Vasculitis: Inflammatory Destruction of Blood Vessels

Vasculitides and Their Ocular Manifestations

• Giant Cell Arteritis (GCA) is the most common vasculitis, affecting large and medium vessels, with a prevalence of 1/10k people over 50, and it primarily affects individuals over 50, with a higher incidence in women.
• GCA presents with symptoms such as headache, jaw claudication, and polymyalgia rheumatica, and it can lead to irreversible blindness, MI, and stroke, with a 35% 5-year survival rate if untreated.
• Ocular manifestations of GCA include ischemic optic neuropathy (ION), cotton wool spots, and retinal artery occlusions, with up to 92% of patients experiencing some form of vision loss.
• Polyarteritis Nodosa (PAN) predominantly affects small and medium arteries, with complications such as peripheral neuropathy, renal failure, and gastrointestinal infarcts, and it is fatal if left untreated.
• Ocular complications of PAN include scleritis, episcleritis, and retinal vasculitis, with a prevalence of 10-20%.
• Granulomatosis with Polyangiitis (GPA) primarily involves small and medium arteries and can lead to chronic rhinitis, lung, and kidney involvement, with a 80-90% survival rate with treatment.
• Ocular manifestations of GPA include orbital pseudotumor, ulcers, and reduced visual acuity, with a prevalence of 50-60%.
• Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disorder that primarily affects skin and joints, with ocular manifestations such as dry eye syndrome, retinopathy, and episcleritis, found in 33% of cases.
• Behcet's Disease/Syndrome, with a prevalence of <1%, primarily affects individuals of Middle Eastern, Turkish, and Asian descent, and it presents with complications such as mouth and genital sores, CNS involvement, and GI ulcerations.
• Ocular manifestations of Behcet's Disease/Syndrome include retinal phlebitis, uveitis, and optic neuritis, with a preference for veins, and it is diagnosed using the Behcetine pathergy test.
• Giant Cell Arteritis (GCA) presents with specific ocular manifestations such as cotton wool spots, amaurosis fugax, and diplopia, and it is diagnosed using duplex sonography of the temporal artery and temporal artery biopsy.
• Polyarteritis Nodosa (PAN) is diagnosed using angiography and tissue biopsy, and it is treated with immunosuppressants, while Behcet's Disease/Syndrome is managed with immunosuppressants due to its autoimmune etiology.