What is refractory CIDP?

In this journal, Kiers et al review treatment options for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) beyond the established first-line therapies of corticosteroids, immunoglobulin and plasma exchange. Their review, framed by the 2021 European association of neurology/ Peripheral nerve society (EAN/PNS) CIDP guidelines, addresses diagnostic challenges in variant presentations and the need to consider alternative diagnoses when first-line treatment response is suboptimal. They discuss current evidence for T-cell and B-cell-directed therapies, including autologous stem cell transplantation, CAR-T cell therapy and proteasome inhibition, as well as novel molecular targets such as FcRN and C1s (complement) inhibitors. Given recent therapeutic developments, limited high-quality evidence for alternatives and associated risks, the review underscores the need to better define ‘refractory CIDP’.