Download Wang outline - American Academy of Optometry

Robert Wang, OD, FAAO
Giant Cell Arteritis? Second Chances...
Abstract: Sixty four year old white male arriving in clinic with signs of giant cell arteritis refusing advanced care and
then lost to follow up. Patient returns to clinic with signs of recalcitrant giant cell arteritis.
I.
Case History
Patient demographics
64 year old white male.
Chief complaints
Reporting to eye clinic with CC of not being able to see out of left eye, onset 4-5 days prior. Patient
notes “flashes of light and big blobs or stuff moving” in his vision, then finally vision is completely
dark.
Ocular, medical history
No significant prior ocular history.
Intervertebral disc disorder, Tobacco use disorder, constipation, chronic low back pain, muscle
weakness, anemia, malnutrition of mild degree, chronic obstructive pulmonary disease, essential
hypertension, peptic ulcer, shoulder pain.
Medications
Hydrocodone/acetaminophen tab, epi-pen, amlodipine besylate, Lisinopril, ranitidine, albuterol,
aldendronate, calcium/vitamin D
Other salient information
None
II. Pertinent findings
Clinical
2/4/13
VA 20/30 ph 20/25+2 right, NLP PH NI left
APD left, confrontation fields fill right, restricted 360 left
IOPs 10mmHg right and 11mmHg left
Anterior segment unremarkable
Posterior segment .2/.2 right, no cup, swollen nerve with flame hemorrhage just inf/temp left
OCT: notes optic nerve head drusen, but also a triangular separation of fluid/edema
characteristic of optic nerve head edema
Physical
Laboratory studies
CRP 30.7
ESR 49
Radiology studies
Ct: no abnormalities
Others
III. Differential diagnoses
Primary/leading
Giant cell arteritis
Non-arteritic ischemic optic neuropathy
Others
IV. Diagnosis and discussion
Elaborate on the condition
Giant cell arteritis is a chronic vasculitis of medium and large size arteries and appears to favor the
smaller branches of the external carotid artery. This eventually leads to endovascular damage,
vessel stenosis and occlusion which then leads to tissue necrosis (Smetana). In the presence of a
swollen nerve in an age group over 50, clinicians should always consider Giant cell arteritis. Most
commonly patients will complain of headaches in 56%, followed by anorexia/weight loss in 52%,
and jaw claudication in 48% (Hayreh). Studies have shown that a useful criteria for normal ESR is
<30 mm/hr in men and <35mm/hr in women with a sensitivity and specificity of 92%. (Hayreh),
however notes that a normal ESR does not rule out the possibility of GCA. CRP appears to be
more reliable test as sensitivity in detecting GCA was noted 100% and the specificity noted at 7983% (Hayreh). Vision loss of varying severity was reported in 92% in a case series (Hayreh).
Visual acuity varies from 20/40 or better to no light perception. The gold standard for GCA arteritis
is a temporal artery biopsy, however even these biopsy’s can be subject to skip lesions where
normal tissue can be found even with GCA. Given this possibility of skip lesions, studies have
emerged on using MRI to specifically search for inflamed portions of the temporal arteries to direct
temporal artery biopsies (Bley).
Expound on the unique features
Patients over 50 with temporal headache and jaw claudication with a swollen nerve are considered
to have giant cell arteritis until proven otherwise.
V. Treatment, management
Treatment and response to treatment
Treatment can precede diagnosis given the threat of further irreversible vision loss. Hayreh
suggests treatment with systemic corticosteroids, starting with a megadose of intravenous steroids
as a loading dose, followed by oral Prednisone. Hayreh also recommends altering the steroid
dosage based on the ESR and CPR reading rather than systemic conditions. Patients should be
warned that usually there is no useful recovery of vision in the effected eye, and that the goal of
treatment is to save the vision in the opposing eye. Many of these patients will be on steroid
therapy for long term treatment.
Recently evidence that MRI with sufficient resolution and an auto injector maybe able to point
areas that are most effected by giant cell arteritis and be markers for possible sites for biopsy.
In our case our patient was walked to the Emergency department in preparation to be admitted to
the hospital for intravenous steroids. Somehow the patient was never admitted, and instead was
given a regiment of oral steroids for treatment. The following temporal artery biopsy was negative.
Patient was lost to follow up until...
4/11/14
Patient returning to eye clinic with eye pain once a week for 2 months. Patient denies
temporal scalp tenderness/pain or jaw claudication.
Va 20/20-3 right, LP without directionality left
APD left
IOPs 14mmHG right and 12mmHg left @ 3:45p
Unremarkable anterior segment
Posterior segment .2r right, .2r left with diffuse pallor, noted optic nerve head drusen both
No clinical signs of giant cell arteritis at this visit, but given history of persistent headache
ordered ESR and CRP. CRP > 55mg/L and ESR 67mmHR. Spoke to ED after hours and
make call to patient regarding lab findings. Patient noted that they still had oral steroids from
the ED as they never finished the treatment. Patient could not return to the hospital that night,
and instead began 80mg steroid PO each day until he could return to the hospital.
4/14/14
Patient arrived in ED, while being on oral steroids 40mg bid po. Patient did not want to have a
temporal biopsy, refused IV steroids and consult to rheumatology. A compromise of sorts was
made with tapering to 60mg for 7 days oral then to 40mg for 30 days and to re-evaluate
following this dosing. ESR 4mg/L, CRP 14.30mmHR. Labs to be done every month or ESR,
CRP and A1C. Monthly optometry visits as well as Primary care visits. Of interesting note,
patient had a longstanding history of shoulder and lower back pain for which he was on
hydrocodone. Also noted was that he had mild malnutrition. The oral steroids had an
astonishing effect on both of these problems. Patient likely had polymyalgia rheumatic as this
occurs in 40-50 percent of giant cell patients. This pain was largely relieved by the oral
steroids to the point that the patient noted that he no longer needed his hydrocodone as
much. In addition because of the steroids the patient was able to put on some weight.
Patient is currently being followed monthly by primary care provider and Optometry, current
goal of treatment is to taper oral steroids as much as possible.
Recent Rheumatology consult noted that they will be pursuing a neuro eval and MRI of the
head to eliminate other possible causes. If no other causes are found they will consider
addition of immunosuppressives to possibly allow the taper of Prednisolone.
Refer to research where appropriate. Bibliography, literature review encouraged
Bley Thorsten, Oliver Wieben, Markus Uhl, Jens Thiel, Dieter Schmidt, and Mathias Langer. "High
Resolution MRI in Giant Cell Arteritis: Imaging of the Wall of the Superficial Temporal Artery."
American Journal of Roentgenology 184 (2005): 283.
Hayreh, Sohan Singh. "Giant Cell Arteritis." . The University of Iowa. N.p., n.d. Web. 12 Nov. 2013.
<http://webeye.ophth.uiowa.edu/dept/GCA/GCA.htm>.
Smetana, Gerald, and Robert Shmerling. "Does this patient have temporal arteritis?." Journal of
the American Medical Association 287 (2002): 92.
VI. Conclusion
Clinical pearls, take away points if indicated
While there are few true optometric emergencies, giant cell can be fairly common, and should be a
differential in all patients with sudden loss of vision over 50. While our patient did not have the
hallmark signs of giant cell (jaw claudication etc), lab testing and other symptoms painted a clear
picture of giant cell arteritis, with an extremely high risk for complete blindness.