Sections View Full Chapter Figures Tables Videos Full Chapter Figures Tables Videos Supplementary Content + Print Section ++ For further information, see CMDT Part 20-18: Polymyalgia Rheumatica & Giant Cell Arteritis + Key Features Print Section ++ ++ Essentials of Diagnosis ++ Age over 50 years Elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CPR) Polymyalgia rheumatica: pain and stiffness in shoulders and hips Giant cell arteritis: headache, jaw claudication, polymyalgia rheumatica, visual abnormalities ++ General Considerations ++ Polymyalgia rheumatica and giant cell arteritis probably represent a spectrum of one disease and frequently coexist The important difference between the two conditions is that polymyalgia rheumatica alone does not cause blindness and responds to low-dose (10–20 mg/day) prednisone therapy, whereas giant cell arteritis can cause blindness and large artery complications and requires high-dose therapy (40–60 mg/day) ++ Demographics ++ Both affect patients over age 50 Giant cell arteritis is more common in northern Europeans and their descendants Incidence of the disease increases with each decade of life + Clinical Findings Print Section ++ ++ Symptoms and Signs ++ POLYMYALGIA RHEUMATICA ++ Pain and stiffness of the shoulder and pelvic girdle areas Fever, malaise, and weight loss Anemia and a markedly elevated sedimentation rate are almost always present Muscle pain much greater than muscle weakness ++ GIANT CELL ARTERITIS ++ Headache, scalp tenderness, visual symptoms, jaw claudication, or throat pain The temporal artery is usually normal on physical examination but may be nodular, enlarged, tender, or pulseless Blindness Results from occlusive arteritis of the posterior ciliary branch of the ophthalmic artery Ischemic optic neuropathy may produce no funduscopic findings for the first 24–48 hours after the onset of blindness Asymmetry of pulses in the arms, a murmur of aortic regurgitation, or bruits heard near the clavicle resulting from subclavian artery stenoses identify an affected aorta or its major branches Forty percent of patients with giant cell arteritis have nonclassic symptoms at presentation, primarily respiratory tract problems (most frequently dry cough), mononeuritis multiplex (most frequently with painful paralysis of a shoulder), or fever of unknown origin The fever can be as high as 40°C and is frequently associated with rigors and sweats Unexplained head or neck pain in an older patient may signal the presence of giant cell arteritis ++ Differential Diagnosis ++ POLYMYALGIA RHEUMATICA ++ Rheumatoid arthritis Polymyositis Chronic infection, eg, endocarditis Plasma cell myeloma Malignancy Fibromyalgia Polyarteritis nodosa ++ GIANT CELL (TEMPORAL) ARTERITIS ++ Migraine Glaucoma Takayasu arteritis Uveitis Carotid plaque with embolic amaurosis fugax Trigeminal neuralgia + Diagnosis Print Section ++ ++ Laboratory Tests ++ An elevated ESR, with a median result of about 65 mm/h, occurs in more than 90% of patients with polymyalgia rheumatica or giant cell arteritis Most patients also have a mild normochromic, normocytic anemia and thrombocytosis ++ Imaging Studies ++ Ultrasonography can detect abnormalities in inflamed temporal arteries but its results are highly operator dependent Magnetic resonance angiography or CT angiography establishes the diagnosis by demonstrating long stretches of narrowing of the subclavian and axillary arteries Imaging of the temporal artery with ultrasound, MRI, or CT angiography can sometimes obviate the need for biopsy ++ Diagnostic Procedures ++ Temporal artery biopsy Diagnostic findings of giant cell arteritis may still be present 2 weeks (or even considerably longer) after starting corticosteroids An adequate biopsy specimen (2 cm in length) is essential, because the disease may be segmental + Treatment Print Section ++ ++ Medications ++ POLYMYALGIA RHEUMATICA ++ Prednisone, 10–20 mg/day orally; if no dramatic improvement within 72 hours, the diagnosis should be revisited Weekly methotrexate may increase the chance of successfully tapering prednisone in some patients ++ GIANT CELL ARTERITIS ++ The urgency of early diagnosis and treatment in giant cell arteritis relates to the prevention of blindness When a patient has symptoms and findings suggestive of temporal arteritis, therapy with prednisone, 60 mg daily orally, is initiated immediately Prednisone should be continued in a dosage of 60 mg/day for 1–2 months before tapering Intravenous pulse methylprednisolone (eg, 1 g/day for 3 days) may help patients with visual loss and may increase chance of remission; however, data supporting this recommendation are preliminary Low-dose aspirin (∼81 mg/day orally) may reduce the risk of visual loss or stroke and should be added to prednisone Tocilizumab, an anti-interleukin-6 receptor monoclonal antibody Phase 2 clinical trials have shown that patients initially treated with tocilizumab and prednisone were able to be tapered off prednisone faster than those who were treated with prednisone alone After 1 year of treatment, tocilizumab achieves corticosteroid-free remission in approximately 50% of patients + Outcome Print Section ++ ++ Follow-Up ++ Course is monitored by a composite of the patient's symptoms and laboratory markers of inflammation (ie, hematocrit, ESR, and C-reactive protein) In adjusting the dosage of corticosteroid, the ESR is a useful but not absolute guide to disease activity. A common error is treating the ESR rather than the patient Within 1–2 months after beginning treatment, the patient's symptoms and laboratory abnormalities will resolve Disease flares are common (50% or more) as prednisone is tapered The total duration of treatment varies considerably but ranges from 6 months to more than 2 years ++ Complications ++ Blindness; once blindness develops, it is usually permanent Thoracic aortic aneurysms occur 17 times more frequently in patients with giant cell arteritis than in normal individuals ++ Prognosis ++ Impact on survival appears small ++ When to Refer ++ Refer to a rheumatologist to establish the diagnosis, plan therapy, and monitor treatment Consult an ophthalmologist for visual changes ++ When to Admit ++ Admit for evaluation and high-dose intravenous methylprednisolone if the patient acutely develops visual loss + References Print Section ++ + +Buttgereit F et al. Polymyalgia rheumatica and giant cell arteritis: a systematic review. JAMA. 2016 Jun 14;315(22):2442–58. [PubMed: 27299619] + +Dejaco C et al. Giant cell arteritis and polymyalgia rheumatica: current challenges and opportunities. Nat Rev Rheumatol. 2017 Oct;13(10):578–92. [PubMed: 28905861] + +Nagel MA et al. Analysis of varicella-zoster virus in temporal arteries biopsy positive and negative for giant cell arteritis. JAMA Neurol. 2015 Nov;72(11):1281–7. [PubMed: 26349037] + +Stone JH et al. Trial of tocilizumab in giant-cell arteritis. N Engl J Med. 2017 Jul 27;377(4):317–28. [PubMed: 28745999] + +Villiger PM et al. Tocilizumab for induction and maintenance of remission in giant cell arteritis: a phase 2, randomised, double-blind, placebo-controlled trial. Lancet. 2016 May 7;387(10031):1921–7. [PubMed: 26952547] + +Watanabe R et al. Immune checkpoint dysfunction in large and medium vessel vasculitis. Am J Physiol Heart Circ Physiol. 2017 May 1;312(5):H1052–9. [PubMed: 28314758]