Cost effectiveness of the type II Boston keratoprosthesis.

PURPOSE: Despite demonstrated cost effectiveness, not all corneal disorders are amenable to type I Boston keratoprosthesis (KPro) implantation. This includes patients with autoimmune diseases, such as Stevens-Johnson syndrome/toxic epidermal necrolysis. Type II KPro is implanted through the eyelids in severe dry eye and cicatricial diseases, and its cost effectiveness was sought. PATIENTS AND METHODS: In a retrospective chart review, 29 patients who underwent type II KPro surgery at the Massachusetts Eye and Ear Infirmary between the years 2000 and 2009 were identified. A total of 11 patients had 5-year follow-up data. Average cost effectiveness was determined by cost-utility analysis, comparing type II KPro surgery with no further intervention. RESULTS: Using the current parameters, the cost utility of KPro from third-party insurer (Medicare) perspective was 63,196 $/quality-adjusted life year . CONCLUSION: Efforts to refer those less likely to benefit from traditional corneal transplantation or type I KPro, for type II KPro surgery, may decrease both patient and societal costs.

The genetics of Adamantiades-Behcet's disease.

Adamanitiades-Behcet's disease (ABD) is a relapsing systemic vasculitis that may involve the eyes, skin, and almost all other organ systems. Current research on the pathogenesis of ABD suggests a genetic basis for the disease, with certain MHC genes such as those coding for HLA-B51 being the most obvious candidates. Environmental factors such as infectious disease are thought to be responsible for triggering an immunological reaction and systemic features of the disease in genetically susceptible individuals. Identification of genes responsible for this susceptibility may lead to more definitive diagnostic tests and new approaches to the management of this potentially blinding condition.

Relative risk for cancer in mucous membrane pemphigoid associated with antibodies to the beta4 integrin subunit.

BACKGROUND: Mucous membrane pemphigoid (MMP) is a mucocutaneous vesiculobullous autoimmune disease characterized by autoantibodies to components of the basement membrane zone (BMZ). Recently, it has been reported that patients with MMP who have autoantibodies to laminin 5, known as anti-epiligrin cicatricial pemphigoid (AECP) have a high incidence of malignancy. OBJECTIVE: The purpose of this study was to determine the association between malignancy and MMP in patients with antibodies to beta4 integrin. METHODS: The incidence of cancer was studied in 79 patients with MMP and/or ocular cicatricial pemphigoid (OCP) who had antibodies to human beta4 integrin subunit. In each patient, the diagnosis was made by histology and confirmed by immunopathology of affected tissues. It was compared to the expected incidence, for age- and gender-matched individuals, in the National Cancer Institute's Surveillance, Epidemiology and End Results (NCISEER) database. RESULTS: Of 79 patients, 3 had cancer. The relative risk (RR) for cancer in patients with MMP and/or OCP, with autoantibodies to human beta4 integrin subunit was 0.29 (95% CI 0.62-8.77). The expected number in the NCISEER database was 10.37. This difference was statistically significant (P < 0.01). CONCLUSION: This incidence of cancer in MMP/OCP patients, with antibodies to human beta4 integrin subunit is considerably lower than expected. Preliminary observations in this and other studies suggest that serological subsets of MMP, based on antigen reactivity, have a different clinical course, prognosis and associations with cancer.