IgG4 related disease is a newly recognised unifying disease entity characterised by tumefactive lesions and tissue infiltration by IgG4 positive plasma cell rich inflammatory infiltrate. The disease was first recognised by the finding of elevated serum IgG4 level in sclerosing pancreatitis by Hamano et al. Since then, the spectrum of IgG4 related disease (IgG4RD) has expanded, and many fibroinflammatory diseases previously regarded as distinct entities are now considered to be related to IgG4. Kuttner's disease, Riedel's thyroiditis, eosinophilic angiocentric fibrosis and retroperitoneal fibrosis are some of the well known examples, being united under IgG4RD. Calcifying fibrous tumour (CFT) is another disease entity which was recently claimed to be IgG4 related.Although IgG4RD is a systemic disease, the condition usually comes to clinical attention due to organ swelling or damage. This entity has now been described in various organs including pancreas, biliary system, salivary glands, periorbital tissues, kidneys, lungs, lymph nodes, central nervous system, meninges, aorta, breast, prostate, thyroid, pericardium and skin. To our knowledge, the disease manifestation in adrenal glands has not been reported in the literature.We recently encountered such a case in which the characteristic histological features of IgG4RD were present in a CFT in the adrenal gland, an apparently unique case at an unusual location.
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