Figure 1.

Immunosuppression strategies (based on Rauz et al¹³). A step–ladder approach to treatment with agents having the fewest side effects to those that have the greatest side effects is adopted according to disease activity (mild, moderate or severe), which is used to guide therapy. Dapsone (25–50 mg twice a day) or sulphapyridine (500 mg twice a day) can be used for mild inflammation; azathioprine (1–2.5 mg/kg/day) or mycophenolate mofetil (500–1000 mg twice a day if intolerant to azathioprine) may be added or substituted for persistent disease. Severe inflammatory disease is treated with cyclophosphamide (1–2 mg/kg/day) and adjuvant prednisolone (1 mg/kg/day with or without supplementary loading doses of 1 g intravenous methylprednisolone preceding oral therapy) for up to 3 months until the optimal effects of cyclophosphamide have taken effect. Patients with refractory disease are managed through intravenous immunoglobulin or ‘biological' agents such as anti-CD 20 (rituximab) or anti-TNFα therapy.