Multicentric Italian survey on daily practice for autoimmune pancreatitis: Clinical data, diagnosis, treatment, and evolution toward pancreatic insufficiency

Abstract

<jats:sec><jats:title>Background</jats:title><jats:p> Autoimmune pancreatitis (AIP) is a rare, and relatively new, form of chronic pancreatitis. The management of AIP can vary considerably among different centres in daily clinical practice. </jats:p></jats:sec><jats:sec><jats:title>Objectives</jats:title><jats:p> The aim of this study is to present a picture of epidemiological, clinical characteristics, outcomes, and the real-life practice in terms of management in several academic and non-academic centres in Italy. </jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p> Data on the clinical presentation, diagnostic work-up, treatments, frequency of relapses, and long-term outcomes were retrospectively collected in a cohort of AIP patients diagnosed at 14 centres in Italy. </jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p> One hundred and six patients were classified as type 1 AIP, 48 as type 2 AIP, and 19 as not otherwise specified. Epidemiological, clinical, radiological, and serological characteristics, and relapses were similar to those previously reported for different types of AIP. Endoscopic cytohistology was available in 46.2% of cases, and diagnostic for AIP in only 35.2%. Steroid trial to aid diagnosis was administered in 43.3% cases, and effective in 93.3%. Steroid therapy was used in 70.5% of cases, and effective in 92.6% of patients. Maintenance therapy with low dose of steroid (MST) was prescribed in 25.4% of cases at a mean dose of 5 (±1.4) mg/die, and median time of MST was 60 days. Immunosuppressive drugs were rarely used (10.9%), and rituximab in 1.7%. Faecal elastase-1 was evaluated in only 31.2% of patients, and was pathological in 59.2%. </jats:p></jats:sec><jats:sec><jats:title>Conclusions</jats:title><jats:p> In this cohort of AIP patients, diagnosis and classification for subtype was frequently possible, confirming the different characteristics of AIP1 and AIP2 previously reported. Nevertheless, we observed a low use of histology and steroid trial for a diagnosis of AIP. Steroid treatment was the most used therapy in our cohort. Immunosuppressants and rituximab were rarely used. The evaluation of exocrine pancreatic insufficiency is underemployed considering its high prevalence. </jats:p></jats:sec>

Efficacy and safety of rituximab in autoimmune pancreatitis type 1: our experiences and systematic review of the literature

“…The eight included articles entailed 391 patients with AIP type 1, of which 110 (28.1%) were treated with RTX [3,10,[19][20][21][22][23][24]. In four studies, data on treatment efficacy, rate of relapses and adverse events were [20,21,23,24] extracted from the cohort analysis of IgG4 patients.…”

“…Adverse effects were described in 6/8 studies and occurred in 11-43% of patients. The most common adverse effects were infusion-related reactions and infections [3,10,[19][20][21][22][23][24].…”

The clinical efficacy of azathioprine as maintenance treatment for autoimmune pancreatitis: a systematic review and meta-analysis

“…A single study [ 32 ] was multinational. Eighteen [ 14 – 18 , 20 – 24 , 26 , 27 , 29 , 33 – 37 ] and eight [ 5 , 13 , 19 , 25 , 28 , 30 – 32 ] studies were conducted as single- and multicenter endeavors, respectively.…”

Diagnostic yield of endoscopic ultrasound-guided tissue acquisition in autoimmune pancreatitis: a systematic review and meta-analysis

“…Obtaining adequate samples to meet the histologic ICDC criteria represents a real challenge for the endoscopist as these criteria are mainly based on findings described on surgical specimens that cannot be easily obtained in even a high-quality biopsy sample. Therefore, in light of the outstanding histological performance of the newer generation FNB needles, our results should push the pathologists to reconsider the histologic diagnostic criteria for AIP in order to define more feasible criteria based on FNB samples 29 .…”