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Published on: December 17, 2010
A Rare Case of Microscopic Polyangitis With Associated Transient Hypophysitis
Mercedes Martinez-Gil1, Monica Rodriguez2, Jeremy Hughes3
1Department of Internal Medicine, Creighton University School of Medicine, Phoenix, AZ 85013, USA.
Abstract:
Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are rare forms of antineutrophil cytoplasmic antibody-associated vasculitides, characterized by systemic inflammation and necrosis of small blood vessels, which can lead to multiorgan damage. GPA is identified by the presence of granulomas and serine proteinase-3 positivity, while MPA is marked by necrotizing vasculitis without granulomas and is associated with myeloperoxidase (MPO) positivity. Central nervous system involvement is more frequent in GPA, affecting 7% to 10% of patients, compared to its occurrence in approximately 1% of MPA cases. In this case report, we present a 41-year-old woman who exhibited symptoms of pituitary mass effect, initially suspected to be a macroadenoma. Further investigation revealed pituitary enlargement due to MPA, confirmed by positive MPO antibodies and lung biopsy findings consistent with MPA. After treatment with prednisone and rituximab, the patient's pituitary gland returned to its normal size, with significant improvement in her symptoms. This case highlights the diagnostic complexities in differentiating MPA from more common causes of pituitary enlargement and underscores the necessity of considering vasculitic origins in similar clinical scenarios. Further research is essential to deepen the understanding of the pathophysiology and to optimize the management of pituitary involvement in MPA.
Insights
Microscopic polyangiitis (MPA), a rare vasculitis, can cause pituitary enlargement. This case report details a successful treatment of pituitary mass effect due to MPA using prednisone and rituximab.
Area of Science:
- Rheumatology
- Endocrinology
- Neurology
Background:
- Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are rare antineutrophil cytoplasmic antibody-associated vasculitides.
- These conditions cause systemic inflammation and small blood vessel necrosis, potentially leading to multiorgan damage.
- MPA is characterized by necrotizing vasculitis and myeloperoxidase (MPO) positivity, distinguishing it from GPA.
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