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Pituitary dysfunction in granulomatosis with polyangiitis.

Daniela Esposito1,2,3, Penelope Trimpou1,2, Dario Giugliano3

  • 1Department of Endocrinology, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden.

Pituitary
|May 26, 2017
PubMed
Summary
This summary is machine-generated.

Granulomatosis with polyangiitis (GPA) can cause pituitary dysfunction, often presenting as hypogonadism or diabetes insipidus. While standard treatments improve imaging, hormonal deficiencies usually persist, requiring ongoing pituitary function assessment.

Keywords:
CyclophosphamideDiabetes insipidusPituitary insufficiencyVasculitisWegener´s granulomatosis

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Area of Science:

  • Endocrinology
  • Rheumatology
  • Neurology

Background:

  • Granulomatosis with polyangiitis (GPA) is a rare multisystem vasculitis affecting primarily the respiratory tract and kidneys.
  • Pituitary dysfunction is an uncommon manifestation of GPA, reported in approximately 1% of cases.

Purpose of the Study:

  • To report clinical features, imaging, treatment, and outcomes in three patients with GPA-related pituitary dysfunction (PD).
  • To review the literature for additional cases of GPA-related PD.

Main Methods:

  • Retrospective analysis of three GPA-related PD cases.
  • Systematic literature review of English medical literature using PubMed.

Main Results:

  • Three female patients (32-37 years) presented with or developed PD. All showed pituitary lesions on MRI.
  • Conventional treatment improved MRI findings but not PD. Literature review identified 51 additional patients.
  • Secondary hypogonadism and diabetes insipidus were common; sellar mass with cystic lesions was frequent.

Conclusions:

  • GPA should be considered in patients with sellar masses, diabetes insipidus, and systemic disease.
  • While systemic symptoms and pituitary imaging improve with treatment, hormonal deficiencies often persist.
  • Regular pituitary imaging and function assessment are crucial for patients with GPA.