Case of the Month
Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO
September 2019
Granulomatosis with Polyangiitis (Wegener’s Granulomatosis)
- Background
- Granulomatosis with Polyangiitis (GPA), formerly known as Wegener’s Granulomatosis, is a systemic multi-system vasculitis associated with c-anti-neutrophil cytoplasmic antibody (C-ANCA) and necrotizing granulomatosis of small and medium vessels1.
- Typically, the disease involves a combination of the ears, nose, throat, lungs, and/or kidneys.
- Clinical Presentation
- Rarely, pituitary involvement is reported, and it is even more rare for pituitary involvement to represent the presenting symptom.1,2 When pituitary involvement is the presenting symptom, the most common manifestation is related to hypopituitarism.
Chronic mastoiditis has been reported with GPA3, although it is not common as a presenting symptom4.
- Rarely, pituitary involvement is reported, and it is even more rare for pituitary involvement to represent the presenting symptom.1,2 When pituitary involvement is the presenting symptom, the most common manifestation is related to hypopituitarism.
- Key Diagnostic Features
- Pituitary: Necrotic inflammatory process with peripheral enhancement, small area of central diffusion restriction, and involvement of the infundibulum and hypothalamus
- Mastoid air cells: enhancing mastoid effusions with an area of coalescence/necrosis consistent with mastoiditis
- Chest: Cavitary nodules and masses
In our case, biopsy of the sellar/suprasellar mass demonstrated acute necrotizing granulomatous inflammation. c-ANCA was positive. These results in combination with the chest and sinus findings led to the diagnosis of GPA.
- Differential Diagnosis
- Statistically, a necrotic sellar/suprasellar mass is most likely to represent a pituitary adenoma with pituitary stalk involvement as in our case, other causes of hypophysitis may also be considered
- Craniopharyngioma can also be considered for a possibly cystic suprasellar mass
- Treatment
- The mainstay of treatment is a combination of corticosteroids and immunosuppressant therapy.3
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