Is there a role for B-cell depletion as therapy for scleroderma? A case report and review of the literature.
Semin Arthritis Rheum
; 40(2): 127-36, 2010 Oct.
Article
in En
| MEDLINE
| ID: mdl-20004954
ABSTRACT
OBJECTIVES:
Rituximab (RTX) has been successfully used in the treatment of several rheumatic diseases with an acceptable safety profile. We present herein a patient with systemic sclerosis (SSc) who exhibited significant improvement of his lung function and skin fibrosis following RTX administration, and review the literature regarding the role of B-cells in SSc and the potential efficacy of RTX in its treatment.METHODS:
We performed an internet search using the keywords systemic sclerosis, scleroderma, rituximab, B-cells, fibrosis, interstitial lung disease (ILD), and therapy.RESULTS:
Our patient, a 40-year old man with severe SSc-associated ILD, received 4 courses of RTX. The patient's lung function improved; forced vital capacity and diffusing capacity of carbon monoxide reached values of 35% and 33%, respectively, compared with 30% and 14% of pretreatment values. Skin thickening assessed clinically and histologically improved as well. Several lines of evidence suggest that B-cells may have a pathogenic role in SSc. B-cells from tight skin mice--an animal model of SSc--exhibit chronic hyperactivity; likewise, B-cells from patients with SSc overexpress CD19 and are chronically activated. Furthermore, studies have revealed that B-cell genes were specifically transcribed in SSc skin and that B-cell infiltration was a prominent feature of SSc-associated ILD. The potential clinical efficacy of RTX in SSc has been explored in a limited number of patients with encouraging results. Preliminary data suggest that RTX may favorably affect skin as well as lung disease in SSc.CONCLUSIONS:
Several basic research data underscore the potential pathogenic role of B-cells in SSc and clinical evidence suggests that RTX might be a therapeutic option in SSc. Large-scale multicenter studies are needed to evaluate the potential clinical efficacy of RTX in SSc.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Scleroderma, Systemic
/
B-Lymphocytes
/
Lymphocyte Depletion
/
Antibodies, Monoclonal, Murine-Derived
/
Immunologic Factors
Type of study:
Clinical_trials
/
Prognostic_studies
/
Systematic_reviews
Limits:
Adult
/
Animals
/
Humans
/
Male
Language:
En
Journal:
Semin Arthritis Rheum
Year:
2010
Document type:
Article
Affiliation country: