Chronic inflammatory demyelinating polyneuropathy and malignancy: A systematic review

Abstract

A systematic review of the literature was performed on the association of chronic inflammatory demyelinating polyneuropathy (CIDP) with malignancy. Hematological disorders are the most common association, particulalry non-Hodgkin lymphoma. CIDP frequently precedes the malignancy diagnosis, and there is a favorable CIDP response to treatment more than 70% of the time. Melanoma is the second most common association and may be accompanied by antiganglioside antibodies; CIDP shows a good response to immunotherapy. Other cancers are rare, with variable timings and presentations but good responses to immunomodulation and/or cancer therapy. Unusual neurological features such as ataxia, distal/upper limb predominance, or cranial/respiratory/autonomic involvement may suggest associated malignancy as may abdominal pain, diarrhea/constipation, poor appetite/weight loss, dermatological lesions, and lymphadenopathy. In the appropriate clinical and electrophysiological setting, CIDP associated with cancer should be considered. Immunomodulatory therapy, cancer treatment alone, or a combination may be effective. Muscle Nerve 57: 875-883, 2018.

Frequency of diabetes and other comorbidities in chronic inflammatory demyelinating polyradiculoneuropathy and their impact on clinical presentation and response to therapy

“…A possible association of CIDP with cancer has been previously reported, although there are no epidemiological data consistent with this association. [9] A medical history of solid cancer was present in 9% of our patients, percentage similar to that observed in the general Italian population with the same age. [28] In only 55% of the cases, the diagnosis of cancer preceded the diagnosis of CIDP by a mean of 10 years (mode 8 years, range 1-25), in 29% the diagnosis of CIDP preceded the diagnosis of cancer by a mean of 8.5 years (mode 7 years, range 1-21), while only in 11% of the patients the two diagnoses were made in the same year (table 2).…”

“…Some previous studies reported an association between CIDP and lymphoma. [7][8][9] We found a low prevalence of lymphoma in our patients with CIDP and did not find difference in demographic and clinical features between patients with and without lymphoma. This data and the lapse of time between CIDP and lymphoma (mean 18 years; range 8-39 years) and vice versa (mean 8 year; range 7-10 years) do not support a possible paraneoplastic mechanism of the neuropathy.…”

“…Commons licence -details of these licences and which Creative Commons licence will apply to this Work are set out in our licence referred to above. [2][3][4][5][6] lymphoma, [7][8][9] solid cancer, [9] monoclonal gammopathy of undetermined significance (MGUS), [10][11][12][13] plasma cell dyscrasias, [9,14] and other disorders. [15][16][17][18] Most of these associations have been reported in isolated cases or small series of patients so that their frequency in CIDP and possible clinical and pathogenic relevance, impact on disability, quality of life (QoL), and response to treatment remains unclear.…”

Chronic Inflammatory Demyelinating Polyradiculoneuropathy

“…101,102 The efficacy of pulsed oral steroids was demonstrated in the PREDICT study, 103 where pulsed oral dexamethasone 40 mg a day for 4 days, every 4 weeks, showed similar efficacy but less adverse effects (i.e., Cushingoid features and sleeplessness) compared with daily prednisolone 60 mg tapered over 6 months Table 5 Associated conditions and mimics Conditions in which coexisting CIDP may be seen. Diabetes, HIV, monoclonal gammopathies, Charcot-Marie-Tooth type 1, other autoimmune disorders (i.e., lupus, inflammatory bowel disease, membranous nephropathy), [91][92][93] chronic hepatitis, 4 post-organ transplant, 94 malignancy, 95 TNF-α inhibitor use (not known to cause neuropathy otherwise). 96 Immune checkpoint inhibitor use 97,98 Other neuropathies that may mimic CIDP clinically and electrodiagnostically Anti-MAG neuropathy, multifocal motor neuropathy, neuropathy in POEMS, hereditary transthyretin amyloid neuropathy.…”

Peripheral Nervous System Involvement in Lymphoproliferative Disorders

“…CIDP frequently precedes the haematological diagnosis and responds to treatments in the same manner as the idiopathic form. 15 …”