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Endocr Pract. 2013 Jan-Feb;19(1):e1-7. doi: 10.4158/EP12181.CR.

Refractory pituitary granulomatosis with polyangiitis (Wegener's) treated with rituximab.

Author information

1
Section of Endocrinology and Metabolism, Yale University School of Medicine, New Haven, CT 06519, USA. jing.hughes@yale.edu

Abstract

OBJECTIVE:

Granulomatosis with polyangiitis (GPA), also known as Wegener's granulomatosis, is an autoimmune disease characterized by inflammation of blood vessels most often seen in the upper respiratory tract, lungs, kidneys, and skin. Central nervous system (CNS) involvement of GPA is rare, particularly in the pituitary, and can be difficult to treat.

METHODS:

Case report.

RESULTS:

We present a 30-year-old woman with pituitary and ocular GPA, whose unusually recalcitrant disease led to the development of pan-hypopituitarism and near-total vision loss. After failing multiple systemic immunosuppressants, she was ultimately treated with the novel immunomodulatory agent rituximab together with pulse corticosteroids, which achieved a gratifying response.

CONCLUSION:

Pituitary and optic chiasm involvement is a rare complication of GPA. We believe this case illustrates the complexity of management of pituitary GPA and provides insight into the potential utility of the biologic agent rituximab in this disease.

PMID:
23186961
DOI:
10.4158/EP12181.CR
[Indexed for MEDLINE]
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