Lymphocytic hypophysitis and hypertrophic pachymeningitis: description of a possible case associated to IgG4 pathologies

Pathologies related to lymphocyte infiltration and inflammatory cells are classified, according to their topography, into idiopathic orbital pathology, idiopathic hypertrophic pachymeningitis, Tolosa-Hunt syndrome and lymphocytic hypophysitis. Here we review the case of a female patient who began with lymphocytic hypophysitis eight years ago and has since presented several episodes of hypertrophic pachymeningitis in a variety of locations. Our study involves a 55-year-old female, being monitored owing to a two-month history of headaches, who was admitted to hospital as the result of a deterioration of her clinical signs and symptoms with paralysis of the whole right-side third cranial nerve, watery eyes and rhinorrhea. A magnetic resonance brain scan revealed the presence of a sellar lesion consistent with lymphocytic hypophysitis. Treatment was established with corticoids with an improvement at first, but two years later the patient's clinical features worsened, with headaches and periocular paraesthesias on the right-hand side. Another MRI scan was performed and several extra-axial lesions were observed, the results of the other studies being normal. Idiopathic hypertrophic pachymeningitis was therefore diagnosed and treatment with corticoids was started again. In the ensuing follow-up the patient presented new lesions in different locations and poor pain control, and so immunomodulator treatment was thus added. Given the association between lymphocytic hypophysitis and hypertrophic pachymeningitis, the study was completed with determination of the IgG4 in serum, which was negative. The association of lymphocytic hypophysitis and hypertrophic pachymeningitis with IgG4-related pathologies have recently been reported. Although the diagnosis has not been confirmed in our patient, this relationship must be taken into account in these idiopathic cases and, above all, if they are associated with other systemic manifestations.