NS is a rare but devastating consequence of certain connective tissue disorders, most commonly RA. The presence of NS mandates that patients be treated with at least one immunosuppressive chemotherapeutic drug. The cure rate is high in such instances after 1 year of freedom from any evidence of recurrent inflammation. Surgical treatment through tectonic scleral and peripheral corneal grafting is rarely (although sometimes) indicated, except in instances of NS that has advanced to the point of perforation of the globe. Various graft materials can be used, including fascia lata, periosteum, split-thickness dermis, Gore-Tex (synthetic), aortic tissue, and autologous or homologous sclera. Surgical treatment alone will not solve the problem for the patient. The physician also must control the underlying immunoregulatory dysfunction that has caused the destruction initially to protect the graft and, subsequently, the patient's eye.
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