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PubMed1983

Surgical considerations in aortitis. Part III: Syphilitic and other forms of aortitis.

The majority of cases involving clinically significant aortitis result from Takayasu's disease, syphilis, and mycotic aneurysms, although aortitis may occur as a part of the clinical manifestation in other diseases of known and unknown etiology. Syphilitic aortitis can be treated successfully if an accurate diagnosis is made early and if appropriate antibiotic therapy is instituted. Untreated or treated inadequately, late cardiovascular complications can occur, namely, aortic aneurysm, aortic valvular insufficiency and coronary osteal stenosis. Surgical treatment should be considered when any of these late complications become manifest. Aortitis can occur with rheumatic fever and, occasionally, in rheumatoid arthritis. Giant-cell arteritis is a disease that can affect large, medium or small arteries, and usually occurs in patients over 50 years of age, chiefly women. The entire aorta may be affected. It is the only form of aortitis commonly associated with dissecting aneurysm of the aorta. Corticosteroids are effective in treating the active form of the disease and can usually prevent the development of later severe complications. Surgery is indicated when a dissecting aneurysm develops.

Journal Article↗
PubMed2000

Study of 52 patients with idiopathic aortitis from a cohort of 1,204 surgical cases.

OBJECTIVE: To determine 1) the frequency of idiopathic aortitis in a large surgical cohort, 2) how often aortitis was associated with a systemic disease, and 3) whether the findings of aortitis in resected specimens predicted future occurrences of clinically apparent vascular injury due to vasculitis. METHODS: Retrospective chart and pathology review of 1,204 aortic surgical specimens that were gathered over a period of 20 years at a tertiary care medical center. A standardized database was used to compare features of aortitis patients with those of controls in whom inflammation was not present. RESULTS: Among 1,204 aortic specimens, 52 (4.3%) were clinically and pathologically classified as idiopathic aortitis. Sixty-seven percent of patients with idiopathic aortitis were women. In 96% of idiopathic aortitis patients with aneurysm formation, aortitis was present only in the thoracic aorta. Among 383 thoracic aortic aneurysms, 12% had idiopathic inflammatory features. In 96% of patients with idiopathic aortitis, symptoms of systemic illness had not been present at the time of surgery. In 31%, aortitis was associated with a remote history of vasculitis and a variety of other systemic disorders. During a mean followup period of 41.2 months, new aneurysms were identified among 6 of 25 patients who were not treated with glucocorticoids. None were identified among 11 patients who were treated with glucocorticoids (mean followup 35.5 months). CONCLUSION: The frequency of idiopathic aortitis in a large surgical cohort was found to be 4.3%. Thoracic aorta aneurysm formation, in the absence of systemic illness, was the most common manifestation. In the setting of a cardiovascular surgery practice, aortitis may first become apparent only after pathologic evaluation of excised specimens. The appropriate medical treatment for patients with incidentally discovered aortitis is not known. Because 17% of our patients subsequently developed new aneurysms, we suggest that it would be prudent for patients with idiopathic aortitis identified at the time of surgery to be periodically evaluated for recurrent or persistent disease.

Adult↗
PubMed1989

Rheumatoid aortitis: a rarely recognized but clinically significant entity.

Aortitis as a feature of rheumatoid arthritis is considered rare. We have, however, identified 10 patients with aortitis from among 188 consecutive autopsy cases of rheumatoid arthritis. There were 5 men and 5 women with a mean duration of rheumatoid arthritis of 9.6 years. Nine were rheumatoid factor positive and had associated nodules. In addition to standard treatment regimens, 9 patients received corticosteroids. Although involvement of the thoracic aorta was most common, involvement of both the thoracic and abdominal aorta was present in 4 cases. Two patients had aneurysmal dilatation of the thoracic aorta and 1 of the abdominal aorta. Microscopic features of aortitis included necrosis of medial smooth muscle and elastica, with an inflammatory infiltrate comprising primarily lymphocytes and plasma cells. A panmural aortitis was seen in 3 cases. Rheumatoid granulomas were noted in the aortic wall in 5. The diagnosis of aortitis was not made until autopsy in any case. Aortitis was hemodynamically significant in 3 patients. Two had congestive heart failure secondary to thoracic aortitis and aortic valvulitis, and 1 had rupture of an abdominal aortic aneurysm at a site involved by aortitis. Seven patients had rheumatoid vasculitis with a mean of 10 organs involved. Six of these died of complications directly related to vasculitis, including 4 patients with coronary arteritis and associated myocardial infarction. Aortitis can be a feature of severe rheumatoid arthritis and is often associated with rheumatoid vasculitis. Hemodynamic compromise does occur and may be fatal.

