Miho Murakami1,2, Takeshi Johkoh3, Seiji Hayashi4, Shiro Ohshima5, Masao Mizuki6, Shin-Ichi Nakatsuka7, Minako Tomobe8, Kazuyuki Kuroyanagi8, Ayako Nakasone8, Norihiro Nishimoto1,2. 1. Osaka Rheumatology Clinic, Osaka, Japan. 2. Department of Molecular Regulation for Intractable Diseases, Institute of Medical Science, Tokyo Medical University, Tokyo, Japan. 3. Department of Radiology, Kansai Rosai Hospital, Hyogo, Japan. 4. National Hospital Organization Kinki Chuo Chest Medical Center, Osaka, Japan. 5. Department of Rheumatology and Allergology, National Hospital Organization, Osaka Minami Medical Center, Osaka, Japan. 6. Department of Chemotherapy, Osaka University Hospital, Osaka, Japan. 7. Department of Diagnostic Pathology and Cytology, Osaka International Cancer Institute, Osaka, Japan. 8. Chugai Pharmaceutical Co., Ltd., Tokyo, Japan; and Safety Evaluation Committee of tocilizumab for Castleman disease.
Abstract
Objectives: To assess the clinicopathologic features of Multicentric Castleman disease (MCD) patients in Japan. Methods: We assessed baseline data for 342 Japanese MCD patients with a biopsy-proven diagnosis, enrolled in a prospective, observational study for tocilizumab treatment. Results: Of 342 patients, 86.0% had plasma-cell type. None had a family history of MCD. Median disease duration of MCD was 3.7 years. Mean body weight and body mass index tended to be lower than those in the general Japanese population. The most common clinical presentations besides lymphadenopathy included fatigue (61.7%), pulmonary involvement (42.7%), and splenomegaly (41.8%). Secondary amyloidosis was reported in 34 patients (9.9%). Laboratory abnormalities included decreased hemoglobin and albumin, and increased acute-phase proteins, serum immunoglobulins, and interleukin-6 (IL-6). IL-6 levels among the MCD patients tested in this study were correlated with levels of albumin, hemoglobin, triglyceride, total cholesterol, C-reactive protein, fibrinogen and immunoglobulin G (Spearman's correlation coefficient, |r| = 0.28-0.59). Conclusion: The clinical features and laboratory abnormalities are similar to those previously reported in other countries, besides higher rates of pulmonary involvement, secondary amyloidosis, and ECG abnormalities. Our results imply that IL-6 is involved in MCD pathogenesis. These findings would be informative for diagnosis and appropriate treatment for MCD.
Objectives: To assess the clinicopathologic features of Multicentric Castleman disease (MCD) patients in Japan. Methods: We assessed baseline data for 342 Japanese MCDpatients with a biopsy-proven diagnosis, enrolled in a prospective, observational study for tocilizumab treatment. Results: Of 342 patients, 86.0% had plasma-cell type. None had a family history of MCD. Median disease duration of MCD was 3.7 years. Mean body weight and body mass index tended to be lower than those in the general Japanese population. The most common clinical presentations besides lymphadenopathy included fatigue (61.7%), pulmonary involvement (42.7%), and splenomegaly (41.8%). Secondary amyloidosis was reported in 34 patients (9.9%). Laboratory abnormalities included decreased hemoglobin and albumin, and increased acute-phase proteins, serum immunoglobulins, and interleukin-6 (IL-6). IL-6 levels among the MCDpatients tested in this study were correlated with levels of albumin, hemoglobin, triglyceride, total cholesterol, C-reactive protein, fibrinogen and immunoglobulin G (Spearman's correlation coefficient, |r| = 0.28-0.59). Conclusion: The clinical features and laboratory abnormalities are similar to those previously reported in other countries, besides higher rates of pulmonary involvement, secondary amyloidosis, and ECG abnormalities. Our results imply that IL-6 is involved in MCD pathogenesis. These findings would be informative for diagnosis and appropriate treatment for MCD.
Entities:
Keywords:
IL-6; inflammation; multicentric Castleman disease; real-world data
Authors: Antonino Carbone; Margaret Borok; Blossom Damania; Annunziata Gloghini; Mark N Polizzotto; Raj K Jayanthan; David C Fajgenbaum; Mark Bower Journal: Nat Rev Dis Primers Date: 2021-11-25 Impact factor: 65.038