Corticosteroid-responsive diabetes mellitus associated with autoimmune pancreatitis

doi:10.1016/S0140-6736(00)02684-2

The Lancet

Volume 356, Issue 9233, 9 September 2000, Pages 910-911

https://doi.org/10.1016/S0140-6736(00)02684-2 Get rights and content

Summary

Autoimmune pancreatitis which can be treated with corticosteroid therapy has the potential to induce diabetes. In a cohort study of 2220 patients with suspected chronic pancreatitis, we found that all four patients with autoimmune chronic pancreatitis also had diabetes. Treatment with prednisolone subsequently improved insulin secretion and glycaemic control in these patients.

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Cited by (152)

Incidence of endocrine and exocrine insufficiency in patients with autoimmune pancreatitis at diagnosis and after treatment: a systematic review and meta-analysis
2022, European Journal of Internal Medicine
Citation Excerpt :
ICDC or HISORT diagnostic criteria were used in 28 studies, while in 22 studies other diagnostic criteria were employed. In five studies the set of inclusion/diagnostic criteria used for patient enrolment was not specified [5,8,12,46,48]. A quality assessment of the included studies based on the Newcastle–Ottawa quality assessment scale is reported in Table 1 and in Table 2.
Autoimmune pancreatitis (AIP) is a rare form of pancreatitis that may lead to endocrine and exocrine insufficiency if left untreated. AIP clinically responds to glucocorticoids (GCs) therapy, but multiple GCs courses are often required to maintain remission with detrimental effects on glycaemic control.
In this systematic review and meta-analysis, we aimed to assess the rate of endocrine and of exocrine insufficiency at diagnosis and at follow up in patients with AIP as well as the impact of GC therapy on pancreatic function in the long-term.
The MEDLINE, SCOPUS, and EMBASE databases were searched from inception to August 2021 to identify studies reporting data on endocrine and exocrine insufficiency in patients with AIP. Pooled events were calculated using a random-effect model and expressed in terms of pooled prevalence rates.
A total of 6522 AIP patients and sixty-two studies were included in the analysis. The pooled estimate rate for the overall prevalence of diabetes in AIP at baseline was 37% (95% CI 32-42, I² 96%). The pooled prevalence rate of exocrine insufficiency was 45% (95%CI 32.9-57.4; I² 97%). The pooled estimate rate of diabetes at follow-up was 44% (95%CI 26.1-62.4) in studies where GCs were given to 100% of patients and 42% (95%CI 30.6-52.9) in studies where GCs were given to less than 100% of patients.
A large proportion of patients with AIP displays concomitant exocrine and endocrine insufficiency at the time of diagnosis. The incidence of diabetes at the longest available follow up tends to increase in patients treated with GCs.
Diabetes mellitus secondary to IgG4-related disease
2022, Endocrinologia, Diabetes y Nutricion
Corticosteroids prevent the progression of autoimmune pancreatitis to chronic pancreatitis
2020, Pancreatology
Patients with autoimmune pancreatitis (AIP) sometimes progress to chronic pancreatitis (CP). We evaluated the ability of corticosteroids to prevent the progression to CP.
We defined patients with definitive findings of CP (stones in the main pancreatic duct [MPD] or multiple pancreatic calcifications) as having severe calcification (SC). A total of 145 AIP patients were enrolled. We measured the duration between AIP diagnosis and SC development and retrospectively compared the time to SC development between patients with and without steroids. Multivariate analysis for factors associated with SC were performed.
Nineteen (13%) patients progressed to SC. Since 95 patients had pancreatic head swelling and SC was found in these patients only, our analysis focused mainly on these at-risk populations. In Kaplan-Meier analysis limited to patients with pancreatic head swelling, the incidence of SC was significantly lower in patients with steroids than in those without (hazard ratio [HR] 0.18, 95% confidence interval [CI] 0.07–0.52; p < 0.001). Multivariate testing of patients with pancreatic head swelling confirmed that steroid therapy was significantly associated with a lower incidence of SC (HR 0.11, 95% CI 0.03–0.34; p < 0.001), while MPD dilation at AIP diagnosis was related to a higher incidence of SC (HR 4.02, 95% CI 1.43–11.7; p = 0.009).
