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Annals of the Rheumatic Diseases | 2016

THU0179 Discontinuation of Nonsteroidal anti-Inflammatory Drugs in Rheumatoid Arthritis Patients with Low Disease Activity

D.J. Go; Kichul Shin; H.J. Baek; Young Mo Kang; Suk-Hwan Kang; S.-H. Park; J.-B. Jun; Yun Jong Lee; Y.W. Song

Background Although nonsteroidal anti-inflammatory drug (NSAIDs) is effective in relieving joint pain in rheumatoid arthritis (RA) patients, long-term use of NSAIDs can cause adverse effects on gastrointestinal or cardiovascular organs. When patients discontinue daily use of NSAIDs, there is often a dichotomous clinical response in patient-reported outcomes (PRO). Objectives We aimed to examine the clinical outcome especially joint pain, and its associated factors in RA patients with low disease activity (LDA) after discontinuing NSAIDs. Methods This study was a 16-week, multi-center prospective open-label trial conducted at eight rheumatology clinics in Korea. RA patients who achieved LDA (DAS28 <3.2) who were on NSAIDs for more than 2 consecutive months, discontinued the NSAID. Acetaminophen (AAP) was used as the rescue medication. During the study period, changes of DAS28 and PRO including pain visual analogue scale (VAS) and Routine Assessment of Patient Index Data 3 (RAPID-3) score were assessed. NSAIDs were restarted when patients 1) pain was uncontrolled with AAP and 2) PRO were aggravated. The endpoint was to analyze the group of patients who continued to withdraw NSAIDs within the study period. Patients were further classified to have “sustained effectiveness” who met the following: 1) 16-week pain VAS ≤30 mm or increase less than 20% from baseline and 2) 16-week RAPID-3 score ≤6 or increase less than 20% from baseline. Results A total of 121 RA patients with LDA were enrolled in the study. Four patients were lost to follow-up. At the end of the study, 18 (15.3%) patients had restarted NSAIDs owing to uncontrolled pain. In general, there was a difference in pain VAS between baseline (median 15 mm (IQR 5–20)) and 16 weeks (median 17 mm (IQR 5–40)) (p=0.022). Changes of DAS28 and RAPID-3 were insignificant in patients taking on-demand AAP (n=99) (0 versus 16 weeks; DAS28, p=0.111; RAPID-3; p=0.339). Moreover, 60% of the total patients (73/117) ended up to show sustained effectiveness without restarting NSAIDs. By using a multivariate logistic regression model, we found out that joint swelling was the detrimental factor compromising sustained effectiveness (OR 0.312, 95% C.I. 0.125–0.777, p=0.012). Conclusions Discontinuation of NSAIDs can be attempted in RA patients with LDA, especially in those without swollen joints at the time point of its cessation. Disclosure of Interest None declared


Annals of the Rheumatic Diseases | 2018

AB0014 Genetic influence of different measure for tumour necrosis factor inhibitors response in rheumatoid arthritis

So-Young Bang; Y-S Park; Kyoung-Soo Kim; Y.B. Joo; S.-K. Cho; Chan-Bum Choi; Y.-K. Sung; T.-H. Kim; J.-B. Jun; D.-H. Yoo; H.-S. Lee; S.-C. Bae

