S1538 A Rare Case of Isolated IG4 Autoimmune Cholangitis

Tagliaferri, Ariana R; Ruparel, Heemani; Melki, Gabriel; Cavanagh, Yana; Grossman, Matthew

doi:10.14309/01.ajg.0000779684.80023.bf

Cited by 1 publication

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“…Biopsy of the common hepatic duct showed attenuated biliary epithelium with active inflammation and no malignancy (Figure 3). These findings were compatible with IgG4-related AC, in the absence of pancreatitis [4]. The patient demonstrated significant clinical and laboratory improvement with steroid treatment, and he was subsequently discharged on prednisone at 45 milligrams per day for four weeks.…”

Section: Figure 1: Endoscopic Retrograde Cholangiography On First Adm...supporting

confidence: 54%

“…His skin, tongue and eyes were jaundiced. On the abdominal exam, there was epigastric and RUQ tenderness upon superficial and deep palpation without rigidity or guarding, no evidence of organomegaly and normoactive bowel sounds [4].…”

Section: Case Presentationmentioning

confidence: 97%

“…Computerized tomography (CT) of the abdomen/pelvis showed no acute abnormalities, including no evidence of pancreatitis. Magnetic resonance cholangiopancreatography (MRCP) revealed normal common bile duct, trace intrahepatic ductal dilatation and attenuation of the ducts near the confluence of the common hepatic duct with concerns for an underlying mass [4].…”

Section: Case Presentationmentioning

confidence: 99%

“…ERCP showed biliary strictures of the common hepatic duct at the level of bifurcation, and the ventral pancreatic duct and common bile ducts were cannulated using plastic stents (Figure 1) [4].…”

Section: Case Presentationmentioning

confidence: 99%

“…However, a study in Japan estimated the annual prevalence of IgG4-RD in AIP patients as 4.6/100,000 of the population in 2011, with 10.3% of patients found to have porta hepatis involvement and 23.5% having intrahepatic disease [1]. Of all cases, only 8% of IgG4 RD accounts for isolated IgG4 AC [1][2][3][4]. A history of allergy, atopy and other autoimmune diseases have been described in isolated IgG4-AC [3].…”

Section: Introductionmentioning

confidence: 99%

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Isolated IgG4 Autoimmune Cholangitis in the Absence of Pancreatitis

Tagliaferri¹,

Ruparel²,

Melki³

et al. 2022

Cureus

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The IgG4-related disease is an uncommon immune-mediated condition affecting multiple organ systems concomitantly; however, it is very rare for a patient to have isolated IgG4 autoimmune cholangitis or IgG4related disease confined to the hepatobiliary system. The majority of cases are associated with pancreatitis and are incidentally discovered in the workup of acute or chronic pancreatitis. When it affects the hepatobiliary system, it develops as inflammatory fibrosclerosing cholangitis, which can mimic other hepatobiliary diseases such as primary sclerosing cholangitis. Herein, we present a case of type 1 IgG4 autoimmune cholangitis in the absence of pancreatitis. Our case is particularly unique because type 1 is the most common type associated with autoimmune pancreatitis; however, our patient had type 1 without any evidence of pancreatic involvement. Additionally, like most cases of isolated IgG4 autoimmune cholangitis, our patient was refractory to standard therapy. This case highlights the clinical significance, rarity and severity of isolated IgG4 autoimmune cholangitis.

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Section: Figure 1: Endoscopic Retrograde Cholangiography On First Adm...supporting

confidence: 54%

Section: Case Presentationmentioning

confidence: 97%

Section: Case Presentationmentioning

confidence: 99%

Section: Case Presentationmentioning

confidence: 99%