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“I've been around for quite some time, and I have not seen the GI involvement of IgG4 disease; it's exceedingly rare.” — Ashkan Farhadi, MD
IgG4-related disease (IgG4-RD) is a chronic inflammatory condition that may be mistaken for Crohn’s disease. [] IgG4-RD may cause diagnostic difficulties due to its overlapping features.
“IgG4 disease [is] a group of autoimmune diseases. This autoimmune disease can affect the pancreas; [it] can affect body systems. There are some reported effects to other parts of the gut, like [the] colon [and] stomach. It can mimic Crohn's disease,” says Ashkan Farhadi, MD, a board-certified gastroenterologist at MemorialCare Medical Group in Orange County, CA, and the author of “The Rhymes of Happiness: Weaving Science, Art and Poetry in the Pursuit of Happiness.”
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What is IgG4-RD?
IgG4-RD is a chronic inflammatory condition characterized by tissue infiltration with IgG4-positive plasma cells. []This can lead to organ dysfunction and fibrosis.
IgG4-RD primarily impacts the pancreas, bile ducts, and salivary glands, but it can also impact parts of the gastrointestinal tract.
“My experience … I've been around for quite some time, and I have not seen the GI involvement of IgG4 disease; it's exceedingly rare," Dr. Farhadi says.
Research suggests that individuals with IBD may exhibit both serological and histological features similar to those of IgG4-RD. But differentiating between the two is possible.
“When we're dealing with some alteration or something in the gut … we do biopsy, and the pathologists are completely familiar with this condition, so I'm not worried you can misdiagnose,” Dr. Farhadi says.
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Dr. Farhadi notes that IgG4-RD can sometimes be dismissed as other conditions, such as alcoholic pancreatitis.
The only part that could be easily misdiagnosed is if a patient comes with pancreatitis, and the doctor is not paying attention to the details of the problem, Dr. Farhadi explains. Particularly nowadays, using alcohol is fairly common, and one of the most common reasons for pancreatitis is alcohol-induced pancreatitis.
"Because many people drink alcohol, [doctors] may misdiagnose [it] as alcoholic pancreatitis, and they blow [patients] off. Then [the patient] come[s] back the next month and the next month, and each time they come back, [the doctors] say, ‘Oh, this is alcoholic pancreatitis.’ Even though the guy quit drinking, he is still experiencing the flares of chronic pancreatitis,” Dr. Farhadi says.
It's critical to pay attention to diagnostic images like a CAT scan that shows, rather than a trophic or calcification pancreas, a bald, bulky, sausage-like pancreas, Dr. Farhadi explains.
"So it's very important for physicians to be aware of this and diagnose it right away, because treatment is … helpful and … totally different from alcoholic pancreatitis or other forms of pancreatitis,” Dr. Farhadi adds.
First-line therapies for IgG4-RD include glucocorticoids; however, disease flares are common at low doses or after tapering. [] Second-line therapy might involve immunosuppressive drugs.
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