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Arthritis & Rheumatism DOI 10.1002/art.38672 Full-length article The running head: Autoimmune-associated hemophagocytic syndrome Title:
Clinical characteristics and treatment outcomes of autoimmune-associated hemophagocytic syndrome in adults Name of the authors: Shunichi Kumakura MD, PhD1, Yohko Murakawa MD, PhD 2 The departments and institutions 1
Department of Medical Education and Research, 2Third Division of Internal Medicine, Faculty of Medicine, Shimane University, Izumo, 693-8501, Japan Conflict of interest, grant or financial support
Dr. Kumakura has received speaking fees from Dainippon Sumitomo Pharma Co., Ltd. (less than $10,000). Dr. Murakawa has received speaking fees from Chugai Pharma Co. Ltd., Eisai Co. Ltd., Mitsubishi Tanabe Pharma, Pfizer Inc, Astellas Pharma Inc, Takeda Pharma Co. Ltd., Janssen Pharma K.K., Santen Pharma Co., Ltd., Bristol-Myers Squibb, Ono Pharma. Co., Ltd., Kissei Pharma. Co. Ltd.. Actelion Pharma Ltd. and Abbvie Japan, and has received research grant supports from Teijin Pharma Ltd., Astellas Pharma Inc, Bristol-Myers Squibb, Mitsubishi Tanabe Pharma, Chugai Pharma Co. Ltd., Takeda Pharma. Co. and Eisai Co. (less than $10,000 each). The corresponding author: Shunichi Kumakura MD, PhD Department of Medical Education and Research, Faculty of Medicine, Shimane University, Izumo, 693-8501, Japan. E-mail: [email protected] TEL +81-853-20-2526, FAX +81-853-20-2542
This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process which may lead to differences between this version and the Version of Record. Please cite this article as an ‘Accepted Article’, doi: 10.1002/art.38672 © 2014 American College of Rheumatology Received: Jun 22, 2013; Revised: Feb 24, 2014; Accepted: Apr 11, 2014
Arthritis & Rheumatology
ABSTRACT Objective. To better define the clinical characteristics and treatment outcomes of autoimmune-associated hemophagocytic syndrome (AAHS) in adults. Methods. Adult cases of AAHS, which were proved by pathological evidence of hemophagocytosis, and did not show any obvious cause other than due to autoimmune disease, were identified by means of a literature review. Results. Of 116 patients identified, underlying diseases included systemic lupus erythematosus (SLE) in 52.3%, adult onset Still’s disease (AOSD) in 26.7%, and dermatomyositis in 6.9%. Fever, lymphadenopathy, hepatomegaly and splenomegaly were found in 86.8, 41.0, 41.8 and 45.5% of patients, respectively. Cytopenia, liver dysfunction and hyperferritinemia developed frequently, and coagulopathy was seen in 50.6%. In patients underlying SLE, normal or low C-reactive protein value was characteristic. Most commonly used therapy was corticosteroids. Corticosteroids were initially administered in 95.7% of patients, with 57.7% responding. Patients being refractory to corticosteroids were usually treated by cyclosporine, intravenous cyclophosphamide (IVCY) or intravenous immunoglobulin G (IVIG), with IVCY being highly effective. Treatment with biologic agents resulted in favorable effects in the majority of patients. The mortality rate was 12.9%. Male-sex (OR, 6.47; 95% CI, 2.06 to 30.39; P
Arthritis & Rheumatism DOI 10.1002/art.38672 Full-length article The running head: Autoimmune-associated hemophagocytic syndrome Title:
Clinical characteristics and treatment outcomes of autoimmune-associated hemophagocytic syndrome in adults Name of the authors: Shunichi Kumakura MD, PhD1, Yohko Murakawa MD, PhD 2 The departments and institutions 1
Department of Medical Education and Research, 2Third Division of Internal Medicine, Faculty of Medicine, Shimane University, Izumo, 693-8501, Japan Conflict of interest, grant or financial support
Dr. Kumakura has received speaking fees from Dainippon Sumitomo Pharma Co., Ltd. (less than $10,000). Dr. Murakawa has received speaking fees from Chugai Pharma Co. Ltd., Eisai Co. Ltd., Mitsubishi Tanabe Pharma, Pfizer Inc, Astellas Pharma Inc, Takeda Pharma Co. Ltd., Janssen Pharma K.K., Santen Pharma Co., Ltd., Bristol-Myers Squibb, Ono Pharma. Co., Ltd., Kissei Pharma. Co. Ltd.. Actelion Pharma Ltd. and Abbvie Japan, and has received research grant supports from Teijin Pharma Ltd., Astellas Pharma Inc, Bristol-Myers Squibb, Mitsubishi Tanabe Pharma, Chugai Pharma Co. Ltd., Takeda Pharma. Co. and Eisai Co. (less than $10,000 each). The corresponding author: Shunichi Kumakura MD, PhD Department of Medical Education and Research, Faculty of Medicine, Shimane University, Izumo, 693-8501, Japan. E-mail: [email protected] TEL +81-853-20-2526, FAX +81-853-20-2542
This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process which may lead to differences between this version and the Version of Record. Please cite this article as an ‘Accepted Article’, doi: 10.1002/art.38672 © 2014 American College of Rheumatology Received: Jun 22, 2013; Revised: Feb 24, 2014; Accepted: Apr 11, 2014
Arthritis & Rheumatology
ABSTRACT Objective. To better define the clinical characteristics and treatment outcomes of autoimmune-associated hemophagocytic syndrome (AAHS) in adults. Methods. Adult cases of AAHS, which were proved by pathological evidence of hemophagocytosis, and did not show any obvious cause other than due to autoimmune disease, were identified by means of a literature review. Results. Of 116 patients identified, underlying diseases included systemic lupus erythematosus (SLE) in 52.3%, adult onset Still’s disease (AOSD) in 26.7%, and dermatomyositis in 6.9%. Fever, lymphadenopathy, hepatomegaly and splenomegaly were found in 86.8, 41.0, 41.8 and 45.5% of patients, respectively. Cytopenia, liver dysfunction and hyperferritinemia developed frequently, and coagulopathy was seen in 50.6%. In patients underlying SLE, normal or low C-reactive protein value was characteristic. Most commonly used therapy was corticosteroids. Corticosteroids were initially administered in 95.7% of patients, with 57.7% responding. Patients being refractory to corticosteroids were usually treated by cyclosporine, intravenous cyclophosphamide (IVCY) or intravenous immunoglobulin G (IVIG), with IVCY being highly effective. Treatment with biologic agents resulted in favorable effects in the majority of patients. The mortality rate was 12.9%. Male-sex (OR, 6.47; 95% CI, 2.06 to 30.39; P
