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Abstract Details

Patients with Anti-Hu/ANNA1 Paraneoplastic Neurological Syndromes Develop Progressive Disability but Have Increased Survival Compared to SCLC Patients Without Paraneoplastic Syndromes
Autoimmune Neurology
S38 - Autoimmune Neurology: Peripheral Autoimmunity, Paraneoplastic Disease, Checkpoint Inhibitors, and Neurosarcoidosis (4:54 PM-5:06 PM)
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ANNA-1/Hu-IgG PNS are rare and factors associated with disability and survival outcomes are unknown. 

We aimed to 1) examine disability and survival in patients with ANNA1/Hu-IgG paraneoplastic neurological syndromes (PNS), and 2) compare survival to small cell lung cancer (SCLC) without PNS.

This is a retrospective cohort study of ANNA1/Hu-IgG+ patients with PNS. Cox proportional hazard models examined clinical predictors of disability and survival. Kaplan-Meier curves examined time to mortality, wheelchair dependence, mRS>2. Log-rank tests assessed differing survival among groups. 

Forty-five patients with ANNA/Hu1-IgG PNS were identified (73% female, mean age=63 years [SD=13]). Phenotypes included; neuropathy (16, 36%), cerebellar ataxia (11, 24%), limbic encephalitis (10, 22%), myasthenia gravis/Lambert-Eaton myasthenic syndrome (3, 7%), myelopathy (2, 4%), enteric neuropathy (2, 4%), myoclonus (1, 2%).

Cancers were identified in 78%; SCLC (26, 74%), non-SCLC (4, 11%), other (5, 14%). Immunosuppressive therapy for PNS was given in 53%.

At PNS onset, disability was mild (mRS=0-2) in 67%, however at 5-year follow-up 72% had mRS>2 and 63% were wheelchair-dependent. Limbic encephalitis had greater hazard of reaching mRS>2 at last follow-up (HR=7.37, [95% CI=2.09-26.00], p=0.002). At 5-year follow-up, 58% had died. Survival was not influenced by phenotype or immunosuppressive treatment. 

Patients with SCLC and ANNA1/Hu-IgG PNS (n=26) compared to SCLC without ANNA1/Hu-IgG PNS (n=1513) had 59% lower hazard of death (HR =0.41, [95% CI = 0.25–0.69], p<0.001). Five-year survival was 36% for ANNA1/Hu-IgG and 13% for SCLC without PNS. A sensitivity analysis of patients matched 3:1 by age and sex demonstrated similar results. 

This study demonstrates that patients with ANNA1/Hu-IgG PNS develop progressive disability and risk of disability is greater in patients with limbic encephalitis. However, patients with SCLC and ANNA1/Hu-IgG PNS survived longer than those without PNS, possibly due to earlier cancer detection or increased antitumor immunity.

Authors/Disclosures
Kimberly DiMauro
PRESENTER
Dr. DiMauro has nothing to disclose.
No disclosure on file
Glen Stevens (Cleveland Clinic Foundation) The institution of Dr. Stevens has received personal compensation in the range of $50,000-$99,999 for serving on a Scientific Advisory or Data Safety Monitoring board for NIH. Dr. Stevens has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for EBSCO. The institution of Dr. Stevens has received research support from National Cancer Institute.
Amy Kunchok (Cleveland Clinic - Mellen Centre) Dr. Kunchok has received personal compensation in the range of $0-$499 for serving as a Consultant for EMD Serono. Dr. Kunchok has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon therapeutics . Dr. Kunchok has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Kunchok has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Neurology.