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

Adolescent perspectives on social support received in the aftermath of sexual abuse: a qualitative study

The extent and quality of social support provided to young survivors of sexual abuse (SA) have only rarely been examined. This qualitative study aimed to investigate adolescent perspectives on social support received in the aftermath of SA. A total of 26 sexually victimized adolescents (15-18 years old) participated in a qualitative face-to-face, in-depth interview that focused on perceived social support. Qualitative content analysis was conducted as per Mayring (2008) using the qualitative data analysis program ATLAS.ti. In addition, quantitative correlational analyses were conducted to identify characteristics of SA and their associations with perceived social support. Although participants perceived parental support as the most necessary type of support, they were much more satisfied with support from peers. In particular, adolescents stated that they wished they had received more emotional support from their parents in order to better cope with the abuse. About half of participants reported having received counseling, and counseling was seen as very helpful in dealing with the consequences of SA. Only a few adolescents mentioned their school as a source of support. Intra-familial abuse, younger victim age at the time of abuse, an adult perpetrator, and severe abuse were all negatively associated with satisfaction with perceived support. Our results suggest that support for young survivors of SA needs to be improved. Prevention of SA needs particular focus on improving parental reactions to SA, facilitating access to professional support, and raising teacher awareness of the importance of their role in the provision of support for sexually victimized children