A 66-year old woman with large vessel vasculitis after a dengue virus infection

Abstract

A 66-year old Caucasian woman with a history of hypertension, primary hypothyroidism and a history suggestive of polymyalgia rheumatica presented with progressive dyspnoea, a few weeks after a dengue fever infection she had caught in Indonesia. Diagnosis of a primo dengue infection was serologically confirmed in Indonesia with a positive dengue IgM and negative IgG. Cardiologic analysis revealed several small aneurysms in the coronary arteries; a critical stenosis of the left ante rior descending artery was treated with bypass surgery. The patient had no family history of cardiovascular dis ease. One month later, she developed dizziness and vascular claudication of both upper and lower extremities. There was no fever; joint inflammation or skin lesions were absent. Physical examination revealed almost impalpable pulsations of the radial and ulnary arteries. Her blood pressure, though difficult to measure, was 120/65 mmHg. Plasma C-reactive protein (79 mg/L) and erythrocyte sedimentation rate (ESR; 50 mm/hr) were increased; leukocyte counts were normal (8.9 x109/L), as was autoimmune serology (including antinuclear and antineutrophil cytoplasmic antibodies). HLA-DR4 and HLA-DRB1*04 alleles were not present. Arterial duplex examination and computer tomography-angiography showed bilateral stenoses of over 50% of subclavian arteries and of over 75% of the axillary arteries (5-6 cm stenosis). Positron emission tomography further revealed increased activity in the entire aorta as well as the carotid, iliac, femoral and axillary arteries, suggestive of vasculitis of the large arteries (Figure 1). The patient’s demographics and lab results made Takayasu arteritis unlikely. Although biopsies of the temporal artery showed no arteritis and the traditional American College of Rheumatology criteria for the classification of giant cell arteritis were not met (only 2 out of 5 criteria: age at onset and ESR), vasculitis of all the large arteries on PET-CT combined with the patient’s age, history of polymyalgia rheumatica and increased ESR are highly suggestive of this diagnosis. 1 Successful treatment was initiated with long-term high doses of prednisone, relieving the patient’s complaints and normalizing C-reactive protein and erythrocyte sedimentation rate, which is sustained to date.