Open AccessMulticentric Italian survey on daily practice for autoimmune pancreatitis: Clinical data, diagnosis, treatment, and evolution toward pancreatic insufficiency
Author(s) -
Barresi Luca,
Tacelli Matteo,
Crinò Stefano Francesco,
Attili Fabia,
Petrone Maria Chiara,
De Nucci Germana,
Carrara Silvia,
Manfredi Guido,
Capurso Gabriele,
De Angelis Claudio Giovanni,
Crocellà Lucia,
Fantin Alberto,
Dore Maria Francesca,
Garribba Alessandra Tina,
Tarantino Ilaria,
De Pretis Nicolò,
Pagliari Danilo,
Rossi Gemma,
Manes Gianpiero,
Preatoni Paoletta,
Barbuscio Ilenia,
Tuzzolino Fabio,
Traina Mario,
Frulloni Luca,
Costamagna Guido,
Arcidiacono Paolo Giorgio,
Buscarini Elisabetta,
Pezzilli Raffaele
Publication year - 2020
Publication title -
ueg journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.667
H-Index - 35
eISSN - 2050-6414
pISSN - 2050-6406
DOI - 10.1177/2050640620924302
Subject(s) - medicine , autoimmune pancreatitis , epidemiology , rituximab , cohort , pancreatitis , retrospective cohort study , radiological weapon , pediatrics , surgery , lymphoma
Background 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. Objectives 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. Methods 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. Results 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%. Conclusions 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.