IgG4-Related Disease: A Retrospective Chinese Study of Features and Treatment Response of 98 Patients Including 4 Rare Cases

Comorbidities and causes of hospitalizations in a cohort of IgG4‐related disease patients from a single center

“…Herein,wefoundthatover90%ofpatientswithIgG4-RDhad at least one comorbidity, with the most prevalent being diabetes mellitus,hypertension,andatopy.Ourstudyshowedahigherprevalenceofdiabetes(47%)comparedwithpreviouslyreportedfigures (16.4%-39%). [6][7][8] We observed a higher prevalence of type 2 and type 3c diabetes in group 4, followed by group 1, which are the phenotypes in which pancreatic involvement is more prevalent. 3 Conversely, another study found diabetes to be more common in groups 1 and 4, despite higher cumulative doses of glucocorti-coidsingroup3.…”

Recent advances in diagnosis, treatment, and prognosis of IgG4-related autoimmune pancreatitis: a narrative review

“…The prevalence of IgG4-RD in Japan is estimated at around 0.28 to 1.08 per 100,000 people. [4] The involvement of the pancreas in IgG4-RD is among 16.3~71%, [7–9] which means the estimated prevalence of type 1 AIP is around 0.3 per 100,000 people. Another pair of data shows that AIP represents 5% of chronic pancreatitis cases with a prevalence of chronic pancreatitis around 25.4–98.7 per 100,000 people, which means the estimated prevalence is approximately 1–5/100,000 people.…”

“…[19] Other less common presentations include lymphadenopathy, chest distress/pain, and proptosis. [7]…”

“…IgG4-RD is a systemic condition, which tends to involve multiple organs, [23] the number of which can range from 1 to 6, [7] even up to 10. [24] The pancreas is one of the most commonly involved organs in IgG4-RD, [25] with a male-female ratio of 3.7 to 4.5:1 in Japan and China.…”

“…The most common onset symptoms of IgG4-related type 1 AIP include jaundice, mild abdominal pain, and weight loss. [5,7,9] Obstructive jaundice with fatigue and weight loss or acute pancreatitis can be the suggestive clinical presentations of type 1 AIP. [19] Other less common presentations include lymphadenopathy, chest distress/pain, and proptosis.…”