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SG IM FO RUM 2016; 39 (11 ) SHARE MORNING REPORT When 50 is Old Michele Fang, MD Dr. Fang is associate professor in the Perelman School of Medicine at the University of Pennsylvania and past president of Midwest SGIM. 56-year-old white female patient orrhage. Medium vessel vasculitis 1 m. Her face is symmetrical. The Ais admitted to the hospital for most typically demonstrates features patient demonstrates left-sided sen - acute onset of painless vision loss. of necrotic lesions and ulcers; nail sory neglect. Strength is 4+/5 in the Her daughter has noticed that the fold infarcts; mononeuritis multiplex; right upper and lower extremity and patient is bumping into objects, but renal infarction; infarction of liver, 0/5 in the left upper and lower ex - the patient denies any problems spleen, or pancreas; myocardial in - tremities. Plantar reflex is extensor with her vision. The patient has a farction; and nasal crusting and si - on the left. There is no clonus. one-year history of anorexia and nusitis. Large vessel vasculitis The patient’s neurological defect upper abdominal pain and a 30- to involves the aorta and its branches is most consistent with Anton’s syn - 40-pound unintentional weight loss. and can lead to symptoms of tempo - drome in which patients deny their Six months prior to admission, the ral headache (temporal arteritis), blindness despite objective evidence patient developed bilateral leg blindness (ophthalmic artery), jaw of visual loss. This typically involves cramps with walking. Evaluation claudication, limb claudication, and confabulation to support the belief demonstrated bilateral peripheral thoracic aortic aneurysms. that they have normal vision. Impor - vascular disease, including aortoiliac Based on this patient’s symp - tant next steps include radiological disease, which required stenting, toms, it is most likely that the patient imaging and serologic tests for and femoral-popliteal stenosis re - has a large vessel vasculitis. Clinical rheumatologic disorders to further dif - quiring bypass. assessment of patients with giant ferentiate amongst the vasculitides. If The patient quit smoking three cell arteritis and Takayasu’s arteritis these are unremarkable, further diag - months prior to presentation but has includes palpation of peripheral nostic information is provided by tem - a 30-pack-year smoking history. She pulses for asymmetry, bilateral blood poral artery biopsy (TAB) in giant cell denies alcohol or illicit drug use. pressure assessment, auscultation arteritis and imaging of the arterial Family history is positive only for di - for bruits, and laboratory tests for ev - tree by conventional angiography, 1 abetes mellitus and hypertension. idence of systemic inflammation . magnetic resonance imaging (MRI), Review of systems is otherwise On physical exam, vital signs are: or positron emission tomography negative. In particular, the patient temperature 37.3 C, P 107, BP (PET) . denies fever or chills, headaches, 154/88, R 20, and PO2 97% on The MRI shows bilateral occipital jaw claudication, genital or oral ul - room air. In general, she is in no diffusion restriction. MRA vascular cers, eye trauma, hematochesia, or acute distress; however, she is con - imaging is significant for verte - melena. Current medications are as - fused and confabulating at times. brobasilar constriction and intracra - pirin 81 mg orally once a day, al - Lungs are clear; heart is regular nial ACA/ICA/MCA vasoconstrictive buterol MDI as needed, and without murmur, gallop, or rub. pattern, suggesting the presence of ezetimibe 10 mg orally once a day. There is slight diffuse abdominal vasculitic lesions. Serologic tests, in - The patient’s history of multi-sys - tenderness. Extremities are without cluding ANA, hepatiis B, RPR, por - tem disease is concerning for a sys - edema and cool with reduced phyria, HIV, ANCA, and dsDNA, are temic vasculitis. Vasculitis is often pulses in the bilateral lower extremi - negative. ESR is 85, hemoglobin difficult to diagnosis because it is ties; she has dark necrotic destruc - 9.7, and WBC 7.6 with absence of relatively uncommon and can pre - tion of the right first and second toe. eosinophilia. BMP, LFT, TSH, and sent in many different ways. The There is no palpable cervical, axillary, lipids are unremarkable. ANA, anti- goal for the initial assessment will or inguinal lymphadenopathy and no DS DNA, rheumatoid factor nega - be to make a diagnosis, categorize obvious rash or other skin lesions. tive, IgA, and C3 and C4 comple- disease severity, and formulate a The neurological exam is significant ment levels are all normal. management plan. for bilateral strabismus; her pupils Highest on the differential, given Small vessel vasculitis can include are responsive to light, but there is the elevated ESR and blindness, is clinical features such as palpable pur - no light perception, and the patient giant cell arteritis . pura, hematuria, and pulmonary hem - cannot count fingers at a distance of continued on page 2 SHARE MORNING REPORT continued from page 1 The patient is promptly started Treatment of GCA (based upon with partial or total blindness on high-dose methylprednisolone. the British Society of Rheumatology denies being visually impaired, An angiogram demonstrates diffuse guidelines) calls for high-dose despite medical evidence to the multifocal medium and small in - steroids for at least three to four contrary. tracranial artery narrowing consis - weeks. Use of aspirin is also recom - tent with vasculitis. Temporal artery mended given the high risk of is - References biopsy demonstrates segmental chemic complications in these 1. Maddula M, Lutton S, Keegan transmural scarring with late-phase patients. Relapses are common. Im - B. Anton’s syndrome due to granulomatous arteritis consistent munosuppressive agents, such as cerebrovascular disease: a case with giant cell arteritis. methotrexate and leflunomide, report. Med Case Reports 2009; The patient is continued on high- should be considered at the third re - 3:9028. dose steroids; however, her vision lapse if not sooner . 2. Suresh E. Diagnostic approach does not return. to patients with suspected Giant cell arteritis (GCA) is a med - Take-home Points vasculitis. Postgrad Med J 2006; ical emergency with permanent vi - 1. Giant cell arteritis (GCA) most 82(970):483-8. sion loss occurring in about 20% of typically occurs in patients older 3. Miller A, Chan M, Wiik A, Misbah patients. Urgent diagnosis and initia - than age 50 and usually (but not SA, Luqmani RA. An approach to tion of high-dose steroids reduce the always) presents with a the diagnosis and management likelihood of this occurring. Other is - headache. of systemic vasculitis. Clin Exp chemic symptoms include diplopia, 2. GCA is a neurological emergency Immunol 2010; 160(2):143-60. transient visual loss, and jaw and and requires a high degree of 4. Kale N, Eggenberger E. Diagnosis tongue claudication. Headache oc - suspicion and prompt treatment and management of giant cell curs in 60% of patients with GCA— with steroids. arteritis: a review. Curr Opin most typically a sudden severe 3. Anton’s syndrome is a form of Ophthalmol 2010; 21(6):417-22. 2 localized pain in the temporal region. anosognosia in which a person SGIM SG IM FO RUM 2016; 39 (11 ).