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.

Keywords: Burden; CIDP; Cost; Epidemiology; QoL; Treatment.

Fig. 1

PRISMA diagram. EXC1: not in CIDP OR: CIDP data only provided pooled with other diseases; EXC2: not relevant for the issues under inquiry, e.g., when the abstract shows no apparent contents of interest; EXC3: does not focus on topics outlined in the inclusion criteria (see Table 1.) EXC4: not in adults (≥ 18); EXC5: not of the following study types: dataset, guideline, meta-analysis, multi-centre study, observational study, practice guideline, review, systematic reviews, clinical trial—phase III, clinical trial—phase IV, pragmatic clinical trial, randomised-controlled trial OR: case reports, conference proceedings, letters, commentaries or editorials; EXC6: not in English; EXC7: papers not published between 2009 and 2019; EXC8: only covers animal or in vitro studies; EXC9: replica/no abstract/no full text

Fig. 2

EQ-5D mean index scores of patients with CIDP [9, 25]. CIDP chronic inflammatory demyelinating polyneuropathy, EQ-5D EuroQoL-5D

Fig. 3

SF-36 scores in a Dutch cohort of patients with CIDP Adapted from Kuitwaard et al. 2009 [7]. The dashed line reflects the normative value of SF-36 by healthy patients: 50 points. Sample size; n = 76. CIDP chronic inflammatory demyelinating polyneuropathy, SF-36 Short Form-36

Fig. 4

Distribution of CIDP direct costs by cost components in the UK and USA [25, 57]. USA costs were calculated on a 2-year basis [57], whereas UK costs are based on an annual estimate [25]