Contributions
Abstract: EP1646
Type: ePoster
Abstract Category: Therapy - disease modifying - 26 Immunomodulation/Immunosuppression
Objectives: We describe the case of a 44-year-old female who developed a previously unreported, corticosteroid-responsive, autoimmune, non-necrotising myositis after receiving alemtuzumab for relapsing remitting multiple sclerosis (RRMS).
Case report: A 44-year-old female with a 13-year history of RRMS was treated with alemtuzumab without any immediate complications in November of 2015. Her baseline EDSS at the time of treatment was 2.0, with MRI showing widespread demyelination affecting the deep white matter of the brain, optic nerves and spinal cord. Seven months later she developed myalgia affecting the arms and legs in a proximal distribution. Clinical assessment and MRI excluded progression of multiple sclerosis (MS). Blood tests showed a grossly elevated creatine-phosphokinase level (CK) of 11015 U/L (< 211 U/L). Hepatic transaminase levels were also elevated at 322 U/L and 129 U/L (< 30 U/L) for AST and ALT respectively.
Electromyography demonstrated myopathic units in keeping with a mild generalized non-necrotizing process. A muscle biopsy was obtained from the left quadriceps. Occasional lymphocytes were seen but there was neither necrosis nor a significant inflammatory infiltrate. Rare fibers were positive for C5b9, the terminal component of complement.
Corticosteroid therapy was started and weaned to 5mg over a 3-month period. The patient's symptoms resolved completely and correlated with decreases in serum CK levels.
Discussion and conclusion: Secondary autoimmunity affecting the thyroid, platelets and rarely the kidneys is well described following immune reconstitution after alemtuzumab. Each of these complications involves antibody-dependent immunity. This case of myositis also appears to be autoimmune and involves humoral immunity. A cellular infiltrate could not be shown on muscle biopsy, but complement activation was present and steroids were highly effective. In the absence of histopathological evidence of T-cells, macrophages or necrosis, we propose this form of myositis may be a unique entity and term the condition, pauci-immune myositis. This case highlights the importance of reporting novel adverse events with emerging immune therapies for MS. Further research is required to understand the mechanisms underlying secondary autoimmunity after alemtuzumab treatment.
Disclosure:
P. Aouad has received travel funding from Biogen, Sanofi Genzyme and Teva
C. Yiannikas is on the advisory board for Biogen Idec-BG12; has received travel funding from Novartis and Biogen; and is a consultant for Allergan
J. Parratt has received honoraria and research grants from Biogen, Novartis, Bayer, Sanofi Genzyme and Merck Serono
Abstract: EP1646
Type: ePoster
Abstract Category: Therapy - disease modifying - 26 Immunomodulation/Immunosuppression
Objectives: We describe the case of a 44-year-old female who developed a previously unreported, corticosteroid-responsive, autoimmune, non-necrotising myositis after receiving alemtuzumab for relapsing remitting multiple sclerosis (RRMS).
Case report: A 44-year-old female with a 13-year history of RRMS was treated with alemtuzumab without any immediate complications in November of 2015. Her baseline EDSS at the time of treatment was 2.0, with MRI showing widespread demyelination affecting the deep white matter of the brain, optic nerves and spinal cord. Seven months later she developed myalgia affecting the arms and legs in a proximal distribution. Clinical assessment and MRI excluded progression of multiple sclerosis (MS). Blood tests showed a grossly elevated creatine-phosphokinase level (CK) of 11015 U/L (< 211 U/L). Hepatic transaminase levels were also elevated at 322 U/L and 129 U/L (< 30 U/L) for AST and ALT respectively.
Electromyography demonstrated myopathic units in keeping with a mild generalized non-necrotizing process. A muscle biopsy was obtained from the left quadriceps. Occasional lymphocytes were seen but there was neither necrosis nor a significant inflammatory infiltrate. Rare fibers were positive for C5b9, the terminal component of complement.
Corticosteroid therapy was started and weaned to 5mg over a 3-month period. The patient's symptoms resolved completely and correlated with decreases in serum CK levels.
Discussion and conclusion: Secondary autoimmunity affecting the thyroid, platelets and rarely the kidneys is well described following immune reconstitution after alemtuzumab. Each of these complications involves antibody-dependent immunity. This case of myositis also appears to be autoimmune and involves humoral immunity. A cellular infiltrate could not be shown on muscle biopsy, but complement activation was present and steroids were highly effective. In the absence of histopathological evidence of T-cells, macrophages or necrosis, we propose this form of myositis may be a unique entity and term the condition, pauci-immune myositis. This case highlights the importance of reporting novel adverse events with emerging immune therapies for MS. Further research is required to understand the mechanisms underlying secondary autoimmunity after alemtuzumab treatment.
Disclosure:
P. Aouad has received travel funding from Biogen, Sanofi Genzyme and Teva
C. Yiannikas is on the advisory board for Biogen Idec-BG12; has received travel funding from Novartis and Biogen; and is a consultant for Allergan
J. Parratt has received honoraria and research grants from Biogen, Novartis, Bayer, Sanofi Genzyme and Merck Serono