Arteritis or idiopathic arterial calcification of infancy.
Explore the source record for details and available documents.
Aortitis↗
Biomedical subjects
J T Lie
Publications and source records attributed to J T Lie.
Pathologic features of Behçet's syndrome: a review of Japanese autopsy registry data.
The data recorded from 170 autopsies of patients with Behçet's syndrome in Japan during the period from 1961 to 1976 were analyzed. The patients had been in the second to the eighth decade of life, and the ratio of males to females was 5 to 2. A wide spectrum of pathologic findings was observed, with involvement of neurologic, ophthalmic, cardiovascular, pulmonary, gastrointestinal, visceral, genitourinary, and mucocutaneous systems. Some of the common acknowledged clinical features of the syndrome, such as oral ulcers, synovitis, retinal and cutaneous vasculitis, and venous occlusions, were underreported. This apparent discrepancy in an autopsy series may be due to the effects of treatment or the healing process, as well as to the possible incompleteness of the postmortem examination. The accessibility of recorded data in a national autopsy registry offers a unique opportunity for review of the pathologic features of Behçet's syndrome.
Adult↗
Takayasu arteritis. A study of 32 North American patients.
Thirty-two patients (26 female, 6 male) with angiographically diagnosed Takayasu arteritis were seen at the Mayo Clinic between 1971 and 1982. Racial composition of this group was 23 North American Caucasians, 4 Mexicans, 3 Orientals, 1 Native American, and 1 patient of Middle Eastern origin. Incidence of the disease in Olmsted County, Minnesota, was 2.6/million/year. Diagnosis was often delayed for long periods of time, with a median delay of 18 months. Patients had both non-vascular symptoms (arthralgias in 56%, fever in 44%, weight loss in 38%) and symptoms of vascular stenosis such as arm claudication (47%) and hypertension due to renal artery stenosis (41%). All patients had either multiple vascular bruits (94%) or absent pulses (50%). Laboratory findings included anemia (44%) and elevations of erythrocyte sedimentation rate (78%). Almost all patients had multiple sites of arterial involvement documented by angiogram with various combinations of stenosis, luminal irregularity and aneurysm formation. Response to corticosteroid treatment was usually very good, with dramatic improvement in non-vascular symptoms and return of pulses in 8 of the 16 patients with absent pulses prior to treatment. Five-year survival rate from time of diagnosis was 94%. Twelve patients underwent surgical procedures involving the carotid arteries (5 cases), subclavian artery (4 cases) and renal arteries (3 cases). Three aneurysms were resected, one had aortic valve replacement for severe aortic regurgitation, and two patients underwent transluminal angioplasty. Pathologic changes were restricted to the media and adventitial layers of the vessel wall and were indistinguishable from those of giant-cell or temporal arteritis. Takayasu arteritis is more common than previously suspected in North America, is not restricted to any one racial group, and is readily treatable with corticosteroids and surgical vascular reconstruction.
Adolescent↗
Pulmonary peliosis.
Peliosis hepatis is an uncommon vascular lesion of unknown etiology, and its occurrence has been linked frequently to therapy with androgenic-anabolic steroids. Extrahepatic peliosis is ever rarer. It is found most commonly in the spleen and usually in conjunction with peliosis hepatis. Documented here is an unusual case of pulmonary peliosis, associated with peliosis hepatis and peliosis of the spleen, in a 40-year-old man who had Fanconi's anemia and who received long-term therapy with fluoxymesterone and methyltestosterone. To our knowledge, this represents only the second reported case of pulmonary peliosis in the English literature.
Adult↗
Inflammatory abdominal aortic aneurysms: a thirty-year review.
The operative records of 2816 patients undergoing repair for abdominal aortic aneurysm (AAA) from 1955 to 1985 were reviewed. Inflammatory aortic or iliac aneurysms were present in 127 patients (4.5%), 123 men and four women. Most patients were heavy smokers (92.1%). Clinical evidence of peripheral arterial occlusive disease and coronary artery disease was found in 26.6% and 39.4%, respectively. Additional aneurysms occurred in half of the patients; iliac aneurysms were the most common (55 patients), followed by thoracic or thoracoabdominal (17 patients), femoral (16 patients), and popliteal aneurysms (10 patients). Ultrasound and computed tomography suggested the diagnosis in 13.5% and 50%, respectively; angiography was not helpful. Excretory urographic findings of medial ureteral displacement or obstruction suggested the diagnosis in 31.4%. The aneurysm was repaired in 126 patients. Only one patient experienced acute aneurysm rupture, but eight patients had chronic contained leakage. When compared with patients who have ordinary atherosclerotic aneurysms, patients with inflammatory aneurysms are significantly more likely to have an elevated erythrocyte sedimentation rate (ESR, 73% vs. 33%, p less than 0.0001); weight loss (20.5% vs. 10%, p less than 0.05); symptoms (66% vs. 20%, p less than 0.0001); and an increased operative mortality rate (7.9% vs. 2.4%, p less than 0.002). The triad of chronic abdominal pain, weight loss, and elevated ESR in a patient with an abdominal aortic aneurysm is highly suggestive of an inflammatory aneurysm and may be beneficial in the preoperative preparation of the patient for aneurysm repair.
