Kaichiro Ishikawa

Diagnostic approach and proposed criteria for the clinical diagnosis of Takayasu's arteriopathy.

The criteria proposed for the clinical diagnosis of Takayasus disease (chronic inflammatory arteriopathy of unknown origin) were based on clinical and angiographic data from 108 Japanese patients: 96 with Takayasus disease and 12 with another disease of the aorta. The criteria consist of one obligatory criterion (age less than or equal to 40 years), two major criteria (left and right mid subclavian artery lesions) and nine minor criteria (high erythrocyte sedimentation rate, common carotid artery tenderness, hypertension, aortic regurgitation or annuloaortic ectasia and lesions of the pulmonary artery, left mid common carotid artery, distal brachiocephalic trunk, thoracic aorta and abdominal aorta). In addition to the obligatory criterion, the presence of two major criteria or of one major plus two or more minor criteria, or of four or more minor criteria suggests a high probability of the presence of Takayasus disease. The criteria had an 84% sensitivity in 96 patients with this disease: 52 (96%) of 54 patients in the active young group, 8 (80%) of 10 in the active older group, 14 (67%) of 21 in the inactive young group and 7 (64%) of 11 in the inactive older group fulfilled the criteria; however, none of the 12 patients with other aortic diseases did so. Use of these criteria has shortened the delay of an accurate diagnosis in patients with Takayasus disease.

Long-term outcome for 120 Japanese patients with Takayasu's disease. Clinical and statistical analyses of related prognostic factors.

BACKGROUND Patients with Takayasus disease, a chronic inflammatory arteriopathy of unknown cause, have variable clinical courses, and predictors of the long-term outcome are not well understood. We studied prognostic factors of this disease, based on follow-up results, and a new prognostic classification was proposed. METHODS AND RESULTS Life-table methods and Cox regression analyses were applied to clinical data on 120 patients who had been prospectively followed for a median of 13 years (range, 1 month to 34 years). The overall survival rate at 15 years after the diagnosis was 82.9% and remained the same for the remainder of the follow-up period. Univariate Cox analyses revealed that of the six dichotomous variables evaluated at diagnosis, four were statistically significant predictors, including complications (Takayasus retinopathy, hypertension, aortic regurgitation, and aneurysm), pattern of the past clinical course, age of the patient, and calendar year of diagnosis. Thus, the 15-year survival was 66.3% versus 96.4% for patients with and without a major complication, 67.9% versus 92.9% for patients with and without a progressive course, 58.3% versus 92.7% for age > 35 years and < or = 35 years, and 79.9% versus 96.5% for patients diagnosed in 1957 through 1975 and in 1976 through 1990, respectively. The delay in diagnosis and the erythrocyte sedimentation rate (ESR) were of marginal significance. The multivariate Cox analysis showed that only two of the above variables were statistically independent predictors, ie, the major complication and the progressive course. In addition to these two factors, ESR was the third predictor, if the Cox stepwise elimination procedure was performed. These three predictors used in various combinations made a total of 1822 classifications theoretically feasible. Of these, a three-stage classification was selected as the best one, based on the Akaike information criterion. The presence of both major complication and progressive course (stage 3) was the worst prognostic indicator (43% survival at 15 years). In contrast, no patient died who had neither of these manifestations or who had a progressive course but an elevated ESR as well (stage 1). CONCLUSIONS The long-term outcome for patients with Takayasus disease seems best predicted by two major prognostic factors, ie, complications and the pattern of the past clinical course, as well as by ESR. Aggressive medical and surgical treatment may be considered for patients with a major complication and a progressive course (stage 3).

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

Eighty-one young Japanese patients with occlusive thromboaortopathy (Takayasus 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 (Takayasus retinopathy, secondary hypertension, aortic regurgitation and aortic or arterial aneurysm) attributed to Takayasus 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 TAkayasus 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) and pregnancy. Clinical course and management of 33 pregnancies and deliveries.

Twenty-seven Japanese patients with occlusive thromboaortopathy (Takayasus disease) associated with 33 pregnancies and deliveries were followed up prospectively, from the prepregnant period. Inflammatory activity of the disease was apparently not enhanced by the pregnancy, but various cardiovascular-related events occurred in the perinatal period. During the intrapartum uterine contractions, marked elevation of systolic blood pressure was associated with 10 pregnancies, including 1 case of subsequent cerebral hemorrhage; this did not occur in 13 normal control gravidas. There were no maternal or neonatal deaths. The birth weight of 25 infants of 21 patients without complications or with a mild single complication of Takayasus disease was 3,023 +/- 442 g (mean +/- standard deviation). The birth weight of 8 infants of 6 patients who had a severe single complication or multiple complications of the disease was 2,599 +/- 394 g (p less than 0.05). Of 83 pregnancies, the present 33 plus 50 other reported cases there were no un-toward events in two fifths. Three fifths had various problems, most of which were related to pressure elevation and heart failure, including intrapartum cerebral hemorrhage in 4. These data should assist in predicting the outcome of pregnancy and delivery in patients with Takayasus disease.

Effects of prednisolone therapy on arterial angiographic features in Takayasu's disease

Abstract Patients with Takayasus disease in the active stage, a time when the inflammatory activity is well reflected by high erythrocyte sedimentation rate, usually respond to corticosteroid therapy. 1–4 There has been no angiographic evidence of any positive attenuation of the arterial stenoses except in 2 cases. 5,6 Long-term prednisolone therapy guidelines have also not been documented. I report here the results of a prospective, long-term follow-up study on 118 patients with Takayasus disease. A comparison of the angiograms obtained at different times in 1 patient was made. Using the life-table method, decrease in the percentage of patients who required prednisolone therapy is estimated. In addition, the method of prednisolone therapy is given attention.

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

Ninety-five Japanese patients with Takayasus 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 Takayasus disease.

Education in angiology. Formation of a medical angiologist.

North American cardiologists, as cardiovascular subspecialists in internal medicine, deal with cardiac as well as vascular disease entities.1 Consequently, cardiologists are also angiologists. However, it has been reported that these physicians spend considerably more of their professional time dealing directly with cardiology rather than angiology-related problems.1,2 Such is also the case in Japan. Medical angiology may be too extensive a field to be the responsibil ity of cardiologists alone, although an excellent report was documented con cerning of guidelines for training cardiovascular subspecialists in internal medicine, as proposed by the Council on General Internal Medicine, American Board of Internal Medicine.3 Therefore, the medical angiologist who wishes to function as a subspecialist for patients with vascular disease should be encour aged. Better trained medical angiologists should enable better treatment and management of related clinical disorders.