Review
Dermatology
Chiamaka L. Okorie, Krithika Nayudu, Vinod E. Nambudiri
Summary: Deficiency of the interleukin-36 receptor antagonist (DITRA) is a rare autoinflammatory disorder caused by mutations in the IL36RN gene, resulting in an overactive immune system and chronic inflammation. Recognition and management of DITRA are crucial, and early identification of cutaneous signs is important for timely treatment. This review provides a comprehensive overview of the manifestations and treatments of DITRA.
EXPERIMENTAL DERMATOLOGY
(2023)
Article
Immunology
Philippe Guillem, Dillon Mintoff, Mariam Kabbani, Elie Cogan, Virginie Vlaeminck-Guillem, Agnes Duquesne, Farida Benhadou
Summary: Hidradenitis Suppurativa (HS) is a chronic suppurative disease of the pilosebaceous unit characterized by hyperkeratinization and inflammation. Environmental factors, genetic predisposition, gender, and skin dysbiosis are considered the main pathogenic drivers. Rare autoinflammatory syndromes associated with HS may shed light on the potential roles of autoinflammation and dysregulated innate immune system in HS.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Medicine, General & Internal
Mohammad Abduljawad, Thamer H. Alsharif, Amin G. Gronfula, Talah K. Magadmi, Lujain I. Khayat, Sarah M. Fageeh, Abdulqader A. Almuallim, Mohammad Ayman Mohammad, Abdullah Albadri
Summary: This study reviewed the available literature on the efficacy and safety of anti-IL-17A therapy for PRP. A total of 19 articles involving 77 cases were reviewed, showing that IL-17 inhibitors had a significant impact on the patients. However, higher-level studies are needed to further evaluate the therapeutic and safety effects of this treatment.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2023)
Article
Dermatology
Guo Jing, Wang Bin, Zhang Zhen Ying
Summary: DITRA, caused by mutations of the IL36RN gene, is characterized by severe symptoms such as acute generalized pustular psoriasis, fever, and leukocytosis, but shows a rapid response to secukinumab in some cases.
PEDIATRIC DERMATOLOGY
(2021)
Review
Cell Biology
Eugenio Sangiorgi, Donato Rigante
Summary: Innate immunity is the initial line of defense in humans, abnormalities of which can lead to autoinflammatory diseases (ADs) caused by gene mutations, resulting in recurrent inflammation. Diagnosis of ADs requires a comprehensive approach involving clinical data, inflammation parameters, therapeutic efficacy of drugs, and genotype analysis.
Review
Cell Biology
Moncef Zouali
Summary: Autoimmune diseases are mainly mediated by T and B cells, while auto-inflammatory syndromes involve various immune cells. This article focuses on B lymphocyte subsets and their roles in auto-inflammatory syndromes.
Article
Dermatology
Chang-Yu Hsieh, Yi-Wei Huang, Yi-Hsuan Huang, Tsen-Fang Tsai
Summary: DITRA is a deficiency of interleukin-36 receptor antagonist (IL36RN) that leads to unopposed pro-inflammatory signaling, resulting in pustular psoriasis. The c.115 + 6 T > C mutation is the most common and important single-nucleotide variant in DITRA among Asian patients. This study presents a case series of 58 DITRA patients with heterozygous or homozygous c.115 + 6 T > C mutation. The patients experienced onset of symptoms at a mean age of 20.74 years, with 20.7% having onset before the age of two. The main clinical phenotype observed was generalized pustular psoriasis with systemic symptoms (56.9%).
EXPERIMENTAL DERMATOLOGY
(2023)
Article
Critical Care Medicine
Silvia M. Ardila, Heidi M. Weeks, Mary K. Dahmer, Niko Kaciroti, Michael Quasney, Anil Sapru, Martha A. Q. Curley, Heidi R. Flori
Summary: This study explores the associations between plasma biomarkers and the number and trajectories of nonpulmonary organ dysfunction (NPOD) in the setting of acute respiratory failure (ARF). The findings suggest that these biomarkers and their trajectory patterns may be useful in evaluating the severity of multiple organ dysfunction syndrome in critically ill children and identifying time-sensitive, treatable traits.
