Ewha Med J.  2016 Jul;39(3):85-88. 10.12771/emj.2016.39.3.85.

Progressive Pituitary Involvement in a Patient with Localized Granulomatosis with Polyangiitis

• Jeon H
• Kim IJ
• Hong YS
• Lim SM
• Cho MS
• Lee J

• KMID: 2344897
• DOI: http://doi.org/10.12771/emj.2016.39.3.85

Abstract

Localized granulomatosis with polyangiitis (loc-GPA) is a milder disease state of GPA restricted to the respiratory tract. Transition from localized form to systemic/generalized disease is predicted to occur in approximately 10% of the patients. We report an unusual case of loc-GPA involving multiple cranial nerves, which in 3 years progressed into systemic disease involving pituitary gland. Initially antineutrophil cytoplasmic antibody (ANCA) was negative, but as symptoms of diabetes insipidus started, ANCA became positive. Clinical course of ANCA negative loc-GPA should be carefully monitored for development of systemic disease. ANCA may be a useful marker for detecting transition from localized to systemic disease.

Keyword

Granulomatosis with polyangiitis; Diabetes insipidus; Pituitary gland

MeSH Terms

Antibodies, Antineutrophil Cytoplasmic
Cranial Nerves
Diabetes Insipidus
Granulomatosis with Polyangiitis*
Humans
Pituitary Gland
Respiratory System
Antibodies, Antineutrophil Cytoplasmic

Figure

• 

  Fig. 1 Histopathologic findings of the nasopharyngeal biopsy. (A) High power field reveals ill-defined granulomatous inflammation with multinucleated giant cells (arrow) (H&E, ×100), (B) Lymphoplasma cells with acute inflammatory cells infiltration (arrow) is observed in small vessel, indicating vasculitis (H&E, ×400).

• 

  Fig. 2 Baseline brain magnetic resonance imaging showing pituitary involvement. (A) T1-weighted midline sagittal image shows loss of normal high signal intensity in neurohypophysis, (B) gadolinium enhanced T1-weighted sagittal image shows diffuse enlargement of pituitary gland (11.5 mm in height) with thickened enhanced pituitary stalk.

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  Fig. 3 Follow-up brain magnetic resonance images. They show slightly decreased size of pituitary gland with no change of diffuse enhancement with thickened pituitary stalk in T1-weighted midline sagittal image (A), and gadolinium enhanced T1-weighted sagittal image (B).

Reference

1. Ahmad I, Lee WC, Nagendran V, Wilson F, Shortridge RT. Localised Wegener’s granulomatosis in otolaryngology: a review of six cases. ORL J Otorhinolaryngol Relat Spec. 2000; 62:149–155.

2. Carrington CB, Liebow A. Limited forms of angiitis and granulomatosis of Wegener’s type. Am J Med. 1966; 41:497–527.

3. Hellmich B, Flossmann O, Gross WL, Bacon P, Cohen-Tervaert JW, Guillevin L, et al. EULAR recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibody-associated vasculitis. Ann Rheum Dis. 2007; 66:605–617.

4. Holle JU, Gross WL, Holl-Ulrich K, Ambrosch P, Noelle B, Both M, et al. Prospective long-term follow-up of patients with localised Wegener’s granulomatosis: does it occur as persistent disease stage. Ann Rheum Dis. 2010; 69:1934–1939.

5. Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994; 37:187–192.

6. De Parisot A, Puechal X, Langrand C, Raverot G, Gil H, Perard L, et al. Pituitary involvement in granulomatosis with polyangiitis: report of 9 patients and review of the literature. Medicine (Baltimore). 2015; 94:e748.

7. De Luna G, Terrier B, Kaminsky P, Le Quellec A, Maurier F, Solans R, et al. Central nervous system involvement of granulomatosis with polyangiitis: clinical-radiological presentation distinguishes different outcomes. Rheumatology (Oxford). 2015; 54:424–432.

8. Sinico RA, Di Toma L, Maggiore U, Bottero P, Radice A, Tosoni C, et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum. 2005; 52:2926–2935.

9. Kapoor E, Cartin-Ceba R, Specks U, Leavitt J, Erickson B, Erickson D. Pituitary dysfunction in granulomatosis with polyangiitis: the Mayo Clinic experience. J Clin Endocrinol Metab. 2014; 99:3988–3994.

10. Hughes J, Barkhoudarian G, Ciarlini P, Laws ER, Mody E, Inzucchi SE, et al. Refractory pituitary granulomatosis with polyangiitis (Wegener's) treated with rituximab. Endocr Pract. 2013; 19:e1–e7.