Associations of pemphigus or pemphigoid with autoimmune disorders in US adult inpatients.

BACKGROUND: The associations and predictors of the gamut of autoimmune conditions in pemphigus and pemphigoid have been examined in few large-scale controlled studies. OBJECTIVE: To examine associations of pemphigus or pemphigoid with autoimmune disorders and related outcomes in adults. METHODS: Data from the 2002-2012 National Inpatient Sample were analyzed, including an ∼20% sample of all US hospitalizations (n = 72,108,077 adults). RESULTS: In multivariable logistic regression models, pemphigus (adjusted odds ratio 1.46, 95% confidence interval 1.30-1.63) and pemphigoid (adjusted odds ratio 1.35, 95% confidence interval 1.24-1.48) were associated with ≥1 autoimmune disorder. Pemphigus was associated with 9 of 29 and pemphigoid with 13 of 32 autoimmune disorders examined in bivariable models. Among pemphigus inpatients, unspecified autoimmune disease, vitiligo, eosinophilic esophagitis, and myasthenia gravis had the strongest effect sizes. Whereas, among pemphigoid inpatients, unspecified autoimmune disease, vitiligo, and chronic urticaria had the strongest effect sizes. There were significant differences of autoimmune comorbidities by age, sex, and race/ethnicity. The estimated excess annual costs of hospital care attributed to autoimmune disorders among inpatients with pemphigus was $2,286,588 and pemphigoid $4,301,681. LIMITATION: Lack of treatment history data. CONCLUSION: Inpatients with pemphigus or pemphigoid had increased odds of multiple cutaneous, extracutaneous, and systemic autoimmune disorders, which were associated with a considerable cost burden.

FR - Doxiciclina, una alternativa efectiva, bien tolerada y de bajo coste como tratamiento de primera línea del penfigoide ampolloso / Doxycycline, a Well-Tolerated, Economic, and Effective Alternative for the First-Line Treatment of Bullous Pemphigoid

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Doxycycline compared with prednisolone therapy for patients with bullous pemphigoid: cost-effectiveness analysis of the BLISTER trial.

BACKGROUND: Bullous pemphigoid (BP) is an autoimmune blistering skin disorder associated with significant morbidity and mortality. Doxycycline and prednisolone to treat bullous pemphigoid were compared within a randomized controlled trial (RCT). OBJECTIVES: To compare the cost-effectiveness of doxycycline-initiated and prednisolone-initiated treatment for patients with BP. METHODS: Quality-of-life (EuroQoL-5D-3L) and resource data were collected as part of the BLISTER trial: a multicentre, parallel-group, investigator-blinded RCT. Within-trial analysis was performed using bivariate regression of costs and quality-adjusted life-years (QALYs), with multiple imputation of missing data, informing a probabilistic assessment of incremental treatment cost-effectiveness from a health service perspective. RESULTS: In the base case, there was no robust difference in costs or QALYs per patient at 1 year comparing doxycycline- with prednisolone-initiated therapy [net cost £959, 95% confidence interval (CI) -£24 to £1941; net QALYs -0·024, 95% CI -0·088 to 0·041]. However, the findings varied by baseline blister severity. For patients with mild or moderate blistering (≤ 30 blisters) net costs and outcomes were similar. For patients with severe blistering (> 30 blisters) net costs were higher (£2558, 95% CI -£82 to £5198) and quality of life poorer (-0·090 QALYs, 95% CI -0·22 to 0·042) for patients starting on doxycycline. The probability that doxycycline would be cost-effective for those with severe pemphigoid was 1·5% at a willingness to pay of £20 000 per QALY. CONCLUSIONS: Consistently with the clinical findings of the BLISTER trial, patients with mild or moderate blistering should receive treatment guided by the safety and effectiveness of the drugs and patient preference - neither strategy is clearly a preferred use of National Health Service resources. However, prednisolone-initiated treatment may be more cost-effective for patients with severe blistering.

The Inpatient Burden of Autoimmune Blistering Disease in US Children: Analysis of Nationwide Inpatient Sample Data.

BACKGROUND: Little is known about the epidemiology of pediatric autoimmune blistering disorders (PAIBD). OBJECTIVE: We sought to determine the inpatient burden and comorbidities of PAIBD. METHODS: We analyzed data from the Nationwide Inpatient Sample from 2002 to 2012, which contained a representative 20% sample of all US hospitalizations. RESULTS: The most common PAIBD with a primary admission was pemphigus (8.0 per million), whereas the most common secondary diagnosis of PAIBD was dermatitis herpetiformis (DH; 9.6 per million). Bullous pemphigoid (BP) was inversely associated with being female and having government or no insurance but positively associated with Black and Hispanic race/ethnicity and more chronic conditions. Pemphigus was associated with being female, Hispanic, having government or no insurance, and having a higher number of chronic conditions. DH was inversely associated with non-White race but positively associated with having government insurance and more chronic conditions. BP was associated with dialysis, hypertension, and diabetes. Pemphigus was associated with osteoarthritis, renal failure, hypothyroidism, and weight loss. DH was associated with herpes simplex virus infection, rheumatoid arthritis, and fungal, viral, and other skin infections. CONCLUSION: PAIBD are associated with a considerable inpatient burden and comorbid health conditions.

