Sarcoidosis最新文献

Comparison of 67Gallium scans of head and neck showed no significant difference in intensity of uptake between patients with Löfgren's syndrome and sarcoidosis patients with similar chest x rays having other modes of onset. Uptake was the same in the 12 white and 18 black patients studied. Thus, the increased bronchoaveolar lavage lymphocytosis which has been shown to be a feature of Löfgren's syndrome is not paralleled by gallium uptake. Gallium scores had no predictive value regarding outcome showing no significant difference between 13 patients who recovered within a year and 15 patients who developed chronic disease. The outcome was more favorable in white patients and in those whose onset was with Löfgren's syndrome.

Finland and Hokkaido resemble each other; the four season climate with cold winters and cool summers is the same as is the frequency of tuberculosis. The size of the population is almost the same. From the 1984 Japanese nationwide sarcoidosis survey we got the data from Hokkaido (288 patients) in order to compare the information with similarly collected data in Finland from 1984 (1,378 patients). The crude prevalence of sarcoidosis was 28.2 per 100,000 in Finland and 3.7 per 100,000 in Hokkaido. The annual incidence was 11.4 per 100,000 in Finland and 1.0 per 100,000 in Hokkaido. The sex distribution was similar; 63% vs 67% women in Finland and Hokkaido, respectively. At diagnosis the Hokkaidoan patients were significantly younger. In Hokkaido more cases were detected via mass X-ray survey (43% vs 34%). Among symptomatic patients eye symptoms were more frequent in Hokkaido, whereas respiratory and joint symptoms and erythema nodosum were more frequent in Finland. Bilateral hilar lymphadenopathy (BHL) was equally distributed (82% vs 84% whereas parenchymal lesions were seen more often in Finland (49% vs 25%) as well as peripheral lymphadenopathy (16% vs 8%).

There are several diseases which present with both ocular and thoracic disease (Table I). Behçet's disease (BD) may be confused with sarcoidosis because they are both multisystem inflammatory disorders presenting with uveitis, polyarthritis, meningitis, cardiovascular disease, erythema nodosum and other cutaneous lesions, and abnormal chest radiographs. The aetiology of both disorders continues to elude us. Hopefully, a careful study of the one may shed light and knowledge on the other.

The aim of this study was to evaluate whether markers of collagen synthesis, hyaluronan (HA) and procollagen type III aminoterminal peptide (PIIINP) in bronchoalveolar lavage fluid (BALF) and serum (S) were correlated to paraclinical markers of disease activity (S-ACE, S-IgG S-IgA S-calcium, chest X-ray (CXR) profusion score, pulmonary function tests (FEV1, FVC, TLC, DLCO)) in pulmonary sarcoidosis. The material comprised 48 patients with biopsy proven sarcoidosis (35 male, 13 female, median age 31 years) and 24 controls (16 male, 8 female, median age 60 years). BAL was performed in the right middle lobe with 250 ml saline. Patients had higher BALF-HA, mean 88 +/- 13 (SEM) micrograms/l, than controls, 39 +/- 2 micrograms/l (p < 0.01), higher BALF-albumin, 121 +/- 13 mg/l, than controls 58 +/- 4 mg/l (p < 0.01), and higher BALF/S-HA ratio, 3.35 +/- 0.51, than controls, 1.23 +/- 0.60 (p < 0.01). There were no significant differences for S-HA, BALF-PIIINP, or S-PIIINP. In patients significant correlations were found between BALF-HA, S-HA, and BALF-albumin; between S-HA and S-ACE; between BALF/S-HA and BALF-albumin; between CXR profusion score and S-HA, S-ACE, S-IgG, S-IgA, FEV1, FVC, TLC and DLCO. The results indicate that measurement of S-HA, BALF-HA, and BALF-albumin may be of value in the monitoring of disease in pulmonary sarcoidosis.

Corticosteroids (CS) are useful drugs for the treatment of cardiac sarcoidosis with severe conducting defects due to sarcoid granuloma. Despite the continuous administration of CS, many patients with severe cardiac involvement may eventually die of congestive heart failure. The purpose of this study was to evaluate the efficacy of CS in patients who had a pacemaker implanted. Questionnaires were obtained from 29 institutes, and 34 cardiac sarcoidosis patients (8 males and 26 females) with pacemaker implantation were enrolled in this survey. We analyzed the survival period in these patients by the Kaplan-Meier method. There was no statistically significant difference in the survival of these patients in terms of their age, sex or disease duration (time from the onset of sarcoidosis to cardiac involvement). However, their survival was affected by the grade of dyspnea, the presence of heart failure, and certain abnormal findings on a myocardial scintigram and echocardiogram. In order to evaluate the effect of CS on the prolongation of survival, we measured the survival of the patients treated with CS and those not treated with CS. However, because of the small number of patients not treated with CS, we were unable to detect any statistically significant difference in survival. Therefore, we analyzed 104 cases in order to evaluate CS therapy: the 34 cases from the questionnaires and 70 cases reported in the literature over the last 10 years.(ABSTRACT TRUNCATED AT 250 WORDS)

We report a case of hypercalcaemic crisis due to sarcoidosis in a 15-year-old boy. The clinical suspicion of sarcoidosis was confirmed by a liver biopsy. At admission serum calcium, 1,25(OH)2 and ACE were elevated and iPTH was suppressed. The levels of serum total and ionized calcium, iPTH, ACE, 1,25(OH)2 and 25-OH were followed and chest X-ray and pulmonary function tests were performed during systemic steroid treatment. The clinical condition improved during treatment and the paraclinical measurements normalised within 5 weeks. The mechanism whereby hypercalcaemia occurs in childhood sarcoidosis is clarified.

Sarcoidosis is a multisystem granulomatous disease of unknown aetiology. The role of radiology is not only to support the clinical diagnosis but also as an adjunct to monitor the course and progression of the disease. The cornerstone of radiological assessment is the plain radiograph, however newer imaging methods are producing more information in relation to the anatomical distribution and pathophysiology which may ultimately have a profound effect on the management of this protean disease.

A follow-up of 254 patients with pulmonary sarcoidosis has been carried out after a median of 27 years from the diagnostic admission. Information on respiratory symptoms at entry was available in 80% of the patients. Cough and chronic bronchitis was present in 39% and 37%, respectively, whereas 41% reported breathlessness when walking and 25% reported breathlessness when undressing. In total, 51% had at least one respiratory symptom. All patients could be traced; eighty patients (31.5%) died during follow-up. A total of 17 died of sarcoidosis and a further 16 died of sarcoidosis-related diseases. All deaths from sarcoidosis were seen among patients with respiratory symptoms at presentation. For sarcoidosis and sarcoidosis-related deaths (N = 33), presence of one or more of the respiratory symptoms increased the risk significantly. For overall mortality, respiratory symptoms were also significant predictors; this was the case even after adjusting for age, sex, x-ray stage and lung function (FEV1 & TLC) at presentation. In conclusion, respiratory symptoms at the time of diagnosis are independently related to vital prognosis in pulmonary sarcoidosis.