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.

Clinical Case Summary

Splenic granulomas are a rare extraintestinal manifestation of Crohn’s disease (CD). In previously reported cases, the diagnosis was confirmed by biopsy. However, due to the increased risk of complications associated with biopsy, confirmation by imaging is desirable.
52-year-old male patient presented with a previously diagnosed, currently untreated CD.
Symptoms:

• Abdominal pain in the right lower quadrant for several weeks, postprandial
• Bowel movements: 4-6 times/day for a few weeks, liquid consistency
• No fever or weight loss
• Palpation: tenderness in the lower right abdomen

Findings:

• C-reactive protein and leukocytes: not elevated
• Calprotectin: 184ug/g. Stool culture and intestinal cytomegaly virus PCR biopsy negative
• Tuberculosis interferon-g release assay: negative
• Colonoscopy and magnetic resonance enterography (MRE): ileitis of terminal Ileum (Fig. 1)
• MRE: multiple T2w-hyperintense splenic lesions, increased in number since last imaging 2024 (Fig. 2)
• B-mode ultrasound: splenomegaly, round nodules of mixed echogenicity (Fig. 3)
• Contrast-enhanced ultrasound (CEUS): 1,5ml contrasting agent (sulphur hexafluoride microbubbles): arterial isoenhancement and late phase hypoenhancement of nodules (Fig. 3), atypical for haemangiomas
• 18-Fluorodeoxyglucose positron emission tomography with computed tomography: no uptake in the spleen (Fig. 4)

After excluding malignancy, abscesses, sarcoidosis, and infectious causes, the splenic granulomatous lesions represented an extraintestinal manifestation of CD. The patient was started on prednisolone, 60mg/day, resulting in marked clinical improvement in 4 days; with reduced abdominal pain and decreased stool frequency. Patient declined recommended maintenance therapy with ustekinumab.
This CEUS pattern is novel to CD and resembles previously reported enhancement patterns of inflammatory lesions. This is a rare reported case of splenic granulomas as an extraintestinal manifestation of CD diagnosed non-invasively by CEUS.

References

Figure 1: Colonoscopy shows 2-4mm aphthous ulcers in the terminal Ileum
Figure 2: (A)T2-Haste and (B)T2-STIR MRE depicting multiple hyperintense splenic lesions
Figure 3: B-mode (left) and CEUS (right) of the splenic lesions. (A) Nodules appear mixed in echogenicity, nodules show isoenhancement during the arterial phase. (B) Nodules exhibit hypoenhancement in the late phase
Figure 4: 18F-Fluorodeoxyglucose (FDG) positron emission tomography with computed tomography shows no uptake of FDG in the spleen
1. Castellani L, Sartini A, Marocchi M, Balbi T, Belluzzi A. Splenic granulomas in a patient with severe Crohn's disease associated with multiple extraintestinal manifestations. Inflamm Bowel Dis. 2011 Jun;17(6):E35-7. doi: 10.1002/ibd.21665. Epub 2011 Feb 11. PMID: 21319276.
2. Schulz C, von Arnim U, Kuester D, Fischbach F, Malfertheiner P. Milzbefall - seltene extraintestinale Manifestation bei Morbus Crohn [Splenic granulomas - rare extraintestinal manifestation of Crohn's disease]. Z Gastroenterol. 2010 Dec;48(12):1367-70. German. doi: 10.1055/s-0029-1245562. Epub 2010 Dec 1. PMID: 21125512
3. Safai Zadeh E, Prosch H, Ba-Ssalamah A, Findeisen H, Alhyari A, Raab N, Görg C. Contrast-enhanced ultrasound of the liver: basics and interpretation of common focal lesions. Rofo. 2024 Aug;196(8):807-818. English, German. doi: 10.1055/a-2219-4726. Epub 2024 Jan 4. PMID: 38176437

Disclosure

Travel grant from Johnson & Johnson