Aged↗
PubMed2005

Type IV thoracoabdominal aortic aneurysm with lymphoplasmacytic aortitis and cystic medial degeneration in a 32-year-old patient with Marfan syndrome.

Aortitis identified in approximately 12% of all thoracoabdominal aneurysms. The most common subtype of inflammatory aortitis is giant cell aortitis, followed by lymphoplasmacytic aortitis. Inflammatory aortitis may occur in isolation or as part of a systemic inflammatory disorder such as Takayasu arteritis, systemic lupus erythematosus, rheumatoid arthritis, and giant cell arteritis. Aortitis has not been described in patients with Marfan syndrome. We report the case of a 32-year-old man with Marfan syndrome and a strong family history of aneurysmal disease who presented with an asymptomatic Crawford type IV thoracoabdominal aneurysm. His aneurysm had no associated dissection, and surgical pathology revealed severe medial degeneration and lymphoplasmacytic aortitis. To our knowledge, this is the first report of such a finding in a patient with Marfan syndrome.

Adult↗
PubMed2004

[Inflammatory aortitis].

PRINCIPLE AND OTHER CAUSES: Takayasu's arteritis, giant cell arteritis and Behçet's disease are the three main causes of inflammatory aortitis. More rarely, aortitis can be observed in Cogan's syndrome, atrophic polychondritis, sarcoidosis, ankylosing spondylitis and in rheumatoid arthritis. RISKS OF PROGRESSION: Takayasu's arteritis is distinct with the development of stenotic lesions of the aorta. With the other causes, aortitis can be complicated by ectasia or even aneurysm, with the risk of rupture. Indeed, during giant cell arteritis, patients are 17 times more likely to develop thoracic aortic aneurysm. Aortic regurgitation is a frequent complication of inflammatory aortitis. Sometimes, aortitis is only manifested by general signs such as fever or an inflammatory syndrome. SUPPLEMENTARY EXPLORATIONS: Recent advances in diagnosis and follow-up of patients with inflammatory aortitis concern the use of non-invasive imaging techniques: Doppler ultrasonography, computed tomography with injection of a contrast product and magnetic resonance imaging, which currently replace the aortography. DIAGNOSTIC PROBLEMS: Infectious aortitis, inflammatory atheromatous aneurysm and retroperitoneal fibrosis are sometimes misleading differential diagnoses.

Aortitis↗
PubMed1999

Surgical treatment for aortic regurgitation caused by non-specific aortitis.

Aortic regurgitation caused by non-specific aortitis is relatively rare, and prosthetic valve detachment after aortic valve replacement has been reported to be one of the most serious complications. The authors investigated the surgical results in patients with aortic regurgitation caused by non-specific aortitis. Between 1978 and 1997, 90 patients with aortic regurgitation secondary to non-specific aortitis underwent surgery. There were 28 males and 62 females. The age of these patients ranged from 19 years to 70 years (mean, 48 years). Sixty-five patients were diagnosed to have Takayasu's arteritis, 10 had Behçet's disease and 15 had aortitis of unknown etiology. The surgical procedures for aortic regurgitation were aortic valve replacement in 62 patients, Bentall-type operation in 27 and remodeling in one. Five patients (5.6%) died during the hospital stay. The follow-up duration ranged from 1 month to 224 months (mean, 98 months). Twenty-one patients died during the follow-up period, and the actuarial survival rate was 83% at 5 years and 68% at 10 years. Prosthetic valve detachment occurred in 12 patients (13.3%). Two patients required a re-aortic valve replacement, eight had a composite graft replacement and two patients underwent a homograft reconstruction. Absence of prosthetic valve detachment was noted in 83% of the patients at 5 years and in 75% at 10 years. The incidence of prosthetic valve detachment was 40% (4/10) in patients with Behçet's disease, 33% (5/15) in aortitis of unknown etiology, 4.6% (3/65) in Takayasu's arteritis and 29% (8/28) in male patients. Surgical techniques for treatment of this condition should be modified for further improvement of the surgical outcome in these patients with Behçet's disease and aortitis of unknown etiology.

