Non-infectious Orbital Vasculitides
Orbital vasculitis is an entity that primarily presents with signs and symptoms of OIS. Most commonly, patients present with exophthalmos, limitation of extraocular movements, conjunctival injection, and/or decreased vision. It can also present with infarction of orbital structures. There are a number of different non-infectious systemic diseases that can be associated with orbital involvement. Although the association between a systemic autoimmune vasculitis and orbital inflammation can be rare, over the past 15 years there has been growing evidence of a relationship between the two.
The evaluation and treatment of non-infectious orbital vasculitides are often complicated and require a thorough understanding of the disease and underlying systemic associations. The long-term prognosis visually and systemically must be weighed against the risks and benefits of the treatment regimen.
A large variety of corticosteroid formulations currently exist and are the mainstay of initial treatment. Traditional steroid-sparing immunosuppressive agents are also an important arsenal against these vasculitides.
Recently, a new class of drugs called biologics, which target the various mediators of the inflammation cascade, may potentially provide more effective and less toxic treatment. The results of biologics for the treatment of systemic rheumatic diseases have led to its off-label use in the treatment of ocular and orbital inflammation. Biologics in general, offer a safer profile compared with conventional immunosuppressive therapies, but clinical experience has demonstrated that they can have unexpected effects, including the risk of tuberculosis activation, invasive fungal, and other opportunistic infections and increase risk of malignancies.
When evaluating a patient with orbital signs, it is important to consider an autoimmune vasculitis as a cause for orbital inflammation or infarction in order to determine the appropriate therapy and potentially diagnose a systemic illness.
Summary and Conclusions
Orbital vasculitis is an entity that primarily presents with signs and symptoms of OIS. Most commonly, patients present with exophthalmos, limitation of extraocular movements, conjunctival injection, and/or decreased vision. It can also present with infarction of orbital structures. There are a number of different non-infectious systemic diseases that can be associated with orbital involvement. Although the association between a systemic autoimmune vasculitis and orbital inflammation can be rare, over the past 15 years there has been growing evidence of a relationship between the two.
The evaluation and treatment of non-infectious orbital vasculitides are often complicated and require a thorough understanding of the disease and underlying systemic associations. The long-term prognosis visually and systemically must be weighed against the risks and benefits of the treatment regimen.
A large variety of corticosteroid formulations currently exist and are the mainstay of initial treatment. Traditional steroid-sparing immunosuppressive agents are also an important arsenal against these vasculitides.
Recently, a new class of drugs called biologics, which target the various mediators of the inflammation cascade, may potentially provide more effective and less toxic treatment. The results of biologics for the treatment of systemic rheumatic diseases have led to its off-label use in the treatment of ocular and orbital inflammation. Biologics in general, offer a safer profile compared with conventional immunosuppressive therapies, but clinical experience has demonstrated that they can have unexpected effects, including the risk of tuberculosis activation, invasive fungal, and other opportunistic infections and increase risk of malignancies.
When evaluating a patient with orbital signs, it is important to consider an autoimmune vasculitis as a cause for orbital inflammation or infarction in order to determine the appropriate therapy and potentially diagnose a systemic illness.
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