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A Rare Case of Adult-Onset Still’s Disease Following Scrub Typhus

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WCIM 2014 SEOUL KOREA 217

Poster Session

The Korean Journal of Internal Medicine Vol. 29, No. 5 (Suppl. 1)

PS 0660 Rheumatology

A Rare Case of Adult-Onset Still’s Disease Following Scrub Typhus

Go-Eun LEE1, Chung-Il JOUNG1, Mi-Hye KWON1 Konyang University Hosptial, Korea1

Adult-onset Still’s disease (AOSD) is a rare autoimmune infl ammatory disease. Typical features include high spiking fever, evanescent rash, and arthritis or arthralgia. Patho- genesis is not well known, but hypothesized that genetic abnormalities confer suscep- tibility and environmental triggers could initiate the disease. As far as we know, there is no report of AOSD associated with scrub typhus, so we report it herein. A 51-year- old woman presented with fever, sore throat, arthralgia, rash, and typical eschar on her posterior arm was diagnosed as scrub typhus and managed at other hospital. She was a farmer, and had history of osteoarthritis of hands. After 2 courses of doxycycline the fever didn’t subsided, she was admitted and treated with antibiotics. Fever persisted and pain on the wrists, knees and ankles developed, she was referred to our hospital.

Vital signs were 120/80 mmHg–102 bpm-18 rates/min-38.3°C. There was urticarial rash on her upper chest and both lower legs and joints were tender but not swollen.

Lab tests showed markedly high serum ESR, CRP, ferritin and low hemoglobin, albumin.

Serum ANA, anti-CCP Ab were weak-positive. Viral markers and triple antibodies for scrub typhus, leptospira and hantan virus were negative. Tests for evaluation of fever such as chest CT, cardiac echocardiography and whole body bone scan were normal and abdomen CT revealed several enlarged lymph nodes. Bone marrow biopsy was refused. She was diagnosed as AOSD by Yamaguchi criteria, treated with low dose of prednisolone without an NSAID due to high transaminases. Intermittent fever re- mained so oral methotrexate was started after short course of IV methylprednisolone 125mg. She had good clinical and laboratory responses, she was discharged. We expe- rienced a case of AOSD following scrub typhus and scrub typhus could be a trigger for development of AOSD.

PS 0661 Rheumatology

Severe Neutropenia in Patients with Rheumatic Diseas- es at a Tertiary Care Hospital in South Korea

Chang-Nam SON1, Ji-Min KIM1, Sang-Hyon KIM1, Soo-Kyung CHO2, Yoon-Kyoung SUNG2, Tae-Hwan KIM2, Jae-Bum JUN2, Sang-Cheol BAE2, Dae-Hyun YOO2

Keimyung University Dongsan Medical Center, Korea1, Hanyang University Hospital for Rheumatic Dis- eases, Korea2

Background: Neutropenia is relatively common in patients with rheumatic diseases.

Some patients with neutropenia require hospitalization in an isolation ward and are ad- ministered recombinant human granulocyte colony stimulating factor (rhG-CSF) or anti- biotics to treat neutropenic fever. Our aim was to investigate the possible causes and the clinical characteristics of severe neutropenia in Korean patients with rheumatic diseases.

Methods: The study participants (n=64) were enrolled from September 2003 to Au- gust 2013 from a population of patients with rheumatic diseases who were admitted to a tertiary care center. These subjects had severe neutropenia and received rhG-CSF at least once during hospitalization. We retrospectively examined data of the subjects including age, gender, initial diagnosis, concomitant medications, serial complete blood count, and bone marrow biopsy.

Results: The most frequent initial diagnoses were SLE (n = 35; 55%), RA (n = 13;

20%), and infl ammatory myositis (n = 6; 9%). The possible causes associated with severe neutropenia were therapeutic drugs (n = 31; 48%), association with lupus (n = 17; 27%), infection (n= 12; 19%), and hemophagocytic syndrome (n = 4; 6%). During hospitalization nine deaths occurred (14%, 9/64). Mortality was higher in patients with sepsis than in patients with neutropenia associated with other causes. Pneumonia was the most common cause of sepsis in patients with neutropenia (58.3%, 7/12). The frequency of sepsis and death was higher in the long-term recovery group (= 28 days) than in the other groups (< 7 days and 7-27 days).

