IgG4-Related Disease

Overview of IgG4-related disease

Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognized immune-mediated condition comprised of a collection of disorders that share particular pathologic, serologic, and clinical features. These disorders were previously thought to
uptodate.com

IgG4-related disease: a systematic review of this unrecognized disease in pediatrics

Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibro-inflammatory condition with an unclear pathophysiological mechanism affecting different parts of…
ped-rheum.biomedcentral.com

Primer: IgG4-Related Diseases

IgG4-related diseases first appeared in the medical literature in 2003, but greater awareness is needed to ensure early diagnosis.
www.rheumatologynetwork.com

Circulating IgG4/IgG ASC percentages of greater than five percent are a more sensitive and reliable marker for disease activity in patients with IgG4-RD, researchers report.
www.rheumatologynetwork.com

Short of tissue histology demonstrating IgG4-secreting plasma cells, serum IgG4 levels would help in diagnosis and monitoring.


History of malignancy more than three times as common than in controls
medpagetoday.com

Invasive cancers like prostate cancer, and those involving immune cells like lymphoma, are postulated to predispose to later development of IgG4-RD as a result of immune dysregulation reacting to neo/auto-antigens.

So far, the evidence does not suggest that IgG4-RD is pre-malignant, nor is it a paraneoplastic phenomenon.


IgG4-related disease: pathophysiologic insights drive emerging treatment approaches

Clin Exp Rheumatol. 2016 Jul-Aug;34(4 Suppl…
ncbi.nlm.nih.gov|By Stone JH


Rituximab is dramatically effective in IgG4-related disease

SNOWMASS, COLO. – If glucocorticoids do not bring patients with immunoglobulin G4-related disease into long-term sustained remission, B cell depletion with rituximab has proved successful, and investigational trials of other drugs are underway, said John H. Stone, MD.
mdedge.com