Table 3.

Considerations for treatment optimization during treatment of CIDP.

Frequent application of outcome measurements, such as strength impairment with grip strength dynamometry and disability with I‐RODS, can be useful to document treatment response, quantify treatment‐related fluctuations, and guide maintenance therapy. Some degree of “wear‐off” may be acceptable and provide reassurance that continued immunotherapy is necessary. However, if clinically meaningful and unacceptable IVIG treatment‐related fluctuations are present, shortening treatment intervals (more so than increasing IVIG dose) is likely to be beneficial. All patients should undergo periodic structured immunotherapy taper trials to assess disease activity. CS should be used only with caution in the motor‐predominant and perhaps Lewis Sumner Syndrome atypical CIDP variants. Worsening following CS exposure has been described in these subsets of patients. Patients with predominantly IgG4 autoantibodies (against nodal and/or paranodal antigens) are unlikely to benefit from IVIG. Treatment with CS or PE should be considered first line in these individuals, with B‐cell depletion therapy reserved for patients refractory or intolerant to CS.

CIDP, chronic inflammatory demyelinating polyneuropathy; CS, corticosteroid; I‐RODS, Inflammatory Rasch‐built Overall Disability Scale; IgG4, immunoglobulin G4; IVIG, intravenous immunoglobulin; PE, plasmapheresis.