Thanks to Eric for presenting the case of a young woman who presented with acute onset of night sweats, chest pain, and fatigue on subacute symptoms of cough and unintended weight loss, with CT chest showing mediastinal fatty infiltration which turned out to be thickening of the aortic arch and descending arteries consistent with Takayasu arteritis!
- The two main large vessel vasculitides are Giant Cell arteritis (GCA) and Takayasu arteritis.
- Takayasu primarily affects young women with the greatest prevalence of disease noted in Asia. GCA primarily affects older patients (>50 years).
- There is no diagnostic test to help with diagnosing Takayasu arteritis and for obvious reasons, biopsy is usually not possible. Some diagnostic criteria have been established to help (see below) and imaging tends to be the most helpful.
- Takayasu is a rare, chronic vasculitis of unknown etiology and primarily affects the aorta and its primary branches.
- Women are affected in 80 to 90 percent of cases, with an age of onset that is usually between 10 and 40 years.
- Though it has a worldwide distribution, the greatest prevalence is in Asia.
- Classification criteria have been developed for Takayasu as a means of categorizing patients for research studies. Rule in the diagnosis if at least three of the following are present:
- Age at disease onset ≤40 years
- Claudication of the extremities (tends to happen later in the disease course and after systemic symptoms of fever and malaise have already started)
- Decreased pulsation of one or both brachial arteries aka “pulseless disease”
- Difference > 10 mmHg in SBP between arms
- Bruit over one or both subclavian arteries (as in our patient!) or the abdominal aorta
- Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities, not due to arteriosclerosis, fibromuscular dysplasia, or other causes
- Imaging of the arterial tree by MRA or CTA is important to help with diagnosis, evaluate the arterial lumen, mintor disease course, and decide on need for surgical intervention.
- Disease can be limited or extensive at diagnosis (see image below) and tends to recur over time (as opposed to GCA which doesn’t usually recur). Poor prognostic indicators include significant arterial narrowing at presentation or fibrosis (inflammation is reversible but fibrosis is irreversible). Arterial wall thickening alone (in the absence of luminal narrowing) has an unknown impact on prognosis.
- The mainstay of treatment is immunosuppression with steroids (typically 40-60mg prednisone daily for average weight adult). Some small studies show added benefit with the addition of disease modifying agents.
- Differential Diagnosis of Takayasu
- Fibromuscular dysplasia (look for string of beads on imaging!)
- Behcet’s disease
- Giant cell (temporal) arteritis (GCA)
- IgG4-related disease
- Infectious aortitis
- Other diseases that can present with large-vessel vasculitis/aortitis such as Cogan’s syndrome, relapsing polychondritis (can cause aortic and mitral regurg), and spondyloarthropathies