Background The genetic studies of tumour necrosis factor inhibitors (TNFi) response in patients with rheumatoid arthritis (RA) have largely relied on the changes in complex disease scores as a measure of treatment response. It is expected that genetic architecture of such complex score is heterogeneous and not very suitable for pharmacogenetic studies. Objectives We aimed to select the most optimal phenotype for TNFi response using heritability estimates using genome-wide association studies (GWAS) in the Korean population. Methods Disease Activity Scores based on 28 joint counts (DAS28) and Clinical Disease Activity Index (CDAI) were assessed at baseline, and after 6 months in 370 Korean RA patients who started TNFi due to moderate or high disease activity. Genotypes were generated on the Illumina HumanOmni2.5Exome array (2.5u2009million variants) in TNFi-treated Korean patients with RA. We estimated heritability using a linear mixed-modelling approach (GCTA) for the TNFi drug-response phenotype ΔDAS28, ΔCDAI and its separate components, such as Δ swollen joint count (SJC), Δ tender joint count (TJC), Δ erythrocyte sedimentation rate (ESR), Δ visual-analogue scale of general health (VAS-GH) and Δ provider global assessment of disease activity (PrGA). Furthermore, a multivariate GWAS approach was implemented, analysing separate DAS28 and CDAI components simultaneously Results The highest heritability estimates were found for ΔPrGA (h2=0.76) and ΔTJC (h2=0.73); lower heritability was found for ΔDAS28 (h2=0.32) with estimates for ΔESR (h2=0.66), ΔSJC (h2=0.62), ΔCDAI (h2=0.60) and ΔVAS-GH (h2=0.53) (all p-value<0.005). Conclusions Our results indicate that multiple SNPs together explain a substantial portion of the variation in change in provider global assessment of disease activity in TNFi-treated patients with RA. In conclusion, optimal phenotype based on heritability suggests the use of changes in clinical disease activity index (CDAI) including provider global assessment than DAS28 in pharmacogenetic study. Disclosure of Interest None declared


Annals of the Rheumatic Diseases | 2018

AB0774 Impact of standardised education program on the accuracy of modified rodnan skin scoring in patients with systemic sclerosis

Jun Won Park; J.-B. Jun; L. Czirják; Yannick Allanore; Marco Matucci-Cerinic; E.B. Lee

Background Modified Rodnan skin score (mRSS) has been used as not only a primary outcome in many clinical trials, but also as an important surrogate marker of disease activity in patients with systemic sclerosis (SSc). Therefore, establishment of well-organised training program of mRSS is essential for the proper management of patients. Recently, Scleroderma Clinical Trials Consortium and the World Scleroderma Foundation published the recommendation for 2-phase mRSS training and emphasised assessing scoring accuracy after the training. Objectives To investigate the effect of modified Rodnan skin scoring (mRSS) education on improving its accuracy Methods Ten rheumatologists (6 professors and 4 fellows) received an education program composed of video education and live demonstration by master instructor (Marco Matucci-Cerinic) at (Seoul in June, 2017). Physicians measured mRSS of 8 patients with SSc 1) before the education, 2) after the video education and 3) after live demonstration without any clinical information of the patients. Accuracy of skin scoring was estimated by the difference from the pre-defined gold-standard score measured by master instructor. Change in accuracy of mRSS during the education course was analysed using linear mixed model. Intra-observer reliability of the mRSS and its change was assessed by intraclass correlation coefficient (ICC).Abstract AB0774 – Table 1 Multivariable analysis indicating effect of the education program on the accuracy of modified Rodnan skin scoring *, Dependent variable = (Examiner’s skin score – gold-standard skin score). Regression coefficient indicates the difference in dependent variable as compared with reference.†, This clinical factor consistently influence the dependent variable irrespective of education course.‡, indicates P value for type 3 fixed effect§, (Physician’s skin score – gold-standard skin score) (95%u2009CI) was estimated as 7.66 (6.03 to 9.29) before the education. Results The number of SSc patients ever experienced by each physician was significantly higher in the professors than fellows but the number of mRSS ever performed was comparable between the two groups. Median (IQR) skin score measured by master instructor was 10.5 (9.0). Mean (SD) difference between skin scores by physicians and master instructor was 7.7 (9.5) units. In the univariable analysis, video education significantly reduced the difference from the gold-standard score (β=−1.96, 95%u2009CI −3.83 to −0.10) whereas live demonstration did not showed additional enhancement in scoring skill. Effect of education program was significantly different according to the physician’s status and patient’s disease type (diffuse vs. limited). In addition, male patient, shorter disease duration and higher gold-standard skin score was associated with more accurate skin scoring irrespective of the education. In the multivariable analysis where above clinical factors were adjusted, video education also led to significantly accurate skin scoring (table 1). When the educational effect was stratified by individual site of examination, face and distal extremities showed greater enhancement of scoring accuracy whereas difference from gold-standard score in proximal extremities was rather increased. ICC of physicians’ skin scores was acceptable over all scoring times (0.63 to 0.88) but was not significantly changed after the education. Conclusions The mRSS education program can significantly enhance the accuracy of mRSS, which is mainly achieved by video education. Reference [1] Khanna D, Furst DE, Clements PJ, et al. Standardization of the modified Rodnan skin score for use in clinical trials of systemic sclerosis. J Scleroderma Relat Disord2017;2(1):11–18. doi: 10.5301/jsrd.5000231 Disclosure of Interest None declared


