Clinical characteristics and prognosis of amyotrophic lateral sclerosis with autoimmune diseases.

Abstract

IntroductionThe occurrence of autoimmune diseases (AIDs) in amyotrophic lateral sclerosis (ALS) patients is widely reported, but little is known about the associated clinical phenotype. This study aims to evaluate the clinical features and prognosis of ALS patients with AID.MethodsThis retrospective study was based on the ALS Registry dataset of Peking Union Medical College Hospital from 2013 to 2020. Clinical features and inflammatory biomarkers at registration were compared between ALS patients with coexisting AIDs and those without (controls). The medical records of immunotherapy were also collected. The Kaplan-Meier method and Cox proportional hazard model were used to study the survival of ALS patients.ResultsThere are 26 (1.6%) ALS patients with AIDs in our database. The ALS patients with AIDs had older ages at onset and poorer respiratory function than controls (pConclusionsPatients with coexisting ALS and AIDs had older onset age and poorer respiratory function but similar overall survival than those with pure ALS.