RT Journal Article
SR Electronic
T1 An eosinophilic variant granulomatosis with polyangiitis involving the dura, bilateral orbits, and mastoids
JF Saudi Medical Journal
JO Saudi Med J
FD Prince Sultan Military Medical City
SP 690
OP 693
DO 10.15537/smj.2016.6.14718
VO 37
IS 6
A1 Al-Hakami, Hasan
A1 Al-Arfaj, Abdurhman S.
A1 Al-Sohaibani, Mohammed
A1 Khalil, Najma A.
YR 2016
UL http://smj.org.sa/content/37/6/690.abstract
AB Granulomatosis with polyangiitis (GPA) formerly called Wegener’s granulomatosis is a chronic necrotizing granulomatous inflammatory disease with systemic vasculitis involving the upper and lower respiratory tract, and kidneys. The typical histopathology is that of necrotizing granulomatous inflammation with palisading histiocytes, neutrophils, and lymphocytes. We report a case of a 57-year-old lady presenting with left eye swelling, left ear pain and discharge, but with no pulmonary or renal symptoms. Investigations revealed positive cytoplasmic antineutrophil cytoplasmic antibodies and proteinase 3 antibodies. The CT and MRI showed meningeal thickening and bilateral structural changes of the orbits and mastoids. Lacrimal gland biopsy showed non necrotizing granulation with an eosinophilic infiltration. She was diagnosed with eosinophilic variant of GPA of the eyes and mastoid bones bilaterally extending to dura and sparing the lungs and kidneys. She responded to corticosteroids and rituximab.