Article
Allergy
Patrick Baum, Sudha Visvanathan, Sandra Garcet, Janine Roy, Ramona Schmid, Sebastian Bossert, Benjamin Lang, Herve Bachelez, Robert Bissonnette, Christian Thoma, James G. Krueger
Summary: This study revealed that the IL-36 pathway plays a significant role in the pathogenesis of generalized pustular psoriasis. Treatment with spesolimab, an anti-IL-36 receptor antibody, resulted in rapid improvement of skin and pustular symptoms in patients with GPP. The treatment also modulated dysregulated molecular pathways associated with GPP and PPP, leading to improved clinical outcomes.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
(2022)
Review
Dermatology
Nilesh Kodali, Isabella Blanchard, Sruthi Kunamneni, Mark G. G. Lebwohl
Summary: Generalized pustular psoriasis (GPP) is a rare subset of psoriasis characterized by sterile pustules, inflammation, and systemic involvement. The IL-1/IL-36 inflammatory pathway plays a critical role in GPP's pathology, with associated genetic mutations including modifications of IL36RN, CARD14, AP1S3, MPO, and SERPINA3 genes. Treatment guidelines for GPP are not well-established, but options include IL-36Ra spesolimab and other biologic therapies, as well as non-biologic options such as retinoids, cyclosporine, and methotrexate. Treatment options differ by country, with Japan having approved biologic therapies specifically for GPP. This review provides an update on the global management of GPP and covers various aspects of the disease.
EXPERIMENTAL DERMATOLOGY
(2023)
Review
Dermatology
Hideki Fujita, Melinda Gooderham, Ricardo Romiti
Summary: Generalized pustular psoriasis is a rare and severe skin disease that poses challenges in diagnosis and treatment. Despite the lack of standardized international guidelines, recent advances in understanding the genetics and pathogenesis of the disease have provided new opportunities for improved diagnosis.
AMERICAN JOURNAL OF CLINICAL DERMATOLOGY
(2022)
Review
Dermatology
Slaheddine Marrakchi, Lluis Puig
Summary: Generalized pustular psoriasis (GPP) is a rare and severe form of the disease, distinct from plaque psoriasis. Dysregulation of the IL-36 signaling pathway appears central to the pathogenesis of GPP. Treatment targeting the IL-36 inflammatory cascade shows promise, although specific therapeutic agents for GPP are currently lacking.
AMERICAN JOURNAL OF CLINICAL DERMATOLOGY
(2022)
Review
Dermatology
Kelly Z. Young, Mrinal K. Sarkar, Johann E. Gudjonsson
Summary: Psoriasis is a chronic, immune-mediated skin disease that affects over 3% of adults in the United States. Generalized pustular psoriasis is an acute, severe form associated with increased morbidity and mortality. Recent advances in genetic and immunological studies highlight the importance of the IL-36 immune axis in the pathogenesis of generalized pustular psoriasis.
EXPERIMENTAL DERMATOLOGY
(2023)
Article
Dermatology
A. David Burden, Robert Bissonnette, Mark G. Lebwohl, Tristan Gloede, Milena Anatchkova, Ismail Budhiarso, Na Hu, Christian Thoma, Anne M. Skalicky, Herve Bachelez
Summary: This study evaluated the reliability, validity, and responder definitions of the Generalized Pustular Psoriasis Physician Global Assessment (GPPGA) and Generalized Pustular Psoriasis Area and Severity Index (GPPASI). The results showed that GPPGA and GPPASI are valid, reliable, and responsive measures for assessing the severity of GPP.
JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY
(2023)
Article
Genetics & Heredity
Piranit Kantaputra, Teerada Daroontum, Mati Chuamanochan, Suteeraporn Chaowattanapanit, Salin Kiratikanon, Charoen Choonhakarn, Worrachet Intachai, Bjorn Olsen, Sissades Tongsima, Chumpol Ngamphiw, Patrizia Pontisso, Timothy C. Cox, Puey Ounjai
Summary: This study found that genetic variants in the SERPINB3 gene are associated with generalized pustular psoriasis (GPP) and adult-onset immunodeficiency (AOID) with pustular skin reaction. The skin of patients with GPP and AOID carrying SERPINB3 mutations showed overexpression of SERPINB3 and SERPINA1, suggesting similar pathogenetic mechanisms between GPP and AOID.
Review
Dermatology
A. David Burden, Siew Eng Choon, Alice B. Gottlieb, Alexander A. Navarini, Richard B. Warren
Summary: Generalized pustular psoriasis (GPP) is a rare neutrophilic skin condition with challenges in accurate assessment and treatment, yet adapting psoriasis disease measures and developing assessment tools specific for GPP severity can lead to more effective monitoring and treatment of GPP patients.
