Takayasu arteritis (TAK) is a rare disease (ORPHA: 3287).1 Despite its well-known clinical characteristics, its etiology remains unknown and shares an inflammatory and genetic background.
An 18-year-old woman was referred to our department for treatment of arterial hypertension secondary to renal artery stenosis. Earlier, at the age of 8 years, she was treated ineffectively with retinoids and cyclosporin A for psoriasis. Remission was achieved with etanercept when the patient was 13–14 years old. Etanercept was reintroduced at the age of 16 years. During routine check-up 2 years later, inflammatory markers were elevated (eg, C-reactive protein, 28 mg/l; reference range <30 mg/l), but there was no exacerbation of psoriasis. One month later, the patient reported back pain that was not relieved by nonsteroidal anti-inflammatory drugs. In addition, the patient reported dyspnea on exertion (New York Heart Association class II). She had temporary anosmia and ageusia during COVID-19.
After 3 months of persistent back pain, magnetic resonance imaging revealed narrowing of the middle section of the abdominal aorta of up to 6 mm at the length of 12 cm, with wall thickening of up to 5 mm (Figure 1A). The patient was admitted to a pediatric cardiology department. Ultrasound examination confirmed these findings (Figure 1B), and also showed left renal artery stenosis (Figure 1C). Computed tomography angiography visualized the abdominal aorta stenosis of up to 6 mm at the level of and below the renal arteries. Moreover, both right and left renal arteries were inflamed and had critically narrowed walls (Figure 1D–1F). Echocardiography revealed reduced left ventricular ejection fraction (LVEF) at 31%. Finally, the diagnosis of middle aortic syndrome in the course of type IV TAK, complicated by severe hypertension and heart failure was made. The patient met the TAK criteria of the American College of Rheumatology / European League Against Rheumatism Classification,2 and obtained 10 points on this score (Supplementary material).
Etanercept therapy was discontinued. Furosemide, spironolactone, metoprolol, and amlodipine were initiated. Prednisone (40 mg/day) and methotrexate (25 mg/week) were started for TAK. The treatment resulted in normalization of blood pressure and LVEF and resolution of the signs and symptoms of heart failure. Thereafter, the patient was referred to our angiology department, where angioplasty of the upper left renal artery was performed because of scintigraphy-detected hypoperfusion in the left kidney. Subsequently, the inflammatory markers normalized, and ultrasound showed complete resolution of the wall thickening in the aorta and renal arteries. The therapy with prednisone and methotrexate was continued, and hypertension was managed with only metoprolol and spironolactone. When the patient was 20 years old, she developed bleeding from the lower gastrointestinal tract, which, based on histopathologic examination of the large intestine was diagnosed as ulcerative colitis.
In the current case report, we present a severe course3 of type IV TAK in a patient predisposed to autoimmune reactions, including a SARS-CoV-2 infection4 during ongoing treatment with etanercept; this was inexplicable because of the proven therapeutic efficacy of tumor necrosis factor α inhibitors in TAK.5 There had been limited reports on patients predisposed to autoimmune reactions in whom the use of etanercept possibly provoked TAK.6
Jacek Lewandowski, MD, PhD, Department of Internal Medicine, Hypertension and Vascular Diseases, Medical University of Warsaw, ul. Banacha 1A, 02-097 Warszawa, Poland, phone: +48 22 599 28 28, email: jacek.lewandowski@wum.edu.pl
October 12, 2023.
November 9, 2023.
November 14, 2023.
None.
None.
None declared.
Łoń I, Leśnowolski Ł, Janiec I, et al. Severe course of type IV Takayasu disease during treatment with etanercept. Pol Arch Intern Med. 2024; 134: 16606. doi:10.20452/pamw.16606
- 1.
- Bhandari S, Butt SRR, Ishfaq A, et al. Pathophysiology, diagnosis, and management of Takayasu arteritis: a review of current advances. Cureus. 2023; 15: e42667.Crossref
- 2.
- Grayson PC, Ponte C, Suppiah R, et al; DCVAS Study Group. 2022 American College of Rheumatology / EULAR classification criteria for Takayasu arteritis. Ann Rheum Dis. 2022; 81: 1654-1660.Crossref
- 3.
- Quan E, Zhao Y. Acute left heart failure caused by Takayasu arteritis: a case report and literature review. Cardiol Young. 2023; 33: 1777-1780.Crossref
- 4.
- Lázaro Mendes J, Venade G, Manuel P, et al. Virus and autoimmunity: can SARS-CoV-2 trigger large vessel vasculitis? Eur J Case Rep Intern Med. 2022; 9: 003486.Crossref
- 5.
- Mekinian A, Biard L, Dagna L, et al. Efficacy and safety of TNF-α antagonists and tocilizumab in Takayasu arteritis: multicentre retrospective study of 209 patients. Rheumatology (Oxford). 2022; 61: 1376-1384.
- 6.
- Kteleh T, Khromachou T, Alfata S, Mouchli A. Development of Takayasu arteritis despite existing treatment of etanercept and questionable paradoxical effect: case report and review of literature. J Med Cases. 2010; 1: 37-38.Crossref