Syphilitic aortitis
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(SYMPHILITIC AORTITIS)
Cardiovascular syphilis is associated with the tertiary stage of syphilis infection and manifests 10–20 years after the initial infection, usually during the 4th–5th decades of life . Syphilitic aortitis is reported to occur in 70%–80% of all cases of untreated syphilitic infection and causes marked cardiovascular complications in 10% of affected patients . Histopathologic examination of the aorta usually reveals a sequence of perivascular plasma cell and lymphocytic infiltrates of the aortic adventitia, which results in obliterative endarteritis, adventitial scarring, and medial necrosis, with destruction of the elastic fibers . The resulting weakened aortic wall gives rise to the late vascular complications of syphilis . The three main associated vascular complications include aortic aneurysm, aortic insufficiency, and coronary ostial stenosis . Aortic insufficiency, whether it is due to direct syphilitic involvement of the aortic valve or dilatation of the aortic annulus, is the most common complication and affects 20%–30% of patients with syphilitic aortitis . It can cause heart failure, for which valve replacement may be required. Coronary ostial stenosis is reported to occur in 20%–26% of patients . Unlike atherosclerosis, syphilitic coronary ostial stenosis is a sequela of fibrosis of the aortic media, and lesions distal to the ostia rarely occur . Although ostial involvement causes symptoms of angina, it rarely results in myocardial infarction, because the slow stenosing process allows the formation of collateral vessels . Patients with coronary ostial stenosis are treated most frequently with bypass graft placement or less commonly with coronary endarterectomy and ostial reconstructive surgery . Aortic aneurysms are detected clinically in only 5%–10% of patients, and 50% of them occur in the ascending aorta; 35%, in the aortic arch; and 15%, in the descending thoracic aorta . The majority of these aneurysms are saccular; however, up to one-third of them can be fusiform. It has been hypothesized that the rich lymphatic arrangement and prevalence of vasa vasorum in the ascending aorta predisposes this artery to mesoaortitis . The manifestations of aortic aneurysms are variable and include chest pain; dyspnea; dysphagia; hoarseness; Horner syndrome; venous obstruction, rupture, or dissection; and death .
The differential diagnosis of aortitis is broad and can be categorized into noninfectious and infectious entities. Giant cell arteritis, a medium-size vessel vasculitis, is the most common cause of aortitis in North America and accounts for greater than 75% of all cases. It typically occurs in patients older than 50 years, and affected individuals present with headache, scalp tenderness, and jaw claudication. Giant cell arteritis classically involves superficial cranial arteries—external carotid branches such as the superficial temporal artery in particular. Vertebral, coronary, and mesenteric arteries, as well as the aorta and its branches, also can be involved. Aortic manifestations typically include annuloaortic ectasia or ascending aortic aneurysm. Acute dissection, aortic valve insufficiency, or abdominal aortic aneurysms also may occur. Takayasu arteritis is a large-vessel vasculitis with a predilection (>80% of cases) for young female individuals; it manifests clinically as diminished peripheral pulses, claudication of the extremities, and differential blood pressure of the arms. Radiologic manifestations include narrowing and occlusion of the aorta and its branches. Concentric thickening of the vessel wall, vessel ectasia, aneurysms, ulcers, and wall calcification also may be seen. Takayasu arteritis most typically involves the abdominal aorta, followed by the descending thoracic aorta and aortic arch. Other rheumatic diseases with which there are high (>10%) prevalences of aortic involvement include ankylosing spondylitis, relapsing polychondritis, and Cogan syndrome. Rheumatoid arthritis, Behçet disease, seronegative spondyloarthropathies, and systemic lupus erythematosus also but less commonly involve well-documented aortic complications .
Infectious aortitis is usually secondary to a preexisting aortic pathologic condition, such as atherosclerotic plaque or an aneurysm sac, that is seeded by infectious organisms. Staphylococcus aureus and Salmonella organisms account for 40% of all cases of infectious aortitis. Other pathogens include Mycobacterium tuberculosis, T pallidum, Listeria organisms, Bacteroides fragilis, Clostridium septicum, and Campylobacter jejuni. Typical imaging findings include wall thickening, soft-tissue accumulation, periaortic fluid, and saccular aneurysm or pseudoaneurysm. Air in the aortic wall is an occasional imaging finding. Pyogenic infections are usually the result of endovascular seeding and most commonly involve the abdominal aorta. Tuberculous aortitis typically involves the distal aortic arch and descending aorta and is usually due to direct extension of the infection, such as that from the mediastinal lymph nodes or empyema, or to the hematogenous or lymphatic spread of distant infection
Syphilis is a sexually transmitted infection caused by the bacterium Treponema pallidum subspecies pallidum. The signs and symptoms of syphilis vary depending in which of the four stages it presents (primary, secondary, latent, and tertiary). The primary stage classically presents with a single chancre (a firm, painless, non-itchy skin ulceration) but there may be multiple sores. In secondary syphilis a diffuse rash which frequently involves the palms of the hands and soles of the feet occurs. There may also be sores in the mouth or vagina. In latent syphilis there are little to no symptoms which can last for years. In tertiary syphilis there are gummas (soft non-cancerous growths), neurological, or heart symptoms. Syphilis has, however, been known as "the great imitator" as it may cause symptoms similar to many other diseases.
symptoms
Although is easy to be overlooked, other symptoms of tertiary syphilis might appear such as gummas and symptoms of neurosyphilis (headache, stiff neck, gait abnormality, dementia etc). Additionally, in rare cases, chest pain and shortness of breath might appear as a result of the damage of the aorta and heart valve.
Tretment
Aortitis caused by infection is rare but can be life-threatening, and must be treated promptly with antibiotics. Aortitis caused by other inflammatory conditions or unknown reasons is typically treated with corticosteroids such as prednisone, and medications that suppress the immune system.Treatment of severe syphilitic aortitis is surgery. The diagnosis of syphilitic aortitis is not currently contemplated by the rarity of the case, because the current treatment with antibiotics is largely complete and, if treated properly, is extremely effective.
National Institute of Medical Coding
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