Clinical Case Summary

A 71-year-old female presented to the gastroenterology clinic following a two year history of dysphagia and sensation of food sticking in the oesophagus. Her dysphagia was limited to solid foods, and symptoms were not exacerbated by positional change. Pain was mostly localised around the sternal notch where she would have the sensation of a food bolus. Over the preceding 2 years, she had experienced about 7.5kg of weight loss with no constitutional symptoms of fevers or fatigue. Her past medical history included hypertension, dyslipidaemia, lower back pain, cold agglutinin disease, monoclonal B lymphocytosis and peripheral arterial disease. On examination, there were no signs of malnourishment or palpable lymph nodes in the axilla, neck or supraclavicular region. Her abdomen was soft and non-tender. Given largely non-illuminating blood results, an oesophageal-gastroduedonoscopy was performed. However, no findings were noted, specifically no oesophageal webs, Zenker diverticulum, gastroparesis, trachealisation of the oesophagus or corrosive pathology. H. pylori was also not isolated on histopathology. A barium swallow was then performed which showed extensive external compression of the proximal third of the oesophagus at the level of the arch of the aorta, most indicative of dysphagia lusoria (Fig 1). Computed tomography angiography (CTA) showed retroesophageal aberrant right subclavian artery bifurcating from the ascending aorta independent of a brachiocephalic artery and causing external oesophageal compression (Fig 2 & 3). Given this definitive diagnosis, the patient was then seen in clinic where management of dysphagia lusoria was explained. Despite ongoing symptoms, the patient decided to continue with conservative management and lifestyle modifications, which included eating smaller bites and increase in fluid intake. Although an extensive compression typically requires surgical intervention, our patient was stable at two-year follow-up (Fig 4 A-B).

References

Figure 1: Barium swallow examination showing extrinsic compression of the proximal third of the oesophagus at the level of the aortic arch (white arrow).
Figure 2: Axial view of computed tomography angiography (CTA) showing the posterior course of the retroesophageal aberrant right subclavian artery.
Figure 3: Axial view of computed tomography angiography (CTA) showing compression of oesophagus (white arrow) by the retroesophageal aberrant right subclavian artery (white arrowhead).
Figure 4: Computed tomography angiography (CTA) multi-planar reconstruction (MPR) showing the posterior course of the right subclavian artery (white arrow), crossing the mediastinum and compressing the oesophagus. A. coronal view B. Sagittal view

Disclosure

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Clinical Case Summary

Introduction: PSC is closely associated with IBD and affects up to 3.6% of Crohn's disease patients, while AIH is also more prevalent in IBD patients, in up to 16% of patients.
Case report: We report a female patient who, at the age of 14, presented with right hypochondrial pain and abnormal liver biochemistry (SGOT: 44 U/l, SGPT: 145 U/l, γ-GT: 300 U/l, ALP: 421 U/l, TBL: 1 mg/dL). The patient underwent MRCP (Figure A) which revealed local strictures with prostenotic dilatation in both intrahepatic and extrahepatic bile ducts, suggestive of PSC. One year later the patient presented with severe abdominal pain and bloody diarrhea. Ileocolonoscopy showed terminal ileitis (Figure B) and segmental colitis. Histological evaluation (Figure C) showed disruption of the architecture, focal erosions of the epithelium, mild mucopenia, inflammatory infiltrate in the dermis, sites of cryptitis and focal formation of crypt abscesses with extension of inflammation to the muscularis mucosae, suggestive of Crohn’s Disease. Two years later, in a regular laboratory test, the patient had abnormal liver function tests (SGOT: 211 U/l, SGPT: 603 U/l, γ-GT: 54 U/l, ALP: 105 U/l, TBL: 0.9 mg/dl). Further evaluation with immunoassay revealed ANA (+) 1/80, AMA (-), SMA (+) 1/160, + F-actin, anti-LKM (-), anti-LC (-), anti-LM (-), p ANCA (+) 1/80, so the patient underwent percutaneous liver biopsy with findings of portal spaces with presence of waxy-containing macrophages, thin fibrous septa, parenchyma with centrolobular necrosis with aggregates of inflammatory cells and waxy macrophages, suggestive of Autoimmune Hepatitis. Today, the patient is in clinical remission under treatment with Ursodeoxycholic acid, Ustekinumab and Mycophenolate Mofetil.
Conclusion: The association of IBD with PSC, AIH and variant forms is now known, however the complexity of the diseases presents a challenge for the clinician and the provision of individualized care.

References

1. Ballotin VR, Bigarella LG, Riva F, Onzi G, Balbinot RA, Balbinot SS, Soldera J. Primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome associated with inflammatory bowel disease: A case report and systematic review. World J Clin Cases. 2020 Sep 26;8(18):4075-4093.
2. Saich R, Chapman R. Primary sclerosing cholangitis, autoimmune hepatitis and overlap syndromes in inflammatory bowel disease. World J Gastroenterol. 2008 Jan 21;14(3):331-7.
3. Rocha HC, Vilela EG. Clinical aspects and prognosis of patients with inflammatory bowel disease associated with autoimmune liver diseases. Gastroenterol Hepatol. 2022 Feb;45(2):83-90. English, Spanish.
4. Malik T, A, Gutierrez A, M, McGuire B, Zarzour J, G, Mukhtar F, Bloomer J: Autoimmune Hepatitis-Primary Sclerosing Cholangitis Overlap Syndrome Complicated by Crohn’s Disease. Digestion 2010;82:24-26.