Isolated IgG4-related sclerosing cholangitis misdiagnosed as malignancy in an area with endemic cholangiocarcinoma: a case report

Rungsakulkij, Narongsak; Sornmayura, Pattana; Tannaphai, Penampai

doi:10.1186/s12893-017-0214-1

Cited by 18 publications

(17 citation statements)

References 42 publications

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“…Whereas PSC and cholangiocarcinoma may be treated through surgical intervention, ISC is treated with a medical approach, specifically high dose steroids. It thus is important to consider, and could have prevented unnecessary surgery in various case reports of patients wrongly diagnosed with other causes of biliary pathology 4 5…”

Section: Discussionmentioning

confidence: 99%

Atypical presentation of IgG₄sclerosing cholangitis

2019

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IgG4 sclerosing cholangitis (ISC) is a rare disease which usually presents in patients in their 60’s or above. Patients often present with obstructive jaundice as the first symptom, especially when associated with autoimmune pancreatitis. We report the case of a 20-year-old man who presented with acute abdominal pain, which was subsequently diagnosed as ISC in addition to autoimmune pancreatitis following magnetic resonance cholangiopancreatography (MRCP). The patient was thus treated with steroids.

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Section: Discussionmentioning

confidence: 99%

Atypical presentation of IgG₄sclerosing cholangitis

2019

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“…There are some characteristic image findings in computed tomography and MRI that are useful for the diagnosis of IAC, including multifocal biliary strictures, a smooth outer margin, a markedly thickened wall of bile duct, or a narrow but visible lumen. [ 23 ] And we will find hyperenhancement during the late arterial phase, homogeneous hyperenhancement during the delayed phase, concurrent gallbladder wall thickening, and lack of vascular invasion. [ 23 – 27 ]…”

Section: Discussionmentioning

confidence: 99%

“…[ 23 ] And we will find hyperenhancement during the late arterial phase, homogeneous hyperenhancement during the delayed phase, concurrent gallbladder wall thickening, and lack of vascular invasion. [ 23 – 27 ]…”

Section: Discussionmentioning

confidence: 99%

Analysis of clinical characteristics and treatment of immunoglobulin G4-associated cholangitis

Xiao

Xu²,

Li³

et al. 2018

Medicine

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Immunoglobulin (Ig)G4-associated cholangitis (IAC) is one of the common organ manifestations of IgG4-related systemic disease (ISD). IAC and autoimmune pancreatitis (AIP) may mimic sclerosing cholangitis, cholangiocarcinoma, or pancreatic carcinoma. Diagnosis is based on a combination of clinical, biochemical, radiological, and histological findings.To study the clinical presentation of and treatment strategy for IAC, we reviewed clinical, serologic, and imaging characteristics, as well as treatment response, in 39 patients with IAC. The majority of patients were men (82%). Clinical features on presentation included obstructive jaundice in 26 patients (67%) and abdominal pain in 20 (51%). Positive IgG4 immunostaining was seen in 27 patients. The median serum IgG4 level before treatment was 769.4 mg/dL (range, 309.1–1229.7 mg/dL). After the steroid therapy, the median serum IgG4 level in 23 patients was 247.0 mg/dL (range, 139.0–355.0 mg/dL). Cholangiograms were available in 36 (92%) patients. Stenosis of the lower part of the common bile duct was found in 26 of 39 patients. Stenosis was diffusely distributed in the intra- and extrahepatic bile ducts in 14 of 39 patients. Additionally, strictures of the bile duct were detected in the hilar hepatic lesions in 27 of 39 patients. AIP was the most frequent comorbidity (35/39 in this study) of IAC. Other affected organs included eyes (n = 6), salivary glands (sialadenitis, n = 10), lymph nodes (mediastinal and axillary, n = 3), kidneys (n = 2), and the retroperitoneum (retroperitoneal fibrosis, n = 2).Regarding treatment, 29 patients were treated with steroids, of whom one underwent pancreatoduodenectomy, and one underwent choledochojejunostomy. Eight patients were treated with biliary stents. The remaining 19 patients took prednisolone alone. Eight patients achieved spontaneous resolution. Four patients with suspected pancreatic cancer or cholangiocarcinoma underwent surgery, including 2 patients who also received postoperative steroids. All patients were regularly followed up for 9 to 36 months. Only 2 patients in the steroids treatment group relapsed to manifest obstructive jaundice and high serum IgG4 levels. These 2 patients were treated with steroids and biliary stents, resulting in complete remission.We also review the diagnostic and therapeutic management and discuss recent pathophysiological findings, which might aid in understanding the molecular mechanisms contributing to IAC and other manifestations of IgG4-related diseases (IgG4-RD). Biomarkers that are more accurate are needed to correctly diagnose IAC and prevent misdiagnoses and unnecessary therapeutic interventions.

