European Guideline on IgG4‐related digestive disease – UEG and SGF evidence‐based recommendations

Abstract

<jats:p> The overall objective of these guidelines is to provide evidence-based recommendations for the diagnosis and management of immunoglobulin G4 (IgG4)-related digestive disease in adults and children. IgG4-related digestive disease can be diagnosed only with a comprehensive work-up that includes histology, organ morphology at imaging, serology, search for other organ involvement, and response to glucocorticoid treatment. Indications for treatment are symptomatic patients with obstructive jaundice, abdominal pain, posterior pancreatic pain, and involvement of extra-pancreatic digestive organs, including IgG4-related cholangitis. Treatment with glucocorticoids should be weight-based and initiated at a dose of 0.6–0.8 mg/kg body weight/day orally (typical starting dose 30-40 mg/day prednisone equivalent) for 1 month to induce remission and then be tapered within two additional months. Response to initial treatment should be assessed at week 2–4 with clinical, biochemical and morphological markers. Maintenance treatment with glucocorticoids should be considered in multi-organ disease or history of relapse. If there is no change in disease activity and burden within 3 months, the diagnosis should be reconsidered. If the disease relapsed during the 3 months of treatment, immunosuppressive drugs should be added. </jats:p>

Risk of Developing Pancreatic Cancer in Patients with Chronic Pancreatitis

“…IgG4-RD, and particularly AIP, could be linked to an increased risk of developing malignant disease relative to the general population. Consequently, current European guidelines recommend lifelong surveillance in patients with IgG4-RD [28]. The overall demographic characteristics, exposures, and outcomes of our cohort are comparable to other studies in the field (Table 4).…”

UEG framework for the development of high‐quality clinical guidelines

“…For example, the QoC TF under the auspices of UEG offers activity grants for the development of guidelines . 14–17…”

United European Gastroenterology guideline: How to manage immunoglobulin G4‐related digestive diseases

“…IgG4‐related digestive diseases (IgG4‐DDs) 4 include various inflammatory disorders in the hepato‐pancreato‐biliary organs 5–7 and digestive tract 8 . They mostly develop in middle‐aged to elderly people (median age 58–65 years) with a male sex predominance, 2 , 3 , 7 but they rarely occur in children 4 . IgG4‐DDs usually show elevated serum IgG4 and typical images, sometimes accompanied with other organ involvement (OOI) 3 , 5 .…”

“…So, they require careful differentiation from malignancies 6 , 9 . Endoscopic ultrasonography‐guided fine‐needle aspiration biopsy is fairly important in cases suspected to be autoimmune pancreatitis (AIP), both to exclude malignancies and to achieve a conclusive histological diagnosis 4 , 9 . Similarly, transpapillary biliary biopsy is effective in cases of IgG4‐related sclerosing cholangitis (IgG4‐SC) 6 .…”

“…A prolonged steroid dosage increases the risk of side effects, such as infection, vertebral fracture, femoral head necrosis, and myopathy 10 . Hence, maintenance therapy is indicated for patients with recurrence risks such as high serum IgG4, diffuse pancreatic swelling, or OOIs (especially dacryoadenitis/sialadenitis, proximal bile duct stenosis, and retroperitoneal fibrosis 3 , 4 , 9 ). In cases with life‐span‐overdosed steroids, rituximab and immunomodulators can be used as steroid‐sparing agents 3 …”