Christina Chadwick, Heather Lehman, Shelby Luebbert, Rabheh Abdul-Aziz, Drucy Borowitz
Cystic fibrosis (CF) clinicians may see patients who have difficult-to-manage symptoms that do not have a clear CF-related etiology, such as unusual gastrointestinal (GI) complaints, vasculitis, or arthritis. Alterations in immunity, inflammation and intraluminal dysbiosis create a milieu that may lead to autoimmunity, and the CF transmembrane regulator protein may have a direct role as well. While autoantibodies and other autoimmune markers may develop, these may or may not lead to organ involvement, therefore they are helpful but not sufficient to establish an autoimmune diagnosis...
March 23, 2023: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Yusuke Manabe, Tomohiko Ishibashi, Ryotaro Asano, Shuichi Tonomura, Yuichi Maeda, Daisuke Motooka, Jin Ueda, Masahiro Yanagawa, Yuko Edamoto-Taira, Tomomi Chikaishi-Kirino, Takeshi Masaki, Tadakatsu Inagaki, Shota Nakamura, Yoshinori Katada, Makoto Okazawa, Masashi Narazaki, Takeshi Ogo, Atsushi Kumanogoh, Yoshikazu Nakaoka
BACKGROUND: Takayasu arteritis (TAK) is an autoimmune large vessel vasculitis that affects the aorta and its major branches, eventually leading to the development of aortic aneurysm and vascular stenosis or occlusion. This retrospective and prospective study aimed to investigate whether the gut dysbiosis exists in patients with TAK and to identify specific gut microorganisms related to aortic aneurysm formation/progression in TAK. METHODS: We analysed the faecal microbiome of 76 patients with TAK and 56 healthy controls (HCs) using 16S ribosomal RNA sequencing...
March 24, 2023: Arthritis Research & Therapy
Satoshi Omura, Takashi Kida, Hisashi Noma, Atsuhiko Sunaga, Hiroaki Kusuoka, Masatoshi Kadoya, Daiki Nakagomi, Yoshiyuki Abe, Naoho Takizawa, Atsushi Nomura, Yuji Kukida, Naoya Kondo, Yasuhiko Yamano, Takuya Yanagida, Koji Endo, Shintaro Hirata, Kiyoshi Matsui, Tohru Takeuchi, Kunihiro Ichinose, Masaru Kato, Ryo Yanai, Yusuke Matsuo, Yasuhiro Shimojima, Ryo Nishioka, Ryota Okazaki, Tomoaki Takata, Takafumi Ito, Mayuko Moriyama, Ayuko Takatani, Yoshia Miyawaki, Toshiko Ito-Ihara, Nobuyuki Yajima, Takashi Kawaguchi, Wataru Fukuda, Yutaka Kawahito
OBJECTIVES: To investigate the association between decreased serum IgG levels caused by remission-induction immunosuppressive therapy of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) and the development of severe infections. METHODS: We conducted a retrospective cohort study of patients with new-onset or severe relapsing AAV enrolled in the J-CANVAS registry, which was established at 24 referral sites in Japan. The minimum serum IgG levels up to 24 weeks and the incidence of severe infection up to 48 weeks after treatment initiation were evaluated...
March 24, 2023: Rheumatology
Jingda Huang, Fang Zeng, Jiajie Li, Wang Xu, Meirong Shen, Qiao Shu, Dehui Liu
RATIONALE: Pneumocystis pneumonia (PCP) is an opportunistic fungal infection that occurs in people with impaired or suppressed immunity such as patients with human immunodeficiency virus or organ transplant. However, the incidence and characteristics of PCP in the population with long-term hemodialysis is poorly described in the literature. PATIENT CONCERNS: We present a case of a 50-year-old female patient being transferred to our hospital in February 2022 with a 20-day history of cough and tight breath...
March 24, 2023: Medicine (Baltimore)
Juhi Gupta, Prashant Jauhari, Atin Kumar, Sheffali Gulati, Biswaroop Chakrabarty, Aditya Kumar Gupta, Rachna Seth
Primary hemophagocytic lympho-histiocytosis (HLH) is a hyperinflammatory syndrome with devastating consequences. Multisystem involvement is a hallmark of HLH; however, HLH may rarely present with signs and symptoms isolated to the central nervous system (CNS). Within the brain, HLH can mimic demyelination, chronic infection, or vasculitis, leading to a diagnostic delay of months to years. We describe here a 7-year-old boy who presented with a history of prolonged fever and multiple focal neurologic deficits, which were being treated as CNS tuberculosis at the referring hospital...
