Papillophlebitis with Uveitis in MPO ANCA Associated Pregnancy

Abstract

Purpose: We aim to describe first case of papillophlebitis with uveitis and vasculitis in perinuclear-anti-neutrophilic-cytoplasmic antibody myeloperoxidase (p-ANCA-MPO) positive term pregnancy reported in INDIA and to underline the importance of disease which should be better known among ophthalmologist. Methods: A 32 years old women visited our outpatient with unilateral defective vision, running nose, multiple joint ache in 3 rd trimester pregnancy. Slit lamp examination revealed unilateral non-granulomatous uveitis, papillophlebitis with vasculitis. She had a positive nasal necrotizing lesion confirmed on biopsy by pathologist and p-ANCA-MPO serology positive. In view of above she was referred to rheumatologist. Results: She was diagnosed with Wegener's granulomatosis (WG) and advised immunosuppresive therapy. She was started on 15mgm/kg/pulse of cyclophosphamide as induction therapy along with steroids and azathioprine 50mgm which were continued for a year postpartum. Anterior uveitis was treated with topical steroids with good effect. The side effects were transitory cushingoid change in mother and growth retardation in the infant. Conclusion: Pappilophlebitis, uveitis and vasulitis may be so mild that they may not cause troubling symptoms and hence in case of term pregnancy with Wegeners granulomatosis as an underlying disease it is commonly underdiagnosed. Great co-operation between ophthalmologist and immunologist is extremely crucial for early diagnosis and initiation of therapy. WG tends to be a multi-organ disease with high rate of recurrences and relapses requiring constant follow up. Early diagnosis would introduce immunosuppressive therapy safeguarding the outcome of pregnancy and lives of mother and foetus.