1. Usha Reddy,MD Discussants Byron Lam, MD Sander Dubovy,MD

2. Case Presentation 8/12/06
87yo female with loss of vision OU (OS > OD) since 8/8/06
3 weeks ago vision was 20/40 OU per visit with optometrist

3. Initial Examination 8/12/06
VA cc
OD: 200 “E” at 2 ½ft
OS: 200 “E” at 5 ½ ft
Pupils
OD: 4mm
OS: 4mm +2 APD
IOP
OD: 13
OS: 14
CVF
L marked complete field defect
R superior altitudinal defect
Motility full OU
SLE- WNL
PCIOL OU
DFE see photos

4. Fundus photos

10. Differential Diagnosis
Arteritic anterior ischemic optic neuropathy (AAION)
Non-arteritic anterior ischemic optic neuropathy (NAAION)
Optic neuritis- MS
Infectious- syphilis, TB
Non-infectious- sarcoidosis
Neoplastic/Infiltrative

11. Case Presentation 8/12/06
Pt described vision loss as beginning in L eye and progressing to R eye with sensitivity to light, followed by having difficulty seeing playing card numbers, and then no longer seeing the floor getting off elevator
Community ophthalmologist for check and started on prednisone 40mg with labs done
Told blood value was “eight times normal” and prednisone increased to 60mg QD on 8/10/06

12. Review of Symptoms Frontal and L sided headache for 2 weeks
Jaw claudication
Loss of 30 lbs over last year
No fevers or chills
History of PMR/arthralgias

13. More History PMHx Allergies SH Past Ocular Hx Medications
NKDA SH
Lives in Miami, daughter in NYC
Quit tobacco 15 yrs ago
Denies ETOH, drugs
Retired
PMHx
Polymyalgia Rheumatica
dx 2004 and tx prednisone for few months, off for 1yr
Osteoporosis
Hypercholesterolemia
Past Ocular Hx
s/p cataract surgery OU
Medications
Zocor 20mg po QHS
Cymbalta 60mg po QD
Melatonin 1.5mg QHS
Forteo

14. Labs
ESR 87
CRP 4
WBC 17.5
Hgb 12.7
Hct 38.4
Plt 610

15. Admission IV steroids MRI/MRA Temporal artery biopsy Anticoagulation
Solumedrol 250mg IV Q6hrs
MRI/MRA
Temporal artery biopsy
Anticoagulation

16. Imaging- MRI MRI Brain w/o & w contrast MRA brain Small vessel disease
No abnormal enhancing lesions
MRA brain
Dominant R vertebral artery with L vertebral artery ending in PICA which is normal anatomic variant
Irregular appearance of L internal carotid artery likely secondary to atherosclerosis

17. Clinical Course 8/13/06- Day 2 OD: CF at 1ft/ “E” at 1 ½ ft
OS: CF at 3ft/ “E” at 3 ft
8/14/06- Day 3
OD: CF at 5ft
OS: CF at 4ft
Temporal artery bx done
8/15/06- Day 4
OD: CF at 1ft
OS: Denies light
ESR 53, CRP < 0.10, ANA-
Solumedrol increased to 500mg IV Q6hrs
Anticoagulation

18. Clinical Course 8/16/06- Day 5 VA unchanged 8/17/06- Day 6
OD: CF at 1ft
OS: NLP
Marked pallor of nerves, reduced edema
ESR 32, CRP 0.15
8/18/06- Day 7
OD: CF at 1 ½ ft
Discharged home on prednisone 80mg

31. Histopathology Inflammatory disease of large and medium sized arteries
Typically granulomatous inflammation involving all layers of vessels- intima, media, adventitia
Skip lesions in vessel
Multinucleated giant cells may or may not be seen
Fragmented elastic layer

32. Bilateral Anterior Ischemic Optic Neuropathy
Diagnosis
Bilateral Anterior Ischemic Optic Neuropathy
Giant Cell Arteritis

33. Diagnosis Also known as: Temporal arteritis Cranial arteritis
Granulomatous arteritis
An Ophthalmic Emergency!

34. GCA presentation Headache 56% Anorexia/weight loss 52%
Jaw claudication 48%
Malaise 38%
Myalgia 29%
Fever 26%
Abnormal temporal artery 20%
Scalp tenderness 18%
Neck pain 16%
Anemia 13%
(Hayreh et al 1997)

35. GCA- Ophthalmic presentations
AION
From posterior ciliary involvement
Optic disc edema
+/- splinter hemorrhages
VF inferior altitudinal defect, inferior nasal sectorial defect
Vascular
Amaurosis fugax
Retrobulbar optic neuropathy
CRAO or BRAO
Choroidal ischemia
Neuro-ophthamic
Diplopia from EOM ischemia
Ptosis
Nystagmus
Pupillary change

36. Occult GCA
Only ocular manifestations
No systemic symptoms

37. American College of Rheumatology GCA Diagnosis Guidelines
Criteria for GCA (3 of 5)
Age of onset of > 50 years
New onset headache
Abnormal temporal arteries- tenderness or decreased pulse
ESR of > 50 mm/hr
Positive biopsy results for GCA

