Journal List > J Rheum Dis > v.20(5) > 1064066

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Yang, Lee, Park, Kwon, Moon, Ko, Chang, Park, Lee, Song, and Lee: Rituximab Treatment for the Patients with Refractory Inflammatory Myopathy
Original Article

J Rheum Dis 2013;20(5):303-309.

Published online: 31 October 2013

DOI: https://doi.org/10.4078/jrd.2013.20.5.303

Rituximab Treatment for the Patients with Refractory Inflammatory Myopathy

Ji Ae Yang, Sang Jin Lee, Jun Won Park, Hyun Mi Kwon, Jin Young Moon, Dong Jin Ko, Sung Hae Chang, Jin Kyun Park, Eun Bong Lee, Yeong Wook Song, Eun Young Lee

Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea

Corresponding to : Eun Young Lee, Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, 101, Daehak-ro, Jongno-gu, Seoul 110-744, Korea. E-mail : elee@snu.ac.kr

Received 23 May 20139 July 2013       Accepted 11 July 2013

Copyright © 2013 The Korean College of Rheumatology

This is a Free Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Objective

To assess the efficacy and safety of rituximab (RTX) on disease activity and muscle strength in patients with inflammatory myopathies refractory to conventional therapy.

Methods

Four inflammatory myopathy patients who had been refractory to glucocorticoids, one or more immunosuppressive therapies and intravenous immunoglob-ulin were treated on an open-label basis. Each patient received two 500 mg doses of RTX 2 weeks apart in one cycle. In one patient who did not respond after the first cycle of RTX, the infusion schedule was modified by the physician. We measured muscle enzyme including CPK, LDH and assessed muscle strength individually to evaluate RTX response. Additionally anti-CD19 antibody was measured.

Results

Three patients responded to the first cycle of RTX treatment with improvements in muscle enzyme and muscle strength, and then maintained physical function over the duration of several infusion cycles. In one patient, muscle enzyme did not decrease after the first cycle of RTX, and a high dose glucocorticoid was given. After modifying the treatment schedule with monthly RTX infusion, his muscle enzyme level and muscle strength improved. Anti-CD19 antibody decreased after RTX generally, but responses were variable. Herpes zoster infection occurred in two patients.

Conclusion

Rituximab may be a therapeutic choice in refractory inflammatory myopathy. However a further trial is needed to confirm the efficacy and prove the safety.

Keywords: Rituximab, Inflammatory myositis

References

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Figure 1.
Serum levels of muscle enzyme (CPK, LDH) at baseline and during followup. (A) In patient 1, (B) In patient 2, (C) In patient 3, (D) In patient 4 (Arrow RTX infusion, total RTX cycle/dose/interval of two infusion).
jrd-20-303f1.tif
Figure 2.
(A) Chest CT on initial presentation shows patchy consolidation on bilateral lower lung field considering BOOP reaction due to DM in Patient 1. (B) Improved lung lesion of CT scan after RTX treatment.
jrd-20-303f2.tif
Figure 3.
In patient 2, immunohistochemical studies performed on affected muscle biopsy. (A) Hematoxylin and eosin stain, magnification ×200 (B) CD20 indicating B lymphocyte staining are positive in perivascular area ×200.
jrd-20-303f3.tif
Figure 4.
Video fluoroscopic swallowing study (VFSS) finding shows pyriform sinus residue and imcomplete clearing in patient 4.
jrd-20-303f4.tif
Table 1.
Baseline characteristics of the inflammatory myositis patients
Patient Underlying disease Sex/Age Disease duration Disease manifestation Previous medication Concomitant medications Therapy after RTX Number of RTX course/dosagex interval Remission (months) Response
1 DM with lung F/39 8 yrs Muscle weakness, Skin findings PDS, MTX, AZA, IVIG PDS PDS 3/500 mg ×2 weeks 56 Y
2 PM M/46 8 yrs Muscle weakness PDS, AZA, MTX, IVIG PDS, MTX PDS, MTX 2/500 mg ×2 weeks 10 Y
3 DM with lung, pharyngeal involvement F/49 3 yrs Muscle weakness, Skin findings, Dysphagia MTX, CYC, IVIG, mPD →PDS PDS PDS AZA, MTX, HCQ 1/500 mg ×2 weeks   Y
4 PM with pharyngeal involvement M/45 3 yrs Muscle weakness, Dysphagia mPD→ PDS, CYC, IVIG PDS, Steroid pulse (solumedrol 500 mg for 3 days) PDS, MTX 1/500 mg ×2 weeks 4/500 mg ×monthly   Y (delayed)

AZA: azathioprine, CYC: cyclophosphamide, HCQ: hydroxychloroquine, IVIG: intravenous immunoglobulin, MMF: mycophenolate mofetil, mPD: methylprednisolone, MTX: methotrexate, PDS: prednisolone, Y: yes, I: indeterminate.

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