Hee Jung Ryu

Fibromyalgia with chronic rheumatic diseases in South Korea: a comparison of clinical and American College of Rheumatology criteria

To describe the prevalence and characteristics of fibromyalgia (FM) in patients with underlying rheumatic disease, and to compare it by three different measures.

Mean platelet volume as a marker for differentiating disease flare from infection in Behçet's disease

The aim of this study was to investigate the association between mean platelet volume (MPV) and clinical manifestations, disease activity or infection in patients with Behçets disease (BD).

AB0577 Mean Platelet Volume is Associated with Behcet's Disease Activity

Background Mean platelet volume (MPV) has been known to be a marker of platelet activity and influenced by inflammation.The correlations of MPV with disease activities were reported in several rheumatic diseases such as rheumatoid arthritis, ankylosing spondylitis, and psoriasis. However MPV is controversial as a marker of their disease activities. Furthermore, the association of MPV with disease activity in Behcets disease (BD) is not determined yet. Objectives The aim of this study is to investigate the association of MPV with clinical manifestations and disease activity in BD. Methods We sequentially retrieved data on 193 patients with BD who visited to our rheumatic clinic from 1999 to 2013 by reviewing of medical records. BD was diagnosed according to International Criteria for BD. The data on demographics, clinical manifestations, and laboratory results including MPV, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were collected. Active BD was defined as it needed prednisolone of more than 0.5 mg/kg/day or to add immunosuppressive agents. Infection was demonstrated by culture or clinical improvement with antibiotic treatment. Statistical analysis was made by t-test and Pearsons correlation. A value of p<0.05 was considered as statistically significant. Results The ratio of female to male was 2:1 and the age of symptom onset was 32.2±11.1 years. The age at diagnosis of BD was 40.9±10.0 years and the follow-up duration was 4.7±3.8 years. Fifty-one patients (26.4%) had major organ involvements such as eye (17.6%), gastrointestinal (7.8%), vascular (4.7%) and nervous system (3.1%). MPV at diagnosis was 8.2±1.2 fl. MPV was not related with clinical manifestations, ESR and CRP. During follow-up, 79 patients had active BD and 12 patients had infections such as pulmonary tuberculosis and pyelonephritis. MPV in active BD (8.1±1.4 fl) was lower than that in inactive disease (8.9±1.3 fl; (p<0.0001). MPV significantly increased when the patients were infected (9.5±1.6 fl; p<0.0001) Conclusions MPV was not related with clinical manifestations of BD. However, there was an association of active BD with lower MPV and of infection with higher MPV. Thus, MPV level may be used as a marker of BD activity and to differentiate infection from active BD. References Are platelet volume indices of clinical use? A multidisciplinary review. Ann Med. 2012 Dec;44(8):805-16. Mean platelet volume in recurrent aphthous stomatitis and behcet disease. Angiology. 2014 Feb;65(2):161-5. Increased mean platelet volume in Behçets disease with thrombotic tendency. Tohoku J Exp Med. 2010 Jun;221(2):119-23. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5121

Infliximab for refractory oral ulcers

Recurrent oral ulcer (ROU) is a common condition that significantly impacts quality of life. It is often related to systemic diseases, such as Behçets disease (BD), Crohns disease, and ulcerative colitis. Treatment of ROU depends on its severity: from topical agents for mild cases to systemic agents, such as corticosteroids, azathioprine, or other immunosuppressants for severe cases. Recently, good results have been reported with infliximab in refractory ROU. However, the optimal dosage and treatment duration have not been determined and the cost and potential side effects should be considered. We report on four patients who received a single-dose infliximab for refractory ROU. Two patients had refractory ROU with no underlying disease; one of them had soft palate perforation accompanied by severe oral ulcers. The two other patients had ROU of BD without major organ involvement. All patients received a single infusion of infliximab and an additional infusion was given on demand in one patient. Infliximab showed a rapid, good response in three patients and was also effective in improving the acute inflammation in the perforation of the soft palate, which had been resistant to conventional therapies. These effects diminished over a few weeks, but the ROU were tolerable and it was not necessary to increase steroids or add another medicine for about 1 year. We suggest that a single infusion of infliximab can be considered for refractory ROU.

Successful Treatment of Concurrent Mucormycosis and Diffuse Alveolar Hemorrhage in a Patient With Newly Diagnosed Systemic Lupus Erythematosus

©2018 Turkish League Against Rheumatism. All rights reserved. Infection is often complicated with systemic lupus erythematosus (SLE) and is one of the leading causes of death in SLE patients.1 When SLE flare coexisting with serious infection is suspected, intensive immunosuppressive therapy is usually a challenge. Mucormycosis is a rare and rapidly progressing infection with high mortality. Diffuse alveolar hemorrhage (DAH) is also one of the rare and serious manifestations in SLE.2,3 We experienced an extremely rare case of SLE that was initially presented with DAH and mucormycosis concurrently.