Aged↗
Catecholamine-secreting paraganglioma of the interatrial septum.
A 29-year-old woman was found to have clinical and biochemical evidence of a functioning paraganglioma (pheochromocytoma) five years after the onset of symptoms. Despite extensive investigation of known potential sites of paragangliomas (including laparatomy), the suspected tumor eluded localization. Adrenergic blocking agents controlled her blood pressure but did not affect the other manifestations of catecholamine excess. Ischemic perforation of the colon and refractory gram-negative sepsis resulted in death. At autopsy, a large paraganglioma was found within the heart; the unique tumor location was an important factor in the fatal clinical course.
Adult↗
Pulmonary lymphangiomyomatosis: with particular reference to steroid-receptor assay studies and pathologic correlation.
The symptoms of progressive dyspnea, hemoptysis, spontaneous pneumothorax, chylous effusions, and cough in conjunction with ventilatory obstruction and abnormal gas exchange in a young woman should prompt the diagnosis of pulmonary lymphangiomyomatosis. Cytosol steroid-receptor assays and postmortem studies were conducted in an extensive investigation of a case of this disease. A biopsy specimen of the lung disclosed evidence of nuclear translocation of [3H]progesterone and the presence of a cytosolic receptor for progesterone, an indication that this disease could be treated effectively with progestin.
Adult↗
Constrictive pericarditis associated with combined idiopathic retroperitoneal and mediastinal fibrosis.
Idiopathic retroperitoneal fibrosis and mediastinal fibrosis are localized expressions of a systemic sclerosing disease of unknown cause which, on rare occasions, may coexist in the same patient. Although pericardial involvement may occur, recurrent constrictive pericarditis that is unrelieved by pericardiectomy has not previously been reported in association with either idiopathic retroperitoneal or mediastinal fibrosis. Reported herein is a case of recurrent constrictive pericarditis that was unrelieved by two pericardiectomies , and autopsy revealed unsuspected combined idiopathic retroperitoneal and mediastinal fibrosis. The clinical, echocardiographic, and pathologic findings are described.
Echocardiography↗
Clinical significance of histopathologic patterns of cardiac amyloidosis.
Cardiac amyloidosis may be asymptomatic or an important cause of progressive heart failure and refractory arrhythmia. To identify the morphologic markers of clinically significant cardiac amyloidosis, we analyzed the hearts of 47 patients with autopsy-proven cardiac amyloidosis (21 with primary amyloidosis [AL] and 26 with senile cardiac amyloidosis [SCA]) histologically for the extent and pattern of amyloid deposits. The extent of amyloid deposition was graded 1 through 4, corresponding with less than 10%, 10 to 25%, 26 to 50%, and more than 50% histologic involvement of the myocardium, respectively. The pattern of deposits was classified as nodular, perifiber, or mixed type, and the presence or absence of vascular involvement was determined. The hearts with primary amyloidosis showed predominantly high-grade deposits (76% grades 3 and 4), a perifiber (65%) or mixed (30%) pattern of deposits, and frequent (90%) vascular involvement. The hearts with senile cardiac amyloidosis tended to have low-grade deposits (62% grades 1 and 2), a nodular pattern (92%) of deposits, and infrequent (4%) vascular involvement. Clinically significant cardiac amyloidosis was associated with grade 2 or greater amyloid deposits in the heart and with involvement of intramyocardial arterioles.
Adult↗
Changing incidence of abdominal aortic aneurysms: a population-based study.
The apparent incidence of abdominal aortic aneurysms among Rochester, Minnesota residents increased seven-fold between 1951 and 1980, while the incidence of thoracic aortic aneurysms declined somewhat. Rates for abdominal aneurysms rose with age and were greater among men. The overall incidence in 1971-1980 was 36.5 per 100,000 person-years. While all clinical classes of abdominal aortic aneurysms became more frequent, the greatest rise in incidence was for small, asymptomatic, and uncomplicated aneurysms which suggested an important role for more complete case ascertainment in recent years. The secular trend in abdominal aortic aneurysm incidence seems to be different from that observed for stroke or for coronary heart disease in the same community.
Adult↗
Continued high incidence of coronary artery disease at autopsy in Olmsted County, Minnesota, 1950 to 1979.
