Definition
A.Part of a group of disorders that result from inflammatory changes to walls of veins and arteries, causing damage to the mural structures which ultimately may cause tissue ischemia and necrosis.
B.Nomenclature is changing for the specific forms of vasculitis based on the Chapel Hill Consensus Conference (CHCC).
C.Two main types.
1.Takayasu arteritis.
2.Giant cell arteritis/temporal arteritis.
Incidence
A.Takayasu arteritis.
1.Common in Asian descent.
2.Women are typically more affected than men.
3.Age of onset tends to be early adulthood.
B.Giant cell arteritis/temporal arteritis.
1.Most common vasculitis (UTD).
2.Afflicts more females than males; age greater than 50 with the most common presenting age between 70 and 79.
3.Common in Scandinavian descent.
Pathogenesis
A.Vasculitis can be a primary pathology or can be secondary to another underlying disease process.
B.Direct injury to the vessel, infectious agents, or immune processes are known to cause vasculitis.
C.Secondary vascular injury may occur from physical agents such as cold and irradiation, mechanical injuries, and toxins (P).
D.Takayasu arteritis.
E.Granulomatous vasculitis of aorta and its major branches (C).
F.Possible immunogenetic predisposition (U).
G.Giant cell arteritis/temporal arteritis.
1.Chronic inflammatory disease of cranial branches of carotid arteries.
Predisposing Factors
A.Female gender, age typically over 60 years old.
B.Some familial pattern.
Subjective Data
A.Common complaints/symptoms.
1.Constitutional symptoms are common with all size vessel vasculitis and include fever, weight loss, malaise, and arthralgias/arthritis.
2.Large vessel vasculitis will often present with limb claudication, asymmetric blood pressures, absence of pulses, bruits, and aortic dilatation.
3.Takayasu arteritis.
a.Decreased pulses.
b.Unequal upper extremity blood pressures.
c.Carotid and subclavian artery bruits.
d.Limb claudication and hypertension.
4.Giant cell arteritis/temporal arteritis.
a.Headache, scalp tenderness.
b.Visual changes, such as amaurosis fugax.
c.Jaw claudication.
d.Throat pain.
e.Temporal artery tenderness.
B.Common/typical scenario.
1.See previous sections for common/typical scenario.
C.Family and social history.
1.Inquire about family history of vasculitis.
D.Review of systems.
1.See previous sections for review of systems.
Physical Examination
A.Cardiology.
1.Decreased pulses.
2.Unequal upper extremity blood pressures.
3.Carotid and subclavian artery bruits.
B.Limb claudication and hypertension.
C.Headache, scalp tenderness.
D.Visual changes, such as amaurosis fugax.
E.Jaw claudication.
F.Throat pain.
G.Temporal artery tenderness.
Diagnostic Tests
A.Takayasu arteritis.
1.Elevated C-reactive protein (CRP) and elevated erythrocyte sedimentation rate (ESR).
2.MRI or CT angiography (CTA) of affected vessels.
B.Giant cell arteritis/temporal arteritis.
1.Normochromic anemia, normal leukocytes, decreased serum albumin, elevated liver enzymes, and elevated CRP and ESR.
2.Best diagnostic test to confirm is temporal artery biopsy (UTD).
3.In patients with normal temporal artery biopsy, consider magnetic resonance angiography (MRA) or CTA for confirmation.
Differential Diagnosis
A.Other forms of vasculitis.
B.Nonarteritic anterior ischemic optic neuropathy (NAAION).
Evaluation and Management Plan
A.General plan.
1.Giant cell arteritis: Early intervention is necessary to prevent blindness, which can become permanent.
B.Patient/family teaching points.
C.Pharmacotherapy.
1.Takayasu arteritis.
a.Prednisone 1 mg/kg orally for 1 month; then tapered over several months to 10 mg orally daily.
b.Methotrexate and mycophenolate mofetil may also be helpful.
2.Giant cell arteritis/temporal arteritis.
D.Must be initiated quickly to avoid permanent blindness; therefore, if clinically suspected begin treatment.
E.Visual loss present at time of diagnosis: Intravenous (IV) methylprednisone 1,000 mg IV daily for 3 days followed by oral steroids (UTD).
F.Prednisone 60 mg po daily × 1 month then taper dose.
G.Low dose aspirin—81 mg po daily.
Follow-Up
A.Giant cell arteritis.
1.Follow-up should be monthly for 6 months if possible and then spaced accordingly.
2.Consider ophthalmology consult for visual symptoms.
B.Takayasu arteritis.
1.Requires regular monitoring.
Consultation/Referral
A.Refer to rheumatology.
Special/Geriatric Considerations
A.Chronic steroid use can cause osteoporosis, so consideration should be made to treat osteoporosis.
Bibliography
Docken, W. P. (2018, August 13). Treatment of giant cell arteritis. In M. Ramirez Curtis (Ed.), UpToDate. Retrieved from https://www.uptodate.com/contents/treatment-of-giant-cell-temporal-arteritis?source=see_link
Docken, W. P. (2018, October 5). Diagnosis of giant cell arteritis. In M. Ramirez Curtis (Ed.), UpToDate. Retrieved from https://www.uptodate.com/contents/diagnosis-of-giant-cell-arteritis?topicRef=8240&source=related_link#H18
Docken, W. P., & Rosenbaum, J. T. (2017, December 8). Clinical manifestations of giant cell arteritis. In M. Ramirez Curtis (Ed.), UpToDate. Retrieved from https://www.uptodate.com/contents/clinical-manifestations-of-giant-cell-temporal-arteritis
Hannon, R. A., & Porth, C. M. (2017). Porth pathophysiology: Concepts of altered health states (2nd ed.). Philadelphia, PA: Wolters Kluwer.
Merkel, P. A. (2019, March 1). Overview of and approach to the vasculitides in adults. In MRamirez Curtis (Ed.), UpToDate. Retrieved from https://www.uptodate.com/contents/overview-of-and-approach-to-the-vasculitides-in-adults
Papadakis, M. A., McPhee, S. J., & Rabow, M. W. (2016). Current medical diagnosis & treatment 2016. New York, NY: McGraw Hill Education.