Contributions
Abstract: EP1313
Type: Poster Sessions
Abstract Category: Clinical aspects of MS - MS Variants
Background: Neuromyelitis optica spectrum disease (NMOSD) is an autoimmune disease with severe potentially life threatening relapses and thus needs long term immunsuppressive therapy to prevent relapses and disability accumulation. However multiple immunosuppressive therapies may increase the risk for side effects and limit further therapy escalation.
Methods: To report a single patient with the diagnosis of NMOSD and concomitant systemic lupus erythematodes (SLE) who showed insufficient response to multiple immunosuppressive therapies. All medical data including clinical disease course, therapies and laboratory results e.g. B-cell counts were continuously recorded and collected.
Results: This female patient was diagnosed with SLE in 1994 at the age of 20. In 1994 bilatereral optic neuritis (ON) occurred resulting in blindness of the right eye which was followed by recurrent myelitis since 1995. Initially ON and myelitis were regarded as a manifestation of SLE. Immunosuppressive treatments with azathioprine, cyclophosphamide methotrexate and ciclosporine), could not stabilize the disease course and led to side effects. At first presentation in our outpatient clinic she tested positive for anti-aquaporin-4 antibodies (AQP-4-Ab) and was diagnosed with NMOSD (EDSS = 3.5). Therapy with rituximab (RTX) was started in 7/2015 with 2 x 1g i.v. two weeks apart and led to B-cell depletion. Five weeks after the first RTX infusion she experienced another relapse with myelitis. B-cells returned six months later, however no complete B-cell depletion could be achieved with ongoing RTX therapy and further relapses occurred. RTX therapy was stopped (normal B-cell count) and treatment with tocilizumab (TZ, 8 mg/kg/body weight) i.v. started in 3/2017. After the second TZ dosage recurrent granulocytopenia (grade 2) associated with bladder as well as bronchial infections occurred and subsequent dosages had to be postponed. She experienced two more relapses (EDSS 4.0) and TZ was stopped after the 6th infusion. Currently, further therapeutic options include mycophenolate mofetil, combination therapies, as well as off label therapy with eculizumab. However this must be weighted against the risk of further side effects.
Conclusions: Insufficient response to RTX and severe side effect to subsequent TZ therapy complicated the clinical course in this patient and illustrates possible risks after frequent and various immunosuppressive therapies in NMOSD.
Disclosure: T. Kümpfel has received travel expenses and speaker honoraria from Bayer Healthcare, Teva Pharma, Merck, Novartis Pharma, Sanofi-Aventis/Genzyme, CLB Behring, Roche Pharma and Biogen as well as grant support from Bayer-Schering AG, Novartis and Chugai Pharma.
I. Meinl has received travel expenses from MedDay and Roche Pharma
Abstract: EP1313
Type: Poster Sessions
Abstract Category: Clinical aspects of MS - MS Variants
Background: Neuromyelitis optica spectrum disease (NMOSD) is an autoimmune disease with severe potentially life threatening relapses and thus needs long term immunsuppressive therapy to prevent relapses and disability accumulation. However multiple immunosuppressive therapies may increase the risk for side effects and limit further therapy escalation.
Methods: To report a single patient with the diagnosis of NMOSD and concomitant systemic lupus erythematodes (SLE) who showed insufficient response to multiple immunosuppressive therapies. All medical data including clinical disease course, therapies and laboratory results e.g. B-cell counts were continuously recorded and collected.
Results: This female patient was diagnosed with SLE in 1994 at the age of 20. In 1994 bilatereral optic neuritis (ON) occurred resulting in blindness of the right eye which was followed by recurrent myelitis since 1995. Initially ON and myelitis were regarded as a manifestation of SLE. Immunosuppressive treatments with azathioprine, cyclophosphamide methotrexate and ciclosporine), could not stabilize the disease course and led to side effects. At first presentation in our outpatient clinic she tested positive for anti-aquaporin-4 antibodies (AQP-4-Ab) and was diagnosed with NMOSD (EDSS = 3.5). Therapy with rituximab (RTX) was started in 7/2015 with 2 x 1g i.v. two weeks apart and led to B-cell depletion. Five weeks after the first RTX infusion she experienced another relapse with myelitis. B-cells returned six months later, however no complete B-cell depletion could be achieved with ongoing RTX therapy and further relapses occurred. RTX therapy was stopped (normal B-cell count) and treatment with tocilizumab (TZ, 8 mg/kg/body weight) i.v. started in 3/2017. After the second TZ dosage recurrent granulocytopenia (grade 2) associated with bladder as well as bronchial infections occurred and subsequent dosages had to be postponed. She experienced two more relapses (EDSS 4.0) and TZ was stopped after the 6th infusion. Currently, further therapeutic options include mycophenolate mofetil, combination therapies, as well as off label therapy with eculizumab. However this must be weighted against the risk of further side effects.
Conclusions: Insufficient response to RTX and severe side effect to subsequent TZ therapy complicated the clinical course in this patient and illustrates possible risks after frequent and various immunosuppressive therapies in NMOSD.
Disclosure: T. Kümpfel has received travel expenses and speaker honoraria from Bayer Healthcare, Teva Pharma, Merck, Novartis Pharma, Sanofi-Aventis/Genzyme, CLB Behring, Roche Pharma and Biogen as well as grant support from Bayer-Schering AG, Novartis and Chugai Pharma.
I. Meinl has received travel expenses from MedDay and Roche Pharma