Endocrine Abstracts

Although the first line therapy for prolactinoma is medical. Visual field defect due to pituitary mass would lead to surgery. Herein, we share the post-operative follow-up of a macroprolactinoma case.

Case: A 57-year-old male patient was operated on for a giant pituitary adenoma, which was compressing the optic chiasm. The postop prolactin (PL) value was found to be higher than 200 ng/ml and cabergoline treatment was initiated. Two months after the operation, the patient was admitted to the hospital for complaints of exophthalmos, headache, fatigue and drowsiness. Blood pressure was 100/60 mmHg, laboratory values revealed Na 128 mEq/l and creatinine 1.82 mg/dl. Adrenal insufficiency was presumed and methylprednisolone was initiated. Cranial CT scan was obtained to exclude secondary complications of operation, and it revealed a 1.5 cm residual adenoma and pseudotumor orbita. When the persistent microscopic haematuria, elevated sedimentation rate (ESR) (80 mm/s) and proteinuria (1.3 g/24 h) were considered, with the clinical finding of pseudotumor orbita, it was believed that all would be indicative of vasculitis and IgG4 related diseases. ANA was negative, PR3ANCA was 3(+) and IgG4 was 224 mg/dl (120–200). Steroid treatment resulted in a significant improvement of ocular and biochemical findings (creatinine 1.4 mg/dl, ESR 36 mm/h). We performed kidney biopsy to exclude Wegener granulomatosis (WG). Biopsy was compatible with WG; IgG4 staining was negative. Pulse steroid therapy and 1 g cyclophosphamide were administered. Haematuria disappeared, IgG4 normalized, creatinine and ESR decreased to 0.9 mg/dl and 29 mm/s, respectively. Cabergoline treatment was switched to bromocriptine to exclude the cabergolin induced vasculitis.

Conclusion: The patient did not have retroorbital biopsy, on the other hand the kidney biopsy supported WG. It has been reported that the PL elevation triggers autoimmunity and clinical remission can be achieved by treatment with dopamine agonist in patients with SLE and RA, whose PL levels are elevated. There are cases in which IgG4 related diseases were identified by the initiation of high dose cabergoline treatment and the relationship between IgG4-related diseases and WG has been firmly stated in the literature. Under the light of literature, vasculitis may have been triggered by prolactinoma and cabergoline treatment in our patient. The orbital pseudotumor may be associated with either IgG4-related disease or WG. A few cases have been reported about WG-associated pseudotumor orbita in adult patients. In this respect, our case remains exciting.