Annals of the Rheumatic Diseases | 2017

AB1028 Prevalence and distribution of sesamoid bones in the hand determined using digital tomosynthesis

Hh Kwon; Bs Koo; Y Song; Y.-K. Sung; S Lee; J.-B. Jun

Background Sesamoid bones are round or oval-shaped bones that are embedded in tendons. The prevalence and distribution of sesamoids in the hand varies between different populations. Conventional radiography (CR) is generally used to identify the sesamoid bones. However, there was no study using digital tomosynthesis (DTS). Objectives The aim of this study was to identify the prevalence and distribution of sesamoid bones in the hand using DTS in comparison to previous studies. Methods Using CR and DTS, hand images (81 left and 100 right) taken at a tertiary hospital were retrospectively reviewed. The sesamoid bones were identified in the distal interphalangeal (DIP), interphalangeal (IP), and metacarpophalangeal (MCP) of the thumb (I), index (II), long (III), ring (IV), and small (V) fingers. Differences in number of sesamoid bones detected on CR and DTS were analyzed. Results Sesamoid bones were observed in MCP I (100%), MCP II (46%), MCP III (2%), MCP IV (2%), MCP V (53%), and IP (53%) on CR. Using DTS, sesamoid bones were found more often in MCP I (100%), MCP II (54%), MCP III (2%), MCP IV (1%), MCP V (59%), and IP (75%). Differences in the mean number of sesamoid bones detected on CR and DTS were statistically significant. Sesamoid bones in DIP joints were frequently observed on DTS, but rarely found on CR. Conclusions Most sesamoid bones in the hand were detected in MCP I, II, V, and IP joints, and were more often detected on DTS than CR. DTS is a reliable tool to evaluate bony structures in the hand. Disclosure of Interest None declared


Annals of the Rheumatic Diseases | 2016

AB0713 Spectrum of Atlantoaxial Ankylosis (AAA) in The Ankylosing Spondylitis (AS): Is It Really Chronological Changes That Occur with Disease Progression or Are There Two Completely Different Pathways?

S. Lee; T.-H. Kim; Y.W. Song; Y.B. Joo; J.-B. Jun

Background The morphologic characteristics of atlantoaxial ankylosis in AS patient and suggests different pathways of involvement resulting in atlantoaxial subluxation in AS patients Objectives To evaluate morphologic characteristics of AAA in AS patient and classify them into categories which reflects the end-stage manifestations of two differents disease pathways. Methods Plain radiographs of cervical spine in 62 AS patients with AAA were reviewed. We classified AAA in AS patients into three subtypes: loss of atlantodental interval (type I), ankylosis of facet joint (type II) and ankylosis of anterior longitudinal ligament or anterior atlantooccipital membrane with cervical spine (type III). And then, we categorized 62 AS patients with AAA into two subgroups: group A (21 patients) with only synovial joint involvement of AS (type I or type II or both) and group B (41 patients) with enthesis involvement of AS with additional synovial joint involvement (type III plus type I or type II or both). We compared the results of mSASSS and disease duration between group A and B. Results The mean cervical mSASSS of patients in group A and B were 27.3 and 16.0 each (p value, 0.196). The mean duration of AS patients in group A and B were 23.7 and 23.6 years each. There was no siginificant differences in cervical, lumbar and total mSASSS or disease duration between the two groups. Conclusions We propose that atlantoaxial joint involvement in the AS patients is not chronological changes that occur with disease progression, but rather it is end-stage manifestation of two different pathways: one only involving synovial joint and the other one involving both synovial joint and enthesis. References Martel W. The occipito-atlanto-axial joints in rheumatoid arthritis and ankylosing spondylitis. Am J Roentgenol Radium Ther Nucl Med 1961;86:223–240 Meijers KA, van Voss SF, Francois RJ. Radiological changes in the cervical spine in ankylosing spondylitis. Ann Rheum Dis1968;27:333–338 Lee HS, Kim TH, Yun HR, Park YW, Jung SS, Bae SC, et al. Radiologic changes of cervical spine in ankylosing spondylitis.Clin Rheumatol 2001;20:262–266 Laiho K, Kauppi M. The cervical spine in patients with ankylosing spondylitis. Clin Exp Rheumatol 2002;20:738 Disclosure of Interest None declared


Annals of the Rheumatic Diseases | 2016

AB0811 MTHFR C677T Polymorphism Is Associated with Increase in Homocysteine but Not with Uric Acid

Il Woong Sohn; S.W. Nam; Chan-Bum Choi; Yong-Hee Kim; J.-B. Jun

Background Serum homocysteine (sHcy) is currently well recognized as an independent risk factor for cardiovascular and cerebrovascular diseases. Hyperhomocysteinemia is reportedly associated with high serum uric acid (sUA) levels. MTHFR C677T polymorphism is assciated with increase in sHcy. Some studies have suggested a positive correlation between MTHFR C677T polymorphism and sUA, but it remains unclear. Objectives We aimed to evaluate the association between sHcy and sUA as well as MTHFR C677T polymorphism and sUA. Methods Patients with memory impairment who visited neurology outpatient clinic were enrolled excluding those who were taking medication for the control of hyperuricemia. Hyperuricemia was defined as >7.0 mg/dl for male and >5.6 mg/dl for female. Hyperhomocysteinemia was defined as >15 μmol/l. Clinical laboratory examinations and genotyping for MTHFR C677T polymorphism was performed. Statistical analyses were performed using chi-square, Pearsons correlation coefficients, and ANOVA. Results Data from 861 subjects (264 male and 597 female) were analyzed. The mean age was 73.2±11.3 years in male and 75.3±10.3 years in female. Male showed significantly higher smoking, alcohol intake, LDL cholesterol, sUA, and sHcy levels, whereas HDL cholesterol, vitamin B12, folic acid were significantly lower compared to female. The prevalence of hyperhomocysteinemia was 38.6% (n=102) in male and 21.4% (n=128) in female. Hyperuricemia was also more common in male with prevalence of 13.3% (n=35) compared to 18.8% (n=112) in female and the mean sUA level was significantly higher in male (5.7±1.4 mg/dl vs 4.6±1.3 mg/dl, p<0.001). sHcy was significantly associated with both folic acid (r=-0.307, p<0.001) and vitamin B12 (r=-0.197 p<0.001). Subjects with elevated sHcy levels had an odds ratio (OR) of 2.7 (95% CI 1.4–5.4) in male and 2.0 (95% CI 1.4–3.1, p=0.001) in female for hyperuricemia. The genotype frequency of MTHFR C677T was 32.8% (n=282) for CC, 49.8% (n=429) for CT, and 17.4% (n=150) for TT. TT genotype showed association with hyperhomocysteinemia when both gender were analyzed. In male, TT genotype (r=0.399, p=0.007) was associated with positive correlation between hyperhomocysteineima and hyperuricemia. In female, CT (r=0.289, p<0.001) and CC (r=0.240, p<0.001) was associated with positive correlation between hyperhomocysteineima and hyperuricemia. MTHFR C677T polymorphism was not associated with hyperuricemia. Conclusions Low serum folic acid and vitamin B12 are significant risk factors for hyperhomocysteinema, which reflects the influence of alcohol consumption and vitamin deficiencies on sHcy level. TT genotype of the MTHFR C677T was associated with hyperhomocysteinemia. A positive association was observed between sHcy levels and sUA levels for both males and females which is in agreement with previous studies. Male having the TT genotype and female having the CT and CC genotype with hyperhomocysteinemia are likely to have higher sUA level. MTHFR C677T polymorphism is not associated with sUA level, which is inconsistent with recent studies. References Assessment of a possible link between hyperhomocysteinemia and hyperuricemia. Eytan Cohen, et al. J Investig Med 2015;63: 534–538. Meta-analysis of the association of the C677T polymorphism of the methylenetetrahydrofolate reductase gene with hyperuricemia. Wen wei, et al. Ann Nutr Metab 2012;60:44–51. Disclosure of Interest None declared


Annals of the Rheumatic Diseases | 2016

AB0107 Association Heterogeneity Mapping Identifies An Asian-Specific Association of The GTF2I Locus with Rheumatoid Arthritis

