INTRODUCTION: Scleritis is often the first clinical manifestation of a systemic disease. Identifying the underlying disease is critical for proper treatment and prognosis. Approximately 43% of scleritis cases are idiopathic, 48% are related to skeletal and connective tissue diseases and 7% are infection related. Malignancy is rarely associated with scleritis. Two previous reports of malignancy (direct scleral involvement with choroidal melanoma and metastatic adenocarcinoma of unknown origin) were associated with an initial presentation of scleritis. The following case illustrates the importance of suspecting malignancy as a paraneoplastic cause of resistant scleritis.
CASE PRESENTATION: An 81 year old man with COPD on supplemental oxygen, hypertension, hyperlipidemia, diabetes mellitus, osteoarthritis and necrotizing scleritis, was referred for further evaluation and management of worsening bilateral eye pain, despite treatment with daily prednisone for two months. He denied fever, weight loss, cough, hemoptysis or other pulmonary symptoms. The tobacco history was significant for 50 pack-years of use. On exam, he was afebrile, with normal heart and respiratory rates, a blood pressure of 150/58 mmHg, and an oxygen saturation of 95% on 2L of supplemental oxygen via nasal cannula. Lung examination revealed a prolonged expiratory phase and decreased breath sounds. Laboratory results including ANCA, ANA, RF, C3, and C4 were within normal limits. Cultures, cytology and histopathology from scleral biopsies were negative for infection and malignancy. A chest radiograph revealed a mass confirmed by CT scan, which showed a 4.3cm spiculated irregular mass in the right upper lobe with multiple areas of lucency and abnormally enlarged lymph nodes throughout the mediastinum. During bronchoscopy, the endobronchial exam was unremarkable, and endobronchial ultrasound guided fine needle aspiration demonstrated malignancy consistent with adenocarcinoma.
DISCUSSION: His paraneoplastic scleritis required acute treatment with bilateral scleral patches with amniotic membrane grafting to both eyes to stabilize the necrotic areas and oral prednisone. Metastatic survey studies were negative. Significant improvement was noted after chemotherapy was started for the lung adenocarcinoma. Currently, three out of twelve chemotherapy treatment sessions have been given. Prednisone has been discontinued. The bilateral eye pain has resolved, vision continues to improve and a steroid sparing agent was never needed.
CONCLUSIONS: Scleritis is an infrequent and rare inflammatory disorder that can present a diagnostic challenge. In half of the cases it is the initial manifestation of a systemic problem. The most common systemic diseases associated with scleritis are rheumatoid arthritis, Wegener’s granulomatosis, polyarteritis nodosa, systemic lupus erythematosus, relapsing polychondritis, and inflammatory bowel disease. Infections with organisms such as pseudomonas, aspergillus, herpes (simplex or zoster), or tuberculosis may cause severe scleritis that is difficult to treat. Rarely, malignancies can present as scleritis either with direct metastatic involvement or as a paraneoplastic syndrome as demonstrated by this case. Effective therapy is dictated by the diagnosis of the associated systemic, infectious, or, as in this case, malignant etiology. An evaluation for malignancy should be considered in a patient presenting with what appears to be idiopathic scleritis that is resistant to treatment.
Reference #1 Kafkala, et al. Masquerade scleritis. Ocular Immunology and Inflammation. 2005;13:479-482.
Reference #2 Fraser Jr DJ, Font RL. Ocular inflammation and hemorrhage as initial manifestations of uveal malignant melanoma: incidence and prognosis. Arch Ophthalmol. 1979;97:1482-1486.
DISCLOSURE: The following authors have nothing to disclose: Hector Payan, Sergio Burguete, Stephanie Levine
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