Patients with atypical presentation of IgG4-RD, such as chronic conjunctival infiltration or scleritis, can suffer from considerable diagnostic delay leading to fibrosis or malignancy development. We report the first case of conjunctival carcinoma in a patient with IgG4-RD.

Purpose Ig4‐related disease is a recently defined entity characterized by a mono or multi organ lymphoplasmocytic infiltrate with IgG4+staining (IgG4+/IgG+ cell ratio>40%), associated with fibrosis or sclerosis and elevated IgG4 serum rate over 135mg/dL. Purpose is to determine the ophthalmological and histological features of IgG4‐RD, and to analyse the outcome under treatment. Methods Patients with biopsy‐proven IgG4‐RD, seen between 2009 and 2011 in a single tertiary center, were retrospectively reviewed. Clinical manifestations, biological and radiological results, histological features and outcome under treatment were analyzed. Results Three caucasian women were included. Mean age was 58 years (range :50‐63). One patient presented with unilateral painful propotosis, associated with third and fifth cranial nerve palsy. One patient had recurrent anterior scleritis, and one patient presented with chronic conjonctival inflitration. None of them had extra‐ocular involvement (FDG‐PET‐scan). Other inflammatory conditions were ruled out. Histopathology demonstrated lymphoplasmocytosic proliferation with expression of IgG4+ plasma cells over 40%. IgG4 serum rate was elevated in one case. All patients primary responded to corticosteroids but 2 of them required the adjunction of an immusuppressive drug(fibrosis or relapse).third one presented with epithelial carcinoma. Conclusion IgG4‐RD is a new and rare entity, challenging to diagnose in case of isolated orbital involvement or in atypical features, such as conjonctivitis and scleritis Histopathology is the gold‐standard for diagnosis if appropriate staining is performed. Treatment is controvertial : corticosteroids are the mainstay, but many cases require associated immunosuppressors.