IgG4-related sclerosing disease (ISD)

A systemic fibroinflammatory disease that has the potential to impact all organ systems, but tends to affect the following organs: pancreas, bile ducts, lacrimal glands, major salivary glands (parotid, submandibular, sublingual), lungs, kidneys, aorta, meninges, and thyroid gland.

Depending on the organ system that is affected, symptoms will vary. Generally speaking, most patients experience lymph node enlargement or weight loss. In patients with pancreatic involvement, there are sometimes instances of jaundice, abdominal pain, flatulence, and nausea. People who experience retroperitoneal fibrosis with this condition may experience back or flank pain. Those with salivary or lacrimal gland involvement might also experience dry mouth and/or eyes, but this is less common.

Acute acalculous cholecystitis, IgG4-related cholecystitis, and pancreatic cancer.

More commonly seen in males than females. Genetic triggers, such as human leukocyte antigen (HLA) haplotypes, may be involved in the condition’s development.

• Incidence, prevalence and mortality of IgG4-related disease in the USA: a claims-based analysis of commercially insured adults (2023)
• Pulmonary hyalinizing granuloma with elevated IgG4 levels, A link to IgG4-related sclerosing disease? (2021)
• IgG4-related sclerosing cholangitis: Rarely, diagnosed but not a rare disease (2021)
• Pathogenesis, clinical presentations and diagnosis of IgG4-related disease: a review (2021)