Isolated Takayasu arteritis (TA) of the pulmonary circulation is extremely rare. We present a case of isolated TA of the pulmonary artery mimicking recurrent pulmonary embolism.
A 30-year-old-man with a history of intermittent fatigue, arthralgias and myalgias for several years presented with fever, night sweats, pleuritic chest pain and a ESR of 100 mm/hr. Vasculitis serology was negative. A ventilation-perfusion scan (V/Q scan) was high probability for pulmonary embolism (PE). He was started on anticoagulation, as well as prednisone and hydroxychloroquine for nonspecific connective tissue disease. Four months later the prednisone was tapered and he presented again with pleuritic chest pain and dyspnea. Physical exam and laboratory data were unremarkable. V/Q scan again showed a perfusion defect. Dopplers of lower extremities were negative for deep venous thrombosis and CT angiogram was negative for thromboembolism. He subsequently complained of progressive dyspnea with exertion. Echocardiogram showed mild pulmonary hypertension. An arteriogram showed diffuse narrowing of the pulmonary arteries compatible with vasculitis. MRA did not show involvement of other large vessels. The patient was started in high doses steroids with some improvement of his symptoms.DISCUSSION: Both giant cell arteritis and TA can present with systemic manifestations and involvement of the pulmonary artery. There are no specific serological markers for these conditions and the diagnosis often needs to be done on a clinical basis. Because of the patient’s age and the lack of classic symptoms of giant cell arteritis, a diagnosis of TA was made.TA is a chronic inflammatory disease of unknown origin that involves the aorta and its main branches. Although abnormal pulmonary angiography is described in 40-85% of the time, only few cases of isolated TA of the pulmonary artery have been described. The combination of symptoms, pulmonary hypertension and abnormal V/Q scan can make difficult the differentiation with chronic thromboembolic disease. Some of the clues to suspect the diagnosis of TA in this case were: the presence of chronic constitutional symptoms, a very high ESR, the absence of risk factors for PE, apparent failure of anticoagulation treatment and the fact that a thrombotic site was never found.
Isolated TA of the pulmonary artery is a rare condition that can mimic the clinical presentation of PE. Failure to respond to anticoagulation, presence of chronic constitutional symptoms, unclear thrombotic site and high ESR should prompt a more extensive work up.
J.E. Morales, None.