Stage IV sarcoidosis: comparison of survival with the general population and causes of death.

The objectives of this study were to compare the survival of sarcoid patients with pulmonary fibrosis with that of the general population and to determine the causes of death and the incidence of evolutive complications. This retrospective cohort included 142 sarcoid patients in radiographic stage IV (74 males; mean ± SD age 48.1 ± 12 yrs). Their survival was compared with that of the general French population, matched for the year and age at diagnosis of stage IV disease, sex and length of follow-up. Expected survival probabilities were calculated year-by-year on the basis of probabilities provided by official demographic data for France. Survival curves were based on the Kaplan-Meier method and compared using the log-rank test. During the follow-up period (7.1 ± 4.8 yrs), pulmonary hypertension (PH) was observed in 29.7% of cases and aspergilloma in 11.3%. Long-term oxygen therapy was required in 12%. Survival was 84.1% at 10 yrs, which was worse than for the general population (p = 0.013). 16 (11.3%) patients died from the following causes: refractory PH (n = 5), chronic respiratory insufficiency (n = 4), acute respiratory insufficiency (n = 2), haemoptysis due to aspergilloma (n = 1), heart sarcoidosis (n = 1), nocardiosis (n = 1) and unknown causes (n = 2). Survival is significantly decreased in stage IV patients. 75% of fatalities are directly attributable to respiratory causes.

[Combined apical emphysema and basal fibrosis syndrome (emphysema/fibrosis syndrome): CT imaging features and pulmonary function tests]. / Syndrome emphysème des sommets et fibrose pulmonaire des bases combinés (syndrome emphysème/fibrose): aspects tomodensitométriques et fonctionnels.

PURPOSE: To describe the high resolution CT (HRCT) imaging and functional features of the emphysema/fibrosis syndrome. PATIENTS AND METHODS: A total of 61 patients were included based on HRCT. We have quantified the extent of fibrosis and emphysema lesions and a combined score was calculated. The scores were correlated to pulmonary function test parameters and specific HRCT features were described. RESULTS: The emphysema and fibrosis scores correlated with functional parameters of obstruction and restriction respectively. The combined score correlated with the reduction in DLCO and degree of pulmonary hypertension. Three HRCT patterns were identified: progressive transition (n=23, 38%) with diffuse emphysema (centrilobular and/or bullous) and zone of transition between bullae and honeycombing; paraseptal emphysema (n=13, 21%) with predominant subpleural bullae of enlarging size at the bases; separate processes (n=14, 23%) with independent areas of fibrosis and emphysema. Eleven patients (18%) could not be classified. The HRCT imaging features changed based on TLC (p=0.04) and FEV1/FVC (p=0.01). CONCLUSION: The emphysema/fibrosis syndrome may be associated with different patterns on HRCT corresponding to specific profiles on pulmonary function tests.

Cost-effectiveness analysis of the terlipressin-glycerin trinitrate combination in the pre-hospital management of acute gastro-intestinal haemorrhage in cirrhotic patients.

OBJECTIVES: To assess the cost-effectiveness of an early treatment of upper gastro-intestinal haemorrages in cirrhotic patients. DESIGN: Utilization data linked to the results of a double-blind, placebo-controlled trial demonstrating the efficacy of the terlipressin-glycerin trinitrate combination (TER-GTN) in the reduction of mortality at day 42 for haemorragic patients due to rupture of oesophageal varices. SETTING: Hôpital Jean Verdier, Bondy, Assistance Publique-Hôpitaux de Paris, France. SUBJECTS: Eighty-four patients included over 2 years by emergency services and hospitalized in an intensive care unit (ICU) for haemorrage, 41 in the "treated" group and 43 in the "placebo" group. MAIN OUTCOME MEASURES: Mortality at day 42, cost per death avoided. RESULTS: The mortality rate in the placebo group was 46.5% versus 27.5% in the treated group. The mean length of stay was 5 days longer in the treatment group. The excess cost per death avoided was 25,849 FF. Of this extra cost 27% was due to treatment and 24% was due to increased length of stay. The excess cost per case treated was FF 5,097, 10% of the total cost per stay for rupture of oesophageal varices (ROV). CONCLUSIONS: Our results are of the same magnitude as those published by Mac Cormick et al. in the United Kingdom for similar treatment. The extra cost appears to be moderate, and much lower than monoclonal antibody therapy for sepsis. The impact on the study hospital budget did not exceed 1.7 10(-4).

Interferon gamma-secreting HCV-specific CD8+ T cells in the liver of patients with chronic C hepatitis: relation to liver fibrosis--ANRS HC EP07 study.

Little is known about the role of specific hepatitis C virus (HCV) CD8+ T cells in liver damage, especially for the progression of fibrosis, during the highly variable course of chronic C hepatitis. The aim of this study was to investigate the presence of HCV-specific CD8+ T cells in the liver of patients with chronic C hepatitis and to examine their clinical significance by relating the response to liver fibrosis and progression rate, serum viral load, serum aminotransferase levels, inflammatory activity and in situ characteristics of the intrahepatic infiltrate. Fifteen patients were prospectively included in the study. Intrahepatic lymphocytes were tested for interferon gamma (IFNg) production in response to HCV class I-restricted epitopic peptides using enzyme-linked immunospot analysis. Liver biopsy samples were evaluated for fibrosis, fibrosis progression rate, activity, and in situ number of CD8+ cytotoxic lymphocytes and apoptotic cells. An IFNg-specific CD8+ T-cell response was detected in the liver samples of 47% of patients which was significantly related to a lower stage of fibrosis (P = 0.02) and a lower progression rate of fibrosis (P = 0.01). It was neither related to the number of cytotoxic lymphocytes infiltrating the liver nor to hepatocyte apoptosis. In conclusion, our results indicate that the presence of HCV-specific IFNg-secreting T cells in the liver of patients with chronic C hepatitis is associated with low liver fibrosis and fibrosis progression rate, suggesting that these IFNg-secreting T cells might limit the progression of liver damage.

Early administration of terlipressin plus glyceryl trinitrate to control active upper gastrointestinal bleeding in cirrhotic patients.

Upper gastrointestinal bleeding (GIB) is a major complication in cirrhotic patients. Endoscopy and oesophageal sclerosis are reference treatments and must be done as soon as possible. However, such treatment is not possible unless the patient is admitted to hospital. In a prospective, randomised, double-blind trial, we compared the efficacy of terlipressin combined with glyceryl trinitrate (TER-GTN), administered as early as possible to 76 patients with cirrhosis who had active GIB (84 bleeding episodes). Infusion was done at the patient's home by the physician on the emergency team (a mobile intensive care unit) if the patient had GIB and a history and clinical signs of cirrhosis. Patients received either an intravenous injection (1 to 2 mg) of TER-GTN or a double-placebo injection, and then another injection at 4 and 8 h. Control of bleeding, rebleeding, and mortality rate at days 15 and 42 were evaluated. In most patients, endoscopy confirmed the rupture of oesophageal varices (75.7%). Bleeding control was significantly better in the TER-GTN group (n = 41) than in the double-placebo group (n = 43) (p = 0.034). Mortality due to bleeding episodes was significantly lower in the TER-GTN group than in the double-placebo group at day 15 (p = 0.035) and at day 42 (p = 0.06). There were no serious side-effects. Early administration of TER-GTN lowers the deleterious consequences of prolonged hypovolaemia on the hepatic function of these patients.