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Type 1 autoimmune pancreatitis and IgG4-related sclerosing cholangitis is associated with extrapancreatic organ failure, malignancy, and mortality in a prospective UK cohort.

Huggett MT, Culver EL, Kumar M, Hurst JM, Rodriguez-Justo M, Chapman MH, Johnson GJ, Pereira SP, Chapman RW, Webster GJ, Barnes E - Am. J. Gastroenterol. (2014)

Bottom Line: The risk of any cancer at diagnosis or during follow-up when compared with matched national statistics was increased (odds ratio=2.25, CI=1.12-3.94, P=0.02).Mortality occurred in 10% of patients during follow-up.The risk of death was increased compared with matched national statistics (odds ratio=2.07, CI=1.07-3.55, P=0.02).

View Article: PubMed Central - PubMed

Affiliation: 1] UCL Institute for Liver and Digestive Health, University College London, London, UK [2] Department of Gastroenterology and Hepatology, University College Hospital, London, UK [3] M.T. Huggett and E.L. Culver contributed equally to this work.

ABSTRACT

Objectives: Type I autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-related SC) are now recognized as components of a multisystem IgG4-related disease (IgG4-RD). We aimed to define the clinical course and long-term outcomes in patients with AIP/IgG4-SC recruited from two large UK tertiary referral centers.

Methods: Data were collected from 115 patients identified between 2004 and 2013, and all were followed up prospectively from diagnosis for a median of 33 months (range 1-107), and evaluated for response to therapy, the development of multiorgan involvement, and malignancy. Comparisons were made with national UK statistics.

Results: Although there was an initial response to steroids in 97%, relapse occurred in 50% of patients. IgG4-SC was an important predictor of relapse (P<0.01). Malignancy occurred in 11% shortly before or after the diagnosis of IgG4-RD, including three hepatopancreaticobiliary cancers. The risk of any cancer at diagnosis or during follow-up when compared with matched national statistics was increased (odds ratio=2.25, CI=1.12-3.94, P=0.02). Organ dysfunction occurred within the pancreas, liver, kidney, lung, and brain. Mortality occurred in 10% of patients during follow-up. The risk of death was increased compared with matched national statistics (odds ratio=2.07, CI=1.07-3.55, P=0.02).

Conclusions: Our findings suggest that AIP and IgG4-SC are associated with significant morbidity and mortality owing to extrapancreatic organ failure and malignancy. Detailed clinical evaluation for evidence of organ dysfunction and associated malignancy is required both at first presentation and during long-term follow-up.

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Multi-system manifestations associated with IgG4-related disease. Prevalence of other organ involvement in IgG4-related disease (a) and associated autoimmune conditions (b).
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Figure 1: Multi-system manifestations associated with IgG4-related disease. Prevalence of other organ involvement in IgG4-related disease (a) and associated autoimmune conditions (b).

Mentions: Forty-one patients (36%) had disease beyond the biliary tree or pancreas (Figure 1). Diffuse lymphadenopathy with enlarged intraabdominal, chest, or peripheral nodes (e.g., axillary or iliac) was the most common manifestation in 43% of patients. IgG4-related sialadenitis was seen in 18%, renal infiltrates or masses in 9%, pulmonary disease with nodules, interstitial lung disease, or pulmonary fibrosis in 6%, retroperitoneal fibrosis in 3%, ocular manifestations with proptosis and retro-orbital disease in 2%, and neurological sequelae including hypopituitarism caused by a pituitary mass and a rapidly progressive autoimmune encephalitis in 2%.


Type 1 autoimmune pancreatitis and IgG4-related sclerosing cholangitis is associated with extrapancreatic organ failure, malignancy, and mortality in a prospective UK cohort.

Huggett MT, Culver EL, Kumar M, Hurst JM, Rodriguez-Justo M, Chapman MH, Johnson GJ, Pereira SP, Chapman RW, Webster GJ, Barnes E - Am. J. Gastroenterol. (2014)

Multi-system manifestations associated with IgG4-related disease. Prevalence of other organ involvement in IgG4-related disease (a) and associated autoimmune conditions (b).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4552254&req=5

Figure 1: Multi-system manifestations associated with IgG4-related disease. Prevalence of other organ involvement in IgG4-related disease (a) and associated autoimmune conditions (b).
Mentions: Forty-one patients (36%) had disease beyond the biliary tree or pancreas (Figure 1). Diffuse lymphadenopathy with enlarged intraabdominal, chest, or peripheral nodes (e.g., axillary or iliac) was the most common manifestation in 43% of patients. IgG4-related sialadenitis was seen in 18%, renal infiltrates or masses in 9%, pulmonary disease with nodules, interstitial lung disease, or pulmonary fibrosis in 6%, retroperitoneal fibrosis in 3%, ocular manifestations with proptosis and retro-orbital disease in 2%, and neurological sequelae including hypopituitarism caused by a pituitary mass and a rapidly progressive autoimmune encephalitis in 2%.

Bottom Line: The risk of any cancer at diagnosis or during follow-up when compared with matched national statistics was increased (odds ratio=2.25, CI=1.12-3.94, P=0.02).Mortality occurred in 10% of patients during follow-up.The risk of death was increased compared with matched national statistics (odds ratio=2.07, CI=1.07-3.55, P=0.02).

View Article: PubMed Central - PubMed

Affiliation: 1] UCL Institute for Liver and Digestive Health, University College London, London, UK [2] Department of Gastroenterology and Hepatology, University College Hospital, London, UK [3] M.T. Huggett and E.L. Culver contributed equally to this work.

ABSTRACT

Objectives: Type I autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-related SC) are now recognized as components of a multisystem IgG4-related disease (IgG4-RD). We aimed to define the clinical course and long-term outcomes in patients with AIP/IgG4-SC recruited from two large UK tertiary referral centers.

Methods: Data were collected from 115 patients identified between 2004 and 2013, and all were followed up prospectively from diagnosis for a median of 33 months (range 1-107), and evaluated for response to therapy, the development of multiorgan involvement, and malignancy. Comparisons were made with national UK statistics.

Results: Although there was an initial response to steroids in 97%, relapse occurred in 50% of patients. IgG4-SC was an important predictor of relapse (P<0.01). Malignancy occurred in 11% shortly before or after the diagnosis of IgG4-RD, including three hepatopancreaticobiliary cancers. The risk of any cancer at diagnosis or during follow-up when compared with matched national statistics was increased (odds ratio=2.25, CI=1.12-3.94, P=0.02). Organ dysfunction occurred within the pancreas, liver, kidney, lung, and brain. Mortality occurred in 10% of patients during follow-up. The risk of death was increased compared with matched national statistics (odds ratio=2.07, CI=1.07-3.55, P=0.02).

Conclusions: Our findings suggest that AIP and IgG4-SC are associated with significant morbidity and mortality owing to extrapancreatic organ failure and malignancy. Detailed clinical evaluation for evidence of organ dysfunction and associated malignancy is required both at first presentation and during long-term follow-up.

Show MeSH
Related in: MedlinePlus