Subcutaneous immunoglobulin treatment for chronic inflammatory demyelinating polyneuropathy

Abstract

Immunoglobulin G (IgG) therapy is an established long-term treatment in chronic inflammatory demyelinating polyneuropathy (CIDP) that is commonly administered intravenously (IVIg). The subcutaneous immunoglobulin (SCIg) administration route is a safe and effective alternative option, approved by the United States Food and Drug Administration (FDA) in 2018, for maintenance treatment of adults with CIDP. Physicians and patients alike need to be aware of all their treatment options in order to make informed decisions and plan long-term treatment strategies. In this review, we collate the evidence for SCIg in CIDP from all published studies and discuss their implications and translation to clinical practice. We also provide guidance on the practicalities of how and when to transition patients from IVIg to SCIg and ongoing patient support. Evidence suggests that IVIg and SCIg have comparable long-term efficacy in CIDP. However, SCIg can provide additional benefits for some patients, including no requirement for venous access or premedication, and reduced frequency of systemic adverse events. Local-site reactions are more common with SCIg than IVIg, but these are mostly well-tolerated and abate with subsequent infusions. Data suggest that many patients prefer SCIg following transition from IVIg. SCIg preference may be a result of the independence and flexibility associated with self-infusion, whereas IVIg preference may be a result of familiarity and reliance on a healthcare professional for infusions. In practice, individualizing maintenance dosing based on disease behavior and determining the minimally effective IgG dose for individuals are key considerations irrespective of the administration route chosen.

Immunoglobulin replacement therapy in patients with immunodeficiencies: impact of infusion method on patient-reported outcomes

“…Despite the improvements in quality of life associated with switching from IVIg to SCIg, some patients may be discouraged because of perceived inconvenience, concerns about AEs at home, and a fear of needles [ 33 ]. A better understanding of the impact of different IgRT infusion methods on treatment satisfaction and well-being will allow for more evidence-based decision making, help guide best practice for IgRT, and facilitate optimization of patient outcomes.…”

Mild COVID-19 in an APECED Patient with Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) and High Titer of Type 1 IFN-Abs: A Case Report

“…Concerning our case, the patient presented an asymptomatic disease, only developing mild manifestations, such as asthenia or headache. IVIg may play a role in rescue therapy for critical COVID-19 cases, so we supposed that chronic therapy with SCIgs for CIPD may have played a possible protective role in the clinical course of our APECED patient [ 4 , 10 , 11 , 12 , 13 ]. Bastard et al in the previous series of 22 patients hypothesized possible protective factors for the 7 patients (34%) who did not become critically ill [ 4 ].…”

“…Interestingly, three patients were receiving monthly IVIg therapy at the time of the infection and another patient was hospitalized prophylactically and treated with subcutaneous recombinant IFN-beta and convalescent plasma therapy [ 4 ]. It could be possible that therapies with IVIg, SCIg, or plasma may decrease the pathogenicity of the autoantibodies against IFN, or they could act through other mechanisms [ 10 , 11 , 12 , 13 , 14 ]. Another hypothesis could be found in the early high dose of corticosteroids treatment, suggesting that it might prevent or attenuate the secondary hyper-inflammatory phase of the disease ( Table 2 ) or prevent adrenal crisis in patient with pre-existing primary adrenal insufficiency.…”

Chronic Inflammatory Demyelinating Polyradiculoneuropathy

“…In addition, many patients appreciate the self-sufficiency and being able to get the treatment when they want it and where they want it. 13 Although 2021 guidelines suggest that 1:1 dose conversion is reasonable, SCIG doses of 0.2 and 0.4 g/kg/wk were studied in a clinical trial and were both found to be effective compared with placebo. 12 Furthermore, long-term efficacy results from the PATH extension study showed lower relapse rates with the higher dose of 0.4 g/kg/wk.…”