Primary Sj?gren's syndrome (SS) shares many features with systemic lupus erythematosus (SLE). Here we investigated the association of the three major polymorphisms in IRF5 and STAT4 found to be associated with SLE, in patients from Sweden and Norway with primary SS. These polymorphisms are a 5-bp CGGGG indel in the promoter of IRF5, the single nucleotide polymorphism (SNP) rs10488631 downstream of IRF5 and the STAT4 SNP rs7582694, which tags the major risk haplotype of STAT4. We observed strong signals for association between all three polymorphisms and primary SS, with odds ratios (ORs) >1.4 and P-values
OBJECTIVES: Mortality rates for Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) have decreased after the introduction of cyclophosphamide. Standardized mortality ratio (SMR) expresses the overall mortality of patients compared with the general population. The aims of this study were to compare survival in an old and a recent cohort of patients with WG and MPA using SMR and to determine predictors for death in both groups combined. DESIGN: Survival analyses were performed by Kaplan-Meier survival curves, SMR and proportional hazards regression models. SETTING: The nephrology and rheumatology clinics at Linköping University Hospital, Sweden. SUBJECTS: All patients diagnosed with WG or MPA in the catchment area during 1978-2005 were divided into two cohorts; patients diagnosed before (n=32, old cohort) and after (n=63, recent cohort) December 31, 1996. RESULTS: The two cohorts differed regarding the proportion of WG (75% vs. 56%, P=0.03) and a tendency for more pronounced kidney involvement in the old cohort: 266 micromol L(-1) (16% dialysis-dependent) vs. 192 micromol L(-1) (5% dialysis-dependent), but were comparable regarding disease severity. SMR at 1 and 5 years were 2.1 (95% CI: 0.43-6.09) and 1.6 (95% CI: 0.6-3.2) in the recent cohort and 5.2 (95% CI: 1.07-15.14) and 2.5 (95% CI: 0.93-5.52) in the old cohort. Five-year survival was 87% and 81%. Serum creatinine, age, end-stage renal disease, diagnosis before 1997 and first relapse were independent predictors for death. CONCLUSION: Patient survival in WG and MPA analysed with SMR may be better than previously believed. Severe renal disease and disease relapse were the major predictors of reduced survival.
Intraoperative and in-hospital mortality after surgery for acute type A dissection depends largely on preoperative conditions, specifically the presence of localized or generalized ischemia. Recently, the Penn classification of patients with acute type A aortic dissection has been described. The primary aim was to validate the Penn classification and to investigate preoperative variables related to mortality.
All consecutive patients operated for acute type A aortic dissection, 1990 to 2009 (n = 360), were included in a retrospective observational study. Univariate and multivariable analyses were used to identify variables related to intraoperative and in-hospital mortality. Propensity scoring was used to adjust for treatment selection bias.
Overall intraoperative mortality was 7% (24 of 360) and in-hospital mortality was 19% (69 of 360). Two hundred nineteen patients (61%) were Penn class Aa (14% in-hospital mortality), 51 (14%) class Ab (24% mortality), 63 (18%) class Ac (24% mortality), and 27 (8%) class Abc (44% mortality), p =0.007. In multivariable analysis, Penn class Ac and Abc were independently related to intraoperative death (odds ratio 5.0 and 5.4, respectively), and Penn class Abc and non-Aa were independently related to in-hospital mortality (odds ratio 3.4 and 2.3, respectively). Concomitant coronary artery bypass grafting, older age, DeBakey type I dissection, and prolonged periods of cardiopulmonary bypass and hypothermic circulatory arrest were also independently associated with mortality.
The Penn classification of acute type A aortic dissection is purposeful and its continued usage encouraged. Penn class indicating localized or generalized ischemia is independently related to intraoperative and in-hospital mortality.
Comment In: Ann Thorac Surg. 2012 Oct;94(4):137623006709
Comment In: Ann Thorac Surg. 2011 Oct;92(4):1382-321958784