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Q J Med 1997; 90: 19-25
© 1997 Association of Physicians
Pro-inflammatory cytokines and the pathogenesis of Gaucher's disease: increased release of interleukin-6 and interleukin-10
1 From the Orthopaedic Research Unit, University of Cambridge, Addenbrooke's Hospital Hills Road, Cambridge, UK 2 From the Department of Medicine, University of Cambridge, Addenbrooke's Hospital Hills Road, Cambridge, UK
Address correspondence to Professor T.M. Cox, Department of Medicine, Addenbrooke's Hospital, Cambridge CB2 2QQ
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Abstract |
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Gaucher's disease is characterized by hepatosplenomegaly, bone-marrow infiltration, osteonecrosis and bone thinning, associated with the presence of pathological macrophages that contain undegraded glycosphingolipids. To investigate the possible role of cytokines in the systemic and local manifestations of established Gaucher's disease, interleukin-1β (IL-1β), interleukin-6 (IL-6), tumour necrosis factor-alpha (TNF
) and interleukin-10 (IL-10) were measured in freshly-separated serum. Samples from eight male and 14 female patients with type 1 Gaucher's disease were compared with sera from 22 healthy age- and sex-matched controls. Concentrations of IL-6 and IL-10 were significantly elevated in sera from patients with Gaucher's disease (11.9 ± 1.8 (SEM) pg/ml and 5.4 ± 0.5 (SEM) pg/ml, respectively) compared with those of controls (4.1 ± 0.9 (SEM) and 0.8 ± 0.3 (SEM) pg/ml, p <0.0001). No significant differences in concentrations of TNF or/IL-1β were identified. IL-6 has been implicated in the development of localized osteolysis in multiple myeloma and in the development of post-menopausal osteoporosis. High concentrations of IL-6 in the serum of patients with Gaucher's disease may thus reflect the development of the bone lesions commonly associated with this disorder. Since IL-6 and IL-10 are important regulators of lymphocyte growth and differentiation, and IL-6 concentrations were significantly raised in patients with oligo- or polyclonal increases in serum immunoglobulins, enhanced release of these cytokines from pathological macrophages provides a pathological link between Gaucher's disease and associated lymphoproliferative disorders.
* Present address: Department of Orthopedic Surgery, SUNY-Health Science Center, 750 East Adams Street, Syracuse, NY 13210, USA
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