Kyung-Tai Kim; So-Young Bang; K. Ikari; D.-H. Yoo; S.-K. Cho; Chan-Bum Choi; Y.-K. Sung; T.-H. Kim; J.-B. Jun; Young-Mo Kang; Chang-Hee Suh; Seung-Cheol Shim; S.-S. Lee; Jue-Yeon Lee; Won-Tae Chung; S.-K. Kim; Shigeki Momohara; Atsuo Taniguchi; Hisashi Yamanaka; Swapan K. Nath; H.-S. Lee; S.-C. Bae

Background Genetic association studies using multiple ancestral cohorts have revealed a large overlap of rheumatoid arthritis (RA)-risk alleles among different ancestries, but there are some exceptional loci showing heterogenic association among populations. Objectives Here we investigated genetic variants with distinct effects on the development of RA in Asian and European populations. Methods Ancestry-related association heterogeneity was examined using the association data from large Korean (n=9,299) and European (n=45,790) rheumatoid arthritis cohorts with Immunochip and genome-wide SNP array data. Novel disease associations detected in Koreans were validated using two independent Asian cohorts (n=5,166) and a meta-analysis. Results We identified significant heterogeneity between the two ancestries for the common variants in the GTF2I locus and showed that this heterogeneity is due to an Asian-specific association effect (PHeterogeneity =9.6×10-9 at rs73366469 [ORMeta =1.37 and PMeta =4.2×10–13 in Asians; ORMeta =1.00 and PMeta =1.00 in Europeans]) in RA. Trans-ancestral comparison and bioinfomatics analysis revealed a plausibly causal SNP (rs117026326; linked to rs73366469), whose minor allele is common in Asians but rare in Europeans. Conclusions We identified the largest effect on Asian RA across human non-HLA regions at GTF2I by heterogeneity mapping followed by replication studies, and pinpointed a possible causal variant. References Okada, Y. et al. Genetics of rheumatoid arthritis contributes to biology and drug discovery. Nature 506, 376–81 (2014). Kim, K. et al. High-density genotyping of immune loci in Koreans and Europeans identifies eight new rheumatoid arthritis risk loci. Ann Rheum Dis 74, e13 (2015). Acknowledgement This study was supported by the Korea Healthcare Technology R&D Project of the Ministry for Health & Welfare (HI13C2124), the Japanese Ministry of Education, Culture, Sports, Science and Technology Grant-in-Aid for Scientific Research (15H04965) and the US National Institutes of Health (R01MD007909 and R01AR060366). *Drs. Kwangwoo Kim and So-Young Bang contributed equally to this work. Disclosure of Interest None declared


Annals of the Rheumatic Diseases | 2016

AB0712 Analysis of Different Manifestations in The Cervical Spine Involvement of Longstanding Ankylosing Spondylitis: Atlantoaxial Ankylosis (AAA) and Atlantoaxial Subluxation (AAS)

