Immunoglobulin G4-related disease: autoimmune pancreatitis and extrapancreatic manifestations

We present a case of immunoglobulin G4 (IgG4)-related disease with pancreatic and extrapancreatic involvement, including the biliary and renal systems. Given the importance of imaging methods for the diagnosis of IgG4-related disease and its differentiation from pancreatic adenocarcinoma, we emphasize important abdominal computed tomography and magnetic resonance imaging findings related to this recently recognized systemic autoimmune disease.

Keywords:
Pancreatitis; Autoimmune diseases; Pancreas; Computed tomography; Magnetic resonance imaging

Authorship

Daniel Alvarenga Fernandes Mailing address: Dr. Daniel Alvarenga Fernandes. Departamento de Radiologia - FCM-Unicamp. Rua Tessália Vieira de Camargo, 126, Cidade Universitária Zeferino Vaz. Campinas, SP, Brazil, 13083-887. E-mail: daniel_alvafer@yahoo.com.br; daniel_ alvafer@icloud.com.

MD, Attending Radiologist in the Radiology Department of the Faculdade de Ciências Médicas da Universidade Estadual de Campinas (FCM-Unicamp), Campinas, SP, Brazil.Universidade Estadual de CampinasBrazilCampinas, SP, BrazilMD, Attending Radiologist in the Radiology Department of the Faculdade de Ciências Médicas da Universidade Estadual de Campinas (FCM-Unicamp), Campinas, SP, Brazil.

Ricardo Yoshio Zanetti Kido

MD, Resident in Radiology and Diagnostic Imaging at the Faculdade de Ciências Médicas da Universidade Estadual de Campinas (FCM-Unicamp), Campinas, SP, Brazil.Universidade Estadual de CampinasBrazilCampinas, SP, BrazilMD, Resident in Radiology and Diagnostic Imaging at the Faculdade de Ciências Médicas da Universidade Estadual de Campinas (FCM-Unicamp), Campinas, SP, Brazil.

Ricardo Hoelz de Oliveira Barros

MD, MSc, Attending Radiologist in the Radiology Department of the Faculdade de Ciências Médicas da Universidade Estadual de Campinas (FCM-Unicamp), Campinas, SP, Brazil.Universidade Estadual de CampinasBrazilCampinas, SP, BrazilMD, MSc, Attending Radiologist in the Radiology Department of the Faculdade de Ciências Médicas da Universidade Estadual de Campinas (FCM-Unicamp), Campinas, SP, Brazil.

Daniel Lahan Martins

MD, Attending Radiologist in the Radiology Department of the Faculdade de Ciências Médicas da Universidade Estadual de Campinas (FCM-Unicamp), at the Pontifícia Universidade Católica de Campinas (PUC-Campinas), and at the Centro Radiológico Campinas - Hospital Vera Cruz, Campinas, SP, Brazil.Universidade Estadual de CampinasBrazilCampinas, SP, BrazilMD, Attending Radiologist in the Radiology Department of the Faculdade de Ciências Médicas da Universidade Estadual de Campinas (FCM-Unicamp), at the Pontifícia Universidade Católica de Campinas (PUC-Campinas), and at the Centro Radiológico Campinas - Hospital Vera Cruz, Campinas, SP, Brazil.

Thiago José Penachim

Nelson Marcio Gomes Caserta

PhD, Tenured Professor in the Radiology Department of the Faculdade de Ciências Médicas da Universidade Estadual de Campinas (FCM-Unicamp), Campinas, SP, Brazil.Universidade Estadual de CampinasBrazilCampinas, SP, BrazilPhD, Tenured Professor in the Radiology Department of the Faculdade de Ciências Médicas da Universidade Estadual de Campinas (FCM-Unicamp), Campinas, SP, Brazil.

Mailing address: Dr. Daniel Alvarenga Fernandes. Departamento de Radiologia - FCM-Unicamp. Rua Tessália Vieira de Camargo, 126, Cidade Universitária Zeferino Vaz. Campinas, SP, Brazil, 13083-887. E-mail: daniel_alvafer@yahoo.com.br; daniel_ alvafer@icloud.com.

