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

I tumori stromali del piccolo intestino. Differenze prognostiche sulla base delle caratteristiche cliniche, morfologiche ed immunofenotipiche

Gastro Intestinal Stromal Tumors (GIST) are rare malignant non epithelial tumors arising from gastro-intestinal tract. Three cases, treated in our departement, are an example of the anatomo-clinical features of these tumors. Prognosis was correlated with the size of tumor at the time of surgery and immunophenotype pattern. The tumors with high expression of nervous antigens ha a worse prognosis

Gastric wall necrosis from organo-axial volvulus as a late complication of laparoscopic gastric banding.

We present a case of massive gastric wall necrosis associated with volvulus of the stomach occurring 17 months after laparoscopic gastric banding. The 19-year-old female was admitted to our hospital with acute abdominal pain with rapid deterioration from massive necrosis of a distended stomach which perforated distal to the gastric band, accompanied by splenic infarction. Total gastrectomy and spenectomy were required. We present this very rare complication which should be recognized and treated early, so that a less drastic operation is possible

Contribution of CT to treatment planning in patients with GIST

Purpose. The aim of this study was to illustrate the morphological and structural computed tomography (CT) patterns of gastrointestinal stromal tumours (GIST) and to discuss the technique's role in identifying lesions at a higher risk for malignant potential, in treatment planning and in the follow-up of patients with GIST. Materials and methods. We retrospectively reviewed the CT scans of 26 patients who underwent surgery for histologically confirmed GIST of the stomach (20 cases), the duodenum (1), the caecum (1), the small bowel (2), the descending colon (1) and the rectum (1). CT exams were performed with a single-slice scanner and a 5-mm collimation before and after the intravenous administration of contrast material. Results. CT allowed us to correctly define the site, size and structure of lesions in all cases and to identify signs of invasion of neighbouring structures in some cases. The lesions exhibited solid density on the unenhanced scan and poor enhancement after contrast-medium administration; lesion structure was homogeneous in ten cases and inhomogeneous in 16; in one case, histology revealed microcalcification that had not been detected by CT. Conclusions. CT, with its panoramic capabilities and high contrast resolution, provides essential information for treatment planning and for the follow-up of GIST patients treated with surgery or chemotherapy