Association between chronic inflammatory demyelinating polyneuropathy and gastrointestinal malignancies
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CLINICAL REVIEW
Association between chronic inflammatory demyelinating polyneuropathy and gastrointestinal malignancies Adnan Malik1 · Rani Berry2 · Brian M. Fung3 · James H. Tabibian4,5 Received: 15 July 2020 / Accepted: 20 October 2020 © Japanese Society of Gastroenterology 2020
Abstract Chronic inflammatory demyelinating polyneuropathy (CIDP) is an uncommon and under-recognized immune-mediated disorder of the peripheral nervous system. It is associated with both infectious and non-infectious etiologies and presents in several variant forms. In rare instances, CIDP has been reported in association with gastrointestinal (esophageal, hepatic, colorectal, and pancreatic) malignancies. The diagnosis of malignancy is typically preceded by weeks to months by that of CIDP, though the inverse may also be seen. As with other etiologies of CIDP, cases associated with gastrointestinal malignancies are often treated with corticosteroids, intravenous immunoglobulins, and/or plasma exchange, with improvement or resolution of neurological symptoms in the majority of cases. In this review, we provide a practical overview of CIDP, with an emphasis on recognizing the clinical association between CIDP and gastrointestinal malignancies. Keywords Peripheral neuropathy · Carcinoma · Autoimmune disease · Gastrointestinal
Introduction Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare, autoimmune neurological disorder characterized by inflammation and destruction of peripheral nerve myelin. It is believed to have a similar pathophysiology to acute inflammatory demyelinating polyneuropathies * James H. Tabibian [email protected] Adnan Malik [email protected] Rani Berry [email protected] Brian M. Fung [email protected] 1
Division of Hepatology, Loyola University Medical Center, Maywood, IL, USA
2
Department of Internal Medicine, UCLA Ronald Reagan Medical Center, Los Angeles, CA, USA
3
Department of Medicine, Olive View-UCLA Medical Center, Sylmar, CA, USA
4
Division of Gastroenterology, Olive View-UCLA Medical Center, 14445 Olive View Dr, Sylmar, CA 2B‑182, USA
5
David Geffen School of Medicine at UCLA, Los Angeles, CA, USA
(AIDPs) such as Guillain–Barre syndrome (GBS), with the main distinguishing factor being the chronicity of the illness. CIDP differs from AIDPs in that it often presents with slow symptom onset and clinical deterioration, usually over the course of eight or more weeks [1–3]. CIDP has an estimated annual incidence of 1–9 cases per 100,000 and prevalence of 2.81 per 100,000 [4, 5]. Unlike many other autoimmune disorders, CIDP appears to affect males more commonly than females, with reported gender ratios between 1.4 and 4.4 [4]. Although significant heterogeneity exists within the presentation of CIDP, the majority of patients experience subacute to chronic numbness, proximal and distal weakness, and loss of reflexes [6]. While the exact etiology of this disease is not clear, most experts now believe that CIDP is caused by a dysregulation of the body’s immu
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