J Rheum Dis.  2013 Oct;20(5):303-309. 10.4078/jrd.2013.20.5.303.

Rituximab Treatment for the Patients with Refractory Inflammatory Myopathy

• Yang JA
• Lee SJ
• Park JW
• Kwon HM
• Moon JY
• Ko DJ
• Chang SH
• Park JK
• Lee EB
• Song YW
• Lee EY

• KMID: 2223006
• DOI: http://doi.org/10.4078/jrd.2013.20.5.303

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 immunoglobulin 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.

Keyword

Rituximab; Inflammatory myositis

MeSH Terms

Antibodies, Monoclonal, Murine-Derived
Appointments and Schedules
Glucocorticoids
Herpes Zoster
Humans
Immunoglobulins
Muscle Strength
Muscles
Myositis*
Rituximab
Antibodies, Monoclonal, Murine-Derived
Glucocorticoids
Immunoglobulins

Figure

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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).

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  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.

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  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.

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  Figure 4. Video fluoroscopic swallowing study (VFSS) finding shows pyriform sinus residue and imcomplete clearing in patient 4.

Reference

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