Kanghoon Lee, Soyeon Jung, Hee Seung Chin
PURPOSE: Frosted branch angiitis (FBA) is a rare form of retinal vasculitis. Four case series and an extensive literature review of 236 cases were conducted to clarify the characteristics of this rare condition. METHODS: Case series and literature review. RESULTS: An analysis of the reported cases revealed that a majority (54.6%) developed FBA in the presence of an underlying disease, with recurrence and complications requiring surgical intervention being rare...
November 28, 2022: Ocular Immunology and Inflammation
Yun Liu, Xiao Zhang, Zongfei Ji, Lili Ma, Huiyong Chen, Lindi Jiang
OBJECTIVES: Accumulating evidence indicates the role of dysregulated circRNAs in autoimmune diseases. In this study, we investigated their role in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) by analysing the expression profiles of circRNA in plasma of AAV patients and exploring their potential as biomarkers of AAV. METHODS: RNA-sequencing (RNA-seq) was performed to identify the plasma circRNA and mRNA expression profiles from five AAV patients and five healthy controls (HCs)...
November 16, 2022: Clinical and Experimental Rheumatology
Xavier Puéchal, Michele Iudici, Elodie Perrodeau, Bernard Bonnotte, François Lifermann, Thomas Le Gallou, Alexandre Karras, Claire Blanchard-Delaunay, Thomas Quéméneur, Achille Aouba, Olivier Aumaître, Vincent Cottin, Mohamed Hamidou, Marc Ruivard, Pascal Cohen, Luc Mouthon, Loïc Guillevin, Philippe Ravaud, Raphaël Porcher, Benjamin Terrier
IMPORTANCE: Results of randomized clinical trials have demonstrated rituximab's noninferiority to cyclophosphamide as induction therapy for antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV), with neither treatment having a specific advantage for granulomatosis with polyangiitis (GPA). However, post hoc analysis results have suggested that rituximab might be more effective than cyclophosphamide in inducing remission in patients with proteinase 3-positive AAV. OBJECTIVE: To compare the effectiveness of rituximab and cyclophosphamide in inducing GPA remission in a large population of unselected patients...
November 1, 2022: JAMA Network Open
C U Wickramasinghe, D M G Fernando, S Wijetunge, H R S D Sumanasekara
Multiple sclerosis (MS) is a debilitating, demyelinating disease of the central nervous system, with manifestations ranging from numbness and blindness to paralysis. Typical MS is a slowly progressive demyelinating disease, causing significant morbidity spanning over many years. In contrast, "Marburg's disease" is a rare variant of MS which demonstrates a malignant monophasic disease progression leading to death within weeks to months. We present a rapidly fatal demyelinating disease with the clinicopathological findings on par with the handful of reported cases of "Marburg's disease" in the literature...
November 28, 2022: Forensic Science, Medicine, and Pathology
Nimet Öner, Elif Çelikel, Zahide Ekici Tekin, Vildan Güngörer, Tuba Kurt, Pakize Nilüfer Tekgöz, Müge Sezer, Cüneyt Karagöl, Serkan Coşkun, Melike Mehveş Kaplan, Merve Cansu Polat, Banu Çelikel Acar
OBJECTIVE: The aim of this study is to evaluate the outcomes of patients who received intravenous immunoglobulin (IVIG) for immunoglobulin A vasculitis (IgAV) with gastrointestinal (GI) tract involvement, and to determine the differences between the groups that responded to IVIG and those that did not. METHODS: This retrospective study comprised 152 patients with IgAV between 2018 and 2022. Sixty-five patients (43%) had GI tract involvement. Patients with IgAV-GI involvement who had been treated with IVIG were evaluated...
November 28, 2022: Clinical and Experimental Medicine
Dennis Tabakovic, Rona Smith, David Jayne, Aladdin J Mohammad
OBJECTIVE: To determine incidence rate, predictors, and outcome of stroke in a population-based cohort of individuals with ANCA-associated vasculitis (AAV). METHODS: The study included 325 patients diagnosed with AAV from 1997 through 2016 in a defined geographic area of Sweden. Patients who suffered stroke were identified from Riksstroke, a national Swedish stroke register established in 1994, and the Skåne Healthcare Register (SHR), which includes data of all inhabitants of Skåne since 1998...
November 28, 2022: Rheumatology
Mats L Junek, Lily Zhao, Stephanie Garner, David Cuthbertson, Christian Pagnoux, Curry L Koening, Carol A Langford, Carol A McAlear, Paul A Monach, Larry W Moreland, Rennie L Rhee, Philip Seo, Ulrich Specks, