Ewha Med J.  2016 Jul;39(3):85-88. 10.12771/emj.2016.39.3.85.

Progressive Pituitary Involvement in a Patient with Localized Granulomatosis with Polyangiitis

Affiliations
  • 1Department of Internal Medicine, Ewha Womans University School of Medicine, Seoul, Korea. leejisoo@ewha.ac.kr
  • 2Department of Radiology, Ewha Womans University School of Medicine, Seoul, Korea.
  • 3Department of Pathology, Ewha Womans University School of Medicine, Seoul, Korea.

Abstract

Localized granulomatosis with polyangiitis (loc-GPA) is a milder disease state of GPA restricted to the respiratory tract. Transition from localized form to systemic/generalized disease is predicted to occur in approximately 10% of the patients. We report an unusual case of loc-GPA involving multiple cranial nerves, which in 3 years progressed into systemic disease involving pituitary gland. Initially antineutrophil cytoplasmic antibody (ANCA) was negative, but as symptoms of diabetes insipidus started, ANCA became positive. Clinical course of ANCA negative loc-GPA should be carefully monitored for development of systemic disease. ANCA may be a useful marker for detecting transition from localized to systemic disease.

Keyword

Granulomatosis with polyangiitis; Diabetes insipidus; Pituitary gland

MeSH Terms

Antibodies, Antineutrophil Cytoplasmic
Cranial Nerves
Diabetes Insipidus
Granulomatosis with Polyangiitis*
Humans
Pituitary Gland
Respiratory System
Antibodies, Antineutrophil Cytoplasmic

Figure

  • Fig. 1 Histopathologic findings of the nasopharyngeal biopsy. (A) High power field reveals ill-defined granulomatous inflammation with multinucleated giant cells (arrow) (H&E, ×100), (B) Lymphoplasma cells with acute inflammatory cells infiltration (arrow) is observed in small vessel, indicating vasculitis (H&E, ×400).

  • Fig. 2 Baseline brain magnetic resonance imaging showing pituitary involvement. (A) T1-weighted midline sagittal image shows loss of normal high signal intensity in neurohypophysis, (B) gadolinium enhanced T1-weighted sagittal image shows diffuse enlargement of pituitary gland (11.5 mm in height) with thickened enhanced pituitary stalk.

  • Fig. 3 Follow-up brain magnetic resonance images. They show slightly decreased size of pituitary gland with no change of diffuse enhancement with thickened pituitary stalk in T1-weighted midline sagittal image (A), and gadolinium enhanced T1-weighted sagittal image (B).


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