Corticosteroids appeared to prevent progression to CP in AIP patients, especially in those with pancreatic head swelling. Patients with both pancreatic head swelling and MPD dilation at diagnosis have a higher incidence of progression to CP. Steroid therapy is suggested for these high-risk cases.
Autoimmune pancreatitis and immunoglobulin g4-related disease
2019, The Autoimmune Diseases
Immunoglobulin G4–related disease (IgG4-RD) is a recently established systemic disease that is characteristically associated with IgG4 and is considered to be caused by autoimmune mechanisms. The clinical features of IgG4-RD can be summarized as (1) systemic distribution; (2) imaging findings of swelling, nodules, and/or wall thickening; (3) high serum IgG4 concentration; (4) abundant lymphoplasmacytic and IgG4-bearing plasma cell infiltration in affected organs; (5) a favorable response to corticosteroid therapy; and (6) combination with other IgG4-RDs simultaneously or in a metachronous fashion. Although IgG4-RD is now attracting worldwide interest, its concept was originally established based on the intense study of IgG4 in autoimmune pancreatitis (AIP) to open the gateway from AIP to IgG4-RD. It has become evident that IgG4-RD has systemic organ manifestations, but the exact prevalence and clinical features of individual IgG4-RDs remain unclear. Numerous pathological, immunological, and genetic investigations have provided considerable insight into the pathogenesis of IgG4-RD, especially regarding autoimmune mechanisms. Accordingly, glucocorticoids are regarded as the first-line agent for active and untreated IgG4-RD, although steroid-sparing agents and B-cell depletion with rituximab may also be considered in exceptional cases. Despite reports of a close association with various malignancies, the long-term prognosis of IgG4-RD appears to be favorable.
Monitoring and predicting disease activity in autoimmune pancreatitis with the M-ANNHEIM-AiP-Activity-Score
2018, Pancreatology
Autoimmune pancreatitis (AiP) is treated by immunosuppressive therapy. Exact description of disease activity of AiP is essential in clinical practice and research, but a score to describe the disease activity is missing. Thus, we aimed to establish an activity score of AiP.
We retrospectively studied long-term disease courses of 29 patients with AiP (Mannheim, Germany), receiving corticosteroid treatment (CST) by analyzing 613 treatment appointments. Two assumptions were made: First, disease activity is higher at emergency treatments; second, disease activity drops under CST. In all patients, we evaluated established activity- and classification-systems of chronic pancreatitis (cP). Based on the most suitable system, we established an activity score of AiP by including AiP-specific parameters identified from our long-term disease courses and the literature. The new AiP-specific activity score was validated in an external cohort of 14 patients with AiP (Stockholm, Sweden).
Within published activity indexes of cP, the M-ANNHEIM-classification most significantly correlated with emergency- and treatment-dependent disease activities (p < 0.001 and p < 0.01, conditional-logistic-regression-analysis). Significant correlations of disease activity were found for several clinical parameters (biliary involvement, extrapancreatic lesions, acute pancreatitis, focal pancreatic mass, pancreatic sausage/mass, focal enlargement, ascites; p < 0.05, Wilcoxon-signed-rank-test). Based on these data and disease features from the literature, the M-ANNHEIM-AiP-Activity-Score (MAAS) was established. CST-induced reduction of MAAS disease activity of more than 60% was associated with lower relapse rates (p < 0.05; Chi-Square-test). The results were validated in the external patient cohort.
The MAAS might represent a useful tool to monitor AiP.
Autoimmune Polyglandular Syndrome Type 3 Complicated with IgG4-related Disease
2024, Internal Medicine

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Research LettersCorticosteroid-responsive diabetes mellitus associated with autoimmune pancreatitis

Summary

Chronic inflammatory sclerosis of the pancreas: an autonomous pancreatic disease?

Am J Dig Dis

Chronic pancreatitis created by an autoimmune abnormality: proposal of the concept of autoimmune pancreatitis

Dig Dis Sci

Research Letters
Corticosteroid-responsive diabetes mellitus associated with autoimmune pancreatitis