IgG4-related disease

A case of
IgG4-related anterior mediastinal sclerosing disease coexisting with autoimmune pancreatitis. Anterior mediastinal mass. Chest computed tomography (CT) revealed a 2.5-cm well-defined homogenous mass in the anterior mediastinum at the time of diagnosis with autoimmune pancreatitis (a), and the tumor decreased in size to 2 cm on CT with oral steroid treatment 6 years ago (b). The tumor had regrown to 3 cm at the time of referral to our department on CT (c, d), and positron emission tomography showed a high maximum standardized uptake of 3.6 by the anterior mediastinal mass lesion (e)
surgicalcasereports.springeropen.com • CC-by-4.0
Abdominal
manifestations of IgG4-related disease: a pictorial review. A patient with a history of mucosa-associated lymphoid tissue (MALT) lymphoma, presenting with raised serum IgG4 and diagnosis of AIP on endoscopic ultrasound (EUS)-guided biopsy. a Axial T2 fat saturated (FS) turbospin echo image shows intermediate to high T2 signal within the pancreas (arrows), but extensive low T2 signal peripancreatic tissue (*). There is diffuse narrowing of the distal pancreatic duct. b Axial T1FS post-contrast arterial phase initially shows reduced enhancement of the peripancreatic tissue (*) compared to the pancreas (arrows). c Axial T1FS post-contrast equilibrium phase showing delayed enhancement of the peripancreatic tissue. d The pancreas and extrapancreatic soft tissue shows marked restricted diffusion with high signal on diffusion-weighted imaging—B800 (arrows), a typical finding in AIP. e The tissue demonstrates corresponding low signal on the ADC map (arrows). f Only the extrapancreatic soft tissue shows high-grade FDG uptake (arrows), a finding which was felt to be atypical for lymphoma
insightsimaging.springeropen.com • CC-by-4.0
Abdominal
manifestations of IgG4-related disease: a pictorial review. Example of extrarenal IgG4 disease. a CECT showing a rind of abnormal soft tissue (*) anterior to the right kidney and encasing the IVC. b) Axial T2 turbo spin echo image of the same patient as in Fig. 9a shows perirenal soft tissue is of low T2 signal (*). c Axial T1FS pre-contrast shows perirenal soft tissue of intermediate T1 signal (*). d Axial T1FS post-contrast arterial phase shows minimal enhancement of the perirenal soft tissue (*). e Axial T1FS post-contrast equilibrium phase shows delayed enhancement related to a cuff of soft tissue around the IVC (arrow) with most of the perirenal soft tissue remaining low T1 signal (*). f The enhancing tissue around the IVC shows restricted diffusion with high signal on DWI—B800 (arrows). g There is corresponding low signal on the ADC map (arrows)
insightsimaging.springeropen.com • CC-by-4.0
Abdominal
manifestations of IgG4-related disease: a pictorial review. Example of IgG4 disease affecting the left kidney. a Axial CECT shows typical wedge-shaped low-density renal cortical lesions in the left kidney (arrows). Both kidneys are stented as there was also IgG4 related retroperitoneal fibrosis. b Coronal FDG PET/CT in same patient as in a showing multifocal high-grade uptake in the left kidney (arrow). c Example of bilateral renal pelvic involvement in a different patient (arrows) on CECT. d Axial T2 turbospin echo image in different patient shows renal lesions (arrows) are of mixed high and low T2 signal (typically reported in the literature as low T2 signal)
insightsimaging.springeropen.com • CC-by-4.0
Abdominal
manifestations of IgG4-related disease: a pictorial review. Example of IgG4-related biliary stricture in a different patient. a Strictures are seen in the common hepatic duct and distal CBD (arrows). Sto stomach, Duo duodenum. b ERCP performed 6 months later on the same patient shows resolution of the extrahepatic strictures after treatment with corticosteroids
insightsimaging.springeropen.com • CC-by-4.0
Abdominal
manifestations of IgG4-related disease: a pictorial review. Example of IgG4 biliary stricture: MRCP shows a long smooth stricture involving the intrapancreatic portion of the CBD (arrow). The proximal pancreatic duct is also attenuated (arrowhead)
insightsimaging.springeropen.com • CC-by-4.0
Abdominal
manifestations of IgG4-related disease: a pictorial review. a Axial T2 balanced gradient echo image shows diffuse enlargement of the body and tail of the pancreas (arrows) with proximal biliary dilatation due to a distal common bile duct (CBD) stricture (*). b Following corticosteroid treatment, the pancreas is less bulky (arrows) and the biliary dilatation is no longer prominent (*) due to resolution of the previous biliary stricture (not shown)
insightsimaging.springeropen.com • CC-by-4.0
Abdominal
manifestations of IgG4-related disease: a pictorial review. Coronal FDG-PET/CT study showing multifocal IgG4 disease with involvement of the right lung, tail of the pancreas and retroperitoneum (arrows)
insightsimaging.springeropen.com • CC-by-4.0
Abdominal
manifestations of IgG4-related disease: a pictorial review. Axial T1FS post-contrast imaging demonstrating late enhancement in diffuse AIP. a The pancreas is relatively hypointense in the arterial phase (arrows). b The pancreas shows more avid enhancement in the portovenous phase (arrows)
insightsimaging.springeropen.com • CC-by-4.0
Abdominal
manifestations of IgG4-related disease: a pictorial review. a CECT showing example of diffuse AIP with sausage shaped configuration to the pancreas and subtle peripancreatic halo of low attenuation (arrows). b Axial CECT in a different patient showing example of focal AIP affecting the head of the pancreas (arrows). c Coronal CECT in the same patient (Fig. 2b) demonstrating focal pancreatic head involvement (arrows)
insightsimaging.springeropen.com • CC-by-4.0
IgG4-related
disease • IgG4-related autoimmune cholangitis, pancreatitis, and pseudotumor - Ganzer Fall bei Radiopaedia
Hartung M, IgG4-related autoimmune cholangitis, pancreatitis, and pseudotumor. Case study, Radiopaedia.org (Accessed on 27 Nov 2022) https://doi.org/10.53347/rID-99511 • CC by-nc-sa 3.0 (modified)
Abdominal
manifestations of IgG4-related disease: a pictorial review. Example of IgG4 retroperitoneal fibrosis. a Axial CECT showing enhancing soft tissue partly encasing the IVC and aorta (arrows). b Axial FDG PET/CT showing increased FDG uptake in the abnormal retroperitoneal soft tissue (arrows). c Axial CECT in the same patient, with reduced periaortic soft tissue, following treatment with corticosteroids. d Corresponding T1FS post-contrast MRI showing residual enhancing soft tissue around the aorta following steroid treatment (arrows)
insightsimaging.springeropen.com • CC-by-4.0
nicht verwechseln mit: IgG4-Autoimmunerkrankungen
Radiopaedia • CC-by-nc-sa 3.0 • de

