rash with sterile neutrophil-filled pustules. By understanding the burden of disease in this population, targeted interventions that improve patient quality of life can be developed. This study describes HCRU in patients with GPP. Methods: Patients were identified as having GPP if they had $1 inpatient or 2 outpatient ICD-10 L40.1 diagnosis codes, separated by 30 to 365 days. All analyses were conducted via the Aetion Evidence Platform TM v3.17, using Optum® Clinformatics TM Data Mart, a US administrative claims database. The study period was from October 1, 2015 to March 31, 2019, with the first diagnosis code marking the index date. A general population matched cohort (MC) of 4:1, based on age and sex, was generated for context. No formal comparisons were conducted. Patient characteristics, treatment, and all-cause HCRU calculated for each visit type (inpatient, outpatient, and emergency department [ED]) during the 12-month follow up (FU) were analyzed. Results: 1669 patients with GPP were identified at baseline, and 1014 had $12 months' FU. Compared with the MC, patients with GPP were more likely to suffer from psoriatic arthritis (GPP: 11.8% vs MC: 0.1%), anxiety (GPP: 11.6% vs MC: 5.8%), and depression (GPP: 10.7% vs MC: 5.0%) at baseline. During the 12-month FU, 449 (44.3%) were treated with a systemic therapy (biologic or non-biologic). Compared with the MC, patients with GPP had more outpatient visits (median: 21 vs 10), a greater frequency of inpatient (22.5% vs 10.4%) and ED (40.5% vs 23.0%) visits, and were hospitalized for longer (median: 8 vs 5 days) during the 12-month FU. Conclusions: This analysis suggests that patients with GPP have more HCRU than those in the MC, highlighting an unmet need among patients with GPP.

Objectives: To study the predictors of absenteeism in adult patients treated for moderate-to-severe atopic dermatitis in Portugal. Methods: Data were drawn from a cross-sectional study conducted in community pharmacies designed to characterize the adult population being treated for moderate-to-severe atopic dermatitis (AD) in Portugal and AD related absenteeism. The questionnaire was self-administered but the data on AD medications was provided by pharmacists. A zero-inflated negative binomial model was used to estimate the predictors of absenteeism related to AD. Results: From the 622 eligible patients, 66.2% were employed, from which 81.8% reported not having missed work due to AD in the previous 6 months. The mean number of absenteeism days was 0.88, with a standard deviation of 3.88. Results of the zeroinflated negative binomial model reveal that patients followed in private setting and patients followed by general practitioners had a higher chance of not missing work. The second part of the model indicates that patients with higher levels of embarrassment and patients on systemic therapy have more days of absenteeism due to AD. Combining both models, we estimated that patients with high levels of embarrassment miss on average 1.46 (CI 90%: 0.41-2.51) days more than patients that don't report any level of embarrassment. Patients who are on systemic therapy miss on average 0.77 (CI 90%: 0.11-1.44) days more than those that are not on systemic therapy. Conclusions: AD is a disease that affects daily life, resulting in productivity losses of patients. The results indicate that the level of embarrassment and the type of medication are predictors of the absenteeism in patients with moderate-to-severe AD.

Background Atypical hemolytic uremic syndrome is a rare disease caused by complement dysregulation that can lead to progressive kidney damage or death if untreated. Owing to its rarity, the impact of atypical hemolytic uremic syndrome and available therapies (eculizumab and ravulizumab) on patients' health-related quality of life is difficult to describe, but such data are required for an economic evaluation. Objective The objective of this study was to estimate utility values for atypical hemolytic uremic syndrome-related attributes in five countries for an economic evaluation. Methods Using discrete choice experiment surveys, key atypical hemolytic uremic syndrome-related attributes (life expectancy, administration frequency, risk of meningitis, need for hospitalization, and risk of kidney impairment) were evaluated in adult general population samples from Australia, Canada, the Netherlands, Sweden, and the UK. Survey choice sets were constructed using a published orthogonal array. A mixed-effects logit model estimated preference strength for each attribute. Utilities were estimated using marginal substitution rates between overall survival and other attributes, weighted against average life expectancy. Results Across all countries (N = 2382), utility weights revealed a consistent pattern: participants were averse to the risk of kidney impairment (disutility/utility weight range: −0.185 to −0.158), risk of meningitis (−0.041 to −0.032), and the need for hospitalization (−0.063 to −0.048), but preferred 8-weekly vs 2-weekly infusions over 1 h (0.013-0.039). Conclusions Although all attributes played a role in determining treatment preferences, the largest drivers were life expectancy and risk of kidney impairment. Participants favored 8-weekly dosing (corresponding to ravulizumab administration frequency) vs 2-weekly dosing. The discrete choice experiment was designed such that estimated (dis)utility weights can be used in future cost-effectiveness models in atypical hemolytic uremic syndrome.

Introduction Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy (TMA) often caused by alternative complement dysregulation. Patients with aHUS can present with malignant hypertension (MHT), which may also cause TMA. Methods This analysis of the Global aHUS Registry (NCT01522183) assessed demographics and clinical characteristics in eculizumab-treated and not-treated patients with aHUS, with (n = 71) and without (n = 1026) malignant hypertension, to further elucidate the potential relationship between aHUS and malignant hypertension. Results While demographics were similar, patients with aHUS + malignant hypertension had an increased need for renal replacement therapy, including kidney transplantation (47% vs 32%), and more pathogenic variants/anti-complement factor H antibodies (56% vs 37%) than those without malignant hypertension. Not-treated patients with malignant hypertension had the highest incidence of variants/antibodies (65%) and a greater need for kidney transplantation than treated patients with malignant hypertension (65% vs none). In a multivariate analysis, the risk of end-stage kidney disease or death was similar between not-treated patients irrespective of malignant hypertension and was significantly reduced in treated vs not-treated patients with aHUS + malignant hypertension (adjusted HR (95% CI), 0.11 [0.01–0.87], P = 0.036). Conclusions These results confirm the high severity and poor prognosis of untreated aHUS and suggest that eculizumab is effective in patients with aHUS ± malignant hypertension. Furthermore, these data highlight the importance of accurate, timely diagnosis and treatment in these populations and support consideration of aHUS in patients with malignant hypertension and TMA. Trial registration details Atypical Hemolytic-Uremic Syndrome (aHUS) Registry. Registry number: NCT01522183 (first listed 31st January, 2012; start date 30th April, 2012). Graphical abstract