Takayasu disease

Takayasu disease: A chronic inflammatory disease of the aorta and its branch arteries. The cause is unknown. The disease is most common in young women of Asian descent and usually begins between 10 and 30 years of age. Symptoms include painful, cool or blanched extremities, dizziness, headaches, chest and abdominal pain, and low-grade fever. The blood pressure is often high. The sedimentation rate (sed rate) may be elevated, reflecting inflammation. Anemia is frequent. The diagnosis is confirmed by an angiogram of the arteries (arteriogram) showing abnormally narrowed and constricted arteries. The disease is treated by suppressing the inflammation with cortisone medication. While most patients improve, some do not or relapse. In cortisone-resistant patients, immunosuppressive drugs may be used. Examples include prednisone, prednisolone, methotrexate (Rheumatrex, Trexall), cyclosporine, cyclophosphamide (Cytoxan), and azathioprine (Imuran). Strict control of elevated blood pressure (hypertension) is important.

Named for the Japanese ophthalmologist Michishige Takayasu (1860-1938) who described it in 1908. (Giovanni Battista Morgagni reported a case consistent with Takayasu disease in 1761.) Also known as Takayasu arteritis, Martorell syndrome, pulseless disease, and aortic arch syndrome.