Idiopathic Mediastinal Fibrosis
Fig. 10.1 Mediastinal tissue biopsy of a patient affected by idiopathic mediastinal fibrosis with elevated serum IgG4 levels. The pictures show fibrotic tissue with linfo-plasmacellular infiltration (a). IgG4 (b) and…
Retroperitoneal Fibrosis and the Spectrum of Chronic Periaortitis
Fig. 8.1 Schematic representation of the clinical spectrum of retroperitoneal fibrosis and chronic periaortitis 8.2 Secondary Forms of RPF The clinical entities included in the spectrum of CP are idiopathic….
Genetics of Fibroinflammatory Disorders
Gene/locus and variation/allele Population Cases Controls Odds ratio P-Value Reference HLA-B (rs3099844) Norwegian 285 PSC 298 4.8 2.6 × 10−26 Karlsen et al. [19] GPC6 (rs9524260) Norwegian 285 PSC 298…
Basic Mechanisms Linking Inflammation and Fibrosis
Fig. 2.1 Myofibroblasts are central to fibrogenesis and are submitted to regulation by pro- and anti-fibrotic cytokines. Schematic and simplified view of the network of cytokines influencing ECM deposition by…
Autoimmune (IgG4-Related) Pancreatitis
Fig. 6.1 (a) Computed tomography (CT) appearance of diffuse AIP: the pancreatic parenchyma appears diffusely hypodense in the body-tail of the pancreas on arterial phase. (b) CT appearance of focal…
Pathophysiology of IgG4-Related Disease
© Springer International Publishing Switzerland 2017Augusto Vaglio (ed.)Systemic Fibroinflammatory DisordersRare Diseases of the Immune System10.1007/978-3-319-41349-5_5 5. Pathophysiology of IgG4-Related Disease Jochen Zwerina1 (1) Ludwig Boltzmann Institute of Osteology at the Hanusch Hospital of WGKK and…
Pathology of Enteric Neuromuscular Disorders
Fig. 17.1 Diagnosis of Hirschsprung disease by rectal suction biopsy. (a) An adequate suction rectal biopsy should be ≥2 mm in greatest dimension and contain a generous sample of submucosa….
Radionuclide Gastrointestinal Transit Tests
Recommended meal (a) 118 mL (4 oz.) of liquid egg whites (e.g., eggbeaters [ConAgra Foods, Inc.] or an equivalent generic liquid egg white) (b) Two slices of toasted white bread (c) 30 g…
Colonic Manometry
Fig. 9.1 Abdominal radiograph showing a high-resolution solid-state colonic motility catheter placed with its tip at the hepatic flexure. The arrow points to the endoclip used to secure the catheter…
Anorectal Manometry
Fig. 10.1 (a) Water-perfusion catheter (Medical Measurement Systems). (b) High-resolution catheter (Medical Measurement Systems). (c) Three-dimensional high-resolution catheter (Sierra Scientific Instruments) (Courtesy of Medical Measurement Systems) The water-perfusion catheter consists…
