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In the central nervous system, the disease causes hypophysitis, hypertrophic pachymeningitis, and intracranial neoplastic lesions.

It was later discovered this disease could affect other regions of the body. This disease was first reported in 2001 in a study on autoimmune pancreatitis associated with high serum IgG4 levels. Immunoglobulin G4 (IgG4)-related disease is a systemic chronic inflammatory condition characterized by elevated IgG4 levels and a marked infiltration of IgG4-positive plasma cells into affected organs. Although no pathological findings indicating a relationship between the two conditions were found, we were able to preoperatively differentiate multiple lesions via detailed diagnostic imaging. This is the first reported case of IgG4-related hypophysitis with PitNET.

Based on the histopathological examination results, steroid therapy was initiated, which reduced pituitary gland size and serum IgG4 levels. The histological features of the resected tumor were consistent with those of gonadotroph PitNET, which was immunohistochemically positive for follicle-stimulating hormone-β and steroidogenic factor-1, and no plasma cell infiltration was observed. A histopathological examination revealed a marked infiltration of plasma cells into the pituitary gland, which was strongly positive for IgG4. The diagnosis was based on an endoscopic transnasal biopsy of the pituitary gland. Because IgG4 level was elevated, a systemic examination was performed multiple nodules were found in both lung fields. Laboratory and loading test results suggested hypopituitarism. Magnetic resonance imaging revealed pituitary body and stalk swelling as well as a small poorly enhanced right anterior lobe mass. Case presentationĪ 56-year-old Japanese man arrived at the hospital with visual impairment, bitemporal hemianopia, and right abducens nerve palsy. We present a case of concurrent IgG4-related hypophysitis and PitNET.

However, biopsy has rarely been performed in these patients, and none have had an associated pituitary neuroendocrine tumor (PitNET).

More than 30 cases of IgG4-related hypophysitis have been reported. Some patients develop immunoglobulin G4 (IgG4)-related hypophysitis associated with systemic diseases.