Adult-onset Still's Disease with Disseminated Intravascular Coagulation and Multiple Organ Dysfunctions Dramatically Treated with Cyclosporine A

Severe systemic manifestations of adult onset Still's disease (AOSD) are often fatal and occasionally related to hemophagocytic syndrome (HS). We describe the case of a 49-yr-old woman with AOSD presenting with non-remitting high fever, confusion, jaundice, hepatosplenomegaly, serositis, azotemia, pancytopenia, coagulopathy with disseminated intravascular coagulation (DIC), hyperferritinemia, acute acalculous cholecystitis and ileocolitis noted in computed tomographic images. The patient had a history of herpes zoster developed prior to the admission, but there is no history of diarrhea or abdominal pain. Although bone marrow examination was not performed due to hemorrhagic diathesis, we suspected AOSD-associated HS on the basis of clinical course without detectable infectious agents in cultures or serologic studies. Intravenous immunoglobulin, pulse methylprednisolone, oral cyclosporine A (CsA) and ceftriaxone brought about transient improvement of fever and confusion, but the disease progressed. After increasing CsA dose, all previously mentioned abnormalities disappeared rapidly. Accordingly, we believe that DIC and multiple organ dysfunctions might have been the complications of HS but not that of sepsis, and that CsA can be used as a first-line therapy in case of life-threatening situations.

Higher Prevalence of Peripheral Arthritis Among Ankylosing Spondylitis Patients

This study was performed to define the clinical spectrum and disease manifestations of ankylosing spondylitis (AS) in a referral hospital setting. We identified the differences in clinical manifestations according to the sex, the age at onset, the presence of peripheral arthritis and the presence of HLA B27. A total 412 patients (357 males, 55 females) were recruited. Eighty-seven percent were men and 155 out of 412 patients (35%) were juvenile-onset. HLA B27 was detected in 385 patients (93%). Peripheral joint involvement was noted in 287 of total AS cases (juvenile- onset ankylosing spondylitis (JOAS), 82%; adult-onset ankylosing spondylitis (AOAS), 61%), and was more common than those reported in other studies. A greater portion of patients with JOAS had peripheral arthritis and peripheral enthesitis than the patients with AOAS. The patients with peripheral arthritis showed a younger age at onset and an increased tendency of having enthesitis and trauma history. The natural history of Korean AS appears largely similar to those seen in Europe and North America, except a few differences. JOAS was quite common and AS was about nine times more common in men than in women. In addition, the HLA B27 antigen frequency was 93%, which is higher than those reported in other studies.

Undifferentiated Spondyloarthropathy in Korea: Focusing on Peripheral Arthritis

Undifferentiated spondyloarthropathy (USpA) includes the forms that do not meet criteria for the established categories of spondyloarthropathy. The clinical spectrum of USpA is therefore wide and few studies have been published on USpA, especially peripheral arthritis. A total of 107 patients fulfilling the European Spondyloarthropathy Study Group criteria for SpA were studied retrospectively by a chart review and interview by a rheumatologist. Peripheral arthritis, excluding hip and shoulder involvement, occurred in 97 of the 107 patients (91%). Joint involvement tended to be monoarticular or pauciarticular, and most frequently developed in peripheral joints including the knee and ankle. Among the 97 patients with peripheral arthritis, only 37 (35%) had a persistent arthritis. HLA-B27 was detected in 80 patients (78%). Peripheral arthritis was found in the lower extremities regardless of symmetry or asymmetry and tended to run a benign course with only a few patients having persistent arthritis

Health-Related Quality of Life in Korean Patients with Ankylosing Spondylitis / 대한류마티스학회지

OBJECTIVE: To assess the health-related quality of life (HRQOL), the correlation among each measurement, and to identify the predictors for HRQOL in Korean patients with ankylosing spondylitis (AS). METHODS: The HRQOL and clinical and laboratory parameters were assessed by Short Form Health Survey-36 (SF-36), EuroQol5 Dimensions (EQ-5D), time trade off (TTO), standard gamble (SG), Centers for Epidemiologic StudiesDepression (CES-D), social support, self-efficacy scale, Bath Ankylosing Spondylitis Functional Index (BASFI), Schober test, occiput to wall test, ESR, and CRP from 90 patients with AS. RESULTS: 78 patients (86.7%) were men with a mean (+/-SD) age of 28.4 (+/-7.9) years. The mean years of disease onset was 10.44 (+/-6.95). The mean scores of SF-36 global, mental component summary (MCS) and physical component summary (PCS) were 53.4 (+/-21.2), 61.8 (+/-26.3), and 51.7 (+/-24.4), respectively. The mean EQ-5D utility and visual analog scale (VAS) score were 0.63 (+/-0.2) and 57.1 (+/-18.6). The mean scores of the TTO and SG were 0.46 (+/-0.6) and 0.46 (+/-0.6). The scores of the SF-36 and EQ-5D in AS patients were lower than those in healthy control. The mean scores of CES-D, self-efficacy scale, social support and social network were 10.5 (+/-6.8), 65.3 (+/-14.6), 2.46 (+/-0.22), and 2.33 (+/-0.47), respectively. The mean score of BASFI was 2.48 (+/-2.13). The BASFI total score was negatively correlated with the scores of SF-36, MCS, PCS, EQ-5D utility and VAS score, social network, and self-efficacy scale, and positively correlated with CES-D. In multivariate models, the predicting variables of SF-36 global and PCS were BASFI and self-efficacy scale and the predicting variables of SF-36 MCS were BASFI, social support, and self-efficacy scale. CONCLUSION: In this study, we observed that HRQOL in Korean patients with ankylosing spondylitis is decreased compare to healthy control. And the efforts to improve HRQOL should be designed to improve the self-efficacy and social support in addition to active treatment to prevent functional disability.