The autopsy records of all people over 30 years old who died and underwent autopsy in Olmsted County from 1950 through 1979 were reviewed (n = 5558, autopsy rate approximately 50%). The hearts of 530 subjects were reexamined for the severity of coronary artery disease and this determination was compared with the recorded grade. After both record and specimen the three major coronary arteries were graded according to percent reduction in luminal area (1 = 0 to 25%; 2 = 26% to 50%; 3 = 51% to 75%; 4 = 76% to 99%; 5 = occlusion). Grades 4 and 5 were designated as significant coronary disease (SCD), and 94% to 99% of diagnoses of SCD were confirmed on reexamination. The percentage of people with SCD increased during the period 1950 to 1969 and remained high in the 1970s. Cohort analysis showed an increase in the disease in all age groups (except in the 30- to 49-year-old group for the later decades of birth). There was no change in the prevalence of myocardial infarction scars over the study period.
Adult↗
Clinical and prognostic significance of vasculitis as an early manifestation of connective tissue disease syndromes.
The courses of 18 patients with arthritis and vasculitis in the first 2 years after onset of disease (mean follow-up, 54 months) were studied. The patients were categorized as having rheumatoid vasculitis, systemic vasculitis, and undifferentiated connective tissue syndrome. These patients cannot be distinguished on basis of organ involvement by vasculitis or histopathologic findings on biopsy, but can be separated clinically by the extent of joint involvement and the presence or absence of rheumatoid factor and antinuclear antibody. Early onset of vasculitis is associated with a poor outcome, especially in patients with rheumatoid arthritis, with rapid progression to vasculitic involvement of the viscera, resulting in death. On the basis of the 54-month follow-up period involving this selected series, the prognosis of patients with systemic vasculitis and undifferentiated connective tissue syndrome appears more favorable than that of patients with rheumatoid vasculitis.
Adult↗
Pathology of amyloidosis and amyloid heart disease.
Amyloidosis is a family of disorders of the immune system. Each member of the family is characterized clinically by a unique syndrome and chemically by a specific amyloid fibril protein. The diagnosis of amyloidosis requires histopathologic identification of amyloid deposits in the affected tissues. Since none of the commonly used histochemical stains is specific for amyloid and their sensitivity is variable, application of a battery of staining methods (two or more) is essential for the histologic diagnosis of amyloidosis. The heart may be affected in any form of systemic amyloidosis and in senile amyloidosis. Cardiac amyloidosis is an important cause of progressive heart failure and refractory arrhythmia of obscure origin, especially in elderly persons. The average survival time of amyloid heart disease after the onset of symptoms is less than 3 years. Clinically, amyloid heart disease may mimic constrictive pericarditis, coronary artery disease, valvular heart disease, and idiopathic hypertrophic or congestive cardiomyopathy. A confirmatory biopsy is needed for diagnosis since cardiac amyloidosis has no pathognomonic symptoms and signs, nor diagnostic electrocardiographic, radiologic, cardioangiographic and echocardiographic findings.
Amyloid↗
The therapeutic impact of temporal artery biopsy.
To evaluate the clinical usefulness of temporal artery biopsy in the diagnosis of giant-cell arteritis we followed up all 134 residents of Olmsted County, Minnesota, who had temporal artery biopsies between 1965 and 1980. Initial biopsies were positive for giant-cell arteritis in 46 cases and negative in 88. A history of jaw pain or claudication and the findings of a palpably abnormal temporal artery were significantly more common in the patients whose biopsy specimens showed giant-cell arteritis. Over a median follow-up period of 70 months (range 1-192) only 8 of the 88 biopsy-negative patients had clinical courses requiring long-term, high-dose corticosteroid therapy for giant-cell arteritis. In this population-based study temporal artery biopsy correctly predicted the subsequent need for corticosteroid therapy in 94% of cases: these findings indicate that biopsy should be done before patients are committed to long-term corticosteroid therapy.
Adrenal Cortex Hormones↗
Occurrence of pheochromocytoma in Rochester, Minnesota, 1950 through 1979.
For the 30 years from 1950 through 1979, the population of Rochester, Minnesota, averaged 45,800, resulting in almost 1.4 million person-years of observation. During that time, 11 cases of pheochromocytoma were diagnosed in this population; thus, the average annual incidence rate was 0.8 per 100,000 person-years. With the inclusion of two additional diagnosed familial cases, the incidence rate would be 0.95 per 100,000 person-years. Five of the 11 cases were diagnosed initially at autopsy.
Adrenal Gland Neoplasms↗
Fibromuscular dysplasia of the right subclavian artery.
Explore the source record for details and available documents.
Adult↗
Aortic dissection in Turner's syndrome.
Explore the source record for details and available documents.
Adult↗