Yongpeng Ge1, Shanshan Li1, Xiaolan Tian1, Linrong He1, Xin Lu1, Guochun Wang2. 1. Department of Rheumatology, China-Japan Friendship Hospital, Yinghua East Road, Chaoyang District, Beijing, 100029, China. 2. Department of Rheumatology, China-Japan Friendship Hospital, Yinghua East Road, Chaoyang District, Beijing, 100029, China. guochunwang@hotmail.com.
Abstract
OBJECTIVES: The purpose of this study was to assess the efficacy of rituximab (RTX) in the management of anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis (DM), with or without rapidly progressive interstitial lung disease (RP-ILD). METHODS: Medical records of DM patients with anti-MDA5 antibodies treated with RTX therapy were reviewed retrospectively. Skin rash data, lung function tests, chest high-resolution computed tomography (HRCT), and serum markers were compared before and after RTX. RESULTS: Eleven consecutive cases, including 5 males and 6 females, were identified. One hundred percent of patients had a typical DM rash and about 45% presented with skin ulceration. All the patients had ILD, 73% had RP-ILD, and 27% had mild or asymptomatic ILD. Ro-52 antibodies were found in 55% of this group. Lymphopenia was present in 10/11 patients (91%). Around half (55%) had a level of ferritin greater than 1000 ng/ml. Nine patients (82%) were refractory. These patients received intravenous RTX (375 mg/m2) at 0 and 14 days (conventional dose) or 100 mg once a week for 4 weeks (low dose). After RTX treatment, 2 patients (18%) with mild ILD showed complete remission, and 6 (55%) showed improvement in lung HRCT and/or lung function. Skin rash in 4 patients (100%) and ILD in 3 (75%) showed improvement in the low-dose group. Infection episodes occurred in four (57%) and one (25%) of the conventional-dose and low-dose group, respectively. CONCLUSIONS: Our study found that RTX is sufficient to improve skin rash and ILD or RP-ILD. Our results also suggest that lower RTX doses may be a useful therapy for anti-MDA5 antibody-positive DM patients. Key Points • To clarify efficacy of RTX in the management of anti-MDA5 antibody-positive DM, we performed a retrospective chart review of DM patients with anti-MDA5 antibodies who were treated with RTX. • This study found that RTX is sufficient to improve skin rash and ILD or RP-ILD. • The results suggest that low-dose RTX in treatment of MDA5-DM results in better responses and fewer adverse events.
OBJECTIVES: The purpose of this study was to assess the efficacy of rituximab (RTX) in the management of anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive dermatomyositis (DM), with or without rapidly progressive interstitial lung disease (RP-ILD). METHODS: Medical records of DMpatients with anti-MDA5 antibodies treated with RTX therapy were reviewed retrospectively. Skin rash data, lung function tests, chest high-resolution computed tomography (HRCT), and serum markers were compared before and after RTX. RESULTS: Eleven consecutive cases, including 5 males and 6 females, were identified. One hundred percent of patients had a typical DM rash and about 45% presented with skin ulceration. All the patients had ILD, 73% had RP-ILD, and 27% had mild or asymptomatic ILD. Ro-52 antibodies were found in 55% of this group. Lymphopenia was present in 10/11 patients (91%). Around half (55%) had a level of ferritin greater than 1000 ng/ml. Nine patients (82%) were refractory. These patients received intravenous RTX (375 mg/m2) at 0 and 14 days (conventional dose) or 100 mg once a week for 4 weeks (low dose). After RTX treatment, 2 patients (18%) with mild ILD showed complete remission, and 6 (55%) showed improvement in lung HRCT and/or lung function. Skin rash in 4 patients (100%) and ILD in 3 (75%) showed improvement in the low-dose group. Infection episodes occurred in four (57%) and one (25%) of the conventional-dose and low-dose group, respectively. CONCLUSIONS: Our study found that RTX is sufficient to improve skin rash and ILD or RP-ILD. Our results also suggest that lower RTX doses may be a useful therapy for anti-MDA5 antibody-positive DMpatients. Key Points • To clarify efficacy of RTX in the management of anti-MDA5 antibody-positive DM, we performed a retrospective chart review of DMpatients with anti-MDA5 antibodies who were treated with RTX. • This study found that RTX is sufficient to improve skin rash and ILD or RP-ILD. • The results suggest that low-dose RTX in treatment of MDA5-DM results in better responses and fewer adverse events.
Authors: Stefan Blum; David Gillis; Helen Brown; Richard Boyle; Robert Henderson; David Heyworth-Smith; Patrick Hogan; Paul Kubler; Cecilie Lander; Nicole Limberg; Peter Pillans; Kerri Prain; Christopher Staples; Michael Walsh; Pamela McCombe; Richard Wong Journal: J Neurol Neurosurg Psychiatry Date: 2010-11-11 Impact factor: 10.154
Authors: Chester V Oddis; Ann M Reed; Rohit Aggarwal; Lisa G Rider; Dana P Ascherman; Marc C Levesque; Richard J Barohn; Brian M Feldman; Michael O Harris-Love; Diane C Koontz; Noreen Fertig; Stephanie S Kelley; Sherrie L Pryber; Frederick W Miller; Howard E Rockette Journal: Arthritis Rheum Date: 2013-02