What is Oriental belle disease?
Oriental belle disease, which is actually the common name for polyarteritis major, is a chronic progressive nonspecific inflammatory disease involving the aorta and its major branches. In the past, it was also known as pulseless disease, atypical aortic constriction, aortic arch syndrome, and Takayasu’s disease.
Is there a significant difference in the incidence of this disease between men and women?
Since it is called oriental beauty disease, the implication is that the disease is more prevalent in young women in Asia (mostly in China and Japan). According to the literature, the ratio of male to female incidence is about 1:7-8, and the age of onset is 15-30 years old.
What are large arteries?
A large artery is a blood vessel with an internal diameter greater than 5 mm and a wall thickness greater than 1 mm. The heart beats and the large arteries beat together to maintain the blood circulation and perfusion of the organs in the body. The major arteries of body circulation include thoracic aorta, abdominal aorta, iliac artery, large arteries of the extremities (subclavian artery, femoral artery, etc.) and large arteries of organs (renal artery, mesenteric artery, carotid artery, coronary artery, pulmonary artery, etc.). The aorta has a three-layer wall structure, namely the intima, mesoderm, and epithelium, of which the mesoderm, also known as the muscular layer, is rich in a certain percentage of elastic fibers and smooth muscle fibers.
What is the pathogenesis of aortitis in terms of pathology?
According to the pathological study, TA is a total inflammation of the arteries, with a staged distribution, early periarteritis and epiarteritis, and later progressing to the middle layer and intima, with massive lymphocyte and plasma cell infiltration and diffuse fibrous tissue hyperplasia in all layers of the affected arteries, or with destruction and rupture of the middle elastic and smooth muscle fibers. Intimal hyperplasia and edema, proliferation of trophoblastic vessels, granuloma formation, and varying degrees of luminal narrowing may occur, followed by thrombosis and occlusion of the lumen, or with abnormal dilation of the artery behind the narrowed segment or aneurysm formation. Subsequently, the diseased artery has side branch formation.
Is there a clear etiology and pathogenesis of this disease?
The etiology of the disease is still unclear. Possible factors that have been considered include rheumatic diseases (rheumatic fever or systemic lupus erythematosus), infections (tuberculosis or syphilis), genetic factors, and estrogen. The pathogenesis is also unclear. Recently, it is thought that the infection may cause a metamorphic reaction in the blood vessel wall. The increase of alpha and gamma globulin and immunoglobulin G in the patient’s blood and the positivity of the blood for anti-arterial antibodies suggest that the disease may belong to the category of autoimmune diseases.
About the diagnosis of Oriental Beauty disease
What are the specific clinical manifestations of this disease?
The clinical manifestations of polyarteritis major are varied, and it usually takes 10 months from the appearance of first symptoms to a definite diagnosis. In children, hypertension, valvular regurgitation, and congestive heart failure are the main features. It has been suggested in the literature that if children present with hypertension, congestive heart failure and elevated hematocrit, the likelihood of suggesting aortitis is 62%. Adults can generally be divided into pre-pulseless (including stage I: pre-vascular inflammation and stage II: vasculitis exudate) and pulseless (stage III: fibrosis) phases. The pre-pulseless stage is mainly non-specific symptoms, mainly fever, general malaise, loss of appetite, sweating, pale skin, dizziness, headache, arthralgia, myalgia, etc.; the pulseless stage is characterized by symptoms and signs of limb or organ ischemia. According to the different parts of arterial involvement can be divided into the following types.
1, head and arm artery type: lesions involving the subclavian artery, carotid artery, and unnamed artery, clinical fatigue, pain, numbness or coldness in the upper limbs, pain in the facial muscles when chewing, emotional agitation, dizziness, headache, memory loss, easy convulsions, vision loss and transient blackness in front of the eyes; weak or absent pulsations of unilateral or bilateral radial, brachial, axillary, cervical or temporal arteries, and normal pulsations of lower limb arteries. Blood pressure in the upper extremities is undetectable or significantly reduced, or the difference in systolic blood pressure between the upper extremities is >2.67 kPa (20 mmHg), while the blood pressure in the lower extremities is normal or increased. Persistent or systolic murmur can be heard at the site of stenosis.
2.Thoracoabdominal aorta type: The lesion involves the thoracic aorta or/and abdominal aorta, clinically there may be numbness, pain, chill sensation in the lower extremities, easy fatigue, intermittent claudication; those with persistently high blood pressure in the upper extremities may have various symptoms of hypertension, weakening or disappearance of pulsation on one or both sides of the arteries in the lower extremities, undetectable or significantly lower blood pressure, and increased blood pressure in the upper extremities; systolic murmurs may be heard in the abdomen or kidney area, and there may be enlarged left ventricle or signs of acute left heart failure may appear.
