Leonardo Furtado Freitas, Osorio Lopes Abath Neto, Joan E Maley, Nitesh Shekhrajka, Bruno A Policeni
No abstract text is available yet for this article.
July 2024: Arquivos de Neuro-psiquiatria
Akhila Arya Pv, Krishnasai Abhishek Madathanapalli, Felipe Carrasco Tenezaca, Andrew Wang
Giant Cell Arteritis (GCA), also known as Temporal Arteritis, is a type of large vessel vasculitis primarily affecting the elderly population. It typically manifests with headaches, visual impairment, and jaw claudication. Although third nerve palsy as the primary presentation of GCA is rare, it has been reported in previous instances. In this report, we describe the case of a patient presenting with pupil-sparing third nerve palsy, ultimately diagnosed with GCA, and successfully managed with steroids and tocilizumab...
May 13, 2024: Rheumatology International
Dorota Suszek, Anna Grzywa-Celińska, Justyna Emeryk-Maksymiuk, Adam Krusiński, Katarzyna Redestowicz, Jan Siwiec
IgA-associated vasculitis (IgAV) known as Henoch - Schönlein purpura (HSP) disease is an inflammatory disorder of small blood vessels. It's the most common type of systemic vasculitis in children which can be associated with the inflammatory process following infections. IgA vasculitis is a rare and poorly understood systemic vasculitis in adults. Coronavirus disease 2019 (COVID-19) has been associated with HSP in both adults and children. A 58-year-old woman was diagnosed with HSP, fulfilling the clinical criteria: palpable purpura, arthritis, hematuria...
May 13, 2024: Rheumatology International
(no author information available yet)
No abstract text is available yet for this article.
May 8, 2024: Kidney International
Jürgen Floege, David R W Jayne, Jan-Stephan F Sanders, Vladimír Tesar, Ethan M Balk, Craig E Gordon, Gaelen Adam, Marcello A Tonelli, Michael Cheung, Amy Earley, Brad H Rovin
No abstract text is available yet for this article.
May 8, 2024: Kidney International
Femke Demolder, Samuel De Bontridder, Shane Hanon
BACKGROUND: Katayama syndrome is an acute manifestation of schistosomiasis, a parasitic infection that manifests itself through a hypersensitivity reaction to migrating larvae and early egg deposition. Left undiagnosed and untreated, acute schistosomiasis can develop into chronic schistosomiasis which can lead to debilitating morbidity such as pulmonary hypertension. This case highlights that Katayama syndrome can also been seen in regions where the parasite is not endemic, as it occurs in travelers returning from endemic regions or in immigrants...
2024: Respiratory Medicine Case Reports
Juan Felipe Coronado-Sarmiento, Juan Pablo Coronado-López, Eduardo Tuta-Quintero, Claudia Marcela Mora, Viviana Mayor
INTRODUCTION: Eosinophilic granulomatosis with polyangiitis (eGPA) is a necrotising vasculitis of small and medium calibre vessels, which affects mostly patients in their fourth to sixth decade of life, and it is a very uncommon aetiology for pulmonary fibrosis. CLINICAL CASE: A Hispanic 72-year-old female patient presents with a history of lower extremities pain, paraesthesia, oedema, and occasional macroscopic haematuria. During her hospitalisation, the patient presents, and images showed findings compatible with pulmonary fibrosis and alveolar haemorrhage, which require a biopsy, establishing the diagnosis of an eGPA...
March 2024: Mediterranean journal of rheumatology
Amira El Ouni, Faiza Ben Messaoud, Rym Khayati, C Abdelkafi, Zeineb Meddeb, Saloua Hamzaoui, Thara Larbi, Sana Toujani, Kamel Bouslama
BACKGROUND: Vascular purpura can be the clinical expression of infectious, inflammatory, drug-related, neoplastic, and endocrine pathologies. To date, there is no consensus codifying the investigation of vascular purpura, especially when it is isolated. PATIENTS AND METHODS: We proposed to study through a retrospective study of 73 cases of vascular purpura, occurring during the period 2004-2019 in our internal medicine department, the contribution of various clinical and paraclinical data to the aetiological diagnosis of vascular purpura...
March 2024: Mediterranean journal of rheumatology
Herwinda Brahmanti, Cesarius Singgih Wahono, Mirza Zaka Pratama, Perdana Aditya Rahman, Riski Bagus Suhendra, Amalia Novia Rizky, Nabilah Hanifah Mukti
BACKGROUND: Cutaneous involvement is common in systemic lupus erythematosus (SLE) patients and may be essential to the disease activity. This study aimed to describe cutaneous manifestations spectrum and determine the association of cutaneous lesions with the disease activity and systemic involvement among SLE patients in Malang, Indonesia. METHODS: A cross-sectional study was conducted using 54 SLE patients from rheumatology outpatient clinic at Saiful Anwar General Hospital Malang, Indonesia...
