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Rituximab as improving survival in macrophage activation syndrome with systemic lupus erythematosus

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Rituximab as improving survival in macrophage activation syndrome with systemic lupus erythematosus

경북대학교 의과대학 내과학교실 류마티스내과

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김나리, 강종완, 은정수, 이상진, 남언정, 강영모

Background: Macrophage activation syndrome (MAS) is a severe, life-threatening complication of systemic inflammatory diseases. MAS is known to be associated with active systemic lupus erythematosus (SLE). Some studies have reported the prevalence of MAS in SLE to be 0.9 to 4.6%. We eval- uated whether rituximab (RTX) treatment reduced mortality compared with conventional treatment in MAS combined with active SLE. Methods: We conducted a retrospective study in patients with SLE admitted to the Kyungpook National University Hospital between April 2002 and March 2015. All patients satisfied the American College of Rheumatology 1997 revised criteria for SLE. MAS was diagnosed according to the HLH-2004 guidelines.

Clinical and laboratory measures of disease activity, and incidence and survival rates of MAS were assessed. Results: Among 138 active SLE patients, 46 patients were treated with the immunosuppressive regimen including RTX and other 92 patients were treated with the immunosuppressive regimen that did not include RTX (control group). There were 23 active SLE patients with MAS. Among 23 patients, 7 patients (30.4%) were treated with RTX.

The mortality was 14.3% in the RTX group (1/7) and 50% in the control group (8/16). There was no significant difference in SLE Disease Activity Index-2K between the RTX group (26.7±8.6) and the control group (27.2±7.8) among the patients with MAS (p=0.001). Conclusion: The present study shows that MAS was strongly associated with active SLE. RTX have efficacy in the reduction of mortality in MAS combined with active SLE.

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Serum KL-6 level reflects severity of interstitial lung disease with connective tissue disease

서울대학교 의과대학 내과학교실 류마티스내과

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이정석, 이은영, 박진균, 이은봉, 송영욱

Background: Measuring severity of interstitial lung disease (ILD) usually depends on the extent or pattern of imaging findings on computed tomog- raphy (CT) and the parameters of pulmonary function test. We aimed to evaluate KL-6 as a potential biomarker reflecting severity of CTD-ILD.

Methods: Study population was a retrospective cohort of 464 Korean patients with rheumatoid arthritis (RA), systemic sclerosis (SSc), inflammatory myositis (IM), Sjogren’s syndrome (SS), and systemic lupus erythematosus (SLE) who had concurrent ILD or not. Serum concentration of KL-6 (U/mL) was measured by Nanopia KL-6 assay (SEKISUI MEDICAL, Tokyo). Temporally nearest CT and PFT results were collected within 2 years from each serum sampling. Semi-quantitative grade of ILD extent was evaluated by CT scan. Results: The patients with CTD-ILD (n=162) had ele- vated serum level of KL-6 compared to CTD without ILD (n=302) (mean±SEM, 737.9±57.5 vs 238.2±10.1U/mL, p<0.001) (Fig 1). In subgroup anal- ysis, RA (544.6±100.3 vs 241.7±19.6, p<0.001), SSc (766.4±103.7 vs 224.0±26.3, p=0.002), IM (808.1±99.8 vs 291.4±33.1, p<0.001), and SS or SLE (914.1±227.1 vs 215.0±10.25, p<0.001) also had significant difference according to the presence of ILD. Semi-quantitative grade of ILD in CT scan was significantly proportional to KL-6 level except between grade 1 and 2 (Fig 2). Conclusion: Serum levels of KL-6 were increased in CTD-ILD and had good correlation with CT grade, FVC, and DLCO. Higher serum level of KL-6 may reflect severity of CTD-ILD.

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