Systematic literature review of burden of illness in chronic inflammatory demyelinating polyneuropathy (CIDP)

Abstract

Background Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare neurological disorder characterised by muscle weakness and impaired sensory function. The present study provides a comprehensive literature review of the burden of illness of CIDP. Methods Systematic literature search of PubMed, Embase, and key conferences in May 2019. Search terms identified studies on the epidemiology, humanistic burden, current treatment, and economic burden of CIDP published since 2009 in English. Results Forty-five full texts and nineteen conference proceedings were identified on the epidemiology (n = 9), humanistic burden (n = 7), current treatment (n = 40), and economic burden (n = 8) of CIDP. Epidemiological studies showed incidence and prevalence of 0.2-1.6 and 0.8-8.9 per 100,000, respectively, depending on geography and diagnostic criteria. Humanistic burden studies revealed that patients experienced physical and psychosocial burden, including impaired physical function, pain and depression. Publications on current treatments reported on six main types of therapy: intravenous immunoglobulins, subcutaneous immunoglobulins, corticosteroids, plasma exchange, immunosuppressants, and immunomodulators. Treatments may be burdensome, due to adverse events and reduced independence caused by treatment administration setting. In Germany, UK, France, and the US, CIDP economic burden was driven by direct costs of treatment and hospitalisation. CIDP was associated with indirect costs driven by impaired productivity. Conclusions This first systematic review of CIDP burden of illness demonstrates the high physical and psychosocial burden of this rare disease. Future research is required to fully characterise the burden of CIDP, and to understand how appropriate treatment can mitigate burden for patients and healthcare systems.

A Rare Case of COVID-19-Induced Chronic Demyelinating Polyneuropathy

“…According to one study, 17% and 74% of patients with CIDP reported severe pain and severe fatigue, respectively [ 12 ]. Another study reported impairments in daily activities like unzipping, using a knife and fork, and requiring some support for outdoor activities like walking and standing [ 13 ]. This case illustrates diagnostic complexity and poor response to treatment due to prolonged ICU stay leading to ICU myopathy and deconditioning.…”

Novel Immunological and Therapeutic Insights in Guillain-Barré Syndrome and CIDP

“…Therapeutic plasma exchange (PLEx) may provide an alternative therapy for patients who do not respond to IVIg and corticosteroids; it can be very effective, especially in patients who have a relapsing disease course or with severe relapses [77,78]. A PLEx course of 5-10 every-other-day sessions within 2-4 weeks is commonly used [9].…”

“…A PLEx course of 5-10 every-other-day sessions within 2-4 weeks is commonly used [9]. Several small trials have shown that between 48 and 81% of CIDP patients have significant short-term improvement after PLEx, but rapid deterioration may occur after completion of treatment [77,79].…”

The neurophysiological lesson from the Italian CIDP database

“…Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic and disabling disease with a range prevalence of 0.8-8.9 cases per 100,000 and incidence of 0.2-1.6/ 100,000 [1]. It is the most frequent acquired immunemediated chronic neuropathy and has a broad spectrum of possible clinical presentation including typical and atypical forms [2][3][4].…”