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Takayasu Arteritis -  Ten Amazing Lesser Known Facts 

Dr Sampurna Roy MD   

 

                                              

(a) Active phase of Takayasu arteritis in descending thoracic aorta showing a granulomatous inflammation at the medio-adventitial junction of aorta destroying the structure of an intercostal artery;

(b) chronic phase of TA in descending thoracic aorta showing media (M) at the outer third with clusters of mononuclear cells. Note marked fibrosis of the adventitia (A) (Hematoxylin and Eosin ).

(c) The cells are lymphocytes, and few plasma cells and histiocytes (Hematoxylin and Eosin)

 

                                               

Healed phase of Takayasu arteritis in descending thoracic aorta:

(a) Loose fibrocellular thickening of the intima (I);

(b) junctional area between intima (I) and media (M) showing destruction of the elastic lamellae/smooth muscle with replacement fibrosis;

(c) similar features are also seen toward the outer third of the media (M) and adventitia (A);

(d) fibrotic adventitia (A) showing endarteritis obliterans of a branch (elastic van Gieson )

 

Source:  Vaideeswar P, Deshpande JR. Pathology of Takayasu arteritis: A brief review. Annals of Pediatric Cardiology. 2013;6(1):52-58.

 

 

(1) Takayasu arteritis, a form of granulomatous arteritis, which affects large- and medium-sized arteries, primarily the aorta and its large branches as well as proximal portions of pulmonary, coronary, and renal arteries, is also known as "Pulseless disease" and "Occlusive thromboaortopathy".   

 

(2) Rokushu Yamamoto, who practiced medicine in Japan, published the first description of Takayasu arteritis in 1830.

 

(3) The first scientific presentation was given in 1905 by Mikito Takayasu, Professor of ophthalmology in Japan.

 

(4) The disease is rare, with 5000 cases reported throughout Japan between 1990 and 2000, while a US study put the incidence at 2.6 cases per 1,000,000 inhabitants per year. The incidence in European countries is unknown. 

 

(5) The condition is most commonly seen in Japan, South East Asia, India, and Mexico. Although early reports suggested that the disease was confined to females from Eastern Asia, it is now recognized worldwide and affects both sexes. Disease manifestations vary between regions, and women are affected in 80–90% of cases in the majority cases.

 

(6) The disease commonly presents in the second or third decade of life, with a delay in diagnosis of between months and years from the onset of first symptoms. However, the illness may also present in childhood.

 

(7) In the early phase patients may complain of systemic symptoms including fever, weight loss, malaise, myalgia, carotidynia, and headaches. These symptoms are often insidious and frequently missed, or their significance is overlooked.

 

(8) The later pulseless phase, commonly appearing months or years later, reflects end-organ ischemia . Focal manifestations differ according to the anatomical location of the affected arteries. Symptoms of upper and lower limbs claudication, light headedness, and chest pain predominate. Decreased or absent peripheral pulses, aortic regurgitation, arterial bruits, and both systemic and pulmonary hypertension are common.

 

(9) Histologically, initially, there are mononuclear cell infiltrations in the adventitia and granulomas with Langerhans cells in the media, followed by disruption of the elastin layer and subsequent massive medial and intimal fibrosis.

These lesions result in segmental stenosis, occlusion, dilatation, and aneurysmal formation in the affected vessels.

 

(10) Ishikawa defined the four most important complications of Takayasu arteritis as retinopathy, secondary hypertension, aortic regurgitation, and aneurysm formation, each graded as mild, moderate, or severe. 

 

 

Source

 

Vaideeswar P, Deshpande JR. Pathology of Takayasu arteritis: A brief review. Annals of Pediatric Cardiology. 2013;6(1):52-58.

 

Töpel I, Zorger N, Steinbauer M. Inflammatory diseases of the aorta: Part 1: Non-infectious aortitis. Gefasschirurgie. 2016;21(Suppl 2):80-86.  

 

Perera AH, Mason JC, Wolfe JH. Takayasu Arteritis: Criteria for Surgical Intervention Should Not Be Ignored. International Journal of Vascular Medicine. 2013;2013:618910. 

 

Johnston SL, Lock RJ, Gompels MM. Takayasu arteritis: a review. Journal of Clinical Pathology. 2002;55(7):481-486.

 

 

 

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