230 32nd World Congress of Internal Medicine (October 24-28, 2014) WCIM 2014
PS 0705 Rheumatology
Clinical Characteristics of Korean Patients with Adult Onset Still’s Disease in a Single Center
Jin Ju KIM1, Su Kyoung CHO1, Chan Bum CHOI1, Yoon Kyung SUNG1, Tae Hwan KIM1, Jae Bum JUN1, Sang Cheol BAE1, Dae Hyun YOO1
The Hanyang University Hospital for Rheumatic Diseases, Korea1
Background: Adult onset Still’s disease (AOSD) is a rare infl ammatory disorder. AOSD mainly occur in young age with slight predominance in female. The predicting out- come is still not defi nite. This study is to evaluate the clinical features in Korean AOSD patients.
Methods: We reviewed medical records of 175 patients, who had disease duration longer than one year. We defi ned poor outcome as polycyclic systemic, chronic articu- lar pattern and death.
Results: Female were predominant (81.7%). The mean age of diagnosis was 37.7 ± 15.1 years old. Forty patients (22.9%) were monocyclic, 83 patients (47.4%) were polycyclic systemic and 52 patients (29.7%) were chronic articular pattern. Clinical manifestations at initial diagnosis were: fever (97.1%), rash (70.3%) and arthritis (58.3%) et al. Involved joint counts were higher in chronic articular pattern than others (P<0.001). Myalgia (P<0.05) and sore throat (P<0.05) were more observed in monocyclic pattern. The mean of serum ferritin was higher in monocyclic pattern than in the others (P<0.05). However, there was no signifi cant fi nding correlated with poor outcome.
Eighteen patients (10.3%) were diagnosed after sixties. Their mean of modifi ed Pouchot’s score (P<0.05) and serum ferritin (P<0.05) were higher than younger ages. Pleuritis was more common in elderly over 60.
The 29 patients (16.6%) had experience of infection during treatment. It was frequently occurred in elderly over 60 (P<0.05). Four patients died because of pneumonia with multi-organ failure, ARDS and MAS.
Conclusions: Female were common and several clinical manifestations could be considered as predictors of disease pattern, but not suffi cient for predictors of poor outcome in this study. And there was considerable occur of disease in elderly over 60 with more clinical activity in this study. Therefore, we need attention in care of elderly with AOSD patients.
PS 0706 Rheumatology
Adult Still’s Disease, Presented with CK Height
Damla ERSOY1
Ümraniye Educational and Research Hospital, Turkey1
Introduction: Adult Still’s Disease is a systemical disease which its both etiology and pathogenic structure is not known clearly. In its etiology, genetic and infectious agents are blamed. During its medical treatment, non steroid anti infl ammatory drugs, ster- oids and immunal modulator drugs are usable. In our case, a 25 Y.O. man has been considered. After getting ecartationed by clinic, laborotary and radiological methods, this disease was considered as still disease diagnosis. Resutls of laboratory searches were as; erythrocyte sedimentation speed 64/ hour, creactive protein 12, AST 65 U/L, ALT 74 U/L, hemoglobin 14.1 g/dl, blood platelet 210.000/mm3, leucocyte 9100/mm3, neutrophil 7240 mm3. CK level 1850 U/l. Atypical cells were not observed in his pe- ripheral spread, neutrophil ratio was obtained as (% 80 neutrophil, % 13 leucocyte, % 6 monocyte, % 1 eosinophil). At his controls, transamination enzymesand CK (creatine kinase) level began to rise while they were declining. Simultaneously, pain and cramps nearly takes half an hour occured at his knee join. There was no patalogic result at his sacroiliac graphy. A fi gure fi tting with a cyst on left tibia was determined at his bilateral knee graphy. It was reported as fi tting with fi broma according to his mag- netic resonance watch and scintigraphy. The sick person had run to a temperature reaching nealy 40 degrees at night and at the same time casts were seen on his body.
Regarding to collagenose disease and vascolyte, ANA (Anti NuclearAntibody), anti ds DNA (antidoublestrandeddeoksiribonucleicacid), RF (Rheumatoid Factor), p-ANCA (per- inuclear anti-neutrophilcytoplasmicantibodies), c-ANCA (cytoplasmicanti-neutrophil- cytoplasmicantibodies) were sent. Result was negative. Ferritin blood test was taken because he was supposed as ESR (erythrocyte sedimentation rate). He was taken under medical treatment by getting >2000 level and relating to Yamaguchi criteria he has 4 major, 2 minor standarts with ESR diagnose.
