OP
Ophthalmology

Giant cell arteritis

December 07, 2011.
Paul A. Monach, MD, PhD,

Educational Objectives

The goal of this program is to improve the diagnosis and treatment of retinal disorders and visual complications of giant cell arteritis. After hearing and assimilating this program, the clinician will be better able to:

1.Diagnose giant cell arteritis on the basis of signs and symptoms, erythrocyte sedimentation rate, levels of C-reactive protein, and biopsy of the temporal artery.

4. Choose appropriate regimens of prednisone to treat giant cell arteritis.

Summary

Symptoms: headache and scalp tenderness occur in 60% to 80%, nonspecific constitutional symptoms, jaw claudication in »50%, polymyalgia rheumatica (eg, in shoulder, hip girdle, and posterior neck), visual loss (permanent in 10%-15%), diplopia, and large vessel involvement (possibly with dizziness or limb claudication)

Signs: scalp tenderness (most common); temporal artery (TA) abnormality (although normal findings do not rule out GCA); loss of pulse or presence of bruit in subclavian and carotid artery; anterior ischemic optic neuropathy (ION; main lesion, occurs in »90%), posterior ION, and central retinal artery occlusions; possible for any branch of extradural carotid artery to be involved (eg, temporal, occipital, and middle meningeal arteries)

Management decisions (treatment and biopsy): normal erythrocyte sedimentation rate (ESR) and levels of C-reactive protein (CRP) in patients with few signs or symptoms suggest biopsy of TA and empiric treatment not needed; high ESR and CRP indicate need for biopsy of TA and early use of empiric prednisone; normal ESR and CRP and negative biopsy make GCA unlikely; if intermediate probability of disease exists, use of biopsy and treatment less clear; biopsy »85% sensitive but only 15% to 20% of all biopsies have positive results; if likelihood of disease high, administer empiric therapy and obtain biopsy

Controversies about biopsy: appropriate size (small to 2 cm) and number of sections needed; unilateral vs bilateral (chance of positive biopsy increases 1% to 10% with bilateral); timing (chance of positive results decreases after 1 or 2 wk of treatment)

Features of temporal arteritis: presence of giant cells not required; inflammation in media of vessel with intimal proliferation indicates temporal arteritis; patients with necrotizing arteritis in small vessels (vasa vasorum) feeding artery may have polyarteritis nodosa (rare); reduplication of internal elastic lamina characteristic; use caution in interpretation of “healed arteritis” as positive result of biopsy

Imaging: magnetic resonance imaging (MRI) sequences with large magnets under development; ultrasonography (used in Europe but not in United States) depends on skill of operator; imaging of large vessels by magnetic resonance angiography (MRA), computed tomography angiography (CTA), or conventional angiography often diagnostic, removing need for biopsy

Treatment: glucocorticoids (prednisone) — remain mainstay, although dosing not established; European League Against Rheumatism (EULAR) recommends 60 mg for 1 mo; protocols in practice vary; taper every 2 to 4 wk for 3 mo, then slowly from 10 to 0 mg; pulsed intravenous steroids used in patients with acute visual loss (data supporting efficacy weak, but toxicity low); aspirin — included in recommendations; based on finding that patients on aspirin for other indications at time of development of GCA have lower rates of blindness and stroke; unclear whether needed after treatment with steroids started; methotrexate — meta-analysis suggests modest steroid-sparing effect, but efficacy controversial; speaker uses for patients requiring high doses of prednisone or who have extreme steroid toxicity even on low doses, and patients with large-vessel disease

Other treatments: mainly as steroid-sparing agents; anti-tumor necrosis factor (TNF) drugs, eg, infliximab (Remicade), not effective; abatacept — blocks costimulation of T cells; used for rheumatoid arthritis; trial under way for patients with new or recurrent GCA that requires ³40 mg prednisone; acts too slowly to reduce need for high initial dose of steroid but may show steroid-sparing effect later; bisphosphonates — (eg, alendronate) used frequently; proton pump inhibitors to prevent peptic ulcer disease possibly unnecessary for patient receiving steroids alone, but steroids and aspirin synergistic; prophylaxis for Pneumocystis — Pneumocystis carinii pneumonia (PCP) seen in 7 patients with GCA at Mayo Clinic over many years; combination of methotrexate and steroids in patients with granulomatosis with polyangiitis (Wegener granulomatosis) carries high risk for PCP; prescribe folic acid for patients taking methotrexate

Follow-up: check frequently for side effects and recurrent disease; most rheumatologists do not increase steroids for asymptomatic rise in ESR and CRP; screen for side effects of steroids and for involvement of large vessels (occurs in 15%-20%); patients have late risk for thoracic aortic aneurysms

Prognosis: many patients experience relapse (usually minor); treat by doubling or tripling dose of steroid and resuming taper; average duration of treatment »2 yr; long-term mortality not increased; morbidity higher from toxicity of steroids than from GCA; visual loss rare after first 2 wk of treatment; most cases of progression of eye disease occur in first week of treatment; use of high-dose intravenous pulsed steroids reasonable, although supporting data not strong

Acknowledgements

Drs. Baumal, Ceron, and Monach spoke at 6th Annual Physician Education Conference “2011 Summit on Posterior Segment Disorders”, held September 10, 2011, in Cambridge, MA, and presented by the Ocular Immunology and Uveitis Foundation (to learn more about CME activities at the Ocular Immunology and Uveitis Foundation, visit www.uveitis.org). The Audio-Digest Foundation thanks the speakers and the Ocular Immunology and Uveitis Foundation for their cooperation in the production of this program.

