyponatremia should be aware of the possibility of delayed neurologic sequelae despite the recovery of neurologic status as well as the degree of hyponatremia in the early treatment course of hyponatremia.

Adult Onset Still's Disease: Clinical Features and Prognostic Factors in Korea / 대한류마티스학회지

OBJECTIVE: This study was undertaken to review the disease course, clinical and laboratory manifestations, prognosis and treatment of adult onset Still s disease (AOSD) in Korea. METHODS: Thirty-two patients with AOSD were enrolled from 1986 to 1997 in Hanyang University Hospital. Diagnosis of AOSD was based on the criteria proposed by Yamaguchi. We classified the disease course into self-limited, inter mittent, or chronic disease course. RESULTS: Twenty-four (75%) patients were female. Skin rash occurred in 28 (88%) patients, lymphadenopathy in 8 (25%), hepatomegaly in 4 (13%), and pericarditis in 2 (6%) out of 32 patients. The most commonly affected joints were knee joints (88%). Elevated LDH was seen in 18 (60%) patients and decreased CK in 17 (61%) patients. Rheumatoid factor was detected in 4 (13%) patients and ANA in 12 (38%) patients. Anemia (Hb < 10 g/dL) was seen in 13 (41%) patients and hypoalbuminemia (<3. 5 g/dL) in 14 (52%) patients. Elevated ferritin (300 ng/mL) level was seen in 23 (79%) patients. Twenty-five (78%) patients had elevated serum transaminase. Bone marrow studies were performed in 16 patients. Nine out of 16 patients showed hyperplasia of the myeloid series and 2 patients displayed the features of a hemophagocytic syndrome. The mean duration of follow up of 32 patients was 32 months (range 3- 108). Eight (27%) patients had a self-limited, 9 (30%) an intermittent, and 13 (43%) a chronic disease course. The hypoalbuminemia was significantly associated with an "intermittent or chronic disease group" (p<0. 05). Thirty-two patients received systemic corticosteroids and 21 patients received single or combination of disease modifying antirheumatic drugs. CONCLUSION: We found that hypoalbuminemia at presentation was significantly associated with an unfavorable outcome, intermittent or chronic disease group. The clinical manifestations and disease course of AOSD in Korea were similar to those previously reported in other countries except significantly lower incidence of lymphadenopathy, hepatomegaly, and pericarditis.

Diffuse Idiopathic Skeletal Hyperostosis in Koreans 9 Cases Report / 대한류마티스학회지

OBJECT: To investigate the characteristics of diffuse idiopathic hyperostosis (DISH) in Koreans. METHODS: The nine patients of DISH were investigated in terms of symptom, involved area in simple X-ray, accompaning disease, serum uric acid level, and serum triglyceride and cholesterol. RESULTS: Eight of nine patients were male, one patient was female, and their mean age was 64. Their symptoms were no symptom(2/9), neck pain(2/9), dorsal pain(2/9), and low back pain(3/9). The involved areas were the cervical vertebrae(1/9), the lower thoracic vertebrae(3/9), or the lower thoracic and upper lumbar vertebrae(5/9). The accompaning diseases were ankylosing spondylitis (1/9), degenerat,ive joint disease (2/9), posterior laminectomy (1/9), diabetes mellitus (1/9), chronic active hepatitis (1/9), cerebrovascular accident (2/9), hypertension(2/9), chronic renal failure(2/9), and gout(2/9). In the cases involving thoracic vertebrae, the ossification was seen dominantly in the right side. Four of nine patients had diabetes mellitus, hyperuricemia (7. 0 mg/dl in male and 6.6 mg/dl in female), or dyslipidemia (194 mg/dl in serum triglycerid level and 275 mg/dl in serum cholesterol level). CONCLUSIONS: In our cases of DISH, it was dominant in male and old age, and the diabetes mellitus or hyperuricemia was more frequently found than in general population.

A case of myelofibrosis

No abstract available.