Abstract

Anti-PIT-1 antibody syndrome has recently been reported and characterized by acquired growth hormone (GH), prolactin (PRL), and thyroid-stimulating hormone (TSH) deficiencies associated with autoimmunity to a pituitary specific transcription factor PIT-1, which plays an essential role in GH-, PRL-, and TSH-producing cells. Although circulating anti-PIT-1 antibody and PIT-1-reactive cytotoxic T cells (CTLs) were detected in the patients, the pathophysiology and precise mechanisms for the autoimmunity remain unclarified. During the follow up, thymoma was diagnosed in all 3 cases with anti-PIT-1 antibody syndrome. Immunohistochemical analysis revealed that PIT-1 was strongly expressed in neoplastic cortical thymic epithelial cells. Importantly, after thymectomy, the titer of anti-PIT-1 antibody decreased and reactivity of CTLs toward PIT-1 diminished. These data strongly suggest that the aberrant expression of PIT-1 in the thymoma plays a causal role in the development of this syndrome. Thus, we define that this syndrome is a novel thymoma-associated autoimmune disease.

Highlights

  • Anti-PIT-1 antibody syndrome has recently been reported and characterized by acquired growth hormone (GH), prolactin (PRL), and thyroid-stimulating hormone (TSH) deficiencies associated with autoimmunity to a pituitary specific transcription factor PIT-1, which plays an essential role in GH, PRL, and TSH-producing cells

  • Because of the advanced age, the tumor has been carefully observed in this patient. These data indicate that one case has not been histologically proven, all the patients with anti-PIT-1 antibody syndrome were associated with thymomas, suggesting that thymoma plays a role in the pathogenesis of this syndrome

  • We have demonstrated that thymoma was associated with all patients with anti-PIT-1 antibody syndrome

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Summary

Introduction

Anti-PIT-1 antibody syndrome has recently been reported and characterized by acquired growth hormone (GH), prolactin (PRL), and thyroid-stimulating hormone (TSH) deficiencies associated with autoimmunity to a pituitary specific transcription factor PIT-1, which plays an essential role in GH-, PRL-, and TSH-producing cells. After thymectomy, the titer of antiPIT-1 antibody decreased and reactivity of CTLs toward PIT-1 diminished These data strongly suggest that the aberrant expression of PIT-1 in the thymoma plays a causal role in the development of this syndrome. The pituitary-specific transcriptional factor-1 (PIT-1, known as POU1F1) is a member of the Pit-Oct-Unc (POU) homeodomain family that plays an essential role in the differentiation of somatotrophs, lactotrophs, and thyrotrophs in the anterior pituitary[8] It regulates the expression of growth hormone (GH), prolactin (PRL), and thyroid-stimulating hormone (TSH) and mutations in PIT-1 gene cause congenital GH, PRL, and TSH deficiencies[9].

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