Behcet's-like syndrome following pembrolizumab: An immune-related adverse event associated with programmed death receptor-1 inhibitor therapy.

INTRODUCTION: The landscape for the treatment of metastatic melanoma has been revolutionized with the introduction immune checkpoint inhibitors. Immune checkpoint inhibitors have now become the standard of care for the treatment of cancers. These immune agents including programmed death receptor-1 inhibitors, programmed death-ligand 1 inhibitors and cytotoxic T-lymphocyte antigen-4 inhibitors have shown promising results but have been associated with numerous immune-related complications. Pembrolizumab, a programmed death receptor-1 inhibitor, has been associated with a number of immune-related adverse events affecting multiple organ systems including integument, ocular, endocrine, cardiovascular, pulmonary, renal, gastrointestinal, and musculoskeletal system. CASE REPORT: We present a case of an 88-year-old Caucasian male with metastatic melanoma of the face with metastasis to the right fifth cranial nerve and into the right cavernous sinus. He underwent resection of the melanoma and was placed on pembrolizumab at 2 mg/kg every three weeks. Interestingly, 24 months on pembrolizumab therapy, he developed corneal erosions, oral and genital ulcerations. MANAGEMENT AND OUTCOME: Patient completed his 24 months of pembrolizumab and was started on prednisone and colchicine with improvement in his symptoms. At his follow-up eight months, he had recurrence of an oral ulcer. DISCUSSION: Here we present a rare case of an elderly male on pembrolizumab who suffered from corneal erosions, oral and genital ulcers, a syndrome similar to Behcet's disease. Given that pembrolizumab and other immune checkpoint inhibitors are being utilized in the treatment of cancers, physicians should be aware of the wide range immune-related adverse events including the possible Behcet's-like syndrome presentation.

Neuroendocrine manifestations of phospholipid antibody disease identified by long-term follow-up study of patients with phospholipid antibodies.

UNLABELLED: Recurrent clinical thrombotic episodes and/or recurrent fetal wastage are the clinical features of phospholipid antibody (aPL) syndrome, which is characterized by a bland thrombosis, but is not inflammatory, as is found in other connective tissue diseases such as systemic lupus erythematosus (SLE). Previous reports have suggested that some patients with primary aPL syndrome may progress to develop other autoimmune diseases, including inflammatory diseases such as SLE. The aim of this study was to determine the long-term outcome of women with aPL antibodies, with regard to progression of their underlying autoimmune disease. To that end, a retrospective study was made of women with aPL and primary aPL syndromes who had been followed at our institution for a minimum of 3 years. Charts were reviewed, patients interviewed, and laboratory tests were performed to determine whether the clinical nature of the disease and/or its autoantibody profile had changed. Thirty patients were enrolled into the study (29 with aPL syndrome, 1 with consistent aPL and no syndrome). Follow-up ranged from 3 to 22 years. Results were as follows: The autoimmune clinical features were unchanged in 27 patients, but 3 patients developed inflammatory disease, presenting with nasal chondritis (2), cutaneous vasculitis (3), and mucosal ulcer (1). In each case, these changes occurred during pregnancy or the immediate postpartum period. One patient fulfilled criteria for SLE as seen by a change in her autoantibody profile. Another incidental finding was that three other patients were diagnosed with papillary thyroid cancer, two being diagnosed during the follow-up period. IN CONCLUSION: (1) Inflammatory disease may develop in some patients with aPL and appears to be set off by pregnancy, a known trigger for clinical thrombotic events in aPL patients. (2) Thyroid cancer may be associated with aPL, and this association warrants further study with larger number of patients.