Alternatives to intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyradiculoneuropathy

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a progressive or relapsing and remitting disease of the peripheral nervous system. It is generally thought to have an autoimmune aetiology and has a global prevalence estimated at 0.8–8.9 per 100,000. In its classical form, it is characterised by a symmetric proximal and distal weakness and sensory dysfunction of all extremities, developing over at least 2 months. Less commonly, CIDP may present with cranial nerve involvement, pure motor, pure sensory, asymmetric or predominately distal limb dysfunction. The optimum treatment for CIDP has long been an area of debate, although with a consensus for the use of intravenous immunoglobulin (IVIg) as an induction and maintenance treatment. Trials have demonstrated that IVIg is effective in the short- and long-term treatment and well tolerated. However, there are considerable cost implications for the long-term use of IVIg, as well as the infrastructure required to administer an infusion for 2–3 days at intervals that may be as short as every 3–6 weeks. Corticosteroids, although known since 1958 to be an effective treatment choice in CIDP with response rates reported between 30 and 90%, have well-recognised cumulative side effects. However, alternative immunosuppressants such as azathioprine, methotrexate or rituximab are commonly viewed as alternatives to IVIg particularly in healthcare systems that may be unable to provide invasive treatments such as plasma exchange or IVIg. As a result, research has focussed on identifying alternative immunomodulating agents that are more convenient, cost effective and improve patient outcomes and autonomy. This month’s journal club will review three treatment options in the management of CIDP. The first paper reviews the use of subcutaneous immunoglobulin (SCIg) in the PATH trial; the second looks at the use of different regimes of corticosteroids and the last reviews the novel use of fingolimod in the FORCIDP trial.