FRI0614 CLINICAL AND LABORATORY FEATURES OF MACROPHAGE ACTIVATION SYNDROME IN PATIENTS WITH ADULT STILL’S DISEASE
Authors: Hiroya Tamai, Yuko Kaneko, Tsutomu Takeuchi
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Adult Still's Disease (ASD) sometimes develops macrophage activation syndrome (MAS). MAS is a severe, fatal complication, however, little is known about its clinical courses and relation to initial symptoms, diagnosis and treatment. <h3>Objectives</h3> To clarify characteristics of MAS in patients with ASD and identify risk factors for developing MAS. <h3>Methods</h3> Consecutive ASD patients diagnosed with Yamaguchi's criteria in our hospital from April 2012 through September 2017 were retrospectively reviewed. Clinical symptoms, laboratory data and treatment information were collected form their charts. Patients were divided into two groups according to the complication of MAS and analyzed. <h3>Results</h3> Sixty five patients with ASD with available information were enrolled in the analysis. The mean age at ASD onset was 45.7 years old, and female was 81.5%. Among them, 12 patients (18.5%) developed MAS during the clinical course. Three patients developed MAS at the same time with the diagnosis of ASD, seven patients after the initiation of glucocorticoid treatment, and two patients at relapse. Five of nine who underwent bone marrow examination proved to be complicated with hemophagocytotic syndrome. The median duration from initial symptoms of ASD to fulfilment of Yamaguchi's criteria was shorter in patients with MAS than those without (10.5 days vs 21.0 days, p=0.077). Drug allergy occurred more frequently in patients with MAS (50.0% vs 22.6%, p=0.056). The maximum levels of ferritin and AST before treatment was higher in patients with MAS than those without (ferritin, 6090ng/mL vs 3434ng/mL, AST 86.5U/L vs 68.0U/L), whereas the maximum levels of CRP was lower in patients with MAS (8.9mg/dL vs 15.9 mg/dL). In nine patients who developed MAS after glucocorticoid treatment, white blood cell count decreased by almost half despite moderate to high dose of glucocorticoid, and the level of ALT increased 10-fold and ferritin increased 2-fold at MAS development. However, the levels of CRP at MAS rather decreased before treatment, suggesting the change in CRP did not reflect MAS development. <h3>Conclusion</h3> The incidence of MAS was 18.5% during the clinical course of ASD. Patients with MAS developed ASD more rapidly than those without. The ferritin and ALT levels predicted and reflected MAS development, whereas CRP levels was not associated with MAS. <h3>Disclosure of Interests</h3> Hiroya Tamai: None declared, Yuko Kaneko Grant/research support from: Abbvie, Eisai, Speakers bureau: AbbVie, Astellas, Ayumi, Bristol-Myers Squibb, Chugai, Eisai, Eli Lilly, Jansen, Kissei, Pfizer, Sanofi, Takeda, Tanabe-Mitsubishi, UCB, Tsutomu Takeuchi Grant/research support from: Astellas Pharma Inc, Chugai Pharmaceutical Co, Ltd., Daiichi Sankyo Co., Ltd., Takeda Pharmaceutical Co., Ltd., AbbVie GK, Asahikasei Pharma Corp., Mitsubishi Tanabe Pharma Co., Pfizer Japan Inc., Eisai Co., Ltd., AYUMI Pharmaceutical Corporation, Nipponkayaku Co. Ltd., Novartis Pharma K.K., Grant/research support from: AbbVie, Asahi Kasei, Astellas, AstraZeneca, AYUMI, Bristol-Myers Squibb, Chugai, Daiichi Sankyo, Eisai, Eli Lilly Japan, Janssen, Mitsubishi Tanabe, Nippon Kayaku, Novartis, Pfizer Japan Inc, Taiho, Taisho Toyama, Takeda, Teijin, Grant/research support from: Astellas Pharma Inc., Bristol Myers Squibb, Chugai Pharmaceutical Co., Ltd., Mitsubishi Tanabe Pharma Co., Pfizer Japan Inc., Santen Pharmaceutical Co., Ltd., Takeda Pharmaceutical Co., Ltd., Teijin Pharma Ltd., AbbVie GK, Asahi Kasei Pharma Corp., Taisho Toyama Pharmaceutical Co., Ltd., SymBio Pharmaceuticals Ltd., Janssen Pharmaceutical K.K., Celltrion Inc., Nipponkayaku Co. Ltd., and UCB Japan, Consultant for: Astra Zeneca K.K., Eli Lilly Japan K.K., Novartis Pharma K.K., Mitsubishi Tanabe Pharma Co., Abbivie GK, Nipponkayaku Co.Ltd, Janssen Pharmaceutical K.K., Astellas Pharma Inc., Taiho Pharmaceutical Co. Ltd., Chugai Pharmaceutical Co. Ltd., Taisho Toyama Pharmaceutical Co. Ltd., GlaxoSmithKline K.K., UCB Japan Co. Ltd., Consultant for: AbbVie, Asahi Kasei, Astellas, AstraZeneca, AYUMI, Bristol-Myers Squibb, Chugai, Daiichi Sankyo, Eisai, Eli Lilly Japan, Janssen, Mitsubishi Tanabe, Nippon Kayaku, Novartis, Pfizer Japan Inc, Taiho, Taisho Toyama, Takeda, Teijin, Consultant for: Astra Zeneca K.K., Eli Lilly Japan K.K., Novartis Pharma K.K., Mitsubishi Tanabe Pharma Co., Asahi Kasei Medical K.K., AbbVie GK, Daiichi Sankyo Co., Ltd., Bristol Myers Squibb, and Nipponkayaku Co. Ltd., Speakers bureau: Astellas Pharma Inc., Bristol Myers Squibb, Chugai Pharmaceutical Co., Ltd., Mitsubishi Tanabe Pharma Co., Pfizer Japan Inc., Santen Pharmaceutical Co., Ltd., Takeda Pharmaceutical Co., Ltd., Teijin Pharma Ltd., AbbVie GK, Asahi Kasei Pharma Corp., Taisho Toyama Pharmaceutical Co., Ltd., SymBio Pharmaceuticals Ltd., Janssen Pharmaceutical K.K., Celltrion Inc., Nipponkayaku Co. Ltd., and UCB Japan, Speakers bureau: AbbVie, Asahi Kasei, Astellas, AstraZeneca, AYUMI, Bristol-Myers Squibb, Chugai, Daiichi Sankyo, Eisai, Eli Lilly Japan, Janssen, Mitsubishi Tanabe, Nippon Kayaku, Novartis, Pfizer Japan Inc, Taiho, Taisho Toyama, Takeda, Teijin, Speakers bureau: AbbVie GK., Bristol–Myers K.K., Chugai Pharmaceutical Co. Ltd., Mitsubishi Tanabe Pharma Co., Pfizer Japan Inc., Astellas Pharma Inc, Diaichi Sankyo Co. Ltd., Eisai Co. Ltd., Sanofi K.K., Teijin Pharma Ltd., Takeda Pharmaceutical Co. Ltd., Novartis Pharma K.K.