Necrobiotic xanthogranuloma scleritis in a case of granulomatosis with polyangiitis (Wegener’s granulomatosis)

Abstract

Narumon Keorochana,1 Kanaspana Klanarongran,2 Kantang Satayasoontorn,3 Sumapa Chaiamnuay4 1Department of Ophthalmology, Ocular Inflammatory and Uveitis Division, 2Department of Ophthalmology, External Disease and Cornea Division, 3Department of Anatomic Pathology, 4Department of Medicine, Rheumatology Division, Phramongkutklao Hospital, Phramongkutklao College of Medicine, Bangkok, Thailand Abstract: The purpose of this study was to describe a case of necrobiotic xanthogranuloma scleritis in a 53-year-old male with unilateral progressive visual loss, scleritis, prolonged fever, and multiple mononeuropathy. Scleral biopsy showed necrosis with small abscess, and the pathological tissues revealed submucosal infiltration of mononucleated foamy histiocytes (xanthoma cells), hemosiderin-laden macrophages, neutrophils, lymphocytes, plasma cells, and erythrocytes without Touton giant cells or cholesterol clefts. Serum protein electrophoresis showed polyclonal gammopathy. All infectious investigations were negative. Afterward, this patient was diagnosed with granulomatosis with polyangiitis based on granuloma found in scleral tissue, vasculitis seen in sural nerve biopsy and positive serologies (C-ANCA and anti-PR3 antibody). He was treated with high-dose corticosteroid and later with intravenous cyclophosphamide monthly. He responded well to treatment, both eye and systemic conditions. Necrobiotic xanthogranuloma scleritis could be an early presentation of granulomatosis with polyangiitis. Keywords: scleritis, uveitis, ocular inflammation, pathology, autoimmune, xanthoma cells, GCA

Keywords

• scleritis
• Necrobiotic Xanthogranuloma
• Granulomatosis with polyangiitis
• Wegener’s granulomatosis
• pathology