3, renal artery type: The lesion involves one or both renal arteries, with persistent, severe or persistent hypertension (systolic blood pressure can be as high as 180-200 mmHg) and various symptoms caused by hypertension, and blood pressure in all four extremities is significantly increased; there may be signs of left ventricular enlargement or left heart failure, and systolic murmurs can be heard in the upper abdomen or renal area.
4.Pulmonary artery type: The milder cases of simple pulmonary artery type may have no obvious symptoms, while the more severe cases may have cyanosis, palpitations, shortness of breath; systolic murmurs in the pulmonary valve area, axillary and dorsal regions, hyperactive second heart sounds in the pulmonary valve area and other manifestations of pulmonary hypertension.
5.Mixed type: The lesion involves two or more groups of vessels mentioned above. Most patients have obvious hypertensive manifestations, and other manifestations vary with the involved vessels.
If this disease is suspected, what tests should be considered first? Are there any specific ancillary tests?
Since the disease is a chronic non-specific inflammatory disease, there are no specific laboratory tests, but important indicators of inflammatory activity, such as rapid blood sedimentation and positive C-reactive protein, are also of great reference value. In some patients, elevated alpha and gamma globulin and immunoglobulin G in the blood and positive anti-big artery antibodies in the blood can be detected. And ultrasonography, CTA, MRA and arteriography (DSA) examinations are necessary to confirm the diagnosis of the disease. Therefore, any patient suspected of this disease should first go to a higher level hospital to check the blood sedimentation, C-reactive protein and other immunological indexes, and if possible, it is better to perform vascular ultrasound or CTA to clarify the vascular condition and also provide reference for future treatment. The figure below shows the typical imaging manifestations of aortitis.
Why should we pay attention to the early diagnosis of aortitis?
Clinical practice shows that if early diagnosis and early treatment are obtained before the vascular lesion enters stage 3 (fibrosis stage), the inflammation of the vessel can be reversed or quiescent without stenosis. Therefore, by early diagnosis we mean that an accurate diagnosis can be made in a timely manner when the pathology is still in the pre-stage 1 vasculitis or stage 2 vasculitis exudation phase.
Why do most cases of aortitis have stenosis or occlusion by the time they are diagnosed?
Because of the chronic onset of the disease and the non-specific nature of the clinical manifestations, for example, in the pre-vascular phase, patients only have non-specific extravascular symptoms such as fever, general malaise, loss of appetite, dizziness, headache, arthralgia, myalgia, rash, etc.; while in the exudative phase of vasculitis, there may be vascular pain and/or tenderness to be identified. Therefore, it is difficult to be detected by us in the early stage of the disease, which brings some difficulties to the diagnosis, especially in rural hospitals with poor medical conditions, if more diagnostic vascular imaging cannot be performed in time, resulting in the disease not being clearly diagnosed in the early stage, and the rate of missed diagnosis is high.
About the treatment of Oriental beauty disease
At present, what are the main means of treatment for aortitis?
For the treatment of TA, it can be divided into non-surgical treatment and surgical treatment at present. Non-surgical treatment, i.e. drug treatment, mainly applies glucocorticoids or immunosuppressants. Surgical treatment includes traditional surgery and the recent rapid development of endoluminal intervention.
When is non-surgical treatment necessary?
Patients with high blood sedimentation and active disease should be treated with glucocorticosteroids for 8-12 weeks, and then gradually reduce the dosage for at least 12 months before considering discontinuing the drug. Immunosuppressive drugs can be added if the results are poor, and surgical treatment can be considered after the inflammatory response is reduced. If the patient is already inactive at the time of consultation, hormones and immunosuppressants are not used. Hormone therapy may also be unnecessary if the blood sedimentation is below 40 mm/h.
What is the appropriate time for surgery?
Generally, it should be after the inflammation has been controlled, the patient has no inflammatory manifestations such as fever and generalized aches and pains, the blood sedimentation and C-reactive protein are normal, and the patient has been stable for at least six months.
Do we have to wait until the active period has passed before surgery can be performed?
No. The timing of surgery should not be too much dependent on whether the disease is in the active phase or not, but should focus on the clinical symptoms, the nature and location of the lesion. If the lesion is critical, such as extensive carotid artery lesions developing to cause significant cerebral ischemia or even complications such as cerebral infarction, or aortic and renal artery obstruction causing intractable hypertension, which can lead to serious cardiovascular and cerebrovascular complications if left untreated, high-dose corticosteroids should be applied while active surgical treatment.
How to choose interventional surgery versus traditional surgery and which one is better?
According to the literature, the long-term prognosis of interventional surgery is not significantly different from that of surgical bypass surgery, and even if the lesion is restenosed or occluded after surgery, balloon dilatation can be performed for the second time to open the stenotic lesion, and endothelial debridement or autologous (artificial) vascular bypass grafting is also feasible for severe occlusive lesions. However, if surgical bypass is performed for the first time, the collateral circulation formed before surgery will gradually become uselessly occluded, which will make secondary surgery very difficult once the ischemic symptoms reappear due to stenosis or thrombosis at the anastomosis. However, even the most skilled interventional surgeons are at their wits’ end when the stenosis is long or multisegmental or when the affected vessel is completely occluded, making it impossible for the guidewire to pass. Therefore, the preoperative evaluation of the disease and the correct analysis of imaging examinations are crucial to the choice of treatment plan. In addition, the application of contrast agents is required in interventional procedures, and interventional procedures may not be performed in patients with acute or chronic renal insufficiency, renal failure or allergy to contrast agents.
