Hyper-IgG4 disease: report and characterisation of a new disease

BACKGROUND: We highlight a chronic inflammatory disease we call 'hyper-IgG4 disease', which has many synonyms depending on the organ involved, the country of origin and the year of the report. It is characterized histologically by a lymphoplasmacytic inflammation with IgG4-positive cells and exuberant fibrosis, which leaves dense fibrosis on resolution. A typical example is idiopathic retroperitoneal fibrosis, but the initial report in 2001 was of sclerosing pancreatitis. METHODS: We report an index case with fever and severe systemic disease. We have also reviewed the histology of 11 further patients with idiopathic retroperitoneal fibrosis for evidence of IgG4-expressing plasma cells, and examined a wide range of other inflammatory conditions and fibrotic diseases as organ-specific controls. We have reviewed the published literature for disease associations with idiopathic, systemic fibrosing conditions and the synonyms: pseudotumour, myofibroblastic tumour, plasma cell granuloma, systemic fibrosis, xanthofibrogranulomatosis, and multifocal fibrosclerosis. RESULTS: Histology from all 12 patients showed, to varying degrees, fibrosis, intense inflammatory cell infiltration with lymphocytes, plasma cells, scattered neutrophils, and sometimes eosinophilic aggregates, with venulitis and obliterative arteritis. The majority of lymphocytes were T cells that expressed CD8 and CD4, with scattered B-cell-rich small lymphoid follicles. In all cases, there was a significant increase in IgG4-positive plasma cells compared with controls. In two cases, biopsies before and after steroid treatment were available, and only scattered plasma cells were seen after treatment, none of them expressing IgG4. Review of the literature shows that although pathology commonly appears confined to one organ, patients can have systemic symptoms and fever. In the active period, there is an acute phase response with a high serum concentration of IgG, and during this phase, there is a rapid clinical response to glucocorticoid steroid treatment. CONCLUSION: We believe that hyper-IgG4 disease is an important condition to recognise, as the diagnosis can be readily verified and the outcome with treatment is very good

Is there hospital variation in long-term incisional hernia repair after abdominal surgery?

Background Currently, hospital benchmarking organizations are often limited to short-term surgical quality comparisons among hospitals. The goal of this study was to determine whether long-term rates of incisional hernia repair after common abdominal operations could be used to compare hospital long-term surgical quality. Study Design This was a cohort study with up to 4 years of follow-up. Patients who underwent 1 of 5 common inpatient abdominal operations were identified in 2005-2008 American College of Surgeons NSQIP data linked to Medicare inpatient records. The main outcomes included occurrence of an incisional hernia repair. A multivariable, shared frailty Cox proportional hazards regression was used to compare each hospital's incisional hernia rate with the overall mean rate for all hospitals and control for American College of Surgeons NSQIP preoperative clinical variables. Results A total of 37,134 patients underwent 1 of 5 common inpatient abdominal operations, including colectomy, small bowel resection, ventral hernia repair, pancreatic resection, or cholecystectomy, at 1 of 216 hospitals participating in American College of Surgeons NSQIP during the 4-year period. There were 1,474 (4.0%) patients who underwent an incisional hernia repair, at a median follow-up time of 16 months (interquartile range 8 to 25 months) after initial abdominal surgery. After risk adjustment, there was no significant difference in the ratio of any one hospital's adjusted hazard rate for incisional hernia repair vs the average hospital adjusted hazard rate. Conclusions Risk-adjusted hospital rates of incisional hernia repair do not vary significantly from the average. This suggests that incisional hernia repair might not be sensitive enough as a long-term quality metric for benchmarking hospital performance

Is there hospital variation in long-term incisional hernia repair after abdominal surgery?

Background Currently, hospital benchmarking organizations are often limited to short-term surgical quality comparisons among hospitals. The goal of this study was to determine whether long-term rates of incisional hernia repair after common abdominal operations could be used to compare hospital long-term surgical quality. Study Design This was a cohort study with up to 4 years of follow-up. Patients who underwent 1 of 5 common inpatient abdominal operations were identified in 2005-2008 American College of Surgeons NSQIP data linked to Medicare inpatient records. The main outcomes included occurrence of an incisional hernia repair. A multivariable, shared frailty Cox proportional hazards regression was used to compare each hospital's incisional hernia rate with the overall mean rate for all hospitals and control for American College of Surgeons NSQIP preoperative clinical variables. Results A total of 37,134 patients underwent 1 of 5 common inpatient abdominal operations, including colectomy, small bowel resection, ventral hernia repair, pancreatic resection, or cholecystectomy, at 1 of 216 hospitals participating in American College of Surgeons NSQIP during the 4-year period. There were 1,474 (4.0%) patients who underwent an incisional hernia repair, at a median follow-up time of 16 months (interquartile range 8 to 25 months) after initial abdominal surgery. After risk adjustment, there was no significant difference in the ratio of any one hospital's adjusted hazard rate for incisional hernia repair vs the average hospital adjusted hazard rate. Conclusions Risk-adjusted hospital rates of incisional hernia repair do not vary significantly from the average. This suggests that incisional hernia repair might not be sensitive enough as a long-term quality metric for benchmarking hospital performance

Using Merged Clinical and Claims Registry Data to Identify High Utilizers of Surgical Inpatient Care 1 Year after Colectomy

Background Under bundled payment initiatives, providers will be held financially responsible for patients' acute and post-acute care costs. Certain patients, termed high utilizers, use disproportionate shares of resources during 1 year. The aim of this study was to identify high utilizers, describe their costs, and determine whether preoperative characteristics predict high utilizer status. Study Design Colectomy patients with 1-year follow-up were identified in a linked clinical (American College of Surgeons NSQIP) and administrative (Medicare inpatient claims) dataset (2005 to 2008). Cost of inpatient care was calculated by multiplying patient Medicare charges in each cost center by cost-to-charge ratios from the Medicare cost reports. A mixed-effects logistic model quantified the association between preoperative characteristics and being a high utilizer after elective and emergent colectomies. Results One thousand and fifty-five of 10,561 colectomy patients accounted for >50% of the inpatient care cost of the entire cohort during 1 year postoperatively. This top decile of patients were labeled high utilizers and had substantially greater costs in the following cost centers: intensive care ($36,322 vs$0), respiratory ($2,875 vs$22), radiology ($649 vs$29), and cardiology ($5,057 vs$166) (all p < 0.001). High utilizers more frequently had emergent index colectomies (43% vs 17%; p < 0.001). Patients with American Society of Anesthesiologists class IV and V had 2-fold increased odds of being high utilizers after both elective (odds ratio = 2.72; 95% CI, 1.89-3.90) and emergent colectomies (odds ratio = 2.09; 95% CI, 1.23-3.55). Conclusions Patients in the top cost decile account for the majority of costs in the year after colectomy, disproportionately accumulate those costs in particular cost centers, and can be identified preoperatively