AMERICAN JOURNAL OF CLINICAL DERMATOLOGY
(2022)
Review
Biochemistry & Molecular Biology
Dominik Samotij, Justyna Szczech, Adam Reich
Summary: Generalized pustular psoriasis (GPP) is a severe immune-mediated disease characterized by sterile pustules all over the body. The genetic background and immunological disturbances play key roles in its pathogenesis, with IL-36 considered a central cytokine. Novel anti-IL-36R antibodies show promise for revolutionizing GPP management.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Dermatology
Jen-Ping Chao, Tsen-Fang Tsai
Summary: Elderly-onset generalized pustular psoriasis (GPP) is rare and has special clinical phenotypes. This study reports seven cases of elderly-onset GPP, showing atypical clinical features and a lower frequency of IL36RN gene mutation. IL36RN mutations may have a significant, dose-dependent effect on the age of onset for GPP.
CLINICAL AND EXPERIMENTAL DERMATOLOGY
(2022)
Review
Dermatology
Siew Eng Choon, Alexander A. Navarini, Andreas Pinter
Summary: Generalized pustular psoriasis (GPP) is a rare and potentially dangerous disease characterized by the appearance of large sterile pustules in widespread areas, with or without systemic inflammation and/or plaque psoriasis. The severity and frequency of flares vary among individuals, and severe flares are often accompanied by systemic symptoms and other extracutaneous manifestations. The clinical course of GPP is highly variable.
AMERICAN JOURNAL OF CLINICAL DERMATOLOGY
(2022)
Article
Dermatology
Rhea Malik, Jeffrey Chen, Charles Lau, Aaron Sandoval, Vinod E. Nambudiri
Summary: This paper is the first study to evaluate the quality, comprehensiveness, and readability of online health information for generalized pustular psoriasis (GPP). The findings show significant gaps in the comprehensiveness and reliability of GPP-specific online health information, with only a small percentage of websites meeting HONcode accreditation and poor readability levels.
EXPERIMENTAL DERMATOLOGY
(2023)
Letter
Dermatology
Patricia Perez-Feal, Noelia Moreiras-Arias, Ana Vega, Jose Manuel Suarez-Penaranda, Maria Monsterrat Lopez-Franco, Isabel Rodriguez-Blanco, Dolores Sanchez-Aguilar, Igor Vazquez-Osorio
Summary: We investigated the sun-protection habits of 61 male individuals who attended a dermatology department in western Ireland. The majority of them had outdoor occupations, either currently or in the past. While most of them wore hats for sun protection, a large number chose baseball caps, which do not offer sufficient coverage for the head and neck. Although SPF products were commonly used, they were not consistently used.
CLINICAL AND EXPERIMENTAL DERMATOLOGY
(2023)
Article
Dermatology
Piranit Kantaputra, Suteeraporn Chaowattanapanit, Salin Kiratikanon, Romanee Chaiwarith, Chareon Choonhakarn, Worrachet Intachai, Natalina Quarto, Sissades Tongsima, James R. Ketudat Cairns, Chumpol Ngamphiw, John A. McGrath, Mati Chuamanochan
Summary: Adult-onset immunodeficiency syndrome (AOID) and Generalized pustular psoriasis (GPP) may share genetic variations that lead to similar immunodeficiency and skin reactions. Variants in SERPINA family members could be associated with the etiology of both AOID and GPP.
JOURNAL OF DERMATOLOGY
(2021)
Review
Medicine, Research & Experimental
Jiahong Zhou, Qing Luo, Yang Cheng, Xia Wen, Jinbo Liu
Summary: Generalized pustular psoriasis is a rare and severe inflammatory skin disease characterized by recurrent pustular eruptions. Recent studies have identified genetic variants associated with the disease, and biological agents targeting the IL-36 pathway show promise as potential treatments.
INTERNATIONAL JOURNAL OF MOLECULAR MEDICINE
(2021)
Article
Immunology
Rena C. Moon, Rachel H. Mackey, Zhun Cao, Seth Emont, Laura L. Schott, Julie Gayle, Craig Lipkin, Ning Rosenthal
Summary: Following an initial decline in adjusted in-hospital mortality for COVID-19 inpatients from April to June 2020, there were two subsequent peaks in mortality rates, with differences observed across age groups from November 2020 to February 2021.
CLINICAL INFECTIOUS DISEASES
(2022)
Article
Dermatology
Bruce Strober, Joyce Leman, Maja Mockenhaupt, Juliana Nakano de Melo, Ahmed Nassar, Vimal H. Prajapati, Paolo Romanelli, Julien Seneschal, Athanasios Tsianakas, Lee Yoong Wei, Masahito Yasuda, Ning Yu, Ana C. Hernandez Daly, Yukari Okubo
Summary: GPP is a rare and potentially life-threatening skin disease characterized by recurrent flares of generalized sterile pustules. Challenges in GPP management include inconsistent diagnostic criteria and a lack of approved therapies. Healthcare providers, especially non-specialists, often lack sufficient knowledge and experience in diagnosing and treating GPP.
