A Global Survey of Disease Burden in Patients Who Carry a Diagnosis of Chronic Inflammatory Demyelinating Polyneuropathy

Abstract

Introduction: The extent to which work productivity, emotional well-being, social interactions, and family life are impacted in patients who self-identify as having chronic inflammatory demyelinating polyneuropathy (CIDP) is not well characterized. Methods: Data from an online survey of 595 individuals with self-reported CIDP, recruited by the Guillain-Barré syndrome (GBS)/CIDP Foundation, were used to assess disease and treatment burden. A total of 37% of patients were classified as ''likely'', 34% as ''somewhat likely'', and 28% as ''unlikely'' CIDP. Results: Of ten symptoms that patients with CIDP may experience, each symptom was experienced by 77-94% of ''likely'', 79-96% of ''somewhat likely'', and 66-91% of ''unlikely'' patients. In ''likely'' CIDP patients 44% stopped working because of their symptoms and 24% moved to a new home. The most common treatments were intravenous immunoglobulin (IVIg) infusion and corticosteroids. IVIg was associated with venous access issues and work/ school absenteeism. Conclusions: CIDP diagnostic confirmation was not performed in any of the survey respondents. Our results do not add any knowledge on the diagnosis or treatment of CIDP. Our findings do provide insight into the symptoms that patients that think they have CIDP or have been told they have CIDP experience, explores how patients that are labeled as having CIDP view treatment expectations, and highlights how these symptoms affect home and work life. We hope that the findings are constructively used to get patients the services they need to improve quality of life, maintain employment, and ensure a safe home environment regardless of diagnostic accuracy.

A longitudinal evaluation of fatigue in chronic inflammatory demyelinating polyneuropathy

“…The frequency of impaired quality of life in patients with CIDP has been well described. Patients with CIDP have consistently been shown to have lower baseline health‐related quality of life compared with historical controls (Allen et al., 2021 ; Gwathmey et al., 2016 ; Maxwell et al., 2013 ; Merkies et al., 2002 ; Padua et al., 2004 ). In one large survey of patients with unconfirmed CIDP, weakness and numbness in the lower limbs, poor balance, and fatigue were among the most bothersome symptoms reported by patients (Allen et al., 2021 ).…”

“…Patients with CIDP have consistently been shown to have lower baseline health‐related quality of life compared with historical controls (Allen et al., 2021 ; Gwathmey et al., 2016 ; Maxwell et al., 2013 ; Merkies et al., 2002 ; Padua et al., 2004 ). In one large survey of patients with unconfirmed CIDP, weakness and numbness in the lower limbs, poor balance, and fatigue were among the most bothersome symptoms reported by patients (Allen et al., 2021 ). While disease burden vis‐à‐vis weakness and sensory loss invariably contribute to poor quality of life, our findings highlight the important role that nonspecific fatigue plays as well.…”

Treatment of chronic inflammatory demyelinating polyneuropathy

“…Pain and fatigue are among the symptoms of greatest concern to patients. 20 Pain has been reported in 7% to 72% of patients. 21 In cases where pain is prominent we approach the diagnosis of CIDP cautiously, and we are careful to exclude other conditions such as POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) syndrome, vasculitis, diabetes, and amyloidosis.…”

Could posturography be a useful outcome measure for chronic inflammatory demyelinating polyneuropathy?

“…Over half of patients with CIDP progress to the point where they are unable to walk without assistance 1 . Balance disturbance is frequently reported by patients as the most bothersome symptom, and the symptom that they most desire to improve with treatment 3 . Gait performance has been shown to correlate with quality of life in patients with CIDP and functional status in patients with other types of peripheral neuropathy 4,5 .…”

“…the most bothersome symptom, and the symptom that they most desire to improve with treatment. 3 Gait performance has been shown to correlate with quality of life in patients with CIDP and functional status in patients with other types of peripheral neuropathy. 4,5 CIDP is treated with immune modulating or immune suppressive treatments with the goal of improving muscle strength and sensation as well as improving daily function.…”