Large vessel vasculitis can be a paraneoplastic disorder. It can involve the aorta or its major branches.
Malignancies associated with large vessel vasculitis:
(1) myelodysplastic syndrome (MDS)
(2) chronic myelomonocytic leukemia
(3) lung cancer
Clinical and laboratory findings:
(1) fatigue
(2) shortness of breath
(3) chest or abdominal pain
(4) elevated ESR and/or C-reactive protein (CRP)
(5) positive serum autoantibodies
Imaging studies show inflammation surrounding the aorta and its major branches, with or without stenosis and/or dilatation. PET/CT is the sensitive for diagnosis and monitoring response to therapy.
Differential diagnosis:
(1) autoimmune large vessel vasculitis
(2) infectious aortitis
(3) degenerative aortic disease
The aortitis can often be controlled by corticosteroids and control of the underlying malignancy.
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