I. Etiology and pathogenesis.
Aortitis, also known as “Takayasu arteritis”, mainly involves the aorta and its primary branches and the pulmonary arteries. The etiology of myocardial damage can be divided into primary and secondary. The former is cytotoxic and immune complex-mediated myocardial damage, while the latter includes hypertension, pulmonary hypertension, aortic valve lesions, coronary artery lesions, and other secondary causes of myocardial damage.
II. Clinical features.
In addition to the clinical manifestations of ischemia and hypoxia of the corresponding organs due to stenosis of the aorta and its primary branches (e.g., dizziness, headache, syncope, intermittent claudication, etc.), symptoms of cardiac insufficiency such as chest tightness and shortness of breath may also occur. Anti-endothelial cell antibodies (AECA) are of some significance.
III. Diagnosis.
Meets criteria 1+2, 3 or 3. criteria 1+2, 4 or 4. criteria 1+2, 3, 4 or 3, 4. diagnosis can be made after excluding other secondary cardiomyopathies.
1. Meet the 1990 American College of Rheumatology (ACR) classification criteria for the diagnosis of aortitis.
2. Clinical manifestations such as chest tightness, shortness of breath, dyspnea, and significantly elevated brain natriuretic peptide (BNP).
3, Echocardiography suggests myocardial hypertrophy, ventricular dilatation, abnormal myocardial echo and decreased left ventricular ejection fraction (LVEF <50%). And exclude cardiomyopathy secondary to hypertension, severe aortic stenosis obstruction, heart valve lesions, coronary artery lesions and pulmonary hypertension. < span="">
4. Myocardial biopsy pathology: mild disorganization of myocardium, hypertrophy of some myocardial cells, enlarged myocardial cell nuclei, vacuolar-like degeneration of some myocardial cells, and a large number of lymphocyte infiltrates were seen.
IV. Risk stratification for sudden death prevention.
Decreased LVEF, worsening NHYA classification, hyponatremia, decreased peak exercise oxygen consumption, decreased Hct%, widened ECG QRS, chronic hypotension, increased resting heart rate, worsening renal function, inability to tolerate conventional therapy and refractory volume overload may suggest a poor prognosis. In addition, significantly elevated or high BNP and or NT-proBNP levels during hospitalization for heart failure, or a decrease of <30%, are indicative of increased risk of rehospitalization and death.
V. Treatment.
Glucocorticoid combined with immunosuppressant (e.g., cyclophosphamide) therapy is the mainstay, and if heart failure has reached the end-state, it can be performed according to the 2014 Chinese Guidelines for the Management of Heart Failure Diagnosis and Management.
1990 ACR classification criteria for nodular polyarteritis.
1, Age of onset ≤ 40 years: age < 40 years at the onset of symptoms or signs.
2. Intermittent limb claudication: weakness, discomfort or worsening of symptoms in one or more limbs during activity, especially in the upper limbs.
3.Weakness of brachial artery pulsation: Weakness of one or both brachial artery pulsations.
4.Difference in blood pressure >10mmHg: Difference in systolic blood pressure >10mmHg in the upper extremities bilaterally.
5.Subclavian artery or aortic murmur: murmur is heard in one or both subclavian arteries or abdominal aorta.
6.Arteriographic abnormalities: stenosis or occlusion of aortic primary branches or large arteries proximal to the upper and lower extremities; lesions are often focal or segmental and are not caused by atherosclerosis, fibromuscular dysplasia or similar causes.
The disease is diagnosed if three of the six items above are met. It is mainly differentiated from congenital aortic stenosis, atherosclerosis, thrombo-occlusive vasculitis, leukoaraiosis, and polyarteritis nodosa.