IgG4-related disease (IgG4-RD) is a systemic disease that is characterized by fibroinflammatory infiltration of various organs, including by plasma cells that express IgG4 (immunoglobulin G subclass 4).

Terminology

This condition has been known by many other names in the past, such as IgG4-related sclerosing disease, IgG4-related systemic disease, IgG4-related systemic sclerosing disease, IgG4-syndrome, IgG4-associated disease, and IgG4-related multi-organ lymphoproliferative syndrome. Since 2012, however, IgG4-related disease has become the preferred term .

Epidemiology

IgG4-related diseases are more common in middle age or elderly males.

Clinical presentation

Clinical manifestations vary by the organ involved. These organ systems may be either synchronously or sequentially involved.

Pathology

Location

The disease spectrum is broad across organ systems:

Microscopic appearance

There are three major histopathologic characteristics of IgG4-related disease :

  • dense lymphoplasmacytic infiltrate
  • at least focally storiform fibrosis
  • obliterative phlebitis

Two of these features are required for a confident pathologic diagnosis of IgG4-related disease, with a few organ-specific exceptions . Additional supporting features are non-obliterative phlebitis and eosinophilia .

Immunophenotype

Immunohistochemistry should demonstrate plasma cells that stain for IgG4. IgG4+ plasma cells must account for more than 40% of IgG+ plasma cells . The absolute density of IgG4+ plasma cells (number per high power field) suggestive of the diagnosis varies by organ. For example, the cutoff is >10/hpf for pancreatic biopsy specimens and >100/hpf for lacrimal and salivary glands .

Markers

Serology is often, but not always, abnormal in IgG4-related diseases. A serum IgG4 level >135 mg/dL is supportive of the diagnosis .

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu IgG4-assoziierte Erkrankung:
Cholezystitis
 
Wikipedia • CC BY-SA 4.0
sclerosing
cholangitis
Wikipedia • CC BY-SA 4.0
Autoimmunpankreatitis
 
Wikipedia • CC BY-SA 4.0
interstitielle
Pneumonie
Wikipedia • CC-BY-SA-3.0
IgG4-assoziierte
hypertrophe Pachymeningitis
bmcneurol.biomedcentral.com • CC-by-4.0
IgG4-assoziierte
Erkrankung des Mediastinums
surgicalcasereports.springeropen.com • CC-by-4.0
IgG4-assoziierte
Cholangitis
Wikipedia • CC BY-SA 4.0