Anterior Segment Manifestations of Microscopic Polyangiitis

Abstract

In this retrospective, non-comparative case series, the medical records of three patients diagnosed with microscopic polyangiitis (MPA) at the Bascom Palmer Eye Institute, Ocular Surface/Uveitis service were analyzed: an 80-year-old female with episcleritis, peripheral ulcerative keratitis, and lung involvement (patient 1); an 83-yearold female with nodular non-necrotizing scleritis (patient 2); and a 19-year-old female with chronic conjunctivitis and nasal inflammation (patient 3). Patient 1 had a diagnosis of MPA on presentation to our service; despite steroid-sparing therapy, she required steroids to treat the ocular flare-up of MPA. Patient 2 was given an MPA diagnosis with the help of rheumatology, based on clinical signs, symptoms and a positive p-ANCA; she was treated with corticosteroids and methotrexate. Patient 3 was diagnosed with MPA based on a second biopsy performed by ENT and a positive p-ANCA; treatment with corticosteroids and methotrexate was initiated. MPA can have conjunctival, episcleral, scleral, and corneal manifestations. Even if lung or renal involvement is not noted clinically, a patient in whom there is a high suspicion for MPA should still undergoes a thorough systemic workup, including a biopsy. Furthermore, a multi-disciplinary approach should be taken when managing these patients in order to avoid vision and life-threatening complications. Microscopic polyangiitis (MPA) is a necrotizing small-vessel vasculitis. Patients are typically male, middle-aged, and develop nonspecific, constitutional signs and symptoms of inflammation. Characteristic features include glomerulonephritis, pulmonary vasculitis, and circulating perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) with myeloperoxidase specificity (MPO). While MPA can affect various parts of the peripheral and central nervous systems, and ophthalmic involvement has been previously reported in scattered reports, ocular findings have not been well documented. MPA is a severe disease with the mortality as high as 27.5%; thus, treatment with immunosuppressant is generally necessary. In this article, we report our experience with three MPA patients who presented with atypical demographics and various ophthalmic findings.