March 24, 2023: Pediatrics
Faith Spratt, Wesley Eilbert
No abstract text is available yet for this article.
April 2023: Journal of the American College of Emergency Physicians open
Yangtengyu Liu, Ding Bao, Meng Meng, Lixia Lu, Honglin Zhu
Behçet disease (BD) is a chronic systemic vasculitis that is clinically characterized by recurrent oral ulcers, genital ulcers, uveitis, and skin lesions. Here, we conducted bulk RNA-seq of skin samples from 4 BD patients and 4 normal controls (NCs). A total of 260 differentially expressed genes (DEGs), including 99 upregulated and 161 downregulated genes, were detected in the skin lesions of BD patients compared to NCs. These DEGs were mainly enriched in the following biological processes: the activation and migration of immune cells, the release of proinflammatory factors, and the IFN-γ signaling pathway...
2023: Frontiers in Pharmacology
Daichi Umemoto, Hiroaki Nishioka
No abstract text is available yet for this article.
April 1, 2023: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
Rona M Smith, Rachel B Jones, Ulrich Specks, Simon Bond, Marianna Nodale, Reem Al-Jayyousi, Jacqueline Andrews, Annette Bruchfeld, Brian Camilleri, Simon Carette, Chee Kay Cheung, Vimal Derebail, Tim Doulton, Alastair Ferraro, Lindsy Forbess, Shouichi Fujimoto, Shunsuke Furuta, Ora Gewurz-Singer, Lorraine Harper, Toshiko Ito-Ihara, Nader Khalidi, Rainer Klocke, Curry Koening, Yoshinori Komagata, Carol Langford, Peter Lanyon, Raashid Luqmani, Carol McAlear, Larry W Moreland, Kim Mynard, Patrick Nachman, Christian Pagnoux, Chen Au Peh, Charles Pusey, Dwarakanathan Ranganathan, Rennie L Rhee, Robert Spiera, Antoine G Sreih, Vladamir Tesar, Giles Walters, Caroline Wroe, David Jayne, Peter A Merkel
OBJECTIVE: Following induction of remission with rituximab in anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) relapse rates are high, especially in patients with history of relapse. Relapses are associated with increased exposure to immunosuppressive medications, the accrual of damage and increased morbidity and mortality. The RITAZAREM trial compared the efficacy of repeat-dose rituximab to daily oral azathioprine for prevention of relapse in patients with relapsing AAV in whom remission was reinduced with rituximab...
March 23, 2023: Annals of the Rheumatic Diseases
Buse Guneri Beser, Hakan Demirci
OBJECTIVE OR PURPOSE: To describe sterile endophthalmitis following intravitreal rituximab (Rituxan, Genentech) injection for treatment of vitreoretinal lymphoma (VRL). DESIGN: Retrospective case series. SUBJECTS, PARTICIPANTS, AND/OR CONTROLS: Medical records of biopsy-proven VRL patients who received intravitreal rituximab (RTX) between January 2011 and December 2021 in Kellogg Eye Center, University of Michigan were reviewed. 3 patients presented with sterile endophthalmitis after the second dose of intravitreal RTX were included...
March 23, 2023: Ocular Immunology and Inflammation
Wolfgang A Schmidt
No abstract text is available yet for this article.
March 23, 2023: Rheumatology
Narumichi Iwamura, Katsumi Eguchi, Tomohiro Koga, Kanako Tsutsumi, Takeshi Araki, Toshiyuki Aramaki, Ayuko Takatani, Kaoru Terada, Yukitaka Ueki
A 61-year-old man with no previous record of autoimmune disease developed fever, polyarthralgia, purpura, and urticaria-like rash 2 weeks after the first dose of the Moderna mRNA-1273 vaccine, and symptoms deteriorated following the second dose. He presented reduced erythrocyte and platelet counts, hyperferritinemia, high sIL-2R levels, and severe hypocomplementemia. We diagnosed hypocomplementemic urticarial vasculitis (HUVS), and his symptoms as well as laboratory findings improved following treatment with mPSL 1000 mg/day for 3 days and PSL 40 mg/day...
March 23, 2023: Immunological Medicine
Maliha Tahir, Ramlah Mehmood, Mirza Sultan Ahmad
Kawasaki disease is a generalized systemic vasculitis that affects blood vessels throughout the body. The aetiology of Kawasaki disease is still unknown but is thought to be related to the combined effects of the immune response, genetic susceptibility, and infections including COVID-19. In this case report, we present a seven months old male infant who presented to us with fever, swollen lips, ulcers in the mouth, enlarged tonsils, strawberry tongue, conjunctivitis and generalised non-blanchable maculopapular rash...
