Author Of 1 Presentation
P0430 - Anti-TNF induced lupus-like disease and progressive multifocal leukoencephalopathy: a unifying underlying mechanism? (ID 1144)
Abstract
Background
Anti-TNF agents have revolutionized the treatment of chronic inflammatory disorders. Nevertheless, several adverse reactions are well recognized including increased rates of infections, lupus-like disease and demyelinating lesions. Progressive multifocal leukoencephalopathy (PML) is a rare but severe demyelinating disease caused by the reactivation and access to the brain of the ubiquitous polyomavirus JC (JCV), which targets oligodendrocytes. It is most commonly associated with immunosuppression, malignancies and systemic lupus erythematosus (SLE).
Objectives
We present the development of both SLE and PML as a result of etanercept treatment for underlying psoriatic arthritis suggesting a need for careful evaluation and close neurological surveillance in patients receiving anti-TNF treatment.
Methods
A brain MRI including MR spectroscopy was performed. To further investigate the lesion, a stereotactic brain biopsy was accomplished, followed by immunohistochemistry.
Results
A 65-year-old female was admitted in the hospital because of a gradual onset of right homonymous hemianopsia. She had a history of psoriatic arthritis treated with etanercept monotherapy for five years. Two years prior to the current admission she developed photosensitivity and antibodies against Ro/SSA and La/SSB. With a presumable diagnosis of anti-TNF induced SLE, etanercept was discontinued and hydroxychloroquine was prescribed. A year later, personality changes were reported and 8 months later, visual disturbances. On current admission, neurological examination revealed a Babinski sign on the right side as well as limb-kinetic apraxia on both sides. MRI showed a T2-hyperintense, non-enhancing, parieto-occipital subcortical lesion of the left hemisphere, spreading through the spleen of the corpus callosum to the right hemisphere. Spectroscopy was compatible with a diagnosis of grade III glioma. Pathology of the brain specimen revealed a demyelinating, macrophage-rich lesion with lysis of Simian-Virus-40 positive glial cells. A diagnosis of PML was made.
Conclusions
In summary, a case of anti-TNF induced lupus which soon developed signs of PML is presented. Induction of type I interferon responses and B cell activation previously shown to be induced by anti-TNF treatment might be involved in mobilization of CD34+ B cell precursors harboring JC virus, a mechanism previously postulated for PML development. Moreover, decreased toll-like receptor signaling and dampened plasmacytoid dendritic cell activation following hydroxychloroquine treatment, could be an alternative explanation. Since the use of immunosuppressive therapy is a known predisposing factor for development of PML, careful evaluation and close neurological surveillance is mandatory.
Presenter Of 1 Presentation
P0430 - Anti-TNF induced lupus-like disease and progressive multifocal leukoencephalopathy: a unifying underlying mechanism? (ID 1144)
Abstract
Background
Anti-TNF agents have revolutionized the treatment of chronic inflammatory disorders. Nevertheless, several adverse reactions are well recognized including increased rates of infections, lupus-like disease and demyelinating lesions. Progressive multifocal leukoencephalopathy (PML) is a rare but severe demyelinating disease caused by the reactivation and access to the brain of the ubiquitous polyomavirus JC (JCV), which targets oligodendrocytes. It is most commonly associated with immunosuppression, malignancies and systemic lupus erythematosus (SLE).
Objectives
We present the development of both SLE and PML as a result of etanercept treatment for underlying psoriatic arthritis suggesting a need for careful evaluation and close neurological surveillance in patients receiving anti-TNF treatment.
Methods
A brain MRI including MR spectroscopy was performed. To further investigate the lesion, a stereotactic brain biopsy was accomplished, followed by immunohistochemistry.
Results
A 65-year-old female was admitted in the hospital because of a gradual onset of right homonymous hemianopsia. She had a history of psoriatic arthritis treated with etanercept monotherapy for five years. Two years prior to the current admission she developed photosensitivity and antibodies against Ro/SSA and La/SSB. With a presumable diagnosis of anti-TNF induced SLE, etanercept was discontinued and hydroxychloroquine was prescribed. A year later, personality changes were reported and 8 months later, visual disturbances. On current admission, neurological examination revealed a Babinski sign on the right side as well as limb-kinetic apraxia on both sides. MRI showed a T2-hyperintense, non-enhancing, parieto-occipital subcortical lesion of the left hemisphere, spreading through the spleen of the corpus callosum to the right hemisphere. Spectroscopy was compatible with a diagnosis of grade III glioma. Pathology of the brain specimen revealed a demyelinating, macrophage-rich lesion with lysis of Simian-Virus-40 positive glial cells. A diagnosis of PML was made.
Conclusions
In summary, a case of anti-TNF induced lupus which soon developed signs of PML is presented. Induction of type I interferon responses and B cell activation previously shown to be induced by anti-TNF treatment might be involved in mobilization of CD34+ B cell precursors harboring JC virus, a mechanism previously postulated for PML development. Moreover, decreased toll-like receptor signaling and dampened plasmacytoid dendritic cell activation following hydroxychloroquine treatment, could be an alternative explanation. Since the use of immunosuppressive therapy is a known predisposing factor for development of PML, careful evaluation and close neurological surveillance is mandatory.