Autoimmune Hypophysitis with Late Renal Involvement: A Case Report

Antonio Aversa,Margherita Vergine,Antonio Brunetti,Alessandro Comi,Gemma Patella,Stefania Giuliano,Sandro La Vignera,Emanuela A Greco,Stefano Iuliano,Michele Andreucci,Maria Carmela Zagari

doi:10.3390/endocrines2020016

Abstract

We report a case of a 50-year-old male admitted to the Endocrinology Unit because of persistent headaches, nausea, feeling tired, sudden weight loss, cold intolerance, decreased appetite, and lack of sex interest. Diagnostic workup showed a 6-millimeter pituitary tumor without signs of compression, and a condition of progressive panhypopituitarism. After 12 months of hormone replacement therapy, the patient was hospitalized because of sudden weight gain, periorbital-peripheral edema, severe dyslipidemia, hypertension, and proteinuria. Corticosteroid therapy was shifted from oral to continuous intravenous infusion, and once the diagnosis of “immune complex-mediated glomerulonephritis with mesangial deposits suggestive for membranoproliferative glomerulonephritis type IIIIgG4-positive” was made, the immunosuppressant mycophenolate (1500 mg/day) was started. After a 6-month follow-up, the complete resolution of renal symptoms was accompanied by the disappearance of a pituitary lesion and the patient was back to prior hormone replacement therapy. Autoimmune hypophysitis (AH) is a rare autoimmune inflammatory disease of the pituitary gland that can impair hormone secretion and function. IgG4-hypophysitis is rare and is usually associated with other IgG4-related diseases. Herein, we describe a rare case of AH associated with late renal disease, and without any other organ involvement.

Highlights

Hypophysitis is a chronic inflammation of the pituitary gland and has many clinical and radiological features common to other non-secreting sellar masses
The pituitary function was normal in only 1.2% of IgG4-related hypophysitis, whereas more than 50% of patients presented panhypopituitarism [5]
We had no clinical explanation for panhypopituitarism, otherother than than the possibility of compressive lesionsatatthe the pituitary pituitary the possibility of compressive lesions once anan autoimmune renal disease waswas diagnosed for this stalk

Summary

Introduction

Hypophysitis is a chronic inflammation of the pituitary gland and has many clinical and radiological features common to other non-secreting sellar masses. Many classifications have been proposed to define hypophysitis. As summarized, this classification is based on anatomic location, histological appearance, and etiology. Primary hypophysitis is the most common form. It can occur in isolation, or as part of a multisystemic disease (i.e., IgG-related systemic disease). IgG4-related diseases are characterized by the infiltration of the lymphocytes and plasma cells in one or more tissues and organs, and elevated plasma IgG4 levels. Many sites may be targeted by IgG-4, including retroperitoneum, salivary glands, and others

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