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| Disease found: | Giant Cell Arteritis |
| Current as of: | October 8, 2024 |
| Disease Overview: | Granulomatous inflammatory vasculitis of medium-to-large arteries with a preference for the temporal artery and other cranial arteries; cause is unknown but theroized to be autoimmune [more info] |
| Signs and Symptoms: | Classically presents >50 years old with new-onset headache, tenderness over the temporal artery, jaw claudication, and artery prominence. May have amaurosis fugax and diplopia. Commonly causes fever and polymyalgia rheumatica in the neck, shoulders, and hips. [more info] |
| Diagnosis: | No one specific test; the American College of Rheumatology recommends at least 6 "points" from the following signs: positive temporal artery biopsy or temporal artery halo sign on ultrasound (+5); ESR ≥50 mm/hour or CRP ≥10 mg/liter (+3); sudden visual loss (+3); morning stiffness, jaw or tongue claudication, new temporal headache, scalp tenderness, temporal artery abnormality, bilateral axillary involvement on imaging, and aortic FDG-PET activity (+2 each). [more info] |
| Treatment: | Immediate corticosteroid therapy is required before any confirmatory testing upon suspicion of disease, to minimize risk of permanent vision loss. IV methylprednisolone is warranted for patients with existing visual/cranial symptoms before tapering on oral therapy. [more info] |
| Clinical Management: | Many patients require ongoing maintenance therapy to prevent relapses/flares. Consider bisphosphonates if prolonged steroid therapy is needed. DMARDs (specifically methotrexate, tocilizumab) can also be used instead of steroids for maintenance. [more info] |
| Referral: | Patients should be referred to rheumatology. The Vasculitis Foundation maintains a database of nationwide and international rheumatology clinics.Referral to Medical Genetics Department, if available. Initial virtual care is also available through organizations like TeleRare Health. |
| Clinical Trials: | Numerous clinical trials are currently recruiting as well as pending activation. |