Background: Autoimmune pancreatitis is a unique clinical entity proposed recently, and is sometimes associated with inflammation of other organs.

Aims: To examine the pathophysiology of the pancreas and other organs in patients with autoimmune pancreatitis.

Patients and methods: We evaluated clinicopathological findings in six resected and one autopsied patient with autoimmune pancreatitis. The pancreas, peripancreatic tissue, bile duct, and gall bladder were examined histologically and immunohistochemically. Biopsied salivary gland and cervical lymph node of one patient were also examined. We also performed similar immunohistochemical examinations in pancreatectomy specimens from 10 patients with alcoholic chronic pancreatitis and biopsied salivary glands from five patients with Sjogren’s syndrome.

Results: Stenosis of the extrahepatic bile duct was detected in all patients. Histological findings were characterised by diffuse lymphoplasmacytic infiltration with marked interstitial fibrosis and acinar atrophy, obliterated phlebitis of the pancreatic veins, and involvement of the portal vein. Immunohistochemically, diffusely infiltrating cells consisted predominantly of CD4 or CD8 positive T lymphocytes and IgG4 positive plasma cells. Similar inflammatory processes also involved the peripancreatic tissue, extrahepatic bile duct, gall bladder, and salivary gland. Lymph nodes were swollen with infiltration of IgG4 positive plasma cells. None of these findings was seen in alcoholic chronic pancreatitis or Sjogren’s syndrome.

Conclusions: The development of the specific inflammations in extensive organs as well as the pancreas in patients with autoimmune pancreatitis strongly suggests a close relationship between autoimmune pancreatitis and multifocal fibrosclerosis.

/pdf/close-relationship-between-autoimmune-pancreatitis-and-460bd7lu6n.pdf

Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis

Pain syndromes of the urogenital and rectal area are well described but poorly understood and underrecognized focal pain syndromes. They include vulvodynia, orchialgia, urethral syndrome, penile pain, prostatodynia, coccygodynia, perineal pain, proctodynia and proctalgia fugax. The etiology of these focal pain syndromes is not known. A specific secondary cause can be identified in a minority of patients, but most often the examination and work-up remain unrevealing. Although these patients are often depressed, rarely are these pain syndromes the only manifestation of a psychiatric disease. This review article presents an overview of the neuroanatomy of the pelvis, which is a prerequisite to trying to understand the chronic pain syndromes in this region. We then discuss the clinical presentation, etiology and differential diagnosis of chronic pain syndromes of the urogenital and rectal area and review treatment options. The current knowledge of the psychological aspects of these pain syndromes is reviewed. Patients presenting with these pain syndromes are best assessed and treated using a multidisciplinary approach. Currently available treatment options are empirical only. Although cures are uncommon, some pain relief can be provided to almost all patients using a multidisciplinary approach including pain medications, local treatment regimens, physical therapy and psychological support, while exercising caution toward invasive and irreversible therapeutic procedures. Better knowledge of the underlying pathophysioloigical mechanisms of the urogenital and rectal pain syndromes is needed to allow investigators to develop treatment strategies, specifically targeted against the pathophysiological mechanism.

The urogenital and rectal pain syndromes

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. 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. 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. 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.

/pdf/hyper-igg4-disease-report-and-characterisation-of-a-new-5gfyop8lz3.pdf

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

A total of 122 patients with a history of non-febrile epileptic seizures were identified in a survey of 6000 persons from a single general practice. The lifetime prevalence was 20.3/1000 including single seizures and 17.0/1000 excluding single seizures; 5.3/1000 had active epilepsy. Diagnosis and classification of seizures in such a survey were difficult. Most patients were seen by a hospital specialist at some point, but hospital follow up was sporadic. Overall patient assessment and monitoring was poor. There is a place for specialised epilepsy clinics that could be organised along the lines of the clinics for diabetes. These provide facilities for initial diagnosis and assessment, for planning long term management, for the selected follow up of difficult cases, and for referral for specific problems. Such a system would improve the long term care of patients with epilepsy.

https://www.bmj.com/content/bmj/287/6393/641.full.pdf

Patrick C. Walshi

Papers

Idiopathic Retroperitoneal Fibrosis