IgG4-related disease

What is it?

IgG4-associated disease is a fibroinflammatory disease first described around the 2000s in the context of autoimmune pancreatitis. Today, it is known that many organs other than the pancreas can be affected. IgG4-correlate disease is characterized by the appearance of tumors affecting one or more organs at the same time, a tendency to chronic relapse, and increased levels of IgG4 (immunoglobulin G subclass 4) in blood tests.

Causes and risk factors

IgG4-related diseases are more common in men than in women and are more common after 40-50 years of age. Pediatric cases are rarely described. Although epidemiological data are still incomplete, IgG4-associated disease appears to be more common in Japan.

Which are the symptoms?

The clinical manifestations of IgG4-associated disease are associated with the compressive effect exerted by the fibrous mass on the affected organ and can be very different from each other.

How is it diagnosed?

Therefore, a final diagnosis requires a biopsy of the affected tissue and a histological examination.

However, there are clinical, radiological, and laboratory elements that can aid in the diagnosis:

  1. allergy in history;
  2. visible swelling of the large salivary glands, lacrimal glands, or orbit;
  3. inflamed pancreas with a "sausage" appearance on CT and MRI;
  4. increase in the level of immunoglobulins IgG4 and IgE in blood tests.

Suggested exams

How is it treated?

The first line of treatment involves taking glucocorticoids (cortisone).

If there is no response or disease recurs, other immunosuppressants such as methotrexate and azathioprine, or biologics such as rituximab may be required.

Where do we treat it?

Within the San Donato Group, you can find IgG4-related disease specialists at these departments:

Are you interested in receiving the treatment?

Contact us and we will take care of you.