Epidemiology
- aka “Pulseless Disease”
- First reported in 1908
- Occurs mainly in Asian (mostly Japanese) females
- Peak age: <40 y/o
- Sex: F:M ratio 9 to 1
Physiology
- granulomatous arteritis of medium and large-sized arteries
- Arterial Involvement:
- Aorta
- Aortic branches: subclavian arteries are the most ocmmonly affected
- Renal vessels
- Large arteries of LE
Pathology
- granulomatous vasculitis involving aorta and branches -> narrowing, occlusion, and aneurysm formation
- Pulmonary arteritis probably occurs in most patients (pulmonary involvement can occur without other organ involvement)
Diagnosis
- ABG: elevated A-a gradient
- CBC: mild anemia/ leukocytosis
- ESR: elevated
- CRP: elevated
- PFT’s: decreased DLCO/ increased Vd/Vt ratio
- MR angiogram: may reveal changes (vessel wall thickening) before frank loss of pulses
- Angiogram: usually diagnostic
ECHO:
– Pulm HTN: may mimic CTEPH
Clinical
- Aortic arch: aneurysm
- CNS: CVA (15% of cases)
- Ocular: visual symptoms (60%)/ blindness
- Pulmonary:
- Pulmonary HTN: PA’s involved in most patients
- Rheumatologic: arthralgias
- Systemic: weight loss, fatigue, fever
- Cardiovascular:
- Hypertension (50%/due to renal artery involvement)
- Syncope (50%)
- Absent UE pulses (“pulseless disease”)
- LV failure
- Limb claudication
- Bruits over large arteries
- MI: although coronary arteries are rarely involved
Symptoms/signs: may occur with or without systemic vascular involvement
1) Optho:
a) Cataracts:
b) AVM’s around optic disks:
c) Blindness:
2) Cardiovascular:
a) Absence of pulses
b) Severe HTN:
c) LV failure:
d) Aortic aneurysm rupture
3) Neuro:
a) CVA
4) Pulmonary:
a) Pulmonary HTN: may mimic CTEPH (as pulmonary involvement can occur without other organ involvement in some cases)
Treatment
- Corticosteroids: disease may progress with blindness, despite treatment
- Cytoxan: for severe cases
- Mycophenolate: may be effective as steroid-sparing agent
- Methotrexate: may be effective as steroid-sparing agent
- Anticoagulation
References
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