Multimodal imaging of granulomatous anterior uveitis with Berlin nodules due to sarcoidosis

Sarcoidosis is a multisystem disorder characterized by the presence of non-caseating granulomas generally involving the lungs, skin, and eyes. Berlin nodules are nodular granulomas in the angle of the anterior chamber and are a less common clinical manifestation of sarcoid uveitis. We here present a case of ocular sarcoidosis who had Berlin nodules on gonioscopic examination. Case Report A 36-year-old male presented with complaints of redness and pain for 3 months. Slit-lamp examination showed the presence of mutton fat keratic precipitates in the right eye, broad-based posterior synechiae, and peripheral iris nodules in both eyes [Fig. 1a-d]. Gonioscopy revealed inflammatory nodules in the angle [Fig. 1e,f and 3a]. Fundus examination was normal. Polymerase chain reaction of aqueous aspirate for Mycobacterium tuberculosis, tuberculin skin test, and QuantiFERON TB gold test was negative. High-resolution computed tomography chest revealed the presence of enlarged mediastinal lymph nodes. Serum angiotensin-converting enzyme level was elevated. The patient was referred to a pulmonologist and was started on oral prednisolone and mycophenolate mofetil. On review after 1 month, eyes quietened, and keratic precipitates and iris nodules disappeared [Fig. 2 and 3b].Figure 1: Slit-lamp images of the right (a and c) and left (b and d) eye showing circumcorneal congestion, peripheral iris nodules, and posterior synechiae with medium-sized keratic precipitates in the right eye. C shows well-circumscribed pearly white nodules with surface vascularization which is characteristic of Berlin nodules. Gonioscopic images of the right (e) and left (f) eye showing tent-shaped peripheral anterior synechiae with Berlin nodules suggestive of ocular sarcoidosisFigure 2: Slit-lamp images of the right (a and c) and left (b and d) eye showing the disappearance of Berlin nodules after treatment with oral steroids and immunosuppressantsFigure 3: Anterior segment optical coherence tomography of the right eye showing a hyporeflective lesion with hyperreflective border at the inferotemporal angle suggestive of Berlin nodule, and keratic precipitates (a), and the absence of the nodular lesion and keratic precipitates after treatment (b)Discussion Ocular sarcoidosis can affect any part of the eye and its adnexa and present as conjunctival granuloma, lacrimal gland enlargement, uveitis, episcleritis, scleritis, optic neuropathy, and orbital inflammation.[1] The anterior segment manifestations according to the revised criteria of the International Workshop on Ocular Sarcoidosis include mutton-fat keratic precipitates (large and small), iris nodules at the pupillary margin (Koeppe) or in the stroma (Busacca), trabecular meshwork nodules (Berlin), and/or tent-shaped peripheral anterior synechia.[2] Berlin nodules seen in sarcoid uveitis can cause an elevated intraocular pressure by anatomic obstruction of the canal of schlemm.[3] Other causes of nodules in the angle of the iris include malignancies such as lymphomas, leukemias, melanomas and metastatic tumors, syphilis, and tuberculosis.[4] Conclusion Berlin nodules, though rare in occurrence, are one of the diagnostic features of ocular sarcoidosis and should be looked for by gonioscopy in suspected cases. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship: Nil. Conflicts of interest: There are no conflicts of interest.