CD_1 Large-Vessel Giant Cell Arteritis: A Report of Three Cases

Abstract

Differences in the epidemiology of arteritis involving the large vessels, especially incidence, have been reported among Japan, Europe, and North America. Giant cell arteritis (GCA) is not often seen in Japan, whereas patients with Takayasu arteritis (TAK) are more commonly seen in Japan than patients with GCA. We describe three Japanese patients with large-vessel GCA and discuss the differential diagnosis of large-vessel arteritis. Patient 1 is a 71-year-old woman with fever around 388C and a 4-kg weight loss over 2 months. She had a white blood cell count of 6800/mm3, hemoglobin of 8.6 g/dl, CRP of 7.53 mg/dl, and ESR of 111 mm/h. ANCA was negative. When she did not improve with antibiotic treatment, she was referred to our hospital. FDP-PET CT revealed aortitis in the ascending to thoracic aorta. Prednisolone (PSL) (5 mg/day) and aspirin (100 mg/day) were administered for 4 months. Inflammation was ameliorated. Patient 2 is a 57-year-old woman with severe pain and ulcers in both lower legs for 9 years. As a result of severe pain, she could not sleep lying on the bed. Narrowing and irregularity of the external iliac, femoral, and popliteal arteries bilaterally were demonstrated with MRA. PSL (5 mg/day) and cyclophosphamide (50 mg/day) were administered. Patient 3 is a 73-year-old woman with mild fever for 2 months treated with antibiotics. CRP was 16.5 mg/dl. She had increased uptake in the bilateral cervical, subclavian thoracic and abdominal aorta. Since she had a history of an L3 compression fracture, she was treated with PSL (5 mg/day) and aspirin (100 mg/day). There are other diseases affecting large vessels besides TAK and GCA described in the 2012 CHCC. The difference between TAK and GCA is controversial. Age at onset of more than versus less than 50 years is likely to differentiate patients with GCA and TAK. However, 'late-onset TAK' has been described (Medicine 2010;89:1-17). 'Large-vessel GCA,' previously 'GCA variant,' has been used to describe GCA patients with extracranial involvement. We discuss 'large-vessel GCA,' which has not been described in the 2012 CHCC.