COMMENTARY

A 30-Year-Old Diagnosis Never Treated: A Case

Stephen Paget, MD

Disclosures

June 08, 2017

Editorial Collaboration

Medscape &

Patient Profile

I was asked to see a 76-year-old Japanese-American woman with Takayasu arteritis, a diagnosis first entertained in 1984 when, at age 33 years, she was found to have multiple arterial bruits. At the time, the patient had a normal erythrocyte sedimentation rate (ESR), and her arteritis appeared to be inactive and did not require steroids. In fact, she has never been treated with steroids for Takayasu arteritis.

In 1989, MRI examination of the chest showed a proximal ascending aorta rotation with a constant diameter of 4 cm, which has remained at that size until now. The descending aorta was normal, and there was no evidence of dissection or aneurysm.

The patient had been seen by many specialists in Japan, some of whom believed she had Takayasu arteritis and others who believed her vascular abnormalities were caused by atherosclerosis, perhaps related to her MTHFR gene status. The patient has also been found to have mild aortic insufficiency; but, aside from when she suffered a saddle pulmonary embolism in 2016, the patient denies chest pain or shortness of breath and has no claudication-type symptoms in the arms and legs. She has had no cerebrovascular accidents but did have a hemorrhage in the right eye.

ESR during the pulmonary embolism episode in September 2016 was 55 mm/hour; in March 2017, the ESR was 70 mm/hour; and today it is normal at 17 mm/hour with a normal C-reactive protein. The patient has had a history of trigger fingers, knee osteoarthritis, low back pain, and osteoporosis. At no time has she had joint inflammation or stiffness or fatigue, and she denies a history of Raynaud phenomenon, skin rash, hemoptysis, fever, or weight loss.

Physical Examination

The patient's vital signs were normal with equal blood pressures in arms and legs. Loud bilateral carotid and subclavian bruits were heard and radial pulses were barely palpable. No ischemic arm or leg findings were noted.

Laboratory Tests—May 10, 2016

  • Comprehensive chemistry panel: glucose, 148 mg/dL; creatinine, 0.9 mg/dL

  • Normal complete blood count

  • Urinalysis, 1+ blood but only 1 red blood cell

  • Spot urine protein/creatinine ratio, 6/33.7; 180 mg/g

  • Cyclic citrullinated peptide antibodies negative

  • Rheumatoid factor negative

  • Anti-Ro, 77 U (normal <20); anti-La, anti-Sm, anti-RNP, all negative

  • Antithyroglobulin, 114 IU/mL (normal <4.11); antithyroid peroxidase, 113 IU/mL (normal <5.61)

  • Antinuclear antibody negative, anti-double-stranded DNA negative

MRI

The patient underwent MRI angiography of her aorta on March 10, 2016, and a chest MRI without and with contrast on March 28, 2017.

She had a left-sided aortic arch with a normal branching pattern of the great vessels. There was no evidence of thoracic aortic dissection or coarctation. Irregular mural thickening was noted at the level of the aortic arch, great vessels, and throughout the descending thoracic aorta, but overall no significant changes were noted. There was stable stenosis at the origin and proximal right brachiocephalic artery and proximal right common carotid artery. The thoracic aorta appeared normal in size.

Views of the left ventricle were very limited; however, normal size and systolic function were demonstrated. Her aortic valve is trileaflet, and she has mild aortic regurgitation and no significant aortic stenosis.

Impressions:

  1. No significant interval change was seen in the appearance of the irregular mural thickening of the aortic arch, great vessels, and throughout the descending thoracic aorta.

  2. Stable stenoses of the right brachiocephalic artery and proximal right common carotid artery were noted.

  3. No evidence was seen of thoracic aortic aneurysm, dissection, or coarctation.

  4. The aortic valve is trileaflet with mild aortic regurgitation.

Comments

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