We present a case of a 72-year-old male with essential hypertension and benign prostatic hyperplasia who presented to the emergency department on January 2018 with an intense right temporal headache with retroocular irradiation, which was pulsatile and associated with scalp hyperesthesia. The patient also reported fatigue, anorexia low-grade fever, night sweats, and non-quantified weight loss over the last two months. He denied any visual symptoms, such as ocular pain or loss of visual acuity. The physical exam revealed a tortuous and indurated right superficial temporal artery that was tender to palpation. An ophthalmology consultation was requested, and anterior ischemic optic neuropathy and other ophthalmological conditions were excluded. The cranial computed tomography (CT) performed in the emergency department was normal, and analytic evaluation showed an elevated ESR of 82 mm/h (normal range < 20 mm/h) as well as an elevated CRP of 20 mg/dL (normal range < 0.5 mg/dL). Furthermore, he also had an inflammatory anemia with a hemoglobin of 11.7 g/L (normal range: 13-17 g/L).
The diagnosis of temporal arteritis was suspected, and the patient was started on prednisolone (1 mg/kg). A biopsy of the right temporal artery was performed on the first week after the initiation of corticotherapy and was negative for GCA. Nevertheless, after the initiation of corticosteroids, there was a remission of symptoms accompanied by a normalization of inflammatory markers with an ESR of 18 mm/h and CRP of 0.4 mg/dL. After two months of 1 mg/kg prednisolone, slow steroid tapering was started. However, at the dose of 20 mg of prednisolone, there was a reappearance of constitutional symptoms, namely fatigue, anorexia weight loss, and night sweats with a new increase of ESR to 80 mm/h. Besides the constitutional symptoms, there were no other organ-specific symptoms, such as headache, visual loss, arthralgias, or others. The corticosteroid dose was increased to the initial dosage (1 mg/kg), but there was no improvement in the symptoms this time.
At this point, due to its persistence, other causes for the constitutional symptoms were excluded. The patient underwent a full-body CT scan, which was negative for neoplastic causes, as well as endoscopic exams, which were also normal. Given the high prevalence of tuberculosis in Portugal, the patient also underwent bronchoscopy and sputum examination, which were negative for this agent (negative direct examination with Ziehl-Neelsen stain and negative polymerase chain reaction for Mycobacterium tuberculosis). The patient was negative for the human immunodeficiency virus, and the venereal disease research laboratory (VDRL) test was also negative. The serologies for Rickettsia, Borrelia, and Brucella were also negative.
Therefore, given the maintenance of constitutional symptoms that caused functional limitation to the patient, a PET scan was performed, which showed a grade 2 aortitis extending from the aortic arch to the emergency of the renal arteries (Figure 1). The transthoracic echocardiogram revealed an ascending aortic dilation of 44 mm (normal range: 17-33 mm) (Figure 2). Furthermore, the antinuclear antibodies were positive with a titer of 1/160, and the rheumatoid factor was negative. The anti-neutrophil cytoplasmic autoantibodies (p-ANCA) to bactericidal/permeability-increasing (BPI) protein were 1.6 UI/ml (positive cut-off value of 1.1 UI/ml). However, there were no manifestations specific to ANCA vasculitis, such as pulmonary-renal syndrome, skin vasculitis, chronic destructive airway disease, pulmonary nodules, or mononeuritis multiplex. The fact that the initial clinical presentation was very suggestive of GCA with temporal involvement which later progressed to aortic involvement, and also as the patient never had any signs or symptoms of ANCA vasculitis, led to the diagnosis of giant cell aortitis. Given the lack of clinical response to corticotherapy, tocilizumab was initiated (162 mg subcutaneous weekly), with a resolution of constitutional symptoms as well as normalization in inflammatory markers. A second PET scan was performed nine months after therapy with an improvement of inflammation in the previously affected areas with grade 1 aortitis (Figure 1).