DERMATOLOGY AND THERAPY
(2022)
Article
Hematology
Anna E. Bortnick, Petra Buzkova, James Otvos, Majken Jensen, Michael Y. Tsai, Matthew Budoff, Rachel Mackey, Samar R. El Khoudary, Elda Favari, Ryung S. Kim, Carlos J. Rodriguez, George Thanassoulis, Jorge R. Kizer
Summary: This study found that high-density lipoprotein (HDL) cholesterol, HDL particle number and size, apoC3-defined HDL subtypes were inversely associated with the long-term incidence and progression of aortic valve calcification (AVC). Further investigation of HDL composition and mechanisms could be useful in understanding pathways that slow AVC.
ARTERIOSCLEROSIS THROMBOSIS AND VASCULAR BIOLOGY
(2022)
Article
Urology & Nephrology
Jay L. Koyner, Rachel H. Mackey, Ning A. Rosenthal, Leslie Ann Carabuena, J. Patrick Kampf, Toni Rodriguez, Aarti Sanghani, Jorge Echeverri, Paul McPherson, Michael J. Blackowicz, Julien Textoris
Summary: A study on inpatients with stage 2 or 3 AKI found a high burden of persistent severe AKI (PS-AKI) and its association with adverse clinical outcomes, with similar results for ICU and non-ICU patients. Interventions to prevent persistence of severe AKI may improve clinical outcomes for these patients.
AMERICAN JOURNAL OF NEPHROLOGY
(2023)
Article
Dermatology
Georgios Kokolakis, Richard B. Warren, Bruce Strober, Andrew Blauvelt, Luis Puig, Akimichi Morita, Melinda Gooderham, Andreas Koerber, Veerle Vanvoorden, Maggie Wang, Dirk de Cuyper, Cynthia Madden, Natalie Nunez Gomez, Mark Lebwohl
Summary: This study evaluated the efficacy and safety of switching to bimekizumab from adalimumab, ustekinumab, and secukinumab. The results showed rapid and durable improvements in clinical responses among nonresponders who switched to bimekizumab. Furthermore, the majority of patients who responded well to previous treatments maintained or improved their response after switching to bimekizumab. Overall, switching to bimekizumab was found to be highly effective and well tolerated for psoriasis patients.
BRITISH JOURNAL OF DERMATOLOGY
(2023)
Article
Dermatology
Diamant Thaci, Ron Vender, Menno A. de Rie, Curdin Conrad, David M. Pariser, Bruce Strober, Veerle Vanvoorden, Maggie Wang, Cynthia Madden, Dirk de Cuyper, Alexa B. Kimball
Summary: The clinical and health-related quality of life responses observed during the first 16 weeks of BE SURE were sustained over 2 years of treatment, regardless of bimekizumab maintenance dosing. Switching from adalimumab to bimekizumab also resulted in sustained improvements up to Week 104. Bimekizumab had a good safety profile with no new safety signals.
BRITISH JOURNAL OF DERMATOLOGY
(2023)
Article
Dermatology
Jonathan I. I. Silverberg, Lars E. E. French, Richard B. B. Warren, Bruce Strober, Kim Kjoller, Morten O. A. Sommer, Philippe Andres, Jakob Felding, Anne Weiss, Deniz Tutkunkardas, Tine Skak-Nielsen, Emma Guttman
Summary: This study examined the PDE4 enzymatic activity and anti-inflammatory effects of orismilast in vitro, ex vivo, and in vivo. The results showed that orismilast selectively and potently inhibited PDE4 and demonstrated broad-spectrum anti-inflammatory activity. These findings support the clinical development of oral orismilast as a novel treatment option for chronic inflammatory skin diseases.
JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY
(2023)
Article
Dermatology
Richard B. B. Warren, Bruce Strober, Jonathan I. I. Silverberg, Emma Guttman, Philippe Andres, Jakob Felding, Deniz Tutkunkardas, Kim Kjoller, Morten O. A. Sommer, Lars E. E. French
Summary: This study aimed to examine the efficacy and safety of orismilast for psoriasis treatment. The phase 2a trial found that orismilast significantly improved the condition of patients with psoriasis. The phase 1 trial tested new formulations to minimize gastrointestinal adverse reactions.
JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY
(2023)
Article
Dermatology
Bruce Strober, Yayoi Tada, Ulrich Mrowietz, Mark Lebwohl, Peter Foley, Richard G. Langley, Richard B. Warren, Maggie Wang, Veerle Vanvoorden, Balint Szilagyi, Valerie Ciaravino, Carle Paul
Summary: It is important to evaluate the long-term efficacy of new treatments for psoriasis. This study found that the majority of patients who responded to bimekizumab treatment at Week 16 maintained a high level of clinical response after 3 years of treatment. These findings demonstrate the long-term effectiveness of bimekizumab in patients with moderate-to-severe plaque psoriasis and its important benefits for health-related quality of life.
BRITISH JOURNAL OF DERMATOLOGY
(2023)
Article
Dermatology
Kristian Reich, Jonathan I. Silverberg, Kim A. Papp, Mette Deleuran, Norito Katoh, Bruce Strober, Lisa A. Beck, Marjolein de Bruin-Weller, Thomas Werfel, Fan Zhang, Pinaki Biswas, Marco D. DiBonaventura, Gary Chan, Susan Johnson, Saleem A. Farooqui, Urs Kerkmann, Claire Clibborn
Summary: This study evaluated the efficacy and safety of long-term abrocitinib treatment for patients with moderate-to-severe atopic dermatitis (AD) for up to 48 weeks. The results showed that abrocitinib treatment significantly improved skin symptoms and pruritus, with a manageable safety profile. This has important clinical implications for long-term treatment of chronic AD.
JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY
(2023)
Article
Dermatology
K. Reich, J. I. Silverberg, K. A. Papp, M. Deleuran, N. Katoh, B. Strober, L. A. Beck, M. de Bruin-Weller, T. Werfel, F. Zhang, P. Biswas, M. D. DiBonaventura, G. Chan, S. A. Farooqui, U. Kerkmann, C. Clibborn
Summary: Long-term treatment with abrocitinib significantly improves symptoms and quality of life in patients with moderate-to-severe atopic dermatitis.
JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY
(2023)
Article
Economics
Jay L. Koyner, Rachel H. Mackey, Ning A. Rosenthal, Leslie A. Carabuena, J. Patrick Kampf, Paul McPherson, Toni Rodriguez, Aarti Sanghani, Julien Textoris
Summary: In hospitalized patients with COVID-19, acute kidney injury (AKI) is associated with higher mortality. This study analyzed the healthcare resource utilization (HRU) and costs related to AKI, community-acquired AKI (CA-AKI), and hospital-acquired AKI (HA-AKI). The findings showed that HA-AKI had higher excess mortality, HRU, and costs than CA-AKI, emphasizing the importance of preventing HA-AKI in COVID-19 patients.
JOURNAL OF HEALTH ECONOMICS AND OUTCOMES RESEARCH
(2023)
Article
Urology & Nephrology
Jay L. Koyner, Rachel H. Mackey, Ning A. Rosenthal, Leslie A. Carabuena, J. Patrick Kampf, Jorge Echeverri, Paul McPherson, Michael J. Blackowicz, Toni Rodriguez, Aarti R. Sanghani, Julien Textoris
Summary: This retrospective observational study compares the costs and healthcare resource utilization of patients with persistent severe acute kidney injury (PS-AKI) and patients with non-persistent severe AKI (NPS-AKI). The study finds that patients with PS-AKI have longer hospital stays and higher costs compared to those with NPS-AKI. Preventing PS-AKI may help reduce hospital stays and costs.
Article
Rheumatology
Lars E. Kristensen, Bruce Strober, Denis Poddubnyy, Ying-Ying Leung, Hyejin Jo, Kenneth Kwok, Ivana Vranic, Dona L. Fleishaker, Lara Fallon, Arne Yndestad, Dafna D. Gladman
Summary: This study analyzed the association between baseline cardiovascular disease risk and the occurrence of major adverse cardiovascular events and malignancies in tofacitinib-treated patients with PsA and PsO. The results showed that patients with increased ASCVD risk and baseline metabolic syndrome had higher incidence rates for MACE and malignancies. It highlights the importance of assessing CV risk and enhancing cancer monitoring in patients with PsA and PsO.
THERAPEUTIC ADVANCES IN MUSCULOSKELETAL DISEASE
(2023)
Article
Gastroenterology & Hepatology
Raymond K. Cross, April N. Naegeli, Ryan W. Harrison, Page C. Moore, Rachel H. Mackey, Margaux M. Crabtree, Celeste A. Lemay, Vipin Arora, Nathan Morris, Angelina Sontag, Cem Kayhan, Joshua R. Korzenik
Summary: Among UC patients in a real-world setting, patients treated with biologics/JAKi had less remission, more moderate/severe disease, and worse patient-reported outcomes than those on 5-ASAs.
CROHNS & COLITIS 360
(2022)