Takayasu arteritis associated with systemic sclerosis

Abstract

Takayasu arteritis (TA), or pulselessness disease, is a necrotizing and obliterative giant cell arteritis whose etiology is unknown; it involves the aorta and its main branches, and coronary and pulmonary arteries. It is diagnosed by clinical findings and angiographic investigation. 1 Systemic sclerosis (SSc) is a connective tissue disease of unknown etiology. Its vasculopathy is manifested by abnormal collection of collagen and extracellular matrix in skin and internal organs, and is caused by immunologic mechanisms. 1 Causes of TA associated with SSc are rarely found. 2 We report the case of a patient with TA who developed SSc 1 year later. This is the third case of Takayasu arteritis associated with systemic sclerosis reported in the literature.A 48-year-old female patient was admitted to our clinic with complaints of malaise and cyanosis on the fingertips, especially in cold environments. One year previously a physical examination for intermittent claudication had shown that she had no pulse on the distal arms. Doppler ultrosonographic investigation showed 85% and 75% narrowing of the right and left subclavian arteries, respectively. The narrowing showed that the left and right subclavian arteries were obliterated on the distal side of the vertebral artery bifurcation region (Fig. 1). Angiographic investigation of the lower extremities revealed that visualization was not possible for the right dorsal pedal artery, the 1/3 distal side of left tibial anterior artery, and the left dorsal pedal artery. The patient was diagnosed as having TA. She had a 3-year history of Raynaud phenomenon in her medical record. Physical examination showed that her blood pressure was 150/70 mmHg on right thigh, pulse rate rhythm 74/min, and upper extremity pulses could not be palpated. There was no murmur on the vessels of the neck. There were dermal indurations on the proximal metacarpopharyngeal joints and fingers of both hands. There were no abnormalities on the other systemic organs.

Late Onset Takayasu Arteritis and Rheumatoid Arthritis

“…The fact that associations with various autoimmune disorders have been described, such as SLE or systemic sclerosis, supports this theory [6, 7]. …”

Unusual overlap of systemic sclerosis with Takayasu arteritis

“…Reports of overlap of Takayasu arteritis with other connective tissue disorders are uncommon 1. There have been very few reports of systemic sclerosis associated with Takayasu arteritis 2–5. We report a case of systemic sclerosis with multiple system involvement that was later found to be associated with Takayasu arteritis.…”