Adult↗
PubMed2005

Involvement of tumor necrosis factor-alpha in the development of T cell-dependent aortitis in interleukin-1 receptor antagonist-deficient mice.

BACKGROUND: Interleukin-1 receptor antagonist-deficient (IL-1Ra(-/-)) mice on the BALB/c background spontaneously develop inflammatory arthropathy that resembles rheumatoid arthritis in humans. These mice also frequently develop aortitis at the root of the aorta, but the mechanism underlying the development of this disease has not been completely elucidated. METHODS AND RESULTS: Using IL-1Ra(-/-) mice (backcrossed 8 generations to the BALB/c background) and wild-type mice, we studied the histopathology and examined the immunologic mechanisms involved in the development of aortic inflammation by cell transplantation experiments. Half of the IL-1Ra(-/-) mice developed aortitis at the root of the aorta, with massive infiltration of macrophages and monocytes and loss of elastic lamellae in the aortic media. Left ventricular hypertrophy and mild aortic stenosis were also shown by transthoracic echocardiography. Transplantation of T cells from IL-1Ra(-/-) mice induced aortitis in recipient nu/nu mice. Bone marrow cell transplants from IL-1Ra(-/-) mice also induced aortitis in irradiated wild-type recipient mice. Furthermore, tumor necrosis factor (TNF)-alpha deficiency completely suppressed the development of aortitis in IL-1Ra(-/-) mice, whereas IL-6 deficiency did not affect pathology. CONCLUSIONS: These observations suggest that IL-1Ra deficiency in T cells activates them excessively, resulting in the development of aortitis in IL-1Ra(-/-) mice in a TNF-alpha-dependent manner.

Animals↗
PubMed1993

Cellular immunocompetence in aortitis syndrome.

Cellular immunocompetence was investigated in 17 cases of aortitis syndrome (3 active, 14 inactive stage). Both the active and inactive groups demonstrated significantly lower interleukin-2 (IL-2) production than healthy volunteers. The active aortitis syndrome group produced significantly more interleukin-1 beta (IL-1 beta) than the inactive group. The proportion of CD11b+ CD8+ cells was significantly lower in the active aortitis syndrome group. Further, the proportions of CD11b- CD8+ cells and CD57+ CD16- cells in the aortitis syndrome patients were significantly higher than the healthy volunteers. These results suggest that there are intrinsic qualitative abnormalities in the T cells that produce IL-2 in aortitis syndrome. Pathogenesis of aortitis syndrome is considered as follows: during the active stage, diminished IL-2 production impairs differentiation and proliferation of suppressor T cells, thus creating abnormalities in the inhibitory functions of immunoregulation and promoting the proliferation of cytotoxic T and natural killer (NK) cells. This presumably initiates inflammation of the aorta and/or artery.

Adult↗
PubMed1982

[A case of aortitis syndrome associated with hypertensive intracranial hemorrhage (author's transl)].

