Takayasu’s arteritis

TL;DR

• Usually Asian females < 40 years old.

• “Pulseless disease” (weak upper extremity pulses), fever, night sweats, arthritis, myalgias, skin nodules, ocular disturbances.

• Granulomatous thickening and narrowing of aortic arch and proximal great vessels B . elevated ESR.

• Treatment: glucocorticoids.

• Arteritis of aorta and its branches → stenosis/aneurysm → claudication. Most often subclavian & innominate arteries (>90%); carotid, coronary, renal, or pulm a. (~50%)

• Epidemiology: most common in Asia; ♀:♂ ~9:1 in Japan but lower elsewhere; age <50 y. Prev 8/million in U.S. w/ ~4:1 ♀:♂ (J Rheumatol 2021;48:952).

• Clinical manifestations: systemic inflamm with fever, arthralgias, wt loss Vessel inflamm w/ pain & tenderness, ↓ & unequal pulses/BPs in extremities, bruits, limb claudication, renovascular HTN (>50%), neurogenic syncope, Ao aneurysm ± AI “Burnt out” or fibrotic period (eg, vascular stenosis)

• Dx studies: ↑ ESR (75%), CRP; arteriography (MRA, CTA) → occlusion, stenosis, irregularity, and aneurysms; carotid U/S Doppler studies; PET-CT; pathology → focal panarteritis, cellular infiltrate with granulomas and giant cells (bx not required for dx)

• Rx: steroids ± MTX, AZA, or anti-TNF; tocilizumab 2nd line (Ann Rheum Dis. 2020;79:19); ASA if critical cerebral stenosis; if surgical/endovascular revasc, preferably done in remission

• Monitoring: MRA, CTA, or PET-CT; ESR/CRP