Chronic inflammatory demyelinating polyneuropathy in end-stage renal disease patients: a case series study
摘要
The occurrence of chronic inflammatory demyelinating polyneuropathy (CIDP) in patients with end-stage renal disease (ESRD) has been rarely studied. This study was intended to explore the clinical, electrophysiological and neuropathological features of six patients with CIDP and ESRD.
MethodsThe medical records collected in Peking University First Hospital between 2021 and 2024 were reviewed before six patients were identified who had developed CIDP with ESRD. The data on their clinical features, electrophysiological tests, nerve ultrasound, laboratory examinations and sural biopsies was retrieved.
ResultsAll the six patients were males ages 35 to 63. Before and after dialysis, all these patients presented with varying degrees of limb sensory impairment and weakness, and reduced or absent tendon reflexes. Albuminocytologic dissociation of cerebrospinal fluid was observed in all of them. Nerve conduction studies identified the demyelinating features while nerve ultrasound showed nerve enlargement of upper limbs. Sural nerve biopsies showed mild to moderate decreases of myelinated nerve fiber density, with differences between different nerve fascicles. Thin myelinated fibers were seen, without onion bulb-like structure. Varying degrees of axonal degeneration with regeneration were identified in some of these patients. Immunotherapy was effective in all these patients.
ConclusionsThe diagnosis of CIDP should be considered if an ESRD patient develops progressive demyelinating neuropathy. Immune therapy has shown clinical efficacy despite the underlying mechanisms that remain poorly understood.