Hospitalization, inpatient burden and comorbidities associated with bullous pemphigoid in the U.S.A.

BACKGROUND: Bullous pemphigoid (BP) is associated with significant disability and comorbid health disorders that may lead to or result from hospitalization. However, little is known about the inpatient burden and comorbidities of BP. OBJECTIVES: To obtain data on the inpatient burden and comorbidities of BP in the U.S.A. METHODS: We analysed data from the 2002 to 2012 National Inpatient Sample, including a representative 20% sample of all hospitalizations in the U.S.A. (72 108 077 adults). RESULTS: The prevalence of hospitalization for BP increased from 25·84 to 32·60 cases per million inpatients from 2002 to 2012. In multivariate logistic regression models with stepwise selection, increasing age, nonwhite ethnicity, higher median household income, being insured with Medicare or Medicaid, and increasing number of chronic conditions were all associated with hospitalization for BP (P < 0·05 for all). The top three primary discharge diagnoses for patients with a secondary diagnosis of BP were septicaemia (prevalence 5·51%, 95% confidence interval 5·03-5·99), pneumonia (4·60%, 4·19-5·01) and urinary tract infection (3·52%, 3·15-3·89). Patients with BP also had numerous autoimmune, infectious, cardiovascular and other comorbidities. Interestingly, BP was associated with multiple neuropsychiatric disorders, including demyelinating disorders, dementias (presenile, senile, vascular and other), paralysis, neuropathy (diabetic, other polyneuropathy), Parkinson disease, epilepsy, psychoses and depression. The mean annual age- and sex-adjusted in-hospital mortality rate was significantly higher in patients with a secondary diagnosis of BP compared with no BP (2·9%, range 2·8-3·9% vs. 2·1%, range 1·9-2·2%). Significant predictors of mortality in patients with BP included increasing age, nonwhite ethnicity and insurance with Medicaid or other payment status (P < 0·05 for all). CONCLUSIONS: Hospitalization for BP increased significantly between 2002 and 2012. Moreover, there were significant ethnic and healthcare disparities with respect to hospitalization and inpatient mortality from BP.

Cost and Resource Use of Pemphigus and Pemphigoid Disorders Pre- and Post-Rituximab.

BACKGROUND: Rituximab (RTX) is increasingly used for the treatment of pemphigus and pemphigoid disorders. The high cost of RTX frequently limits its use and access. OBJECTIVE: To determine the health system resources and costs associated with RTX treatment of pemphigus and pemphigoid. METHODS: Health system resources and costs attributed to a convenience sample of 89 patients with either pemphigus or pemphigoid were identified, quantified, and valued 6 months prior to and following RTX initiation between May 2006 and August 2012. Overall cohort costs and costs per patient were calculated (2013 Can$). RESULTS: The overall cohort cost for 6 months pre-RTX was $3.8 million and for 6 months post-RTX was $2.6 million. The average cost per patient decreased from $42,231 to $29,423 (30.3% decrease). The main cost driver was intravenous immunoglobulin. CONCLUSIONS: Our findings suggest that RTX is effective in reducing health system resources and the costs associated with the treatment of pemphigus and pemphigoid.

Comparison of cost of immune globulin intravenous therapy to conventional immunosuppressive therapy in treating patients with autoimmune mucocutaneous blistering diseases.

Autoimmune mucocutaneous blistering diseases (AMBD) are a group of potentially fatal diseases that affect the skin and mucous membranes. AMBD have different target antigens as well as variable clinical presentation, course, and prognosis. The mainstay of conventional immunosuppressive therapy (CIST) for AMBD is long-term high-dose systemic corticosteroids and immunosuppressive agents. Such therapy has proven effective in many patients; however, in some patients, the disease continues to progress with significant sequelae such as blindness, loss of voice, anal, and vaginal stenosis which causes poor quality of life. Furthermore, the CIST may have some serious side effects including opportunistic infections which may cause death. Immune globulin intravenous (IGIV) therapy has been reportedly used in the management of patients with AMBD refractory to CIST. IGIV has shown to be more clinically beneficial than CIST by bringing about long-term clinical remission and less recurrence. The high cost of the IGIV is of concern to patients, physicians, and insurance companies. In this report, we compare the cost of IGIV to that of CIST in treating a cohort of 15 mucous membrane pemphigoid (MMP), 10 ocular cicatricial pemphigoid (OCP), 15 bullous pemphigoid (BP), and 32 pemphigus vulgaris (PV) patients. In each cohort of patients, CIST had significant side effects, many of which were hazardous and required prolonged and frequent hospitalizations. Some of these side effects were severe enough to require discontinuation of the treatment. We consider the total cost of CIST to be the actual cost of the drug, plus the cost of management of the side effects produced by CIST. In the same patient cohort, no significant side effects to IGIV were observed. None of the IGIV treated patients required physician visits, laboratory tests, or hospitalizations specifically related to IGIV therapy. Hence, the total cost of the IGIV therapy is the actual cost of the IGIV only. The mean total cost of treatment of IGIV therapy is statistically significantly less than that of CIST during the entire course of the disease and on an annual basis. In conclusion, IGIV therapy is a safe, clinically beneficial, and a cost effective alternative treatment in patients with AMBD, non-responsive to CIST.