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NMOSD: What Are The Effects Of Comorbidities On Relapse Rates?
Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune demyelinating disorder of the central nervous system marked by recurring episodes of optic neuritis and transverse myelitis.1 Rare and chronic, its prevalence is estimated to be between 0.037 and 10 per 100,000 individuals, varying by geographic region and population.2 NMOSD often involves antibodies against aquaporin-4 (AQP4), the main water-channel protein in the brain, a finding that sets it apart from multiple sclerosis (MS).2
The Bottom LineTo shed light on whether the presence of malignancies and autoimmune diseases influences the annualized relapse rate (ARR) in NMOSD patients, investigators in Taiwan tapped data from the country's largest hospital system to analyze demographic characteristics, relapse rates, and comorbidities among 485 NMOSD patients.1 Most of these patients (n=466) were adults; the rest were children (n=19). They were identified from data collected between 2006 and 2021.
What the research team discovered
The median ARR was found to be 0.51 (interquartile range [IQR] 0.26 to 1.11) for adults and 0.39 (IQR 0.21 to 0.77) for pediatric patients.1 Among the adult NMOSD patients, 6.7% had malignancies, while 21.7% had other autoimmune diseases. The study noted a significantly higher risk of malignancy in adult NMOSD patients compared to the general population, with a relative risk (RR) of 5.99 (95% confidence interval 2.71 to 9.23; P<.01).
Breast cancer was the most frequently reported malignancy (19%), followed by brain (16%), liver (6%), and colorectal cancers (6%). Among autoimmune disorders, Sjogren's syndrome was the most frequent, affecting 12.7% of adult patients (n=59) and 21.1% of pediatric patients (n=4).
Although the ARR was higher for the entire cohort during the 3-year period following a diagnosis of NMOSD, there was no significant difference in ARR between patients with malignancies or autoimmune diseases and those without. This was contrary to the findings of previous studies, which indicated potential increases in relapse rates and disease severity due to comorbidities.
"The discrepancy between our results and previous studies may be attributed to a significantly higher percentage of using rituximab in NMOSD patients with autoimmune disease than those without," the authors explained in their report, which was published in Multiple Sclerosis and Related Disorders.1
Implications for clinical practice
The results underscore the need for vigilant monitoring for malignancies in NMOSD patients. In fact, the authors were very specific with regard to timing: "Using age as a predictor to perform the discrimination analysis in NMOSD with and without malignancy, we found that 43.3 years was the best cutoff age to consider the possibility of malignancy in NMOSD, with a sensitivity and specificity of 0.84 and 0.46, respectively," they wrote.1
This is significant, because detecting and managing comorbid conditions early can influence overall patient outcomes and quality of life. "We found that 6.7% of patients with NMOSD had malignancy, which could occur before or after the diagnosis of NMOSD, with a median time interval of approximately 2.3 years," the authors noted.1
Again, the study found no significant differences in ARRs when comparing NMOSD patients with and without autoimmune diseases. This suggests that while comorbidities are prevalent among NMOSD patients, their presence does not necessarily exacerbate the disease's relapse rate within the initial years post-diagnosis.
Strengths and limitations
The study's strengths include its large sample size and extensive time frame, which provided a robust data set for analysis. Additionally, the researchers employed rigorous standards to ensure accurate diagnosis and classification of NMOSD, distinguishing it from MS, which can often present similarly in clinical settings.
A limitation of the study was the fact that it wasn't population-based, making the findings potentially not generalizable to the broader population. The researchers also acknowledged difficulties in pinpointing exactly when symptoms began, relying instead on the date of a confirmed diagnosis, which could affect the accuracy of the relapse timing analysis.
Why these results matter
This study provides valuable insights into the relationship between comorbidities and NMOSD relapse rates. The finding that comorbidities like malignancies and autoimmune diseases do not significantly affect the relapse rate within 3 years post-diagnosis is crucial for clinicians managing NMOSD.
Early and vigilant screening for malignancies in NMOSD patients, particularly as they approach middle age, is recommended to improve patient outcomes. The study's findings enhance the understanding of NMOSD and its management, emphasizing the need for comprehensive care approaches that include diligent monitoring for comorbid conditions.
To build upon these important insights, the researchers recommended further studies to explore the fundamental pathways linking NMOSD with malignancies. Understanding these connections, they suggest, could lead to more-effective management strategies and improved patient outcomes.
Finally, there's a need for more-comprehensive studies that include a broader population base to validate these findings—and to explore their applicability in different demographic settings.