S. Lee; T.-H. Kim; Yoseb Song; Y.B. Joo; J.-B. Jun

Background Atlantoaxial ankylosis (AAA) is another late manifestation of cervical spine involvement in longstanding AS and suggest that higher cervical mSASSS is more related with the development of AAA than atlantoaxial subluxation (AAS). AAA does not cause as much cervical instability as AAS. Objectives To analyze radiologic and clinical findings of atlantoaxial ankylosis and atlantoaxial subluxation in ankylosing spondylitis Methods A total of 150 AS patients (145 men, 5 women, mean age, 36.8 years) who had AAA (60 men and 2 women, mean age, 60 years) or AAS (85 men and 3 women, mean age, 34 years) were randomly gathered and underwent at least two plain radiographs of lateral cervical spine on flexion. The atlantodental interval more than 3mm was considered as AAS. Loss of atlantodental interval was considered as AAA. The plain radiographs of lateral cervical spine were independently analyzed by two radiologists. Interobserver reliability between reader 1 and 2 in scoring mSASSS in AS was analyzed. We compared the results of mSASSS between AAS and AAA to see if it was related to severity or duration of the disease. Results The mean mSASSS of AS patients with AAA and AAS were 40.1 and 16.5 respectively and the mean duration of AS patients with AAA and AAS were 19.3 and 13.7 years each. There was a significantly (p<0.05) higher total mSASSS, especially cervical spine mSASSS, for AS patients with AAA than those with AAS after mSASSS was corrected for age and duration. But, disease duration was not significantly related with the development of AAA compared with that of AAS. Conclusions We found that it has incidence as high as AAS and both are similar in that they are late presentation of AS and occur in patient with long duration. We found that AAA is another manifestation of cervical spine involvement in longstanding AS and is related to severity of the AS reflected by higher cervical mSASSS. References Martel W. The occipito-atlanto-axial joints in rheumatoid arthritis and ankylosing spondylitis. Am J Roentgenol Radium Ther Nucl Med 1961;86:223–240 Meijers KA, van Voss SF, Francois RJ. Radiological changes in the cervical spine in ankylosing spondylitis. Ann Rheum Dis 1968;27:333–338 Lee HS, Kim TH, Yun HR, Park YW, Jung SS, Bae SC, et al. Radiologic changes of cervical spine in ankylosing spondylitis. Clin Rheumatol 2001;20:262–266 Laiho K, Kauppi M. The cervical spine in patients with ankylosing spondylitis. Clin Exp Rheumatol 2002;20:738 n> Disclosure of Interest None declared


Annals of the Rheumatic Diseases | 2016

THU0514 Seasonal Variations of Episodes of Acute Gouty Arthritis: A Systematic Review and Meta-Analysis

J.-B. Jun; Kyu Yong Park; Hong-Kyu Kim; Hyeong Sik Ahn; Sun Hee Kim; Shin-Young Yim

Background Acute gouty arthritis is the inflammatory arthritis with abrupt and intermittent nature of attack. Objectives The aim of this study was to analyze the seasonal variations of episodes of acute gouty arthritis. Methods We searched MEDLINE, EMBASE, the Cochrane library, and KoreaMed for all articles published before November 2015. Studies with quantitative data on episodes of acute gouty arthritis by months and/or seasons were included. Meta-analysis for episodes of acute gouty arthritis was done by seasons and months. Subgroup analyses by the confirmatory methods of acute gouty arthritis were done by seasons. Results Ten studies published between 1920 and 2015 were included, containing a total of 29,863 subjects. Plotting of episodes of acute gouty arthritis by months showed higher episodes during the period from March to July than other months. The risk ratio of episodes of acute gouty arthritis in spring was 1.21 (95% CI: 0.95 to 1.55, I2=86%; p=0.12), 1.32 (95% CI: 0.98 to 1.78, I2=90%; p=0.07), 1.27 (95% CI: 1.02 to 1.59, I2=81%; p=0.03) when compared to summer, fall and winter, respectively. Therefore, spring had significantly higher episodes of acute gouty arthritis than winter. Subgroup analyses by confirmatory methods of episodes of acute gouty arthritis were as follows: 1) The risk ratio of acute gouty arthritis confirmed by the presence of monosodium urate in synovial fluid in spring was 1.46 (95% CI: 1.22 to 1.76, I2=0%; p<0.0001), 1.28 (95% CI: 1.07 to 1.54, I2=7%; p=0.007), 1.24 (95% CI: 0.93 to 1.65, I2=59%; p=0.15) when compared to summer, fall and winter, showing that spring had significantly higher episodes of acute gouty arthritis than summer and fall; 2) The risk ratio of acute gouty arthritis confirmed by the presence of inflammation in synovial fluid was 1.53 (95% CI: 1.31 to 1.80, I2=0%; p<0.00001), 1.44 (95% CI: 1.07 to 1.93, I2=69%; p=0.02), 1.28 (95% CI: 1.05 to 1.55, I2=40%; p=0.01) when compared to summer, fall and winter, showing that spring had significantly higher episodes of acute gouty arthritis than 3 other seasons. Conclusions This seems to be the first systematic review and meta-analysis on seasonal variations of acute gouty arthritis. Based on our study, the acute gouty arthritis might be developed more frequently during the period from spring. Increase in physical activity, dehydration, and seasonal variation of cortisol level could be related with this seasonal variation of episodes of acute gouty arthritis. References Choi HJ, Lee CH, Lee JH, Yoon BY, Kim HA, Suh CH, Choi ST, Song JS, Joo H, Choi SJ, Lee JS, Shin K, Jun JB, Baek HJ. Seasonality of gout in Korea: a multicenter study. J Korean Med Sci. 2015 Mar;30(3):240–4. Elliot AJ, Cross KW, Fleming DM. Seasonality and trends in the incidence and prevalence of gout in England and Wales 1994–2007. Ann Rheum Dis. 2009 Nov;68(11):1728–33 Disclosure of Interest None declared


