Natural history and classification of occlusive thromboaortopathy (Takayasu's disease).

Fifty-four Japanese patients with occlusive thromboaortopathy (OTAP), including four males, were classified according to evidence of complications attributed to OTAP at the time when the diagnosis was established: Group I, uncomplicated OTAP with or without the involvement of the pulmonary artery. Group II, mono-complicated OTAP: presence of a single complication together with uncomplicated OTAP. Group II was subdivided according to severity into group IIa - mild or moderate form, and group IIb - severe form. Group III, multi-complicated OTAP with two or more complications as well as uncomplicated OTAP. The five-year survival rate after established diagnosis was 83.1%. Seven patients died of OTAP within five years, but all had belonged to group IIb or III.     

Cardiac involvement in nonspecific aortoarteritis (Takayasu's arteritis).

Fifty-four patients (18 males and 36 females, ages 2 to 37 years) with nonspecific aortoarteritis (NSAA) were studied. Evaluation revealed hypertension in 35, congestive heart failure (CHF) in 24, mild to moderate mitral regurgitation in six, and mild aortic regurgitation in two. Erythrocyte sedimentation rate was raised (greater than 35 mm in the first hour) in 38 patients. The arterial lesions included type I in seven, type II in eight, and type III in 34. Pulmonary artery involvement was present in 4 (type IV) of the 20 patients in whom it was studied. Selective coronary angiography was done in 11 patients and revealed 90% left main stenosis in one patient. Hemodynamic data revealed raised (greater than 7 mm Hg) mean right atrial pressure in nine, raised mean pulmonary artery pressure (greater than 20 mm Hg) in 29, and raised left ventricular filling pressure (greater than 12 mm Hg) in 27 patients. Radionuclide ventriculography revealed reduced (less than 45%) left ventricular ejection fraction (LVEF) in 27 patients. The myocardial morphology as evaluated on right ventricular endomyocardial biopsy revealed normal histology in nine, features of inflammatory myocarditis in 24, and nonspecific changes suggestive of dilated cardiomyopathy in six patients. Marked right ventricular endocardial thickening was present in three. All patients with CHF had some histologic abnormality. We emphasize that myocardial involvement including myocarditis is common in NSAA and may precipitate CHF in these patients.     

Myocardial involvement and its response to immunosuppressive therapy in nonspecific aortoarteritis (Takayasu's disease)--a study by endomyocardial biopsy

Myocardial involvement in nonspecific aortoarteritis was evaluated in 16 patients (age 7-37 years, 2 males, 14 females) with the help of endomyocardial biopsy obtained from the right ventricle using the Cordis bioptome introduced from the right femoral vein. Morphological features of myocarditis were present in 8, endocardial thickening in 2, mild to moderate myofibre hypertrophy in 11, and a normal biopsy in 3 patients. Myocarditis was present in 8/11 cases with active disease and in none with inactive disease. Five of the 8 patients with myocarditis presented with congestive cardiac failure with 3 of them having no associated hypertension or valvar involvement to account for it. Immunosuppressive therapy was given to all patients with myocarditis. Serial studies (ongoing) showed clinical, haemodynamic and morphological improvement. Myocarditis appears to occur commonly in nonspecific aortoarteritis during the acute phase of the disease and may precipitate congestive cardiac failure in some patients. Immunosuppressive therapy shows promise and merits further evaluation.     

Systolic hypertension and cardiac mortality of Takayasu's aortoarteritis.

Long-term observations of a 35-year-old woman who died from refractory congestive heart failure due to Takayasu's aortoarteritis are reported. Severe systolic hypertension was documented in the pre-pulseless phase. After the loss of all limb pulses, but relative sparing of carotid vessels, serial ophthalmodynamometric retinal pressures suggested that central aortic pressure remained high during her terminal cardiac illness. Postmortem pathologic examination showed a narrowed, severely atherosclerotic aorta with variable occlusions of all branch vessels. The loss of capacitance and volume of the aorta appears to be the cause of systolic hypertension and refractory pump failure of the left ventricle.     