March 2024: Mediterranean journal of rheumatology
Moaz O Moursi, Wael Hamam, Adnan Hajjar, Mohammad Es-Salim, Soha Aboukhalaf, Omar Jamil, Muhammad Zahid
With syphilis resurgence, physicians should be more vigilant to infection-induced cerebral vasculitis in high-risk patients presenting with neurological symptoms. In this case, neurosyphilis should not be missed. Thorough serologic screening and lumbar puncture are crucial for diagnosis, and further research is needed for safe and effective treatments in these populations.
May 2024: Clinical Case Reports
G S Madhuram, T Geetha, G Bharathiraja, P Arunkumar, M Prabagar, K Subramaniam
BACKGROUND: Peripheral T cell lymphoma (PTCL), not otherwise specified (NOS) is a heterogenous group of predominantly nodal T cell lymphomas that generally presents with lymphadenopathy with or without extra nodal involvement. Acral vascular syndrome clinically presents as digital ischemia with Raynaud's phenomenon and acral cyanosis. Although, this condition is commonly associated with connective tissue disorder, smoking and vasculitis, its association with lymphoid malignancy is very rare...
January 2024: Journal of the Association of Physicians of India
Gonzalo Borrego-Yaniz, Lourdes Ortiz-Fernández, Adela Madrid-Paredes, Martin Kerick, José Hernández-Rodríguez, Sarah L Mackie, Augusto Vaglio, Santos Castañeda, Roser Solans, Jaume Mestre-Torres, Nader Khalidi, Carol A Langford, Steven Ytterberg, Lorenzo Beretta, Marcello Govoni, Giacomo Emmi, Marco A Cimmino, Torsten Witte, Thomas Neumann, Julia Holle, Verena Schönau, Gregory Pugnet, Thomas Papo, Julien Haroche, Alfred Mahr, Luc Mouthon, Øyvind Molberg, Andreas P Diamantopoulos, Alexandre Voskuyl, Thomas Daikeler, Christoph T Berger, Eamonn S Molloy, Daniel Blockmans, Yannick van Sleen, Mark Iles, Louise Sorensen, Raashid Luqmani, Gary Reynolds, Marwan Bukhari, Shweta Bhagat, Norberto Ortego-Centeno, Elisabeth Brouwer, Peter Lamprecht, Sebastian Klapa, Carlo Salvarani, Peter A Merkel, María C Cid, Miguel A González-Gay, Ann W Morgan, Javier Martin, Ana Márquez
BACKGROUND: Giant cell arteritis is an age-related vasculitis that mainly affects the aorta and its branches in individuals aged 50 years and older. Current options for diagnosis and treatment are scarce, highlighting the need to better understand its underlying pathogenesis. Genome-wide association studies (GWAS) have emerged as a powerful tool for unravelling the pathogenic mechanisms involved in complex diseases. We aimed to characterise the genetic basis of giant cell arteritis by performing the largest GWAS of this vasculitis to date and to assess the functional consequences and clinical implications of identified risk loci...
May 8, 2024: Lancet Rheumatology
Yeliz Yagiz Ozogul, Yesim Ozguler, Sinem N Esatoglu, Didar Ucar, Ugur Uygunoglu, Zekayi Kutlubay, Vedat Hamuryudan, Gulen Hatemi
OBJECTIVE: Assessment of damage accrual over time is important for evaluating and comparing long-term results of treatment modalities and strategies. Retrospective studies may be useful for assessing long-term damage, especially in rare diseases. We aimed to validate Behçet's Syndrome Overall Damage Index (BODI) for use in retrospective studies by evaluating its construct validity, reliability, and feasibility in retrospectively collected data. Additionally, we aimed to determine missing items by evaluating Behçet's syndrome patients with different types of organ involvement and long-term follow-up...
May 11, 2024: Rheumatology
Hao Cheng Shen, Khai-Tuan Andrew Bui, Rachel Richard, Nader Toban, Marianne Lévesque, Rosalie-Sélène Meunier, Carolyn Ross, Jean-Paul Makhzoum
OBJECTIVE: Positive antineutrophil cytoplasmic antibodies (ANCAs) may occur in the setting of interstitial lung disease (ILD), with or without ANCA-associated vasculitis (AAV). We aim to compare the characteristics and clinical course of patients with ILD and positive ANCA (ANCA-ILD) to those with negative ANCA. METHODS: We performed a single-center retrospective cohort study from 2018 to 2021. All patients with ILD and ANCA testing were included. Patient characteristics (symptoms, dyspnea scale, and systemic AAV), test results (pulmonary high-resolution computed tomography and pulmonary function tests), and adverse events were collected from electronic medical records...
