Clinical factors, diagnostic delay, and residual deficits in chronic inflammatory demyelinating polyradiculoneuropathy

Abstract

Management of patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is complicated by the challenging diagnosis, monitoring of disease activity, and treatment response. Real world data about the current practice of care in CIDP are rare, but important to improve clinical guidelines. In this study, we determined the current practice of diagnosis and treatment in relation to the clinical outcome of patients with CIDP in a cross‐sectional study in The Netherlands. All patients registered at the Dutch neuromuscular patient organization and patients at the Erasmus Medical Center were approached. CIDP diagnosis was confirmed by using patient files and expert consensus. The response rate was 137/197 (70%) and the diagnosis CIDP was confirmed in 112 patients. The time from onset of symptoms until CIDP diagnosis was median 5 months and > 12 months in 26% of the patients. Patients with a late diagnosis (>5 months) compared to patients with an early diagnosis (≤5 months) more frequently had another initial diagnosis (P < .001), multifocal abnormalities (P = .011), final diagnosis made in university hospitals (P = .001), and more (residual) complaints, also illustrated via two patient reported, validated clinical outcome measures. Although 97% of patients received treatment, 88% reported (residual) neurological symptoms and deficits. CIDP diagnosis is often delayed, especially in patients with atypical CIDP variants. Diagnostic delay may result in delayed initiation of treatment. A more rapid and accurate diagnosis of CIDP may prevent or at least reduce residual neurological deficits and accompanying disability in CIDP.

Underdiagnosis and diagnostic delay in chronic inflammatory demyelinating polyneuropathy

“…CIDP underdiagnoses, may lead to quite wide-ranging delay in appropriate diagnosis and offering immunotherapeutic treatment as shown in our study, in line with previous studies [3,5]. Moreover, axonal loss in CIDP worsens with time and adversely affects treatment response [22], again prompting the need for early diagnosis and intervention to limit the disability.…”

“…We investigated the frequency and causes of pre-referral underdiagnoses of CIDP in consecutive patients attending our specialist inflammatory neuropathy service, and also determined the diagnostic and treatment delay. This aspect of underdiagnoses in treatment responsive CIDP has been explored rarely [3,5]. To the best of our knowledge, this is the first study focussing on specific clinical and electrophysiological features of CIDP contributing In our cohort, 23.3% cases had a pre-referral diagnosis of GBS which was corrected to acute-onset CIDP.…”

“…The delay in diagnosis of CIDP has been reported to range from 2 to 64 months [4]. A more recent Dutch study showed diagnostic delay of more than 12 months in 26% of cases [5]. However, the precise frequency and the factors leading to underdiagnoses (i.e., patients receiving an initial erroneous non-CIDP diagnosis and treated as such and having a final diagnosis of CIDP) of treatment-responsive CIDP is unknown, which in turn may have major consequences for offering immunomodulatory therapy to potentially eligible patients at an early stage when it is most likely to be effective.…”

Diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy in clinical practice: A survey among Dutch neurologists

“…1,2 Despite the published European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) 2010 diagnostic criteria for CIDP, the diagnosis can be challenging, leading to both over-and underdiagnosis. [3][4][5][6][7][8] The extent to which patients can differ in clinical presentation has become more visible in the last decade, resulting in an extended group of atypical CIDP variants, such as distal predominant and asymmetric, for which clear definitions are lacking. 1,9 In addition, not all patients with a clinical suspicion of CIDP completely fulfil the EFNS/PNS 2010 (electro) diagnostic criteria for CIDP.…”

The comparison of MRN, electrophysiology and progression among typical CIDP and atypical CIDP subtypes

“…Second, the ethnical differences and genetic backgrounds between Italy and China may represent reasons for the different rates of disease progression. Finally, several studies have shown that different treatment initiation times and treatment regimens may also lead to different results 26 – 28 . The uneven distribution of medical resources and limited therapeutic levels in China lead to the late start of treatment for patients, and most patients are treated with hormones; only a few patients have received a single course of IVIg (intravenous immunoglobulin).…”