Localizations of Takayasu's disease (author's transl)

Localizations of Takayasu's disease are described and discussed in relation to 10 cases. The affection causes panaortitis which produces stenosis, or less often dilatation, of the thoracic and/or abdominal aorta, and stenosis of occlusion of the arteries arising from the aorta. Lesions of the aortic arch and superior aortic branches, mainly subclavian and carotid, are the most frequently observed form of the disease, but coronary stenosis and aortic incompetence are sometimes noted. Lesions in the descending thoraco-abdominal aorta are frequently present, and stenosis and occlusion of the renal arteries are usually associated with the abdominal aortitis. Stenosis of the terminal aorta and iliofemoral axes is a more rare occurrence. The association of lesions in the aortic arch and its branches, and the descending aorta and its branches, was found in about 2/3rds of cases. Pulmonary arteries are affected in about half the cases according to recent reports.     

Endovascular stent implantation for severe pulmonary artery stenosis in aortoarteritis (Takayasu's arteritis).

Symptomatic pulmonary artery stenosis is a relatively uncommon manifestation of aortoarteritis. We describe a patient of aortoarteritis with severe proximal right pulmonary artery stenosis who presented with dyspnea on exertion and central cyanosis. The pulmonary artery stenosis was successfully relieved by percutaneous transluminal balloon angioplasty and implantation of a balloon-expandable stent. This resulted in immediate improvement in oxygen saturation, disappearance of cyanosis, relief of dyspnea, and marked improvement in right lung perfusion as demonstrated by pre- and postangioplasty technetium lung ventilation-perfusion scans. The changes in the pulmonary arterial wall morphology were detected precisely by intravascular ultrasound imaging.     

Natural history of diverticular disease

PURPOSE: The natural history of patients admitted because of acute diverticulitis is largely unknown, and the selection of patients for surgical treatment varies notably. This study presents our experience concerning the outcome for 366 patients admitted during a 10-year period. METHODS: Three hundred sixty-six patients admitted to our hospital with acute diverticulitis from 1981 to 1990 were identified from a computer database, and their clinical data up to the end of 1996 were reviewed from the database and patient records. RESULTS: There were significantly more males than females in the age group less than 50 years old, and young males underwent surgical treatment during the first treatment period more frequently than the others. Young patients were operated on without mortality, and all their temporary colostomies were closed. Older patients died more often of diseases unrelated to the diverticular disease during the years after the first episode of acute diverticulitis. Recurrences of diverticular disease developed in 22 percent of patients, and they were significantly more common in patients less than 50 years old than in the older age groups. Males less than 50 years old more often developed complications of diverticular disease after two hospital admissions. CONCLUSIONS: Males first admitted when less than 50 years of age undergo more primary operations and develop more recurrences of diverticular disease than do older people. Based on our data, however, we recommend surgery for all patients after two episodes of acute diverticulitis that resolves after conservative treatment with antibiotics.     

Natural history of Alzheimer's disease.

Alzheimer's disease (AD) is the principal cause of dementia in the elderly, and affects about 15 million people worldwide. The earliest symptom is usually an insidious impairment of memory. As the disease progresses, there is increasing impairment of language and other cognitive functions. Problems occur with naming and word-finding, and later with verbal and written comprehension and expression. Visuospatial, analytic and abstract reasoning abilities, judgment, and insight become affected. Behavioral changes may include delusions, hallucinations, irritability, agitation, verbal or physical aggression, wandering, and disinhibition. Ultimately, there is loss of self-hygiene, eating, dressing, and ambulatory abilities, and incontinence and motor dysfunction. Before diagnosis of AD, individuals may have memory complaints, which represent a period of mild cognitive impairment (MCI). Before MCI, there is a prodromal, ill-defined presymptomatic period of disease ('pre-MCI"). In this review, we particularly focus on these earliest stages. We also discuss the more advanced stages of AD, and address factors that may influence disease course. Understanding the natural history of AD will allow better targeting of the disease-modifying treatments that are on the horizon.     

Nonspecific aortoarteritis (classification and surgical treatment)

Three hundred cases of nonspecific aortoarteritis are reviewed. A new classification of the condition is proposed identifying 3 stages (acute inflammation, subacute recurrent inflammation and chronic condition), 3 types of morphologic changes (stenosing, deforming and aneurysmatic), 3 sites of lesion (aortic branches, the thoracoabdominal aorta and combined lesions) and 4 degrees of organ ischemia. Ten syndromes are distinguished in the clinical pattern, and their relative incidences are specified. A total of 253 operations, including 218 reconstructive ones, were performed: 83 on aortic branches, 87 on thoracoabdominal aorta, 40 on the abdominal aorta, and 11 for aortic aneurysms. Reconstructive surgery of renal arteries was performed in 89 patients, and of visceral arteries, in 55. The need for anti-inflammatory treatment is emphasized. Surgical treatment produces better results, as compared to conservative treatment alone.     

