IgG4-related disease with multiple organs involvement was effectively controlled by glucocorticoids: a case report

Abstract

Immunoglobulin G4 (IgG4) related disease is a rare autoimmune disease involving multiple organs and tissues. A diagnosis of IgG4-related disease (IgG4-RD) is mainly based on serum IgG4 concentration, imaging, pathology, and effective glucocorticoids therapy. In this paper, we report a 53-year-old male with typical signs and symptoms of IgG4-RD successfully treated with glucocorticoids. This patient had experienced bilateral mumps for more than 8 months and intermittent abdominal pain spreading to his lower back for 2 months before his admission. During his hospitalization, based on the characteristic appearance of magnetic resonance imaging (MRI), magnetic resonance cholangiopancreatography (MRCP), and computed tomography with positron emission tomography, a diagnosis of autoimmune pancreatitis (AIP), cholangitis with biliary obstruction, lachrymal adenitis, and submandibular adenitis was made. A high serum IgG4 concentration further supported a diagnosis of IgG4-RD. Then, the treatment was promptly initiated with corticosteroids. MRI, MRCP, and IgG4 concentration were re-examined during his follow up, suggesting that glucocorticoid treatment resulted in a resolution of his disease. The dosage of glucocorticoid had been gradually decreased. Now, he is stable with oral low-dose glucocorticoids. Certainly, long-term follow up of this patient with such a rare disease is very essential to observe the possibility of disease recurrence and glucocorticoids related complications.

Keywords: IgG4-related disease (IgG4-RD); Mikulicz’s disease (MD); autoimmune pancreatitis (AIP); biliary cholangitis; glucocorticoids.

Figure 1

Magnetic resonance imaging (MRI) suggested diffuse thickening of the pancreas and dilatation of the pancreatic duct. (A) MRI performed on July 30, 2018 suggested diffuse thickening of the pancreas and dilatation of the pancreatic duct. (B) MRI performed on August 23, 2018 suggested that diffuse thickening of the pancreas and dilatation of the pancreatic duct slightly improved. (C) MRI performed on December 12, 2018 suggested that diffuse thickening of the pancreas and dilatation of the pancreatic duct had little change from before. (D) MRI performed on March 27, 2019 suggested that diffuse thickening of the pancreas and dilatation of the pancreatic duct had little change from before.

Figure 2

Magnetic resonance cholangiopancreatography (MRCP) indicated local dilatation of the intrahepatic bile duct with an internal diameter of dilated bile duct reaching 1.2 cm, a beak-like stenosis at the end of the bile duct, and unclear proximal pancreatic duct. (A) MRCP performed on July 6, 2018 suggested that the left and right intrahepatic bile ducts were locally dilate and the end of common bile duct was locally narrowed. (B) MRCP performed on August 23, 2018 suggested the internal diameter of the dilated bile duct reaching 1.1cm, which was a bit better than before. (C) MRCP performed on December 12, 2018 suggested that biliary stricture was not significantly changed than before. (D) MRCP performed on March 27, 2019 suggested that biliary stricture was not significantly changed than before.

Figure 3

Computed tomography with positron emission tomography (CT-PET) upon admission. It suggested diffusely increased metabolism in pancreas (SUVmax: 4.0), bilateral submandibular gland (SUVmax: 4.4), tear ducts (SUVmax: 5.4), and parotid gland (SUVmax: 2.9).

Figure 4

Dynamic change of serum immunoglobulin G (IgG) (A), serum amylase (B), serum lipase (C), serum alanine aminotransferase (ALT) (D), serum aspartate aminotransferase (AST) (E), serum total bilirubin (TBIL) (F), serum direct bilirubin (DBIL) (G), and r-glutamyl transpeptidase (r-GGT) (H).