COX-2 Specific Inhibitor and Cognitive Function: a Pilot Study / 대한류마티스학회지

OBJECTIVE: New nonsteroidal anti-inflammatory drugs (NSAIDs) with highly selective cyclooxygenase-2 (COX-2) inhibition afford protection against gastropathy, but their acute and long-term effects on the central nervous system are unclear. Our aim was to investigate the influence of COX-2 specific inhibitor (celecoxib) on cognitive function. METHODS: Within the context of a randomized controlled parallel trial of NSAIDs for osteoarthitis (OA), we performed a battery of neuropsychological tests in consecutive 10 osteoarthritis patients with celecoxib (200 mg/day) and 13 osteoarthritis patients with diclofenac (100 mg/day) before and after 4 weeks by clinical psychologists who were not involoved in the study and unaware of study protocols and treatment allocation. The tests were performed randomly in sequence in order to minimize learning effect. The examed cognitive domains included memory, reasoning/problem solving, simple and complex attention, visual-spatial processing, and psychomotor speed. RESULTS: Demographic characteristcs (age, sex, disease duration, functional status measured by patient's and physician's global assessment and KWOMAC, CES depression score, education level) were not significantly different between both treatment groups. In all cognitive domains, we did not find out significant cognitive decline before and after treatments either with celecoxib or diclofenac. There was no difference in the change of cognitive function between both treatment groups. CONCLUSION: The short-term use of COX-2 specific inhibitor as well as conventional NSAID may not impair cognitive function. The long-term follow up study using large number of patients is in progress.

Hepatic Safety of Methotrexate in Rheumatoid Arthritis Patients with Hepatitis B Surface Antigen / 대한류마티스학회지

OBJECTIVE: To investigate the hepatic safety of methotrexate (MTX)and useful parameters to assess the risk of hepatotoxicity in rheumatoid arthritis (RA) patients with hepatitis B surface antigen (HBsAg). METHODS:19 HBsAg positive (B group)and 54 HBsAg negative (non-B group)RA patients taking MTX were prospectively followed up for 2 years. Aspartate aminotransferase (AST)and alanine aminotransferase (ALT)were checked at 2-month interval.In B group,HBeAg,HBeAb and levels of hepatitis B virus DNA (HBV DNA)were additionally checked,and liver biopsy was performed in seventeen of 19 patients.Change of AST or ALT to more than 1.5 times the upper limit of normal were considered as a hepatic event.The data were analyzed by stepwise linear and logistic regression method. RESULTS:The findings of liver biopsy in B group were classified as grade I (n=8),II (n=6),and IIIA (n=3)according to the Roenigk Classification Scale. Mean cumulative dose of MTX in B group was 2054mg in cases with hepatic event and 1780mg in cases who stopped taking MTX.The frequency of hepatic event (47.4%vs 7.4%,p=0.0001)and MTX withdrawal (26.3%vs 1.9%, p=0.0008)was higher in B group than in non-B group.HBV DNA (+)group (n=9)showed the increased tendency of hepatic event and MTX withdrawal compared with HBV DNA (-)group (n=10).Baseline HBV DNA was negative in all HBeAg (-)patients (n=14),but HBV DNA was positive during MTX therapy in 7 of 14 HBeAg (-)patients.Serum levels of AST and ALT returned to normal range within 2 months after MTX withdrawal in twelve of 13 patients with hepatic event. CONCLUSION:The use of MTX in HBsAg positive RA patients requires close monitoring of AST and ALT.HBV DNA seems to be a useful marker to predict hepatic event and viral replication,especially in HBeAg (-)patients.

Synovectomy of the Rheumatoid Knee Using Intra-articular Injection of 165Dy Hydroxide Macroaggregates / 대한정형외과학회잡지

165Dy Hydroxide Macroaggregates(165Dy HMA) has a short half life(2.3 hours) and a size range of 3-5µm that give the advantage of reduced leakage and a shorter hospital stay. This report will show the results of a prospective open study on the efficacy and safety of 165Dy HMA in 178 knees of 141 patients with chronic synovitis refractory to conventional antirheumatic therapy. The final global assessment was classified as good, fair or poor. Extra-articular leakage of 165Dy HMA was determined by the scintigraphic evaluation of liver, groin and knee joints. The optimum radiation dose was 250 mCi. The mean follow up periods were 32.4(14-112) weeks. Thirty seven percent of the knees showed good results, 48% fair results and 15% poor results. In the knees with stage I radiographic changes, 82% showed improvement including 32% of the patients with good results. In the knees with stage II radiographic changes, 90% showed improvement including 42% of the patients with good results. The mean period of improvement for the 158 knees that responded to treatment was 41.4(24-106) weeks. Leakage of radioactivity from the injected joint was minimal. Adverse reactions were rare(radiation burn : 4 cases, transient postinjection swelling : 14 cases). In conclusion, 165Dy HMA radiation synovectomy is a safe and useful therapy for chromic synovitis of the rheumatoid knees.