Conclusions: In patients with rheumatic diseases, drug toxicity was the most common cause of severe neutropenia. Among the causes of neutropenia, sepsis is of greatest con- cern; therefore, physicians need to pay attention to the early detection of infection.

PS 0662 Rheumatology

Hepatic Sarcoidosis in a Patient with Chronic Hepatitis B Virus Infection

Hye-Sun PARK1, Sooyoung JUNG1, Seunghee HAN1, Hyemin KIM2, Sanghoon AHN3, Yongbeom PARK1, Sookon LEE1, Sangwon LEE1

Division of Rheumatology, Department of Internal Medicine, Severance Hospital, Korea1, Department of Pathology, Severance Hospital, Korea2, Division of Gastroenterology, Department of Internal Medicine, Severance Hospital, Korea3

Sarcoidosis is a systemic infl ammatory granulomatous disease which affects multiple organs including liver, spleen, heart, eyes and skin. Liver involvement can be found in 11.5% of the cases and there have been many studies reporting the association be- tween hepatitis C virus infection and sarcoidosis. However, the role of hepatitis B virus infection as a trigger for sarcoidosis has never been reported before. We describe a case of hepatic sarcoidosis in a patient with chronic hepatitis B infection with possible link between them. It is the fi rst case reporting a patient with INF-a-naïve chronic HBV infection presenting hepatic sarcoidosis accompanied by portal hypertension and liver cirrhosis.

PS 0663 Rheumatology

The Seasonality of Gout Onset and Flares in Korea

Hyo-Jin CHOI1, Chan Hee LEE2, Joo Hyun LEE3, Bo Young YOON3, Hyoun Ah KIM4, Chang Hee SUH4, Sang Tae CHOI5, Jung Soo SONG5, HoYeon JOO6, Sung Jae CHOI7, Ji Soo LEE8, Kee Chul SHIN9, Jae Bum JUN10, Han Joo BAEK1

Gachon University Gil Hospital, Korea1, NHIS Ilsan Hospital, Korea2, Inje University Ilsan Paik Hospital, Korea3, Ajou University School of Medicine, Korea4, Choong Ang University Hospital, Korea5, Inha University Hospital, Korea6, Korea University Ansan Hospital, Korea7, Ewha Womans University Medical Center, Korea8, SNU Boramae Medical Center, Korea9, Hanyang University,Hospital for Rheumatic Dis- eases, Korea10

Background: To evaluate seasonality of gout onset and fl ares in Korea.

Methods: We retrospectively examined data from 330 patients seen at nine rheuma- tology multicenter clinics, under urate lowering therapy (ULT) more than one year after stopping prophylactic medication. Demographic data, clinical and laboratory features and seasonality of gout fl are were collected. Season was classifi ed as three-month time intervals (the period from March through May was defi ned as spring).

Results: The mean age was 52.2 years and mean disease duration was 26.8 months.

The male to female count was 318:12. The season of gout onset was most common in summer (28.5%). During ULT, 113 of the 330 patients (34.2%) experienced at least one gouty attack in the period from stopping prophylaxis to one year later. Total gout fl ares were one hundred forty-seven (male 97.3%). Gout fl ares were most common in summer (30.6%), and then fall (25.2%), spring (22.4%), and winter (21.8%). The most common month of fl are was June (13.6%), following by August (10.2%), September (9.5%) and February (9.5%). The aggravating factor was identifi ed in 60 fl ares; alcohol (68.3%), concomitant drugs (13.3%), food (10.0%), and work/exercise (8.3%). The pro- portion of alcohol in summer was higher (88.9%) than other seasons. In the patients with attained to the target serum uric acid (< 6mg/dL) at the end of prophylaxis (n=52), gout fl ares were most common in fall (35.8%) and September (17.0%).

Conclusions: Gout onset of Korea was most common in summer. The summer and fall were most common seasons of gout fl are during ULT. We suggested that increased physical activity, changes of temperature, dehydration and alcohol consumption might be associated with our results.

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