A case of aortitis syndrome associated with hypertensive intracranial hemorrhage is reported. A 38-year-old female was admitted in 1980 suffering from hypertension and blood pressure difference between the left and right arms which had existed for 10 years. Two weeks prior to admission, she noticed weakness and numbness of the right arm and leg. She was orientated and alert at the time of admission. Her right upper and lower extremities could not function with full strength against resistance. Also, right hemihypesthesia and hemiparesthesia were present. Left arm systolic blood pressure was 195 mmHg. Her pulse was palpable in both radial arteries. CT scans revealed a small high density area in the posterior crus of the internal capsule with a slight mass effect. There was no ventricular dilatation. She was given conservative treatment, such as with antihemorrhagic and antihypertensive drugs, and was discharged with good recovery after three months. Various studies were performed during her hospital stay. Based on angiographic findings, the most probable diagnosis was an extensive type of aortitis syndrome. Hypertension resulted from narrowing of the abdominal aorta and the right renal artery. Pulmonary scintiscanning using 99mTc-macroaggregated albumin revealed that her pulmonary arteries were also involved. Aortitis syndrome is not infrequent, but intracranial hemorrhage following after aortitis syndrome is rare. This may be because the carotid artery is involved in a high incidence of such cases, and therefore the intracranial blood flow is disturbed in most cases. Although the left common carotid artery was involved in the subject case, hypertensive intracranial hemorrhage occurred in the left posterior crus of the internal capsule. In a case of aortitis syndrome, therapy resulting in recirculation to the carotid artery involved is generally carried out in order to improve poor intracranial blood flow. However, it is suggested that control of blood pressure is necessary to prevent intracranial hemorrhage in aortitis syndrome.

Adult↗
PubMed2003

Aspergillus aortitis after cardiac surgery.

OBJECTIVES: The aim of this study was to describe the clinical characteristics of Aspergillus aortitis in a small series of consecutive patients. BACKGROUND: Aspergillus infection of the ascending aorta after cardiopulmonary bypass surgery has rarely been reported and has always resulted in death. METHODS: Aspergillus aortitis was confirmed by pathologic and microbiologic analysis in eight men (61 +/- 8 years) of 9,375 consecutive patients who underwent cardiac surgery between 1975 and 2000. RESULTS: Patients presented with Aspergillus aortitis after aortic valve replacement (n = 5), coronary revascularization (n = 2), or both (n = 1). Initial symptoms appeared between the immediate postoperative period and up to two years after surgery. All patients had prolonged fever. Ante-mortem diagnosis was established in only three patients for whom transthoracic echocardiography was suggestive of aortic pseudoaneurysm and was confirmed by thoracic computed tomography or aortography. All patients had negative peripheral blood cultures. Seven patients died at short-term follow-up, and the one surviving patient was promptly treated by surgery and antifungal drugs. Pathologic examination confirmed Aspergillus aortitis with multi-organ dissemination without heart involvement in all patients except for two, in whom aortic valve endocarditis was found. Fungal cultures confirmed the presence of Aspergillus fumigatus in all patients. CONCLUSIONS: Aspergillus aortitis is typically found after aortic valve or coronary surgery. It commonly leads to lethal multi-organ dissemination without involvement of the intracardiac structure. This entity should be considered in patients with persistent fever and negative blood cultures after open-heart surgery involving significant aortic wall damage, irrespective of the postoperative period.

Adult↗
PubMed1996

Lupus aortitis: a case report and review of the literature.

Systemic lupus erythematosus is a complex immunological and rheumatological disease that has numerous complications. Central to the pathogenesis of systemic lupus erythematosus is immune complex formation and deposition in blood vessels and end organs. This is a case report of an autopsy of a patient with systemic lupus erythematosus, end stage renal disease, peripheral vascular occlusive disease, pancreatitis, and aortitis. The aortitis was found to be immune complex mediated with deposition of IgG, C3, as well as fibrinogen in the wall of the aorta as shown by immunofluorescence. The hypercoagulable state of the patient is discussed with particular emphasis on the role of anticardiolipin antibodies, antiphospholipid antibodies, and anti-beta-2-glycoprotein I in the pathogenesis. This case is unique in that the immune complex mediated aortitis has not been described in the literature over the past 25 years. We recommend that the diagnosis of immune complex mediated aortitis be considered in the differential diagnosis of aortitis, particularly in the background of a patient with systemic lupus erythematosus.

Adult↗