PS 0707 Rheumatology
Characteristic Findings of Reactive Hemophagocytic Syndrome in Adult-Onset Still’s Disease
Ju-Yang JUNG1, Chang-Bum BAE1, Hyoun-Ah KIM1, Chang-Hee SUH1 Ajou University Hospital, Korea1
Background: Hemophagocytic syndrome is a potentially life-threatening complication of systemic infl ammatory disorders. Adult-onset Still’s disease (AOSD) is the main sys- temic autoimmune diseases related to reactive hemophagocytic syndrome (RHS).
Methods: One-hundred nine patients with a diagnosis of AOSD were evaluated ret- rospectively. We reviewed clinical data and laboratory fi ndings including the results of biopsies of 21 AOSD patients with RHS. Also, 17 hemophagocytic lymphohistiocytosis (HLH) patients were compared with RHS patients.
Results: Twenty-one patients developed RHS during the course of AOSD and only 7 patients were confi rmed by bone marrow, liver, or lymph node biopsy. The AOSD patients with RHS showed signifi cantly higher frequencies of splenomegaly, hepato- megaly and lymphadenopathy compared with those without RHS. The patients with RHS showed signifi cantly higher relapse rate than those without (61.9% vs. 18.2%, p<0.001). Possible trigger factors inducing hemophagocytosis were detected in 16 of 21 RHS patients; disease fl are in 12 patients, infection in 3 patients and drug in 1 patient. The AOSD patients with RHS showed higher frequency of leukopenia, anemia, thrombocytopenia, hypoalbuminemia, hypofi brinogenemia, hypertriglyceridemia, and elevated lactate dehydrogenase than those without. Multivariate logistic regression showed that platelet, hemoglobin, and hepatomegaly were independent predictor of RHS. Patients with defi nite RHS and patients with probable RHS were almost compa- rable. Compared to RHS patients, HLH patients showed poor prognosis such as higher death rate.
Conclusions: When an AOSD patient shows at least 2 cell lineages cytopenia and hepatomegaly, RHS needs to be suspected. Even though RHS is known as a life-threat- ening complication of AOSD, the long term prognosis seemed similar to those without RHS. Diagnostic confi rmation by biopsy may not be essential if there are the typical clinical and laboratory findings of RHS. Moreover, the prognosis of HLH diagnosed with 3 cell lineages cytopenia at admission was much worse than RHS.
PS 0708 Rheumatology
Anti -N-Methyl-D-Aspartate Receptor Antibody is Associated with Fibromyalgia in Patients with Systemic Lupus Erythematosus
Dong-Jin PARK1, Shin-Seok LEE1, Ji-Hyoun KANG1, Jung-Ho CHOI1, Yi-Rang YIM1, Jeong-Won LEE1, Kyung-Eun LEE1, Lihui WEN1, Tae-Jong KIM1, Yong-Wook PARK1, Yukitoshi TAKAHASHI2
Chonnam National University Hospital, Korea1, Shizuoka Institute of Epilepsy and Neurological Disor- ders, Japan2
Background: The high concordance of systemic lupus erythematosus (SLE) with fi bro- myalgia (FM) suggests common underlying mechanisms related to pain and distress in both patient groups. This study was aimed to evaluate roles of NMDAR antibodies in development of FM in SLE patients
Methods: Sera from 104 SLE patients, 112 FM patients, and 110 healthy controls were analyzed to detect titers of antibodies to N-terminus of 2B subunit of NMDAR (GluN2B). Clinical, laboratory data and concomitant diseases were found by reviewing the patient charts. We underwent clinical examination and neuropsychiatric evalua- tion, and interviewed SLE patients using a structured questionnaire that included FM and neuropsychiatric symptoms.
Results: 18 patients (17.3 %) of total 104 SLE patients were revealed having FM. The titer of anti-GluN2B antibodies was signifi cantly higher in SLE patients than FM pa- tients and healthy controls (P < 0.001). In SLE patients, patients with concomitant FM showed higher titers of anti-GluN2B antibodies (P < 0.05). The titers of anti-GluN2B antibodies were correlated with tender point count (Spearman’s rho = 0.238, P = 0.016) and widespread pain index (Spearman’s rho = 0.276, P = 0.005), but not with other symptom scales. Anti-GluN2B antibody-positive SLE patients were more likely to have NPSLE and concomitant FM (P < 0.05). In multivariate analysis, Anti-GluN2B antibody was independent predictor of concomitant FM and NPSLE.
Conclusions: This is the fi rst study to present that antibodies to NMDAR may be as- sociated with pathogenesis of FM in SLE patients.
