Background Juvenile idiopathic arthritis (JIA) is classified as an acquired autoinflammatory disease. The interleukin-1 and interleukin-6 play an pivotal role in pathogenesis of this disease (1). The systemic manifestations as well as arthritis in sJIA are related to IL-6 action. Il-6 appears to be responsible for the anemia seen in sJIA, mainly inducing the production of hepcidin. Hepcidin prevents the release of iron from the macrophages, hepatocytes, and enterocytes to the plasma, causing a decrease in serum iron levels (2). Thus, the effective blockade of IL-6R signaling is expected to decrease hepcidin levels and increase iron availability for hemoglobin production (3), but in a beta thalassemic trait subject it may be unexpected.
Objectives Our objective is to evaluate the efficacy of tocilizumab therapy in a JIA patient with beta thalassemic trait.
Methods A.M. is a 25 yrs. caucasian male, affected by JIA since ten years and beta thalassemic trait (mother and brother too). The patient received over the time different traditional and biologic DMARDs, with an inadeguate control on arthritis’ flares, inflammatory values and anemia. He was treated with MTX in monotherapy (2001-2002); Etanercept in association with MTX first, then Cyclosporine and at last Leflunomide (2002 – 2005); Anakinra (2005 – 2007); Adalimumab (2007 – 2008); Etanercept again (2008 – 2009); Abatacept (2009 – 2010). In 2009 he started Abatacept therapy with DAS28CRP value of 5,27 (TJC: 6, SJC: 2, CRP 75 mg/dL, PGA 70 mm) and hemoglobin was 8,8 g/dL. Because of recurrent arthritis’ flares, on April 2010 he started Tocilizumab monotherapy (8 mg/kg monthly i.v.) after two months wash out.
Results At the baseline of Tocilizumab treatment DAS28CRP and HAQ were respectively 4,17 (TJC: 4, SJC: 2, CRP 6,84 md/dL, PGA 71 mm), and 1 and hemoglobin was 8,6 g/dL. At 18th month was present a remission since about six months and the hemoglobin was 11,6 g/dL, as reported in Table 1.
Conclusions Although in JIA patients there is an anemia due to chronic inflammation, the patient of this report has also a beta thalassemic trait as confounding factor. After Tocilizumab therapy, we obtained a low disease activity after three months and a complete remission after one year, otherwise previous biologic therapies. In addition to this result, the increased of hemoglobin concentration (3 g/dL respect basal value) suggest that Tocilizumab therapy is able to reset low hemoglobin levels due to chronic inflammation.
Berent Prakken et al. Juvenile idiopathic arthritis. The Lancet, 2011: 377; 9783:2138 - 2149
Gurion R.et al. Systemic arthritis in children: a review of clinical presentation and treatment. Int. J Infl. 2012;2012:271569
M. C. Genovese et al. Arthritis&Rheum 2008:58(10);2968–2980.
Disclosure of Interest None Declared
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