title: Pituitary involvement in granulomatosis with polyangiitis: report of 9 patients and review of the literature.
abstract: Pituitary dysfunction is a rare manifestation of granulomatosis with polyangiitis (GPA) (Wegener). The main aim of this multicenter retrospective study was to describe the characteristics and outcomes of pituitary manifestations in patients with GPA included in the French Vasculitis Study Group database.Among the 819 GPA patients included in the database, 9 (1.1%) had pituitary involvement. The median age at diagnosis of GPA and pituitary involvement was 46 and 50.8 years, respectively. Pituitary involvement was present at onset of GPA in 1 case and occurred later in 8 patients after a median follow up of 58.5 months. When pituitary dysfunction occurred, 8 patients had active disease at other sites including ENT (n = 6), eye (n = 4), or central nervous system (n = 3) involvement. The most common hormonal dysfunctions were diabetes insipidus (n = 7) and hypogonadism (n = 7). Magnetic resonance imaging was abnormal in 7 patients. The most common lesions were an enlargement of the pituitary gland, thickening of the pituitary stalk, and loss of posterior hypersignal on T1-weighed images. All patients were treated with corticosteroid therapy and 8 patients received immunosuppressive agents for the pituitary involvement, including cyclophosphamide (n = 3), rituximab (n = 2), and methotrexate (n = 3). After a median follow-up of 9.2 years, GPA was in complete remission in 7 patients, but 8 patients were still under hormone replacement therapy. Among the 5 patients who had a subsequent MRI, 2 had complete resolution of pituitary lesions.By combining our study and the literature review, the frequency of hypogonadism and diabetes insipidus, among the patients with pituitary dysfunction, can be estimated at 78% and 71% respectively. Despite a high rate of systemic disease remission on maintenance therapy, 86% of the patients had persistent pituitary dysfunction. The patients who recovered from pituitary dysfunction had all been treated by cyclophosphamide.Pituitary disease in GPA occurs mostly several months or years after diagnosis. There is no correlation between hormonal, radiologic, and systemic outcome. Although immunosuppressive drugs improve the systemic disease, hormonal deficiencies usually persist. It is therefore important to shorten diagnostic delays and treat these patients early in the course of disease before irreversible damage occur.
authors.affiliation: From the Department of Internal Medicine, Hôpital de La Croix-Rousse and University Claude Bernard Lyon 1, 103 Grande rue de la Croix-Rousse 69317 Lyon Cedex 04 (ADP, PS); Department of Internal Medicine, Centre de Référence des maladies systémiques autoimmunes rares, Hôpital Cochin, University Paris-Descartes, Assistance Publique - Hôpitaux de Paris, Paris (XP, LG); Department of Endocrinology, Groupement Hospitalier Est and University Claude Bernard Lyon 1, 59 Boulevard Pinel 69677 Bron Cedex, Lyon (CL, GR); Department of Internal Medicine, Centre Hospitalier Régional Universitaire de Besançon, 2 Place Saint-Jacques, Besançon (HG); Department of Internal Medicine, Hôpital Edouard Herriot and University Claude Bernard Lyon 1, Lyon (LP); Department of Internal Medicine, Hôpital de Hautepierre, 1 Avenue Molière, Strasbourg (LG); Department of Internal Medicine, Centre Hospitalier Universitaire de Dijon, 14 rue Paul Gaffarel, Dijon (SB); Department of Neuroradiology, Groupement Hospitalier Est and University Claude Bernard Lyon 1, 59 Boulevard Pinel 69677 Bron Cedex, Lyon (OT); Department of Rheumatology, Hôpital de la Maison Blanche, 45 rue Cognacq-Jay 51092 Reims Cedex (JPE); and Department of Internal Medicine, Groupe Hospitalier Mutualiste de Grenoble, 8 Rue Docteur Calmette 38000 Grenoble, France (SV).
authors.forename: Audrey
authors.initials: A
authors.lastname: De Parisot
authors.forename: Xavier
authors.initials: X
authors.lastname: Puéchal
authors.forename: Corinne
authors.initials: C
authors.lastname: Langrand
authors.forename: Gerald
authors.initials: G
authors.lastname: Raverot
authors.forename: Helder
authors.initials: H
authors.lastname: Gil
authors.forename: Laurent
authors.initials: L
authors.lastname: Perard
authors.forename: Guillaume
authors.initials: G
authors.lastname: Le Guenno
authors.forename: Sabine
authors.initials: S
authors.lastname: Berthier
authors.forename: Olivier
authors.initials: O
authors.lastname: Tschirret
authors.forename: Jean Paul
authors.initials: JP
authors.lastname: Eschard
authors.forename: Stephane
authors.initials: S
authors.lastname: Vinzio
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
authors.forename: Pascal
authors.initials: P
authors.lastname: Sève
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010900:Pituitary Diseases / Q000150:complications*; D010907:Pituitary Hormones / Q000097:blood; D012189:Retrospective Studies
chemical_list: D007166:Immunosuppressive Agents; D010907:Pituitary Hormones