May 11, 2024: ACR open rheumatology
Serena D'Aniello, Arianna Rustici, Laura Ludovica Gramegna, Claudia Godi, Laura Piccolo, Mauro Gentile, Andrea Zini, Alessandro Carrozzi, Raffaele Lodi, Caterina Tonon, Massimo Dall'Olio, Luigi Simonetti, Raffaella Chieffo, Nicoletta Anzalone, Luigi Cirillo
BACKGROUND: To describe high-resolution brain vessel wall MRI (VW-MRI) patterns and morphological brain findings in central nervous system (CNS) vasculitis patients. METHODS: Fourteen patients with confirmed CNS Vasculitis from two tertiary centers underwent VW-MRI using a 3T scanner. The images were reviewed by two neuroradiologists to assess vessel wall enhancement characteristics and locations. RESULTS: Fourteen patients were included (six females; average age 48 ± 19 years)...
April 29, 2024: Diagnostics
Diana Shu Yee Tan, Yibeltal Akelew, Matthew Snelson, Jenny Nguyen, Kim Maree O'Sullivan
The gut microbiota and short chain fatty acids (SCFA) have been associated with immune regulation and autoimmune diseases. Autoimmune kidney diseases arise from a loss of tolerance to antigens, often with unclear triggers. In this review, we explore the role of the gut microbiome and how disease, diet, and therapy can alter the gut microbiota consortium. Perturbations in the gut microbiota may systemically induce the translocation of microbiota-derived inflammatory molecules such as liposaccharide (LPS) and other toxins by penetrating the gut epithelial barrier...
April 28, 2024: International Journal of Molecular Sciences
Qiuyu Xu, Gang Chen, Yubing Wen, Xiaohu Shi, Wenling Ye, Yan Qin, Xuemei Li
PURPOSE: Cryoglobulinemia is a pathological condition characterized by the presence of cryoglobulins in the blood, with cryoglobulinemic glomerulonephritis being the most frequent form of renal involvement. Fanconi syndrome presents as a generalized dysfunction of the proximal tubule, characterized by the presence of polyuria, phosphaturia, glycosuria, proteinuria, proximal renal tubular acidosis, and osteomalacia. We aimed to present five cases co-occurring with Fanconi syndrome and cryoglobulinemia...
May 10, 2024: Clinical Rheumatology
Mayu Shiomi, Ryu Watanabe, Shogo Matsuda, Takuya Kotani, Ayana Okazaki, Yuichi Masuda, Tsuneyasu Yoshida, Mikihito Shoji, Ryosuke Tsuge, Keiichiro Kadoba, Ryosuke Hiwa, Wataru Yamamoto, Akitoshi Takeda, Yoshiaki Itoh, Motomu Hashimoto
OBJECTIVES: To determine the current retention rate of mepolizumab (MPZ) and identify factors associated with drug retention in patients with eosinophilic granulomatosis with polyangiitis (EGPA) in the Kansai multicentre cohort (REVEAL cohort). METHODS: Sixty patients diagnosed with EGPA and treated with MPZ between December 2016 and June 2023 were enrolled. The clinical characteristics, including laboratory data, treatments administered, and disease course outcomes were collected retrospectively...
May 10, 2024: Modern Rheumatology
Bangtao Li, Weiming Chen, Xiaodi Cai, Yuanping Hai, Qiming Pang, Wei Xiang, Zhengzheng Zhang
X-linked lymphoproliferative disease (XLP) is a rare genetic disorder characterized by immune dysregulation. The three most common clinical phenotypes are EBV-associated infectious mononucleosis (FIM), abnormal gammaglobulinemia, and lymphoma. We present a rare case of XLP1 with neurovasculitis, which is non-EBV-related and involves multiple systems, a condition rarely seen in children. The patient initially presented with an unsteady gait, which progressively evolved into language and consciousness disorders...
2024: Frontiers in Immunology
Chun-Hui She, Dan Hu, Jun Zou, Hua-Fang Bao, Yan Shen, Jian-Fei Cai, Jing-Fen Ye, Dan Luo, Lei-Lei Jian, Hai-Fen Ma, Cheng-Cheng Hou, Yong Chen, Jian-Long Guan
Behçet's syndrome (BS) is a variant vasculitis that can involve multiple organs with inflammatory manifestations. This study aimed to provide a more comprehensive analysis of the clinical phenotypes and characteristics of BS patients. We enrolled 2792 BS patients referred from China nationwide to Huadong Hospital Affiliated to Fudan University from October 2012 to December 2022. Detailed assessments of demographic information, clinical manifestations, laboratory results, gastroscopy, and medical imaging were conducted...
May 10, 2024: Clinical Rheumatology
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