Severe Paraneoplastic Anterior and Posterior Scleritis Associated with Myelodysplastic Syndrome

To describe a rare case of severe anterior and posterior scleritis that led to a diagnosis of high-risk myelodysplastic syndrome.

We present a case report along with a literature review on cases of paraneoplastic scleritis to highlight commonalities in etiology, presentation, treatment response, and outcomes.

A 59-year-old male presented with a severe case of unilateral anterior and posterior scleritis with ciliochoroidal detachment and secondary angle closure. His workup revealed severe macrocytic anemia and thrombocytopenia. A subsequent bone marrow biopsy demonstrated cytogenetic analysis consistent with high-risk myelodysplastic syndrome (MDS). The patient was started on high-dose prednisone but was unable to be tapered below 30 mg without recurrence. Rituximab infusions were initiated followed by resolution of ocular symptoms and a successful taper of prednisone. He initiated chemotherapy and was to receive allogenic bone marrow transplantation. However, while on chemotherapy, he clinically deteriorated and passed away.

This case of severe scleritis was suspected to be a paraneoplastic syndrome secondary to myelodysplastic syndrome. Paraneoplastic syndromes represent a rare etiology of scleritis. Patients with hematologic abnormalities such as unexplained cytopenias, severe manifestations at presentation, or steroid refractory scleritis may warrant comprehensive evaluation for malignancy, expedited systemic workup with attention to blood counts, and interdisciplinary collaboration between ophthalmology, hematology, and internal medicine. If an underlying malignancy is identified, definitive treatment of the scleritis involves treatment of the cancer.