Granulomatosis with polyangiitis: Possible endocrine manifestations

Abstract

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is a necrotizing vasculitis of small and medium-sized blood vessels characterized by diffuse inflammation of vascular structures and perivascular and extravascular granulomatosis. In its systemic form, GPA predominantly affects the ear, nose and throat, with lung and kidney involvement with typically rapidly progressive necrotizing glomerulonephritis with extracapillary crescents, while the absence of kidney damage at the time of diagnosis is defined as a limited form of GPA with a more favorable prognosis (1, 2). Antineutrophil cytoplasmic antibodies (c-ANCA) with specificity for proteinase 3 (PR3) represent a biochemical diagnostic criterion. They are detected in 90% of generalized forms and in about 50% of limited forms of granulomatosis with polyangiitis (1, 2). In the absence of treatment, GPA is a disease of progressive evolution. Systemic corticosteroid therapy and immunosuppressive therapy significantly changed the prognostic aspect of the disease. Only a few sporadic observations have been published on endocrine disorders associated with GPA. We present a case of a man, 39 years old, with Wegener's granulomatosis who developed autoimmune thyroiditis 8 years after the initial diagnosis.

Keywords

• granulomatosis with polyangiitis
• autoimmune thyroiditis
• antineutrophil cytoplasmic
• antibodies small vessel vasculitis