Suggested Reading

Abbate M et al: Prevention and treatment of diabetic retinopathy: evidence from clinical trials and perspectives. Curr Diabetes Rev 7:190, 2011; Augustin AJ: Upcoming therapeutic advances in diabetic macular edema: an intravitreal dexamethasone drug delivery system. Expert Opin Drug Deliv 8:271, 2011; Campochiaro PA et al: Long-term benefit of sustained-delivery fluocinolone acetonide vitreous inserts for diabetic macular edema. Ophthalmology 118:626, 2011; Elman MJ et al: Expanded 2-year follow-up of ranibizumab plus prompt or deferred laser or triamcinolone for diabetic macular edema. Ophthalmology 118:609, 2011; Gilles MC et al: Intravitreal triamcinolone prior to laser treatment of diabetic macular edema: 24-month results of a randomized controlled trial. Ophthalmology 118:866, 2011; Goslin BJ, Chung MH: Temporal artery biopsy as a means of diagnosing giant cell arteritis: is there over-utilization? Am Surg 77:1158, 2011; Hassan N et al: Giant cell arteritis. BMJ 342:d3019, 2011; Klingel R: RheoNet registry analysis of rheopheresis for microcirculatory disorders with a focus on age-related macular degeneration. Ther Apher Dial 14:276, 2010; Kuno N, Fujii S: Dry age-related macular degeneration: recent progress of therapeutic approaches. Curr Mol Pharmacol May 6, 2011 [Epub ahead of print]; Landa G et al: Qualitative spectral OCT/SLO analysis of drusen change in dry age-related macular degeneration patients treated with Copaxone. J Ocul Pharmacol Ther 27:77, 2011; Lugo JZ et al: Demographic and laboratory data may predict positive temporal artery biopsy. J Surg Res 170:332, 2011; Meisner RJ et al: How to diagnose giant cell arteritis. Int Angiol 30:58, 2011; Montero JA et al Intravitreal anti-VEGF drugs as adjuvant therapy in diabetic retinopathy surgery. Curr Diabetes Rev 7:176, 2011; Olson JH et al: Nutritional supplementation and age-related macular degeneration. Semin Ophthalmol 26:131, 2011; Pearson PA et al: Fluocinolone acetonide intravitreal implant for diabetic macular edema: a 3-year multicenter, randomized, controlled clinical trial. Ophthalmology 118:1580, 2011; Villa-Forte A: Giant cell arteritis: suspect it, treat it promptly. Cleve Clin J Med 78:265, 2011; Witkin AJ, Brown GC: Update on nonsurgical therapy for diabetic macular edema. Curr Opin Ophthalmol 22:185, 2011; Yehoshua Z et al: Current clinical trials in dry AMD and the definition of appropriate clinical outcome measures. Semin Ophthalmol 26:167, 2011; Zhang K et al: Ciliary neurotrophic factor delivered by encapsulated cell intraocular implants for treatment of geographic atrophy in age-related macular degeneration. Proc Natl Acad Sci U S A 108:6241, 2011.

Readings

Disclosures

In adherence to ACCME Standards for Commercial Support, Audio-Digest requires all faculty and members of the planning committee to disclose relevant financial relationships within the past 12 months that might create any personal conflicts of interest. Any identified conflicts were resolved to ensure that this educational activity promotes quality in health care and not a proprietary business or commercial interest. For this program, the following has been disclosed: Dr. Monach has received research/grant support (in the form of drug) from Bristol-Myers Squibb. The planning committee reported nothing to disclose. In this lecture, Dr. Monach presents information related to off-label or investigational use of a product, therapy, or device.

Acknowledgements

Dr. Monach spoke at 6th Annual Physician Education Conference “2011 Summit on Posterior Segment Disorders”, held September 10, 2011, in Cambridge, MA, and presented by the Ocular Immunology and Uveitis Foundation (to learn more about CME activities at the Ocular Immunology and Uveitis Foundation, visit www.uveitis.org). The Audio-Digest Foundation thanks Dr. Monarch and the Ocular Immunology and Uveitis Foundation for their cooperation in the production of this program.

CME/CE INFO

Accreditation:

The Audio- Digest Foundation is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.

The Audio- Digest Foundation designates this enduring material for a maximum of 0 AMA PRA Category 1 Credits™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Audio Digest Foundation is accredited as a provider of continuing nursing education by the American Nurses Credentialing Center's (ANCC's) Commission on Accreditation. Audio Digest Foundation designates this activity for 0 CE contact hours.

Lecture ID:

OP492303

Expiration:

This CME course qualifies for AMA PRA Category 1 Credits™ for 3 years from the date of publication.

Instructions:

To earn CME/CE credit for this course, you must complete all the following components in the order recommended: (1) Review introductory course content, including Educational Objectives and Faculty/Planner Disclosures; (2) Listen to the audio program and review accompanying learning materials; (3) Complete posttest (only after completing Step 2) and earn a passing score of at least 80%. Taking the course Pretest and completing the Evaluation Survey are strongly recommended (but not mandatory) components of completing this CME/CE course.

Estimated time to complete this CME/CE course:

Approximately 2x the length of the recorded lecture to account for time spent studying accompanying learning materials and completing tests.

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