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Figures

Figures (4)

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Figure 1
Axial contrast-enhanced CT scan of the abdomen, nephrographic phase. A: diffuse pancreatic enlargement (solid arrow). Note also a biliary stent in the common bile duct and hypoenhancing cortical nodules in both kidneys (dashed arrows). B: Retraction and obliteration of the pancreatic tail, together with a hypoenhancing halo (solid arrow), in addition to the renal nodules.

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Figure 2
Axial abdominal MRI scan. A: T1-weighted image without contrast. Diffusely increased, slightly hypointense pancreas with loss of the normal pancreatic roughness (solid arrow). Note also a hypointense renal nodule. B: Contrast-enhanced T1-weighted image, portal phase. Hypoenhancing peripancreatic halo (solid arrow), together with hypoenhancing, partially ill-defined renal nodules. C: T2-weighted image. Diffusely increased pancreas, shortened pancreatic tail, and loss of the normal pancreatic roughness (a “sausage-like” pancreas), as well as diffuse, irregular narrowing of the main pancreatic duct (solid arrow), together with hypoenhancing, partially ill-defined renal nodules. D: Diffusion restriction in the entire pancreas, with an ADC value of 0.839 × 10–3 mm²/s (solid arrow), as well as in the renal nodules (dashed arrows).

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Figure 3
Magnetic resonance cholangiography showing bile duct dilatation up to the intrapancreatic common bile duct (arrow).

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Figure 4
Axial abdominal MRI scan taken after corticosteroid therapy. A: Contrast-enhanced T1-weighted image, portal phase. Normally sized pancreas without the peripancreatic halo seen before (solid arrow). No renal nodules were seen on T1-weighted images. B: T2-weighted image. Normal-sized pancreas without the main pancreatic duct changes seen previously (solid arrow). Small nodules remain, seen only on T2-weighted images (dashed arrows) and smaller in size than those observed before corticosteroid therapy.

Figure 1 Axial contrast-enhanced CT scan of the abdomen, nephrographic phase. A: diffuse pancreatic enlargement (solid arrow). Note also a biliary stent in the common bile duct and hypoenhancing cortical nodules in both kidneys (dashed arrows). B: Retraction and obliteration of the pancreatic tail, together with a hypoenhancing halo (solid arrow), in addition to the renal nodules.

Figure 2 Axial abdominal MRI scan. A: T1-weighted image without contrast. Diffusely increased, slightly hypointense pancreas with loss of the normal pancreatic roughness (solid arrow). Note also a hypointense renal nodule. B: Contrast-enhanced T1-weighted image, portal phase. Hypoenhancing peripancreatic halo (solid arrow), together with hypoenhancing, partially ill-defined renal nodules. C: T2-weighted image. Diffusely increased pancreas, shortened pancreatic tail, and loss of the normal pancreatic roughness (a “sausage-like” pancreas), as well as diffuse, irregular narrowing of the main pancreatic duct (solid arrow), together with hypoenhancing, partially ill-defined renal nodules. D: Diffusion restriction in the entire pancreas, with an ADC value of 0.839 × 10–3 mm²/s (solid arrow), as well as in the renal nodules (dashed arrows).

Figure 3 Magnetic resonance cholangiography showing bile duct dilatation up to the intrapancreatic common bile duct (arrow).

Figure 4 Axial abdominal MRI scan taken after corticosteroid therapy. A: Contrast-enhanced T1-weighted image, portal phase. Normally sized pancreas without the peripancreatic halo seen before (solid arrow). No renal nodules were seen on T1-weighted images. B: T2-weighted image. Normal-sized pancreas without the main pancreatic duct changes seen previously (solid arrow). Small nodules remain, seen only on T2-weighted images (dashed arrows) and smaller in size than those observed before corticosteroid therapy.

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Publicação do Colégio Brasileiro de Radiologia e Diagnóstico por Imagem Av. Paulista, 37 - 7º andar - conjunto 71, 01311-902 - São Paulo - SP, Tel.: +55 11 3372-4541, Fax: 3285-1690, Fax: +55 11 3285-1690 - São Paulo - SP - Brazil
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