Open AccessClinical characteristics and prognosis of amyotrophic lateral sclerosis with autoimmune diseases
Author(s) -
Jin-Yue Li,
Xiaohan Sun,
Dongchao Shen,
Xunzhe Yang,
Mingsheng Liu,
Liying Cui
Publication year - 2022
Publication title -
plos one
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.99
H-Index - 332
ISSN - 1932-6203
DOI - 10.1371/journal.pone.0266529
Subject(s) - medicine , amyotrophic lateral sclerosis , hazard ratio , medical record , immunotherapy , proportional hazards model , cancer , retrospective cohort study , survival analysis , disease , propensity score matching , clinical trial , respiratory failure , oncology , confidence interval
The 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. Methods This 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. Results There 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 ( p <0.05). After propensity score matching by sex, onset age, and disease duration, the difference in respiratory function remained significant between groups. We found no differences in overall survival between ALS patients with and without AIDs before and after matching ( p = 0.836; p = 0.395). Older age at onset, rapid disease progression, and lower erythrocyte sedimentation rate (ESR) were associated with shorter survival ( p <0.05). Among ALS patients with AIDs, 8 (30.8%) had a history of immunotherapy and showed slightly prolonged survival compared with those without immunotherapy, but the results did not reach statistical significance ( p = 0.355). Conclusions Patients with coexisting ALS and AIDs had older onset age and poorer respiratory function but similar overall survival than those with pure ALS.