IgG4-related disease of the head and neck

IgG4-related disease is a rare immune-mediated
condition characterized by fibrosis and infiltration
of IgG4-positive plasma cells in a single or as a
generalised condition. Clinically, IgG4-related disease
may mimic malignant and inflammatory disorders.
The head and neck region is overall the second most
common site, followed by the pancreas. In the head
and neck this condition may involve salivary glands,
the orbit, lymph nodes, the thyroid gland, the upper
respiratory tract and skin. Many disorders previously
regarded as single-organ diseases, such as Küttner’s
tumour, Mikulicz’s disease and Riedel’s thyroiditis are
now regarded as IgG4-related diseases. This paper
presents diagnostic criteria, clinicopathological
features depending on the affected site as well as the
treatment protocols. Due to insufficient awareness
in the medical community the IgG4-related disease
is often unrecognised, and in consequence leads to
serious organ damage as a result of dense fibrosis, and
may even cause death of the patient