Introduction

IgG4-related disease of the liver, pancreas, and bile ducts (IgG4-HPB) can lead to fibrosis and organ damage. Liver stiffness measurement (LSM) by vibration-controlled transient elastography and Enhanced liver fibrosis (ELF) score are validated, non-invasive methods for measuring liver fibrosis in other chronic liver diseases, but little is known about their utility in IgG4-HPB.

Aims & Methods

The aim of this study is to assess LSM using Fibroscan® and ELF in patients with IgG4-HPB. This is a retrospective observational cohort of patients meeting the ACR/EULAR classification criteria for IgG4-Related Disease at Oxford University Hospitals. Clinical data collected included demographics, organ involvement, clinical flares, serum biomarkers and organ damage. LSM was recorded in 55 IgG4-HPB patients, and sequential LSM was recorded in 32 patients. Serum ELF was measured in 23 patients. Wilcoxon rank, Mann-Whitney U, and Fisher’s exact tests were performed.

Results

Ninety-seven patients with IgG4-HPB were included; median age was 68 years ±19, and 79% were male. Among the entire cohort, 69% had established organ dysfunction and/or damage and, when subdivided by organ involvement, damage was present in 72% of those with liver and biliary disease (IgG4-HB) and 65% of those with pancreatic disease only (IgG4-AIP) (Table 1).

	Overall cohort (n = 97)	HPB cohort (n = 57)	AIP cohort (n = 40)	p-value
Median age (IQR)	67.5 (19)	68.5 (19)	65.5 (18)	0.91
Men	77 (79%)	44 (77%)	33 (82%)	0.61
Established organ damage	67 (69%)	41 (72%)	26 (65%)	>0.99
Diabetes	33 (34%)	26 (46%)	12 (29%)	0.14
Cardiometabolic disease	41 (42%)	22 (39%)	20 (49%)	0.11
MASLD	19 (20%)	13 (23%)	6 (15%)	0.59
Mean cumulative steroid dose (SD)	7326 (17468)	9012 (21263)	4260 (3074)	0.32
Median number of flares (IQR)	1 (2)	1 (2)	0 (1)	0.25

LSM was recorded in 55 patients: IgG4-HB (n = 38) had a higher mean LSM compared with IgG4-AIP (n = 17) (6.1 kPa vs 5 kPa, p = 0.04). IgG4-HB were risk-stratified by LSM: 66% had LSM <8kPa, 21% had LSM ≥8 but <12kPa, and 13% had LSM ≥12kPa. There was a higher prevalence of metabolic associated steatotic liver disease (MASLD) and its metabolic factors (diabetes, hypertension, hypercholesterolemia, and ischaemic heart disease) in those with more advanced liver fibrosis (LSM≥12 kPa). Furthermore, survival analysis showed that patients with LSM ≥ 8kPa develop organ damage faster than those with LSM < 8kPa (p=0.0086).

In longitudinal analysis, 32 patients with IgG4-HPB had serial LSMs. Of those with IgG4-HB (n = 22), 23% showed an increase in LSM over a median of 2 years (IQR 1-4). The proportion of patients meeting criteria for MASLD increased from 20% to 25% over a median of two years. In those with paired readings during active disease and remission (n = 8), there was no discernible variation in LSM.

Twenty-three patients with IgG4-HPB had an ELF test and were stratified by the score: 13/23 (56%) <9.8 and 10/23 (43%) ≥9.8. ELF accurately identified patients with more significant liver fibrosis; no patient with LSM ≥ 8kPA was seen in the <9.8 group (p = 0.0021). There was no association of ELF with disease activity or serum biomarkers.

Conclusion

This is the first study assessing the role of LSM by Fibroscan® and of ELF score in patients with IgG4-HPB. An increased LSM over time was associated with the accumulation of cardiometabolic risk factors, evidence of MASLD, and end-organ damage, while ELF score ruled out advanced fibrosis in this cohort concurrently.

Disclosure

EC: Speaking Fees: Horizon Therapeutics, Advanz (Intercept), Albireo, Dr Falk Pharma, Gilead, GSK; Consulting Fees: Advanz (Intercept), Horizon Therapeutics, Ipsen, Mirum, Moderna, Sanofi, Zenus Pharma; Grant Support: Jansen, Innovate UK, PSC Support, Wellcome Trust; Institutional Funding Support: BRC Oxford NIHR (UK), Oxford Charitable Fund, Research Capability Fund.
SK, RM and CM have nothing to declare.

Introduction

Inflammatory Bowel Diseases (IBD) can lead to various complications, with Acute Severe Colitis (ASC) being among the most critical. ASC is a medical and surgical emergency that poses a significant risk to life, necessitating prompt and effective diagnosis and management. The first-line treatment typically involves intravenous corticosteroids. However, some cases are resistant to corticosteroids and require second-line medical therapy or immediate surgical intervention.

Aims & Methods

The objective was to identify factors associated with the failure of first-line medical treatment in patients with ASC. A retrospective descriptive and analytical study was conducted over ten years (January 2014 to March 2024) in the gastroenterology department of Charles Nicolle Hospital. The study included patients with IBD complicated by ASC, defined by the Truelove and Witts criteria and a Lichtiger score greater than 10.Patients with bacterial superinfection were not included.

Results

Forty-five patients over 18 years old were included, with a mean age of 37.87 years (range: 18–74 years). There was a slight female predominance (53%), with a male-to-female ratio of 0.87. In 31% of cases, ASC was the initial presentation of the disease. Endoscopic signs of severity at admission were observed in 49% of patients. All patients received intravenous corticosteroid therapy with hydrocortisone hemisuccinate as the first-line treatment. The response rate to first-line treatment was 64.5%. Second-line medical therapy was administered to 24.4% of patients, consisting of cyclosporine in 11.1% and infliximab in 13.3%. Five patients (11.1%) underwent subtotal colectomy due to corticosteroid failure.
In univariate analysis, factors associated with corticosteroid failure included male gender (p=0.011), absence of extraintestinal manifestations (p=0.017), higher average number of stools on the first day (p=0.013), Lichtiger score >10 on the third (p=0.011) and fifth day (p<0.001), hemoglobin levels >11 g/dL on the first day (p<0.001), >10 g/dL on the third day (p=0.009), >9 g/dL on the seventh day (p=0.013), white blood cell count >10,000/mm³ on the first day (p=0.008), >12,000/mm³ on the third (p=0.001) and seventh day (p=0.004), C-reactive protein (CRP) level >30 mg/L on the seventh day (p=0.005), and persistent endoscopic signs of severity after three to five days of corticosteroid therapy (p=0.001).
Multivariate Analysis showed that only the presence of severe endoscopic signs on follow-up endoscopy after three to five days of corticosteroid therapy was predictive of corticosteroid failure (Odds Ratio = 42.2; p=0.028).

Conclusion

Acute Severe Colitis is a potentially life-threatening complication. The presence of severe endoscopic signs after five days of corticosteroid therapy should prompt the initiation of second-line therapy. Understanding the predictive factors for corticosteroid failure is essential for individualized management to improve both immediate and long-term outcomes.

Disclosure

The authors declare no conflicts of interest.