A case of classic neuromyelitis optica (Devic’s syndrome) triggered by pegylated-interferon α
© Mangioni et al.; licensee BioMed Central Ltd. 2014
Received: 14 August 2014
Accepted: 24 September 2014
Published: 30 September 2014
Despite recent development of direct acting antivirals for treatment of hepatitis C, the current standard of care may still include pegylated-interferon, which is associated with frequent and, at times, serious adverse events.
Here we report for the first time on a severe case of classic neuromyelitis optica (i.e., optic-spinal form) in a 32 year-old Egyptian man with chronic hepatitis C treated with pegylated-interferon α2a for 4 months.
Treating physicians must be alerted on rare but important unexpected complications of interferon, in order to consider carefully its use especially when they deal with patients not in dire need of urgent treatment.
KeywordsAdverse events HCV Neuromyelitis optica Pegylated-interferonα
Hepatitis C virus (HCV) chronically infects approximately 160 million people worldwide and ranks among the leading causes of liver disease. Even with recent licensing of first-generation protease inhibitors, pegylated-interferon α (Peg-IFNα) plus ribavirin remains the cornerstone of HCV treatment, especially in genotype 1 and 4 HCV infection. Adverse events, including a number of neurological and autoimmune conditions, may be treatment-limiting and, rarely, life-threatening[2, 3].
On July 2013, after 4 months of physiotherapy and steroid treatment, the patient showed full recovery of sphincter control and had recommenced walking with assistance. At that time he still suffered from a deficit in proprioceptive sensibility, as well as pronounced fatigability during movement.
Our examinations ruled out viral myelitis, neurological complications of systemic diseases such as systemic lupus erythematous (SLE), Sjogren’s syndrome and sarcoidosis and atypical presentation of multiple sclerosis (MS). The radiological findings on spinal cord MRI and, later, evidence of AQP4-Ab confirmed the diagnosis of NMO. Devic’s disease is an idiopathic autoimmune syndrome of the CNS characterized by severe attacks of optic neuritis and myelitis. Although the NMO phenotype can occur in contexts of MS or several systemic autoimmune diseases, it is considered a distinct syndrome, especially since the identification of the disease-specific antibody anti-aquaporin 4 (AQP4-Ab, or NMO-Ig) in more than 70% of patients with Devic’s disease. AQP4-Ab is thought to play a critical role in producing the lesions by triggering an immune attack against the aquaporine channel type 4, which is the predominant water channel in the CNS and particularly expressed in the optic nerves and spinal cord. Thus, to our knowledge this is the first description of a severe case of classic NMO (i.e., optic-spinal form) triggered by Peg-IFNα.
We can infer that a prolonged exposure of the patient’s immune system to the immunomodulatory activity of Peg-IFNα could have broken the immune tolerance and started an autoimmune reaction, eventually leading to the clinical manifestations of the neuromyelitis optica. In support of our inference, it has been observed that patients with autoimmune and inflammatory diseases (mainly SLE, but also reumatoid artrithis and MS) possess endogenous IFNα inducers (immune complexes that activate natural IFNα producing cells), which cause continuous IFNα production and subsequent chronic stimulation of the immune system[6, 7].
Worldwide, Peg-IFNα will likely remain a predominant treatment for chronic hepatitis C over the next few years, even though its side effects are sometimes unpredictable and severe. We report this case in order to alert treating physicians about rare but important unexpected complications of interferon. Those kind of life-threatening or long term disabling side effects should be of concern, making it advisable to achieve balance between benefits and risks of IFNα-based regimen, especially in patients not in urgent need of starting therapy, in the hopes that quick development of (and broader access to) new tolerable directing-acting antivirals will make IFNα-free therapy possible in a near future.
Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.
Hepatitis C virus
Magnetic resonance imaging
Central nervous system
Antibody anti-aquaporin 4
Systemic lupus erythematous
We are grateful to Anlaids Sezione Lombarda for their continuous support. Thanks to all the staff of the Division of Infectious Diseases, ward and outpatient clinic.
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