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