Contributions
Abstract: EP1289
Type: ePoster
Abstract Category: Clinical aspects of MS - 1 Diagnosis and differential diagnosis
Introduction: Over the past years, the use of anti-tumor necrosis factor- α (TNF- α) agents and IL-6 blockade by tocilizumab for the management of Neuro-Behçet disease (NBD) has increased progressively, but still remains off-label. However, some patients remain refractory and warrant a different approach to treatment.
Objective: To describe a case of NBD refractory to traditional immunosuppressive agents with secondary failure of anti-TNF-α and anti-IL-6 therapies.
Methods and results: We present the case of a 32-year-old male patient with a history of recurrent oral ulcerations, bilateral anterior uveitis, papulo-pustular skin lesions, erythema nodosum on both legs. He was diagnosed with NDB after presenting T2/FLAIR hyperintense white matter lesions in the midbrain and hypothalamus showing Gadolinium-enhancement, responsible for cerebellar syndrome, diplopia and hypersomnia. The International Criteria for BD were fulfilled and azathioprine (AZT) 150mg/day combined with oral steroids started. Following 2 relapses at 12 and 18 months respectively after starting AZT, monthly pulses of intravenous cyclophosphamide (1000 mg) were started but the patient relapsed again 8 months later. Given the aggressive disease course, anti-TNFα therapy with infliximab (INFX) was started (5 mg/kg every 8 weeks), in combination with 100 mg of AZT and oral steroids. The patient remained relapse-free both clinically and radiologically until he presented a severe spinal cord relapse with paraplegia and paresis of upper limbs 26 months after starting INFX. Tocilizumab infusions were started (8 mg/kg/month) but did not lead to remission as 2 subsequent relapses were observed within 3 months. The patient was started on another anti-TNF-α agent (golimumab). He remains free of disease activity 4 months later.
Conclusion: NBD is a devastating CNS inflammatory disease for which there is an unmet need for effective therapies in patients failing several lines of immunosuppressive treatment to avoid unfavourable outcome. Randomized controlled trials or pragmatic trials are needed to determine the best treatment algorithm.
Disclosure:
Frédéric London received travel grants from Biogen, Merck and Sanofi.
Vincent van Pesch received travel grants from Biogen, Bayer Schering, Genzyme, Merck, Teva Sanofi and Roche. His institution receives honoraria for consultancy and lectures from Biogen, Bayer Schering, Sanofi, Merck, Roche, Teva and Novartis Pharma as well as research grants from Novartis Pharma, Bayer Schering, Sanofi and Roche.
Abstract: EP1289
Type: ePoster
Abstract Category: Clinical aspects of MS - 1 Diagnosis and differential diagnosis
Introduction: Over the past years, the use of anti-tumor necrosis factor- α (TNF- α) agents and IL-6 blockade by tocilizumab for the management of Neuro-Behçet disease (NBD) has increased progressively, but still remains off-label. However, some patients remain refractory and warrant a different approach to treatment.
Objective: To describe a case of NBD refractory to traditional immunosuppressive agents with secondary failure of anti-TNF-α and anti-IL-6 therapies.
Methods and results: We present the case of a 32-year-old male patient with a history of recurrent oral ulcerations, bilateral anterior uveitis, papulo-pustular skin lesions, erythema nodosum on both legs. He was diagnosed with NDB after presenting T2/FLAIR hyperintense white matter lesions in the midbrain and hypothalamus showing Gadolinium-enhancement, responsible for cerebellar syndrome, diplopia and hypersomnia. The International Criteria for BD were fulfilled and azathioprine (AZT) 150mg/day combined with oral steroids started. Following 2 relapses at 12 and 18 months respectively after starting AZT, monthly pulses of intravenous cyclophosphamide (1000 mg) were started but the patient relapsed again 8 months later. Given the aggressive disease course, anti-TNFα therapy with infliximab (INFX) was started (5 mg/kg every 8 weeks), in combination with 100 mg of AZT and oral steroids. The patient remained relapse-free both clinically and radiologically until he presented a severe spinal cord relapse with paraplegia and paresis of upper limbs 26 months after starting INFX. Tocilizumab infusions were started (8 mg/kg/month) but did not lead to remission as 2 subsequent relapses were observed within 3 months. The patient was started on another anti-TNF-α agent (golimumab). He remains free of disease activity 4 months later.
Conclusion: NBD is a devastating CNS inflammatory disease for which there is an unmet need for effective therapies in patients failing several lines of immunosuppressive treatment to avoid unfavourable outcome. Randomized controlled trials or pragmatic trials are needed to determine the best treatment algorithm.
Disclosure:
Frédéric London received travel grants from Biogen, Merck and Sanofi.
Vincent van Pesch received travel grants from Biogen, Bayer Schering, Genzyme, Merck, Teva Sanofi and Roche. His institution receives honoraria for consultancy and lectures from Biogen, Bayer Schering, Sanofi, Merck, Roche, Teva and Novartis Pharma as well as research grants from Novartis Pharma, Bayer Schering, Sanofi and Roche.