Annals of the Rheumatic Diseases | 2015

SAT0403 Clinical Characteristics and Outcomes of Diffuse Alveolar Hemorrhage in Patients with Systemic Lupus Erythematosus

Junghyun Choi; D.H. Kim; S.-K. Cho; Chan-Bum Choi; T.-H. Kim; J.-B. Jun; D.-H. Yoo; S.-C. Bae; Y.-K. Sung

Background Diffuse alveolar hemorrhage (DAH) is a life-threatening though rare manifestation of systemic lupus erythematosus (SLE). Early diagnosis is difficult since the occurrence is abrupt and both symptoms and radiographic findings of diffuse lung infiltration on thoracic CT are nonspecific. Since DAH has high early mortality, it is important to find the clinical features indicating DAH among SLE patients with diffuse lung infiltration and factors influencing its outcomes. Objectives To identify the clinical features of DAH in SLE patients compared with other causes of acute diffuse lung infiltration in SLE patients, and to determine the influencing factors of early outcome of SLE-DAH. Methods We retrospectively collected the medical records of SLE patients, who had acute diffuse lung infiltration on thoracic CT in a single university hospital between January 2004 and August 2014. DAH was defined based on the clinical diagnosis, and we divided patients into two groups; DAH vs. non-DAH group. We compared the demographic and clinical characteristics and presenting symptoms between two groups. And then, we further analyzed the early mortality of SLE-DAH patients and factors affecting mortality in DAH patients. Results Among the 815 SLE patients who checked thoracic CT, 47 patients with acute diffuse lung infiltration were selected for this study. Among them, 24 SLE patients (51.1%) were diagnosed as DAH, the rest of 23 patients (48.9%) formed non-DAH group; non-DAH group included various diseases such as CMV infection (n=2), pneumocystis infection (n=3), unspecified pneumonia (n=9), SLE associated lesions (n=7), and others (n=2). All of them were female and young: 29.6 years old in DAH group and 29.8 years old in non-DAH group. In the presenting symptoms, hemoptysis and dyspnea were more common in DAH group, while fever was more common in non-DAH group, though statistically not significant. In laboratory findings, hemoglobin level (8.2±0.3 vs. 10.0±0.4 g/dL, p<0.01) and C3 level (41.0±4.2 vs. 63.8±7.9, p=0.03) of DAH group were significantly lower than those of non-DAH group, while acute phase reactants and autoantibody profiles were comparable between two groups. The other factors indicating high SLE activity such as SLEDAI-2K score (11.5±1.7 vs. 10.1±1.1), C4 level (11.2±1.5 vs. 17.2±2.5), and CH50 level (12.0±3.4 vs. 25.0±4.6) were different between two groups, but these differences didnt reach statistical significance. During follow-up, 7 among 24 DAH patients died with mean duration of 27.1 days from DAH development; 6 patients died within 3 weeks from DAH development. In comparison between the survivor and the deceased, lupus nephritis was more common in the deceased (100% vs. 35.3%, p<0.01), and they had been taken more intensive treatment with plasmapheresis (71.4% vs. 17.6, p=0.02), IVIG (85.7% vs. 35.3%, p=0.07), transfusion (100% vs. 47.1%, p=0.02), or ventilator (71.4% vs. 0%, p<0.01) compared with survivors. Conclusions Among SLE patients who had diffuse lung infiltration in thoracic CT, 51% was DAH and they tend to have lower level of hemoglobin and high SLE disease activity compared with non-DAH patients. DAH in SLE patients showed high early mortality and it was associated with comorbid condition of lupus nephritis Disclosure of Interest None declared

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S.-K. Kim

Catholic University of Daegu

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S.-S. Lee

Chonnam National University

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