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“…The rate of benign strictures misdiagnosed preoperatively as cholangiocellular carcinoma is high, variant data can be found in the literature: about 7-18%, even up to 50% [4,15,16]. The term 'malignant masquerade' was first used in 1985 by Hadjis et al, they described the malignant-behaving, but histologically proven benign bile duct strictures with this expression, the lesions of which cannot be distinguished by clinical or imaging features [17].…”

Section: This Classification Was Described Bymentioning

confidence: 99%

WITHDRAWN: Double Rarity: Malignant Masquerade Biliary Stricture in a Situs Inversus Totalis Patient. A Case Report

Eitler

Máthé

Papp

et al. 2020

Preprint

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Background. Situs inversus totalis is a rare anatomical variation of both the thoracic and the abdominal organs. Common bile duct strictures can be caused by malignant and benign diseases as well. 7-18% of the latter ones are 'malignant masquerade’ cases, as pre-operative differentiation is difficult. Case presentation. We present the case of a 68y male patient with known situs inversus totalis and a recent onset of obstructive jaundice caused by a malignant behaving common bile duct stricture. Technically difficult endoscopic retrograde cholangiopancreatography, brush cytology, magnetic resonance cholangiopancreatography, endoscopic ultrasound, and percutaneous transhepatic drainage with stent implantation were performed for proper diagnosis. Cholecystectomy, common bile duct resection with hilar lymphadenectomy and a hepatico-jejunostomy has been performed following multidisciplinary consultation. The final histology report did not confirm any clear malignancy, the patient is doing well.Conclusion. In situs inversus patients, both diagnostic and therapeutic procedures can lead to various difficulties. Benign biliary strictures are frequently misdiagnosed preoperatively as cholangiocellular carcinoma. The surgery is usually unavoidable, involving a significant risk of complications.The co-existence of these two difficult diagnostic and therapeutic features made our case challenging.

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Isolated IgG4-related sclerosing cholangitis misdiagnosed as malignancy in an area with endemic cholangiocarcinoma: a case report

Cited by 18 publications

References 42 publications

Atypical presentation of IgG₄sclerosing cholangitis

Atypical presentation of IgG₄sclerosing cholangitis

Analysis of clinical characteristics and treatment of immunoglobulin G4-associated cholangitis

WITHDRAWN: Double Rarity: Malignant Masquerade Biliary Stricture in a Situs Inversus Totalis Patient. A Case Report

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Isolated IgG4-related sclerosing cholangitis misdiagnosed as malignancy in an area with endemic cholangiocarcinoma: a case report

Cited by 18 publications

References 42 publications

Atypical presentation of IgG4sclerosing cholangitis

Atypical presentation of IgG4sclerosing cholangitis

Analysis of clinical characteristics and treatment of immunoglobulin G4-associated cholangitis

WITHDRAWN: Double Rarity: Malignant Masquerade Biliary Stricture in a Situs Inversus Totalis Patient. A Case Report

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Atypical presentation of IgG₄sclerosing cholangitis

Atypical presentation of IgG₄sclerosing cholangitis