Molecular Mechanisms Underlying the Therapeutic Role of Vitamin E in Age-Related Macular Degeneration.

The eye is particularly susceptible to oxidative stress and disruption of the delicate balance between oxygen-derived free radicals and antioxidants leading to many degenerative diseases. Attention has been called to all isoforms of vitamin E, with α-tocopherol being the most common form. Though similar in structure, each is diverse in antioxidant activity. Preclinical reports highlight vitamin E's influence on cell physiology and survival through several signaling pathways by activating kinases and transcription factors relevant for uptake, transport, metabolism, and cellular action to promote neuroprotective effects. In the clinical setting, population-based studies on vitamin E supplementation have been inconsistent at times and follow-up studies are needed. Nonetheless, vitamin E's health benefits outweigh the controversies. The goal of this review is to recognize the importance of vitamin E's role in guarding against gradual central vision loss observed in age-related macular degeneration (AMD). The therapeutic role and molecular mechanisms of vitamin E's function in the retina, clinical implications, and possible toxicity are collectively described in the present review.

Esophageal Stricture Recalcitrant to Repeated Dilation in Complex Rheumatological Disease.

We report a case of severe refractory esophageal strictures in the setting of rheumatological disease found to be secondary to isolated esophageal autoimmune bullous disease. She had a history of Sjogren syndrome and esophageal strictures with many previous dilations. After rheumatological workup, she was diagnosed with mixed connective tissue disease. Biopsy showed complement and immunoglobulin G deposition in the basement membrane consistent with bullous lupus or bullous pemphigoid. She had no cutaneous bullae and was diagnosed with isolated esophageal bullous disease. She required multiple dilatations over several months of treatment and was started on mycophenolate mofetil with clinical improvement.

Hyper IgG4-Related Disease Presenting with Orbital Tumor and Immune Deficiency.

We report a case of IgG4-RD in a patient with high IgG4 levels, low functional antibodies, and low IgM levels. He presented with bilateral orbital pseudotumors and, after initial improvement on corticosteroids, relapsed with recurrent pleural effusion and pelvic pseudotumor. He had a grossly elevated serum IgG (1905 mg/dl) with elevations in all IgG subclasses but marked elevation in IgG4 (412 mg/dl), low IgM, and low pneumococcal antibodies. Orbital mass biopsy showed polyclonal lymphocytic infiltration and increased IgG4 plasma cells. The patient was started on prednisone and tried several immunosuppressive medications including mycophenolate mofetil, methotrexate, hydroxychloroquine, and azathioprine with decrease in size of the orbital pseudotumor. During a period when the patient stopped his medications, the pseudotumor enlarged with new development of recurrent pleural effusions. He was also found to have a pelvic mass that was biopsy positive for IgG4 proliferation. This case with progression to multiorgan involvement highlights the importance of identifying patients with IgG4-related disease. In contrast to previous cases with normal-to-high IgM, the IgM was low with impaired functional antibodies.