• „ CLINICAL VIGNETTE

When Takayasu mimics pulmonary hypertension — severe pulmonary artery stenosis — what to do?

Mateusz Polak, Marek Grabka, Wojciech Wróbel, Iwona Woźniak-Skowerska, Katarzyna Mizia-Stec

1st Department of Cardiology, School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland

A 27-year-old female, without a history of cardiac diseases, with increasing fatigue and dyspnea, up to class IV of New York Heart Association (NYHA) during the previous two weeks, was admitted to the cardiology department with suspicion of pulmonary arterial hypertension (PAH).

On admission, the patient presented resting dyspnea, tachycardia 120 bpm, and blood pressure 100/60 mm Hg. Capillary blood oxygen saturation was 80%. A loud systolic murmur was present over the entire chest. Physical examination did not reveal peripheral edema nor features of venous thromboembolism in the lower extremities.

Laboratory tests revealed amino-terminal pro-brain natriuretic peptide of 10745 pg/ml (n = 0–125 pg/ml), increased inflammatory parameters (C-reactive protein 25 mg/l; white blood cells 13 × 103/µl), serum concentration of IgG4 of 60 mg/dl. Echocardiography showed the overload of the right ventricle, tricuspid annulus dilatation with torrential regurgitation. Calculated systolic pulmonary artery pressure was 110 mm Hg. Computed tomography angiography (angio-CT) scan revealed inflammatory infiltration of the pulmonary trunk, involving the pulmonary arteries and causing critical stenoses (Figure 1A–B). No embolic material was found on the scan.

Based on clinical and angiographic criteria proposed by the Japanese Research Committee on Vasculitis Syndromes [1], the initial diagnosis of Takayasu arteritis (TA) was stated. These criteria included: (1) angiographic evidence of narrowing or occlusion of the aorta or large arteries on CT or magnetic resonance imaging; (2) early age of onset; (3) presence of inflammatory markers; and (4) exclusion of atherosclerosis, other inflammatory diseases, or congenital vascular abnormalities.

The patient received 15 mg of prednisone per day and two intravenous infusions of 400 mg cyclophosphamide, achieving reso­lution of resting dyspnea. During the next 6 months, the patient received a total dose of 4.8 g cyclophosphamide and achieved total remission of clinical symptoms. Control angio-CT scan showed substantial regression of stenosis (Figure 1C–D). Figure 1E shows control parameters. Further hospitalizations were planned to perform imaging examinations and evaluate the effects of the treatment.

TA is a large-vessel vasculitis affecting the aorta and its primary branches: subclavian artery (33.7%), the renal artery (25.3%), and the common carotid artery (21.7%); the pulmonary artery comprised only 0.8%. Inflammatory processes in the acute phase cause thickening of the arterial wall. The chronic phase causes vascular fibrosis, stenosis, and occlusion, resulting in congestive heart failure and pulmonary hypertension [1].

We present a rare case of TA involving pulmonary arteries that clinically mimicked signs of severe PAH. Clinical presentation: age, sex, symptoms, amino-terminal pro-brain natriuretic peptide (NT-proBNP) level, and echocardiographic signs suggested PAH. Detailed echocardiographic assessment and the CT-scan allowed to reveal that the PAH-like features of right ventricular overload were due to severe pulmonary arteries stenosis. In the differential diagnosis, it was essential to exclude another inflammatory artery stenosis, mainly giant cell arteritis (GCA). While TA is seen in young females, GCA affects older patients. In GCA, inflammation processes often involve the external carotid artery, which is not seen in TA. Arterial wall thickening can be due to retroperitoneal fibrosis and therefore mimics TA, but TA lacks peritoneal diffusion [2]. IgG4 related disease was also taken into consideration. With a serum concentration of IgG4 of 60 mg/dl, it was unlikely (96% negative predictive value) [3].

The first line of treatment for TA is glucocorticoids. Half of the patients require second-line agents: cyclophosphamide, methotrexate, or biologic drugs [4]. Endovascular or surgical interventions in artery stenosis may be necessary once irreversible stenosis starts to develop. Since the rates of complications are the highest in patients with acute inflammatory lesions, interventional therapy should be avoided during the acute phase of the disease [5].

Article information

Conflict of interest: None declared.

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How to cite: Polak M, Grabka M, Wróbel W, et al. When Takayasu mimics pulmonary hypertension — severe pulmonary artery stenosis — what to do? Kardiol Pol. 2021; 79(9): 1046–1047, doi: 10.33963/KP.a2021.0059.

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