Published: October 10, 2024
Adam Ash is an emergency medicine physician who practices in New York. His 16 years of experience in education, administration, and research allow him to write about a broad spectrum of medical topics.
Eculizumab Effectiveness In NMOSD And Risks Of Postvaccination Attacks And Infections
Photo Credit: Asd
The following is a summary of "Eculizumab Use in Neuromyelitis Optica Spectrum Disorders: Routine Clinical Care Data from a European Cohort," published in the October 2024 issue of Neurology by Ringelstein et al.
Neuromyelitis optica spectrum disorders (NMOSDs) are managed by preventing attacks, with eculizumab (ECU), a terminal complement cascade inhibitor, shown to be effective in aquaporin-4 (AQP4)-IgG seropositive (+) cases in clinical trials.
Researchers conducted a retrospective study evaluating the effectiveness and safety of eculizumab in patients with AQP4-IgG+ NMOSD.
They reviewed data of patients with AQP4-IgG+ NMOSD treated with ECU (December 2014 and April 2022) at 21 centers in Germany and Austria. Primary outcomes included annualized attack rate (AAR), MRI activity, Expanded Disability Status Scale (EDSS), and safety.
The results showed 52 patients (87% female, age 55.0 ± 16.3 years) received ECU for a median of 16.2 months (IQR 9.6 – 21.7). After meningococcal vaccination, 7 of 36 patients (19%) had attacks, while no attacks occurred in those on prednisone. Eculizumab reduced the median AAR from 1.0 to 0 (P<0.001), and MRI activity and EDSS remained stable. Serious infections occurred in 13% of patients, and 10% died (primarily older, severely disabled).
They concluded that ECU effectively prevented NMOSD attacks, though risks such as infections and post-vaccination attacks must be considered, especially in patients with comorbidities.
Source: neurology.Org/doi/10.1212/WNL.0000000000209888
NMOSD: Current Perspectives
Neuropsychological assessments are key to diagnosing executive cognitive impairment in patients with neuromyelitis optica spectrum disorder.
Using 15 years' worth of data from the largest hospital system in Taiwan, a team of researchers examined the impact of comorbidities on relapse rates among 485 patients with neuromyelitis optica spectrum disorder.
For patients living in Denmark, a diagnosis of aquaporin-4 antibody-seropositive neuromyelitis optica spectrum disorder (AQP4-Ab+ NMOSD) translates into a much shorter life expectancy, say the results of this study.
In this meta-analysis, serum vitamin D levels were lower in patients with neuromyelitis optica spectrum disorder than in healthy controls. That said, the authors cited several caveats that need further investigation.
According to the results of a new study from researchers in South Korea, plasma sphingolipids may help monitor disability and disease activity in patients with neuromyelitis optica spectrum disorder (NMOSD) and assist clinicians in choosing therapies.
A recent analysis found an elevated risk of type 2 diabetes among patients with neuromyelitis optica spectrum disorder, with steroid use possibly contributing to this greater risk.
This survey of people with NMOSD found that older age, higher pain scores, use of walking aids, lower wages pre-diagnosis, interference by NMOSD in daily activities, and depression were correlated with loss of income and/or work hours.
Although rare, cases of neuromyelitis optica spectrum disorder are on the rise and the condition is affecting some races more than others.
Nearly two-thirds of patients with neuromyelitis optica spectrum disorder experience delays in aquaporin 4 antibody testing and diagnosis, which could negatively impact early treatment initiation to prevent neurologic disability.
In a new study, 1 in 3 patients with neuromyelitis optica spectrum disorder (NMOSD) had evidence of cognitive dysfunction. Further investigation, however, is warranted to better understand which cognitive domains are most affected by NMOSD.
Investigators revealed a significant association between vitamin D deficiency and increased risk of neuromyelitis optica spectrum disorder, highlighting the possible impact of vitamin D on NMOSD pathogenesis.
A large data analysis found a significantly heightened relative risk of optic neuritis in NMOSD, MOGAD, and other inflammatory diseases, supporting prompt evaluation of visual complaints in these patients.
A comparison between high sensitivity cell-based assays and a rapid immunodot assay for detecting AQP4-IgG in NMOSD patients showed promising results for the rapid test.
Medication switching for medical, nonmedical, or tolerability reasons is common among patients with neuromyelitis optica spectrum disorder (NMOSD). Timing is everything, though, and delays may contribute to disease progression.
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