Case Report: Isolated Pauci-immune Vasculitis of the Pituitary Gland Revealed by Stereotactic Biopsy

Abstract

Purpose: To demonstrate a case of isolated pauci-immune vasculitis of the pituitary gland in which the diagnosis was attained by stereotactic biopsy.

Methods: We report a 36-year-old man who presented with diabetes insipidus and secondary hypogonadism. Cranial MRI revealed an enhancing lesion of the pituitary stalk. A histological sample was obtained by stereotactic biopsy and pathological work-up provided the diagnosis of isolated pauci-immune vasculitis. No further organ manifestations or serologic signs of systemic vasculitis were identified. There was no response to immunosuppressive therapy and the patient refused treatment with Rituximab.

Conclusions: Vasculitis of the pituitary gland is a rare condition. Less than 50 cases have been described to date in patients with granulomatosis with polyangiitis (GPA, or Wegener’s granulomatosis) and few case reports exist on pituitary involvement in other systemic vasculitis like Behcet’s disease and Cogan’s syndrome [1] [2] [3] [4] [5] [6] [7] [8] [9]. Pituitary involvement in GPA predominantly affects the posterior pituitary gland resulting in central diabetes insipidus (DI) but global or partial anterior pituitary dysfunction and compression of the optic chiasm has also been described (10). Relapse and permanent residual pituitary insufficiency is common despite immunosuppressive therapy [7]. Vasculitis of the pituitary gland without manifestation in other organs has been described in few patients and diagnosis of GPA was attained by the presence of proteinase-3 specific ANCA and/or transsphenoidal biopsy in these cases [10] [11] [12].

The case presented here demonstrates that pauci-immune vasculitis of the pituitary can occur in the absence of systemic disease. The diagnosis can be made by stereotactic biopsy but therapy remains a challenge.

Key words

vasculitis - pituitary - GPA

• References

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