2023: Pakistan Journal of Medical Sciences Quarterly
Mari Sato, Masakiyo Yatomi, Ikuo Wakamatsu, Shogo Uno, Chiharu Hanazato, Tomomi Masuda, Koichi Yamaguchi, Haruka Aoki-Saito, Norimitsu Kasahara, Yosuke Miura, Hiroaki Tsurumaki, Kenichiro Hara, Yasuhiko Koga, Noriaki Sunaga, Takuhisa Okada, Hayato Ikota, Takeshi Hisada, Toshitaka Maeno
Eosinophilic granulomatosis with polyangiitis (EGPA) is systemic vasculitis caused by eosinophilia affecting small to medium-sized blood vessels, which damages the organs. Antineutrophil cytoplasmic antibody-associated vasculitis EGPA treatment guidelines added anti-interleukin-5 antibody mepolizumab to the standard treatment protocol for active-non-severe EGPA based on the MIRRA study. Nevertheless, the role of mepolizumab in treating patients with active severe EGPA has not been established. We treated a patient with EGPA complicated with small intestine perforation using steroid pulse intravenous, high-dose glucocorticoids, intravenous high-dose immunoglobulin therapy, and mepolizumab without immunosuppression agents; the patient went into remission, suggesting that mepolizumab is an effective therapeutic agent that could lead to remission in severe EGPA...
2023: Respiratory Medicine Case Reports
Sascha T Bender, Peter R Mertens, Christian Gross
No abstract text is available yet for this article.
April 2023: Kidney International
L Floyd, L Byrne, A D Morris, A C Nixon, A Dhaygude
Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) can be associated with a high burden of morbidity and mortality in an ageing population. It is increasingly recognised that individualised management is needed. Few studies have looked specifically at frailty related outcomes in AAV and a gap remains in understanding the application of frailty assessment tools in these patients. We carried out a single centre, cohort study between 2017 to 2022. Forty-one patients who had newly diagnosed or relapsing AAV and aged ≥65 years were included...
2023: Journal of Frailty & Aging
Sergei Elber-Dorozko, Yackov Berkun, Abraham Zlotogorski, Alexander Maly, Ariel Tenenbaum
No abstract text is available yet for this article.
March 2023: Israel Medical Association Journal: IMAJ
Wen-Juan Sun, Yong-Hong Sun
Kawasaki disease (KD), also known as mucocutaneous lymph node syndrome, is a systemic acute vasculitis belonging to autoimmune disease. Up to now, the specific pathogenesis of this disease remains unclear, and it may involve various factors such as immune response, inflammatory response, and vascular endothelial injury caused by the activation of the nuclear factor-kappa B (NF-κB) signaling pathway. In particular, children with KD and cardiac injury tend to have a poor prognosis, and researchers hope to explore the specific pathogenesis of cardiac injury in KD to provide new options for clinical diagnosis and treatment and reduce the incidence rate of this disorder...
March 15, 2023: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
Fu-Yong Jiao, Zhi-Long Mu, Zhong-Dong DU, Xiao-Dong Yang, Jie Shen, Wei Xiang, Hong Wang
Kawasaki disease (KD) is a febrile disease mainly observed in children aged <5 years, with medium- and small-vessel vasculitis as the main lesion. Although KD has been reported for more than 50 years and great progress has been made in the etiology and pathology of KD in recent years, there is still a lack of specific indicators for the early diagnosis of KD, especially with more difficulties in the diagnosis of incomplete Kawasaki disease (IKD). At present, there are no clear diagnostic criteria for IKD, which leads to the failure of the timely identification and standardized treatment of IKD in clinical practice and even induce the development of coronary artery lesion...
March 15, 2023: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
Zehra Özsoy, Emre Bilgin, Melek Seren Aksun, İmdat Eroğlu, Umut Kalyoncu
BACKGROUND: The objectives were to define the distribution of rheumatic diseases in patients with erythrocyte sedimentation rate (ESR) ≥ 100 mm/h and to find variables that can differentiate main study groups from others. METHODS: Charts of patients admitted with ESR ≥ 100 mm/h between 2015 and 2020 were reviewed. Patients were divided into four diagnostic groups based on etiology: infection (without a rheumatic diagnosis), oncologic (without a rheumatic diagnosis), rheumatic, and no definitive diagnosis...
December 2022: Turkish Journal of Medical Sciences
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