Panhypophysitis and infundibulitis associated with granulomatosis with polyangiitis

Area of Science:

• Endocrinology
• Rheumatology
• Neurology

Background:

• Granulomatosis with polyangiitis (GPA) is a rare autoimmune vasculitis.
• Pituitary involvement in GPA (hypophysitis) is an uncommon but serious manifestation.
• GPA-associated hypophysitis can present with diverse hormonal deficiencies and neurological symptoms.

Purpose of the Study:

• To highlight the rare but significant pituitary involvement in GPA.
• To emphasize the diagnostic challenges and importance of early recognition of GPA-associated hypophysitis.
• To discuss the management and long-term implications of pituitary dysfunction in GPA.

Main Methods:

• Case report of a 39-year-old woman with GPA and pituitary involvement.
• Clinical presentation including headaches, vision loss, and hormonal deficiencies.
• Diagnostic workup involving c-ANCA, PR3 antibodies, biochemical tests, and pituitary MRI.
• Treatment with corticosteroids and rituximab, followed by hormone replacement therapy.

Main Results:

• The patient presented with symptoms suggestive of GPA and subsequent pituitary dysfunction.
• Biochemical evaluation revealed central adrenal, thyroid, and gonadotropin deficiencies.
• Pituitary MRI showed characteristic findings of inflammation and necrosis.
• Hormonal deficiencies persisted despite remission of systemic GPA activity, necessitating long-term hormone replacement.

Conclusions:

• Pituitary involvement is a rare but critical complication of GPA that requires prompt diagnosis and management.
• GPA-associated hypophysitis can lead to persistent hormonal deficiencies requiring lifelong hormone replacement.
• Awareness of hypophysitis in GPA is essential for timely intervention and improved patient outcomes.