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.

Common Symptoms

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.

Coexisting Diseases and Conditions

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

Risk Factors and Prevalence

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


  1. Article Sources
    1. Chen, L. Y. C., Mattman, A., Seidman, M. A., & Carruthers, M. N. (2019, March). Igg4-related disease: What a hematologist needs to know. Haematologica. https://www.haematologica.org/article/view/8802

    2. Divatia, M., Kim, S. A., & Ro, J. Y. (2012). IgG4-related sclerosing disease, an emerging entity: a review of a multi-system disease. Yonsei medical journal, 53(1), 15–34. https://doi.org/10.3349/ymj.2012.53.1.15

    3. Nakazawa, T., Naitoh, I., Hayashi, K., Miyabe, K., Simizu, S., & Joh, T. (2013). Diagnosis of IgG4-related sclerosing cholangitis. World journal of gastroenterology, 19(43), 7661–7670. https://doi.org/10.3748/wjg.v19.i43.7661

    4. Ryu, J. H., Sekiguchi, H., & Yi, E. S. (2011). Pulmonary manifestations of immunoglobulin g4-related sclerosing disease. European Respiratory Journal, 39(1), 180–186. https://doi.org/10.1183/09031936.00025211