Wu, Ruizhe

Abstract Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by life-threatening infections and inflammatory conditions. Hematopoietic cell transplantation (HCT) is the definitive treatment for CGD, but questions remain regarding patient selection and impact of active disease on transplant outcomes. We performed a multi-institutional retrospective and prospective study of 391 patients with CGD treated either conventionally (non-HCT) enrolled from 2004 to 2018 or with HCT from 1996 to 2018. Median follow-up after HCT was 3.7 years with a 3-year overall survival of 82% and event-free survival of 69%. In a multivariate analysis, a Lansky/Karnofsky score <90 and use of HLA-mismatched donors negatively affected survival. Age, genotype, and oxidase status did not affect outcomes. Before HCT, patients had higher infection density, higher frequency of noninfectious lung and liver diseases, and more steroid use than conventionally treated patients; however, these issues did not adversely affect HCT survival. Presence of pre-HCT inflammatory conditions was associated with chronic graft-versus-host disease. Graft failure or receipt of a second HCT occurred in 17.6% of the patients and was associated with melphalan-based conditioning and/or early mixed chimerism. At 3 to 5 years after HCT, patients had improved growth and nutrition, resolved infections and inflammatory disease, and lower rates of antimicrobial prophylaxis or corticosteroid use compared with both their baseline and those of conventionally treated patients. HCT leads to durable resolution of CGD symptoms and lowers the burden of the disease. Patients with active infection or inflammation are candidates for transplants; HCT should be considered before the development of comorbidities that could affect performance status. This trial was registered at www.clinicaltrials.gov as #NCT02082353.

Abstract Background African Americans face a disproportionate incidence and prevalence of hidradenitis suppurativa (HS) in the United States, but HS severity and outcomes across racial and ethnic groups have not been well‐established while controlling for potentially confounding factors. In this retrospective cohort study, we investigated the associations of race and ethnicity with HS severity, emergency department (ED) visits, hospitalizations, and surgeries for HS while controlling for age, sex, body mass index (BMI), tobacco use, and insurance type. Methods We reviewed 1190 patients seen at the Medical College of Wisconsin with ≥3 encounters for HS between 1/1/2002 and 3/19/2019, excluding those without race data or an encounter in which HS was treated. Results A total of 953 patients were included; 470 patients were Black or African American non‐Hispanic (49%), 39 Hispanic (4%), 418 White non‐Hispanic (44%), and 26 other race or ethnicity (3%). Controlling for age, sex, BMI, tobacco use, and insurance type, Black patients had 2.8 times the odds of having Hurley stage III disease (95% CI 1.76–4.45, P < 0.001), 2.86 times the risk for experiencing an ED visit for HS (95% CI 2.12–3.88, P < 0.001), 2.25 times the risk for experiencing a hospitalization for HS (95% CI 1.42–3.56, P < 0.001), and 1.61 times the risk for experiencing a surgical encounter for HS (95% CI 1.34–1.95, P < 0.001) when compared to White patients. Conclusions African Americans face significant disparities in HS severity, ED visits, hospitalizations, and surgeries. The causes of these disparities must be further investigated and addressed.