Phys Clinical Cases · rheumatological

IgG4-Related Disease — DCE Clinical Case

DCE long-case clinical station: comprehensive management of a 62-year-old retired engineer who presents with six weeks of painless obstructive jaundice, three months of progressive painless submandibular gland enlargement and a rising creatinine from multi-organ IgG4-related disease (type 1 autoimmune pancreatitis, IgG4-related sclerosing cholangitis, Mikulicz syndrome, IgG4-related kidney disease and early retroperitoneal fibrosis) — the one-disease-many-organs concept, the clinicopathological diagnosis built on the histopathology triad, the correct use and limits of serum IgG4, the exclusion of pancreatobiliary malignancy and Sjogren syndrome, the 2019 ACR and EULAR classification criteria, the decompress-first principle for ureteric obstruction, and the integrated management plan of glucocorticoids and rituximab, with probing-question discussion and a short-case station on the salivary and lacrimal gland examination for Mikulicz syndrome.