TY - JOUR
T1 - Evaluation of a Case Series of Patients with Generalized Pustular Psoriasis in the United States
AU - Noe, Megan H.
AU - Wan, Marilyn T.
AU - Mostaghimi, Arash
AU - Gelfand, Joel M.
AU - Agnihothri, Ritesh
AU - Armstrong, April W.
AU - Bhutani, Tina
AU - Bridges, Alina
AU - Brownstone, Nicholas
AU - Butt, Melissa
AU - Duffin, Kristina P.Callis
AU - Carr, Christian
AU - Creadore, Andrew
AU - Deniro, Katherine L.
AU - Desai, Sheena
AU - Dominguez, Arturo R.
AU - Duffy, Emily K.
AU - Fairley, Janet A.
AU - Femia, Alisa
AU - Gudjonsson, Johann E.
AU - Kaffenberger, Jessica A.
AU - Katz, Kimberly L.
AU - Le, Stephanie T.
AU - Martinez, Edgar
AU - Maverakis, Emanual
AU - Myers, Bridget
AU - Naik, Haley B.
AU - Nelson, Caroline A.
AU - Ortega-Loayza, Alex G.
AU - Plovanich, Molly E.
AU - Rangel, Lauren K.
AU - Ravi, Vignesh
AU - Reddy, Vidhatha D.
AU - Saleh, Jamal Z.
AU - Kirby, Joslyn S.
AU - Sandhu, Jeena K.
AU - Shakshouk, Hadir
AU - Shields, Bridget E.
AU - Sharif-Sidi, Zakariyah
AU - Smith, Jacob
AU - Steahr, Amanda
AU - Toussi, Atrin
AU - Wanat, Karolyn A.
AU - Wang, Bo
AU - Wei, Brian M.
AU - Weinhammer, Annika
AU - Worswick, Scott D.
AU - Yang, Alexander
N1 - Funding Information:
reported serving as a consultant for Pfizer, hims, Digital Diagnostics, Concert, Lilly, and AbbVie; receiving licensing and royalty fees from Pfizer and Concert unrelated to this work; serving on the medical advisory board for hims and hers; and being an Associate Editor at JAMA Dermatology. Dr Gelfand reported serving as a consultant for Bristol Myers Squibb, Boehringer Ingelheim, GlaxoSmithKline, Janssen Biologics, Novartis Corp, Regeneron, UCB, Sanofi, and Pfizer (grants or honoraria for which were paid to the trustees of the University of Pennsylvania) from AbbVie, Janssen, Novartis Corp, Sanofi, Celgene, OrthoDermatologics, and Pfizer; and payment for continuing medical education that was supported indirectly by Lilly and Ortho Dermatologics; being a co–patent holder of resiquimod; and serving as deputy editor for the Journal of Investigative Dermatology and chief medical editor for Healio Psoriatic Disease. Dr Armstrong reported serving as a research investigator and scientific adviser to AbbVie, Boehringer Ingelheim, Bristol Myers Squibb, Incyte, Leo, UCB, Janssen, Lilly, Novartis, Ortho Dermatologics, Sun, Dermavant, Dermira, Sanofi, Regeneron, Pfizer, and Modmed. Dr Bhutani reported serving as a consultant for Pfizer, AbbVie, Novartis, Arcutis, Leo, Boehringer Ingelheim, and Lilly and being a principal investigator on studies funded by Celgene, Janssen, Pfizer, and Galderma. Dr Duffin reported being an investigator, adviser, or consultant to AbbVie, Amgen, Bristol Myers Squibb, Celgene, Lilly, Janssen, Novartis, Pfizer, Sienna Biopharmaceuticals, Stiefel Laboratories, UCB, Ortho Dermatologics, Regeneron Pharmaceuticals, AnaptysBio, and Boehringer Ingelheim. Dr Fairley reported serving as a consultant for AstraZeneca. Dr Femia reported serving as a consultant for Octagon Therapeutics. Dr Gudjonsson reported serving as a consultant for Novartis, Eli Lilly, Almirall, Bristol Myers Squibb–Celgene, AnaptysBio, Boehringer Ingelheim, AstraZeneca, and Sanofi and receiving research grants from Kyowa-Kirin, Almirall, Eli Lilly, and SunPharma. Dr Kaffenberger reported being the principal investigator for work funded by AbbVie, Celgene, Corrona, Eli Lilly, Janssen, Regeneron, Pfizer, and UCB. Dr Naik reported receiving grant support from AbbVie, consulting fees from 23andme, AbbVie, and DAVA Oncology, and advisory board fees from Boehringer Ingelheim; being an Associate Editor for JAMA Dermatology; and serving as an unpaid board member of the US Hidradenitis Suppurativa Foundation. Dr Ortega-Loayza reported serving on the advisory boards for Janssen and Bristol Myers Squibb. Dr Kirby reported serving as a consultant for AbbVie, ChemoCentryx, Incyte, Janssen, Novartis, and UCB. No other disclosures were reported.
