ANCA-negative granulomatosis with polyangiitis-like intraocular and cutaneous vasculitis secondary to myelodysplastic syndrome

Abstract

An elderly woman presented with subacute, bilateral, severe vision loss and pronounced panuveitis. She also noted a skin lesion on her ankle, and biopsy of the lesion revealed necrotising vasculitis and granulomatous inflammation of small vessels. Although the organ involvement in this case is atypical, a granulomatosis with polyangiitis (GPA)-like syndrome was suspected. Systemic and topical ophthalmic steroids were initiated, with effective but incomplete reduction in intraocular inflammation. Cytogenetic analysis of bone marrow aspirate demonstrated myelodysplastic syndrome (MDS). Systemic chemotherapy with azacitidine was then initiated, achieving haematological stability and quiescence of intraocular inflammation. Therefore, this is a case of paraneoplastic, GPA-like syndrome involving both eyes, secondary to an underlying MDS. With oncological treatment, vision recovered significantly.

Keywords: Autoimmunity; Vasculitis.