PEDIATRIC CRITICAL CARE MEDICINE
(2023)
Article
Rheumatology
Jerold Jeyaratnam, Anna Simon, Inmaculada Calvo, Tamas Constantin, Anna Shcherbina, Michael Hofer, Marco Gattorno, Alberto Martini, Brigitte Bader-Meunier, Bas Vastert, Jeremy Levy, Elise Dekker, Fabrizio de Benedetti, Joost Frenkel
Summary: The study evaluated the long-term efficacy and safety of canakinumab in patients with mevalonate kinase deficiency during the open label extension of the CLUSTER trial. Canakinumab effectively controlled disease activity and prevented flares in these patients over a 72-week period. No new safety concerns were reported, and the majority of patients experienced minimal or no disease activity at the end of the study.
Review
Pediatrics
Rebecca Hetrick, Melissa Oliver
Summary: Autoinflammatory bone disorders are characterized by sterile osteomyelitis. The diseases include chronic nonbacterial osteomyelitis, Majeed syndrome, and deficiency of the interleukin-1 receptor antagonist. These disorders result from dysregulation of the innate immune system and cytokine imbalance, leading to inflammasome activation and excessive bone remodeling. This review provides an overview of the immunopathogenesis of pediatric autoinflammatory bone diseases, focusing on genetics and inborn errors of immunity, and briefly discussing clinical manifestations, management, and areas for future research.
FRONTIERS IN PEDIATRICS
(2023)
Article
Dermatology
M. Oymanns, A. Kreuter, C. Assaf
Summary: This case illustrates the potential benefit of using anakinra in the treatment of pyoderma gangrenosum concurrently with local suppurative disease.
Review
Rheumatology
Jeremie Delaleu, Samuel Deshayes, Francois Rodrigues, Lea Savey, Etienne Riviere, Nicolas Martin Silva, Achille Aouba, Serge Amselem, Marion Rabant, Gilles Grateau, Irina Giurgea, Sophie Georgin-Lavialle
Summary: In most cases, TRAPS is revealed by AA, with AA diagnosed before TRAPS in 96% of cases. A proportion of patients with TRAPS and AA require renal replacement therapy, with a mortality rate of 14%. Biologic treatment for AA shows varied outcomes, with some patients showing improvement in renal function while others worsened or remained stable. Some patients may experience relapse of AA after kidney transplantation.
Article
Rheumatology
Julia Kuehn, Susanne Schleifenbaum, Michaela Hendling, Sandra Siebenhandl, Julie Krainer, Sabrina Fuehner, Antje Hellige, Carolin Park, Claas Hinze, Helmut Wittkowski, Dirk Holzinger, Lorenz Thurner, Andreas Weinhaeusel, Dirk Foell, Christoph Kessel
Summary: This study found that aberrant or incomplete polarization of T helper cells may contribute to the pathological processes in systemic juvenile idiopathic arthritis (sJIA).
ARTHRITIS & RHEUMATOLOGY
(2023)
Article
Immunology
Eleni Karakike, George N. Dalekos, Ioannis Koutsodimitropoulos, Maria Saridaki, Chryssa Pourzitaki, Georgios Papathanakos, Antigone Kotsaki, Stamatios Chalvatzis, Vasiliki Dimakopoulou, Nikolaos Vechlidis, Elisabeth Paramythiotou, Christina Avgoustou, Aikaterini Ioakeimidou, Elli Kouriannidi, Apostolos Komnos, Evangelia Neou, Nikoletta Rovina, Eleni Stefanatou, Haralampos Milionis, George Nikolaidis, Antonia Koutsoukou, Georgia Damoraki, George Dimopoulos, Vassileios Zoumpos, Jesper Eugen-Olsen, Karolina Akinosoglou, Nikolaos K. Gatselis, Vasilios Koulouras, Eleni Gkeka, Nikolaos Markou, Mihai G. Netea, Evangelos J. Giamarellos-Bourboulis
Summary: This study investigated the effect of personalized immunotherapy on critically ill COVID-19 patients. The results showed that anakinra had a favorable clinical improvement in patients with features of MALS. However, the tocilizumab treatment group had a higher incidence of secondary infections.
JOURNAL OF INNATE IMMUNITY
(2022)
Article
Endocrinology & Metabolism
Taghreed A. Ayash, Seline Y. Vancolen, Mariela Segura, Marie-Julie Allard, Guillaume Sebire
Summary: Group B Streptococcus (GBS) infection is a major risk factor for fetal organ injuries, preterm birth, and neurodevelopmental impairments. Blocking interleukin-1 (IL-1) can alleviate the inflammatory response and improve the weight gain of pregnant women.
FRONTIERS IN ENDOCRINOLOGY
(2022)
