Pituitary dysfunction in granulomatosis with polyangiitis

Abstract

PurposeGranulomatosis with polyangiitis (GPA) is a multisystem disease, characterized by necrotizing small-vessel vasculitis, which mainly affects the respiratory tract and the kidneys. Pituitary involvement in GPA is rare, present in about 1% of all cases of GPA. To date, only case reports or small case series have been published. Herein we report clinical features, imaging findings, treatment and outcomes in three patients with GPA-related pituitary dysfunction (PD).MethodsA retrospective analysis of three cases of GPA-related PD was conducted, followed by systematic review of the English medical literature using PubMed.ResultsThe three cases include three women aged between 32 and 37 years. PD was the presenting feature in one and two developed PD in the course of the disease. All patients had a pituitary lesion on MRI. Conventional treatment with high doses of glucocorticoids and cyclophosphamide led to resolution or improvement of the MRI abnormalities, whereas it was not effective in restoring PD. A systematic review identified 51 additional patients, showing that GPA can lead to partial or global PD, either at onset or, during the course of the disease. Secondary hypogonadism is the predominant manifestation, followed by diabetes insipidus (DI). Sellar mass with central cystic lesion is the most frequent radiological finding.ConclusionGPA should be carefully considered in patients with a sellar mass and unusual clinical presentation with DI and systemic disease. Although conventional induction-remission treatment improves systemic symptoms and radiological pituitary abnormalities, hormonal deficiencies persist in most of the patients. Therefore, follow-up should include both imaging and pituitary function assessment.

Less known aspects of central hypothyroidism: Part 1 – Acquired etiologies

“…Wegener’s granulomatosis, also known as granulomatosis with polyangitis, is a rare disorder with unknown etiology, which is characterized by vasculitis involving small vessels. The pituitary is rarely affected, CH being reported in half of cases [82].…”

“…Bexarotene also increases the peripheral metabolism of thyroid hormones by inducing glucuronyl transferases and sulfotransferases [96]. Consequently, hypothyroid patients on bexarotene require a higher replacement dose (up to two times) of levothyroxine and due to the suppression, TSH concentrations cannot be used to monitor the treatment dose [82]. The TSH-suppressing effect was not reported for 13- cis -retinoic acid (isotretinoin), which is used for severe acne, whereas it was observed in 2 of 4 patients in a pilot study on oral alitretinoin for congenital ichthyosis [99].…”

Granulomatosis with Polyangiitis Manifesting as a Symptomatic Sellar Mass in a Young Woman

“…A challenging aspect of the case was the initial diagnosis of Rathke's cleft cyst based on the first MRI, which might have delayed the accurate diagnosis of her pituitary lesion. Previously reported cases of pituitary GPA have had highly varied MRI findings, with T1 hypo-or hyperintensity, T2 hyperintensity, and heterogeneous enhancement seen [3,[9][10]. This degree of variability is typical for granulomatous diseases, and imaging findings can often be confused with malignancy [11].…”

Severe localised granulomatosis with polyangiitis (Wegener’s granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus: a case report and literature review

“…Thickened stalk. Hyperprolactinaemia ENT, pulmonary MPO +ve Esposito 2017 [ 57 ] 28540625 3 37* 37 F Sellar mass extending into the suprasellar cistern with peripheral enhancement and central cystic lesion. Stalk deviation.…”