Patterns of symptoms and prognosis in occlusive thromboaortopathy (Takayasu's disease)

Ninety-five Japanese patients with Takayasu's disease were classified according to four patterns of severity of symptoms in the period from the onset of symptoms to diagnosis. Included were pattern A in 34 patients with a plateau course after the insidious onset of symptoms, pattern B in 18 patients with a decrescendo course after the sudden onset of severe symptoms, pattern C in 5 patients who had no severe symptoms during the years between the early and late periods of severe symptoms and pattern D in 38 patients with a crescendo course after the onset of symptoms and the following period of years of no severe symptoms. Fifteen patients died during the prospective follow-up period of 8.6 +/- 6 years (mean +/- SD) after admission, but 12 had had patterns C and D and the remaining 3 had had patterns A and B. The cardinal signs and symptoms are given in detail, particularly those at the onset. These data should aid in early diagnosis and prediction of the prognosis in Takayasu's disease.     

Survival and morbidity after diagnosis of occlusive thromboaortopathy (Takayasu's disease)

Eighty-one young Japanese patients with occlusive thromboaortopathy (Takayasu's disease), including nine men, were followed up prospectively for 7.4 +/- 5.8 years (mean +/- standard deviation) after the established diagnosis. These patients were classified according to the presence and severity of four major complications (Takayasu's retinopathy, secondary hypertension, aortic regurgitation and aortic or arterial aneurysm) attributed to Takayasu's disease at the time when the diagnosis was established: no complications (group I) or mild single complication (group IIa) and severe single complication (group IIb) or multiple complications (group III). When the data were analyzed with the left table method, severe events attributed to TAkayasu's disease, as well as death, were used as end points. Seven of the 81 patients were surgically treated; 2 of the 7 died and 1 had severe events postoperatively. In the remaining 74, the 10 year cumulative eventless survival rate after the diagnosis in combined groups I and IIa (44 patients) and combined groups IIb and III (30 patients) was 97.0 +/- 2.9 (mean +/- standard error of the mean) and 58.6 +/- 11.3 percent, respectively (p less than 0.002). These data are useful for predicting death or severe events, or both, and provide basic information regarding possible elective surgery for patients with this intractable disease.     

Occlusive thromboaortopathy (Takayasu's disease): clinical findings and angiographic classification

We report the findings in 26 cases of occlusive aortopathy (Takayasu's disease) encountered during eight years. The mean age was 25.3 years and female to male ratio was 2.7:1. Chest and local pain, fever, hypertension (70%) and absent pulses were accompanied by high erythrocyte sedimentation rate (ESR) 38 to 126 mm, eosinophilia 4 to 26%. All immunological tests were negative except tuberculin-test (PPD) with 65% positive. EKG was normal in three cases, 23 showed left ventricular hypertrophy, ST-T changes in two and acute myocardial infarction in two cases. The classification: Type I cervico-brachial or arch type with five cases, type II thoraco-abdominal type with six cases, type III peripheral type with four cases and generalized types combination of two or more of three types and/or pulmonary artery involvement, showed good correlation with clinical findings and prognosis. The occlusive aortopathy is an inflammatory disease of unknown origin, an auto-immune or genetic etiology may be involved.     

An unusual case of occlusive thromboaortopathy (Takayasu's disease)--a case report

A case of Takayasu's disease presenting in a young man as a solid abdominal mass is described. The literature is reviewed and classifications of the disease are discussed. Occlusive thromboaortopathy, also known as "pulseless disease" or Takayasu's disease, was first described in 1908 by Takayasu, who observed cataracts and peculiar arteriovenous anastamoses around the optic papillae in a young woman. It is a vasculitic disorder of uncertain etiology occurring mainly in young women and may involve part or most of the aorta, as well as the pulmonary artery. Early features include fever, malaise, weight loss, and a high ESR, and, later on, absent pulses, aneurysms, hypertension, and heart failure may occur. The authors describe an unusual case of Takayasu's disease in a young black South African man.