Acquired enophthalmos with systemic lupus erythematosus

Ocular involvement sometimes occurs with systemic lupus erythematosus (SLE) but enophthalmos with SLE is rare. We report a case of enophthalmos with SLE. A 25-year-old male was admitted for two weeks of fever, sore throat, arthralgia, chest pain and right arm weakness with pain. We diagnosed him with SLE with malar rash, arthritis, pleural effusion, proteinuria, leukopenia, positive antinuclear antibody, anti-dsDNA, and lupus anticoagulant. The patient was prescribed high-dose prednisolone and hydroxychloroquine 400 mg. One week after discharge, he complained about a sensation of a sunken right eye. CT showed right enophthalmos, a post-inflammatory change and chronic inflammation. Proteinuria increased to 3.8 g/day after the patient stopped taking prednisolone. Cyclophosphamide therapy was administered for three months without improvement. We decided to restart prednisolone and change cyclophosphamide to mycophenolate mofetil. Proteinuria decreased but enophthalmos remains as of this reporting.

FRI0120 A Decrease in C-Reactive Protein Does not Guarantee Favorable Treatment Response Enough to Retard Bony Progression in the Patients with Axial Spondyloarthritis

Background Axial spondyloarthritis (aSpA) is a chronic inflammatory disease that can lead to irreversible spinal bone damage and loss of spine mobility. However, it is controversial whether the inflammation is coupled with bony change in aSpA. C-reactive protein (CRP) is useful for monitoring disease activity, if it is elevated at baseline. Objectives To investigate the association of CRP with radiographic spinal progression in aSpA Methods We reviewed the data of 178 patients with aSpA who visited the rheumatology clinic at our hospital between January 2009 and February 2013. They had at least 2 sets of lateral cervical and lumbar radiographs a minimum of two years apart for assessing the modified Stoke Ankylosing Spondylitis Spine Score (mSASSS). We calculated annual mSASSS change using two different mSASSS. CRPs at the time of taking radiographs were also collected. High CRP was defined as it was ≥10 mg/L. We divided the patients into 4 groups in terms of CRP at baseline and follow-up during treatment: persistently low (LL), persistently high (HH), decreased CRP to low level after treatment (HL), and increased CRP after treatment (LH). Results Age of the patients was 40.2±12.0 years and female to male was 1:4. Disease duration was 13.3±9.0 years. The patients with HLA-B27 positivity were 91%; those with ankylosing spondylitis were 81%. Radiographs were taken at intervals of 4.5±2.6 years. Seventy four patients were included in LL, 27 in HH, 55 in HL and 5 in LH. The duration of decrease of CRP to low level was 8.9±10.7 months in HL. (Among In the patients of LL, the frequencies of male, initial spinal syndesmophytes, and use of TNF inhibitors decreased compared with other groups.) New spinal syndesmophytes were observed during follow-up in 24% (LL), 44% (HH), 56% (HL) and 40% (LH). Annual mSASSS change was 0.6±1.0 (LL), 1.4±1.5 (HH), 1.5±1.6 (HL), and 0.6±0.8 (LD). The patient with initial high CRP showed significantly more new syndesmophyes and annual mSASSS change than those with initial low CRP. When the patient with high CRP were classified to two groups (48 patients in 10≤ and <30 mg/L, and 37 patients in ≥30 mg/L), however, there was no statistical difference new syndesmophyes and annual mSASSS change between them. Comparing HH and HL, annual mSASSS change did not differ between them. Although it was not statistically significant, new syndesmophytes were more common in HL than in HH. Conclusions Baseline high CRP (≥10 mg/L) is associated with radiographic spinal progression compared with low CRP. However, the higher CRP was not correlated with the more progressive spinal disease. In addition, there was no difference in spinal bony change between the patients with persistent high CRP and those with a reduction of CRP. Thus, while monitoring the aSpA patients with high baseline CRP, physicians should keep in mind that a decrease in CRP does not guarantee favorable treatment response enough to retard bony progression. Further disease-modifying drugs may be needed to be given for them. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.3470

FRI0365 Cross-cultural adaptation and validation of the behcet’s disease current activity form in korea

Objectives This study was to perform a cross-cultural adaptation of the Behcet’s disease current activity form (BDCAF, version 2006) to Korean language and to evaluate its reliability and validity in a population of Korean patients with Behcet’s disease (BD). Methods The BDCAF was translated into the Korean language by two translators, who were aware of its objectives and it was translated back into the English language by two different translators. The Korean BDCAF was administered to thirty-six patients with BD who were attending the outpatient rheumatology clinic. The second BDCAF was done by mail in thirty patients (83%) one day after visit. The test-retest reliability was analyzed by computing k statistics. Kappa scores higher than 0.6 indicated good agreement. To assess validity, we compared BDCAF score with the patient’s/clinician’s perception of disease activity (0~7 visual scale) and Korean version of behcet’s disease quality of life (BDQOL) which was validated at 2008. Results The male to female was 10:26, and the mean age of patients was 52.0 ± 8.1 years (± SD). The mean disease duration was 5.8±4.8 years. About test-retest reliability, good agreement were achieved BDCAF items of headache, oral/genital ulcer, eye involvement, arthralgia, arthritis, moderate agreement were erythema nodosum, nausea/vomiting/abdominal pain, skin pustules and diarrhea with altered/frank blood per rectum (all p< 0.005), and poor agreement were nervous system and major vessel involvement items. Cronbach’s alpha coefficient was 0.692 for BDCAF first assessment, and 0.710 for BDCAF second assessment, indicating acceptable levels of internal consistency for both assessments. Significant correlations were obtained BDCAF score with the patient’s/clinician’s perception of disease activity and BDQOL (all p< 0.05). Conclusions The Korean version of BDCAF is a reliable and valid instrument for measuring current activity in BD patients of Korea. Disclosure of Interest: None Declared