A 40-year-old woman presented with complaints of a 4-year history of recurrent central chest pain.
Initial Presentation: Recurrent chest pains.
Description: A 40-year-old woman presented with complaints of a 4-year history of recurrent central chest pain. She gave a history of having a prolonged fever for about 3 months accompanied by arthralgia at the age of 13 years, but the fever resolved gradually.
Need more information?
The pain was unrelated to food or exertion, and occurred intermittently during both day and night. The patient had no medical history of diabetes, hypertension, hypercholesterolaemia or hypothyroidism. She did, however, give a history of having a prolonged fever for about 3 months accompanied by arthralgia at the age of 13 years. She said that at the time she had extensive investigations for infections and possible malignancies—but the fever resolved gradually without any cause being identified. She said that she had had no further problems until the current complaint began 4 years ago.
What is the most likely diagnosis?
- Hyperparathyroidism
- Idiopathic hypercalcaemia
- Aortic aortitis
- Hyperlipidaemia
Figure legend. Chest x-ray (A) and CT angiogram (D) show calcification (indicated by arrows in A). MRI angiograms (B and C) show narrowing of the aorta and features suggestive of active inflammation (indicated by arrows).
Answer: Aorta arteritis
Breakdown: On clinical examination, her blood pressure was 130/90 mm Hg in both upper limbs, pulses were easily palpable, and there were no bruits or murmurs heard on auscultation. A chest x-ray showed calcification of the wall of the aortic arch and descending thoracic aorta, with narrowing at the level of the diaphragmatic hiatus (figure). Full blood count, erythrocyte sedimentation rate, C-reactive protein, lipid profile, and parathyroid hormone levels were normal.
MRI angiograms showed narrowing of the aorta in the post-subclavian area, in the descending thoracic aorta at the level of the diaphragm, and at the level just above the origin of the left renal artery (figure); T2-weighted images showed a thickened and oedematous thoracic aorta, suggesting active inflammation (figure), and a CT angiogram showed extensive calcification (figure). Taken together these findings led us to making a diagnosis of aorta arteritis type III with active disease.
Takayasu arteritis or aorto-arteritis is a large vessel vasculitis of the aorta and its branches. The disease may manifest itself with only non-specific features like fever, myalgia, arthritis, and malaise during the acute phase, which can last for several months. In the chronic phase, in the absence of clinical features secondary to vessel occlusion or ischaemia, the diagnosis maybe further delayed. However, disease activity may persist in the aortic wall despite the patient being asymptomatic and all inflammatory markers remaining within normal limits. A high index of suspicion is required to make the diagnosis during the acute phase, and regular monitoring is necessary to avoid serious long-term complications.
The disease takes its name from Mikito Takayasu, who described the first case in 1908 at the Annual Meeting of the Japan Ophthalmology Society.
Source: All content for this “Diagnostic Puzzle” was sourced from The Lancet. The article was written by Mahesh Janarthanan, MD and Jebaraj Rathinasamy, MD
Original article is available at:
https://www.thelancet.com/article/S0140-6736(18)31997-4/fulltext
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