Differentials
Giant cell arteritis (GCA)
SIGNS / SYMPTOMS
Patients are usually older; average age is 74 years. May have polymyalgia rheumatica with proximal myalgia. Jaw claudication is common. Lower extremity involvement is less common.
INVESTIGATIONS
Imaging with CT or magnetic resonance angiography; GCA is more likely to have cranial artery involvement and less likely to have lower extremity involvement.
Essential hypertension
SIGNS / SYMPTOMS
Intact pulses and the absence of bruits. No marked difference in blood pressure between each side.
INVESTIGATIONS
Clinical diagnosis.
No stenoses on vascular imaging.
Syphilis
SIGNS / SYMPTOMS
Firm painless ulcer at site of primary inoculation, usually genital region. Symmetric non-itchy rash accompanies systemic symptoms.
INVESTIGATIONS
Positive syphilis serology.
Catheter or CT angiogram: typical calcification of the proximal ascending aorta.
Tuberculosis (TB)
SIGNS / SYMPTOMS
Persistent productive cough. Recent travel to an endemic area.
INVESTIGATIONS
Mantoux test: elicits delayed hypersensitivity reaction, which will be positive in people with latent infection, previously cleared infection, or in those previously immunized.
Chest x-ray: may show evidence of pulmonary TB foci.
Sputum culture: takes 4 to 12 weeks to culture acid-fast bacilli but is diagnostic if found.
Interferon-gamma release assay: blood test to detect interferon gamma production when incubated with TB bacilli. Can identify active and latent infection.
Spondyloarthropathy
SIGNS / SYMPTOMS
Back pain and stiffness lasting more than 1 hour, particularly in the mornings. Peripheral arthritis. May be preceded by urethritis or cervicitis in the case of reactive arthritis. Accompanying symptoms may include psoriasis, palmar-plantar pustulosis, iritis, uveitis, or conjunctivitis.
INVESTIGATIONS
X-ray of spine: may demonstrate sacroiliitis.
X-ray of peripheral joint affected by arthritis may show periarticular osteolysis in psoriatic arthritis.
Behçet disease
SIGNS / SYMPTOMS
A triad of oral and genital ulceration with uveitis. Often accompanied by a peripheral arthritis. May have thrombotic arterial and venous occlusions.
INVESTIGATIONS
Angiography: reveals saccular dilation of involved arteries or thrombotic occlusion.
Cerebrospinal fluid examination supportive but not diagnostic; increased inflammatory cells and protein.
Kawasaki disease
SIGNS / SYMPTOMS
Typically affects children under age 5 years. High-grade fever with strawberry-tongue-marked lymphadenopathy. Red eyes with uveitis or conjunctivitis. Rash and peeling of the skin on the palms and soles may be seen.
INVESTIGATIONS
Clinical diagnosis using set criteria.
Angiography reveals saccular dilation of coronary arteries if affected.
Marfan syndrome
SIGNS / SYMPTOMS
Typically tall people with long limbs. May have a family history of Marfan syndrome. Susceptible to lens dislocation.
Systemic signs and symptoms absent.
INVESTIGATIONS
Clinical diagnosis.
Family history.
Genetic testing rarely carried out.
Ehlers-Danlos syndrome
SIGNS / SYMPTOMS
May have hypermobile joints or paper-thin skin scars.
Systemic signs and symptoms absent
INVESTIGATIONS
Angiography: if vascular wall collagen affected, may reveal saccular dilation of involved arteries.
Genetic testing.
Atherosclerosis
SIGNS / SYMPTOMS
More common in men, may have associated risk factors of hypertension, smoking, diabetes mellitus, and raised cholesterol. Typically, patients are aged over 40 years.
INVESTIGATIONS
Angiography: typical abrupt narrowing of artery rather than tapered narrowing. Lesions usually at the vessel origin and carotid bifurcations.
Fibromuscular dysplasia
SIGNS / SYMPTOMS
Pulses present but may be diminished. Hypertension common and most commonly affects renal and carotid arteries.
INVESTIGATIONS
Angiography: characteristic beading of affected arteries. Aorta not usually involved.
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