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

Surviving Sepsis Campaign: International guidelines for management of severe sepsis and septic shock: 2008

SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Robot-assisted ureterolysis, retroperitoneal biopsy, and omental wrap: Pilot series for the treatment of idiopathic retroperitoneal fibrosis

Background and Purpose: Retroperitoneal fibrosis (RPF) is an uncommon disease of vague cause distinguished by a chronic inflammatory response. Traditionally, RPF with ureteral involvement has been managed with open ureterolysis and transposition, with excellent success rates. More recently, laparoscopic ureterolysis has been described. Here, we report our experience of managing idiopathic RPF with robot-assisted ureterolysis, retroperitoneal biopsy, and ureteral omental wrapping. Patients and Methods: We performed robot-assisted ureterolysis, retroperitoneal biopsy, and ureteral omental wrapping on five consecutive patients between April and October 2006. The same technique was used for all five patients, except for the omental wrapping. Initially, omental wrapping was performed laparoscopically, but in the last two patients, it was performed entirely robotically. We analyzed our patients\u27 data retrospectively. Results: The mean operative time was 220.5 minutes and 390 minutes for unilateral and bilateral cases, respectively; mean blood loss 33.4 mL; mean length of stay 2.8 days; and mean follow-up was 5.6 months. All patients have remained free of obstruction since surgery and no longer need pain medication. Conclusions: Our study suggests that robot-assisted ureterolysis with laparoscopic or robot-assisted omental wrapping is a feasible alternative to the more morbid open procedure and compares favorably to the purely laparoscopic technique with respect to operative times, estimated blood loss, length of stay, and postoperative relief of obstruction. This procedure can be performed entirely robotically, which provided several advantages over the other techniques. © Mary Ann Liebert, Inc. 2008