What are the main means of interventional treatment?
Currently, interventional treatment for stenosis or occlusive lesions, including aortitis, consists mainly of percutaneous balloon dilatation angioplasty (PTA) and endovascular stenting. Interventional treatment can be applied to all patients with symptomatic aortitis, but the best indication for PTA is a single, limited stenosis in a large or medium vessel. Currently, PTA has been considered the treatment of choice for renal vascular hypertension due to renal artery stenosis. In addition, PTA is also a more appropriate treatment for single limited stenosis in carotid arteries, upper limb arteries, and aorta. However, certain lesion types with high risk, allergy to contrast agents, active arteritis, advanced tumors, and economic factors are relative contraindications.
Why do I need to put stents after PTA?
PTA is effective in narrowed lesions, but there is still a significant gradient effect in patients who only undergo PTA, while the application of stenting can eliminate the gradient effect at the narrowed area and obtain a better intraluminal diameter, and balloon dilation can cause local intimal tears, and if the torn intimal pieces float in the vessel after stenting, it is easy to obstruct blood flow and cause hemodynamic changes and possible secondary thrombosis. And the incidence of thrombosis or stenosis is significantly lower after stenting. PTA is currently recommended for vessels with small stenotic segments, while the addition of stents for vessels with long stenotic segments, chronic complete stenosis and lesions at the arterial opening may improve the success rate, increase the intraluminal diameter and reduce the restenosis rate. The operative technique of intra-arterial stenting is not complicated. On the basis of PTA, a metal mesh self-expanding stent or a ball-expanding stent of appropriate length and diameter is delivered to the stenosis and released after accurate positioning.
What is the efficacy and safety of interventional procedures?
Interventional treatment of aortitis is safe and effective. PTA is effective for limited stenosis of the aorta, with a technical success rate of 90%. The incidence of postoperative restenosis varies depending on the site of the lesion and ranges from 30% to 70%, but can be treated with additional PTA or stent placement. The success rate of stenting is about 99%, and the 5-year patency rate of stenting is about 90%. The total complication rate is 2%-6%, and the main complications include vascular perforation or rupture, hematoma and pseudoaneurysm at the perforation site, arterial entrapment, cerebral infarction, renal infarction, and embolization of the distal end of the treated artery.
What are the current traditional surgical procedures?
1.Endarterectomy with autologous vein piece repair: Applicable to segmental stenosis or occlusion at the beginning of common carotid artery, internal carotid artery, renal artery, etc. The carotid artery should be sutured with an autologous vein piece to enlarge the caliber of the stenotic segment and prevent postoperative stenosis. Since the wall of the diseased artery is hardened with adhesions and the hyperplastic endothelial layer is poorly demarcated, surgical debridement is more difficult, so it is seldom used.
2, revascularization, bypass grafting: also known as bypass surgery, that is, the use of autologous or artificial blood vessels, across the lesion to establish bypass circulation, the specific procedure can be based on the lesion site, the severity of the decision. Revascularization and bypass grafting do not require extensive separation of adhesions, and the procedure is relatively simple. However, there is evidence that 44% of the biopsies performed around the anastomosis are still TA after the anastomosis in the area of vessels with normal angiographic morphology, because the disease mainly affects the intima and damages the elastic fibers, so if the diseased vessel is used as the anastomosis, it is very difficult to operate either with synthetic grafts or autologous veins, and it is prone to serious anastomotic complications, such as aneurysms, with an incidence of 12-20%. In addition, post-operative anastomotic intimal hyperplasia and thrombosis leading to anastomotic restenosis is also one of the important problems that need to be solved in bypass surgery.
3.Autologous kidney transplantation: It is suitable for those who have more lesions above and below the opening of the renal artery in the proximal part of the renal artery and the abdominal aorta in polyarteritis major, and cannot perform renal artery reconstruction.
4.Nephrectomy: including partial nephrectomy and total nephrectomy. The latter is suitable for those who have normal kidney on one side and serious lesions on the other side, and the abnormally high blood pressure can be significantly reduced after removal of the diseased kidney.
In what cases should only traditional surgery be chosen?
Interventional surgery, although less invasive and more effective, is not a panacea after all. Moreover, the current cost of interventional surgery is high, and it is still difficult for most patients to accept such high medical costs. In cases of long stenosis or severe occlusive lesions where interventional surgery may not be possible, or where the patient’s own condition (contrast allergy, renal insufficiency) or economic factors make interventional surgery inappropriate, surgical treatment may be considered depending on the situation.