Publisher Copyright:
© 2021 American Medical Association. All rights reserved.
PY - 2022/1
Y1 - 2022/1
N2 - Importance: Generalized pustular psoriasis (GPP) is a chronic, orphan disease with limited epidemiological data. Objective: To describe the clinical characteristics, treatments, longitudinal disease course, and disease-specific health care utilization among patients with GPP across the United States. Design, Setting, and Participants: A retrospective longitudinal case series involving 95 adults who met the European Rare and Severe Psoriasis Expert Network consensus definition for GPP and were treated at 20 US academic dermatology practices between January 1, 2007, and December 31, 2018. Main Outcomes and Measures: The primary outcome is to describe the patient characteristics, associated medical comorbidities, treatment patterns complications, and GPP-specific health care utilization. Results: Sixty-seven of 95 patients (70.5%) were women (mean age, 50.3 years [SD, 16.1 years]). In the initial encounter, 35 patients (36.8%) were hospitalized and 64 (67.4%) were treated with systemic therapies. In total, more than 20 different systemic therapies were tried. During the follow-up period, 19 patients (35.8%) reported hospitalizations at a median rate of 0.5 hospitalizations per year (IQR, 0.4-1.6). Women had a decreased risk of an emergency department or hospital encounter (odds ratio, 0.19; 95% CI, 0.04-0.83). Conclusions and Relevance: Generalized pustular psoriasis is a rare, chronic disease without standard treatment and is associated with continued health care utilization over time.
AB - Importance: Generalized pustular psoriasis (GPP) is a chronic, orphan disease with limited epidemiological data. Objective: To describe the clinical characteristics, treatments, longitudinal disease course, and disease-specific health care utilization among patients with GPP across the United States. Design, Setting, and Participants: A retrospective longitudinal case series involving 95 adults who met the European Rare and Severe Psoriasis Expert Network consensus definition for GPP and were treated at 20 US academic dermatology practices between January 1, 2007, and December 31, 2018. Main Outcomes and Measures: The primary outcome is to describe the patient characteristics, associated medical comorbidities, treatment patterns complications, and GPP-specific health care utilization. Results: Sixty-seven of 95 patients (70.5%) were women (mean age, 50.3 years [SD, 16.1 years]). In the initial encounter, 35 patients (36.8%) were hospitalized and 64 (67.4%) were treated with systemic therapies. In total, more than 20 different systemic therapies were tried. During the follow-up period, 19 patients (35.8%) reported hospitalizations at a median rate of 0.5 hospitalizations per year (IQR, 0.4-1.6). Women had a decreased risk of an emergency department or hospital encounter (odds ratio, 0.19; 95% CI, 0.04-0.83). Conclusions and Relevance: Generalized pustular psoriasis is a rare, chronic disease without standard treatment and is associated with continued health care utilization over time.
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U2 - 10.1001/jamadermatol.2021.4640
DO - 10.1001/jamadermatol.2021.4640
M3 - Article
C2 - 34878491
AN - SCOPUS:85121237930
SN - 2168-6068
VL - 158
SP - 73
EP - 78
JO - JAMA Dermatology
JF - JAMA Dermatology
IS - 1
ER -