38. Proposed Ophthalmology Guidelines by Hayreh et al
Jaw claudication
CRP >2.45 mg/dl
Neck pain
ESR > 47mm/hr
-CRP more sensitive than ESR and a combination of two provides best specificity (97%)

39. GCA Causes Genetic Infectious Autoimmune Link to PMR not understood
Possible spectrum of disease

40. Pathophysiology GCA mediated primarily by cellular immunity
CD4+ T-helper cell response to macrophage-presented antigens leads to inflammation typically beginning in the adventitia and progressing to involve the whole vessel wall

41. Epidemiology of GCA Older the age the greater the prevalence
Women more than men
Caucasians

42. Relevant Tests Labs Imaging Color duplex ultrasonography ESR CRP
Fibrinogen
CBC for Anemia, Thrombocytosis, Leukocytosis
Imaging
CT/MRI
Color duplex ultrasonography

43. ESR interpretation
Miller et al created a formula to calculate normal ESR
Age/2 for males
(Age +10) /2 for females
Hayreh et al suggested normal ESR as <30 mm/hr in men and <35 mm/hr in women, with a sensitivity and specificity of 92%

44. Goal of therapy Prevention of contralateral vision loss
Occurs in 95% of cases if untreated

45. GCA Treatment Steroids Cyclosporine/Azathioprine or MTX
Prednisone dose 1mg/kg/day = daily
IV methylprednisolone of 1g/day for 3-5 days in cases of vision loss
No prospective controlled trials to define whether oral or IV treatment is best
Treatment for 1-2 years with monitoring of ESR, CRP and clinical symptoms
Cyclosporine/Azathioprine or MTX
Anticoagulation for reactive thrombocytosis

46. Complications of steroid therapy
Osteoporosis
Increased susceptibility to infection
Impaired wound healing
Hyperglycemia
Hypertension
Cataracts
Glaucoma
Psychiatric disorders

47. Prognosis
Visual improvement is better with early diagnosis and treatment with steroids, although improvement is rare
Steroid therapy for prevention of further visual loss with stabilization usually after 5 days

48. Patient Follow-up 8/21/06 VA Pupils Optic nerves with marked pallor
OD: CF at 2 ½ft
OS: NLP
Pupils
OS: 3+ APD
Optic nerves with marked pallor
ESR 25, CRP 0.10
Continue Prednisone 80mg PO QD
RTC 2 weeks

49. Summary Giant cell arteritis is an ophthalmic emergency
Important to keep in the differential, particularly in elderly patients
Key diagnostic factors include visual and systemic symptoms as well as labs for ESR and CRP
Temporal artery biopsy is definitive diagnostic test
Early intervention is crucial for preservation of vision
Treatment is with corticosteroids
Therapy course based on clinical symptoms and labs

50. Abstract Title: “Eight times normal” Diagnosis: Giant cell arteritis
Key Words, Giant cell arteritis, Anterior ischemic optic neuropathy
Abstract: 87yo female with h/o PMR who presented with visual loss in the L eye which progressed to the R eye with VA 200 “E” at 5 ½ ft OS and 200 “E” at 2 ½ ft OD. Accompanying symptoms included headache, jaw claudication, and weight loss. She was started on prednisone as an outpatient by community ophthalmologist. Upon presentation at BPEI, DFE revealed chalky white nerves OU and labs ESR 87, CRP 4. She was admitted to BPEI for presumptive diagnosis of giant cell arteritis for IV steroid therapy, temporal artery biopsy, and further studies. During her hospital course her visual loss deteriorated and stabilized at NLP in L eye and CF in R eye with bilateral giant cell arteritis confirmed. She was discharged on oral prednisone with regular clinical follow-up and labwork.

51. References
Chan, CCK, Paine M, O’Day J. Steroid management in giant cell arteritis. Br J Ophthalmol 2001; 85:1061–1064.
Hayreh SS, Podhajsky PA, Raman R, Zimmerman B. Giant cell arteritis: Validity and reliability of various diagnostic criteria. Am J Ophthalmol. 1997;123:
Hayreh SS, Zimmerman B, Kardon RH. Visual improvement with corticosteroid therapy in giant cell arteritis: Report of a large study and review of literature. Acta Ophthalmol Scand 2002;80:
Hayreh SS, Zimmerman B. Visual deterioration in giant cell arteritis patients while on high doses of corticosteroid therapy. Ophthalmology 2003;110:
Hayreh SS, Zimmerman B. Management of giant cell arteritis: Our 27-Year Clinical Study; New Light on Old Controversies. Ophthalmologica 2003;217:
Hunder GG, Bloch DA, Michel BA, Stevens MB, Arend WP, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY, Lie JT, Lightfoot RW, Masi AT, McShane DJ, Mills JA, Wallace SL, Zvaifler NJ. The American College of Rheumatology 1990 criteria for the classification of giant cell arteritis. Arthritis Rheum 1990;33: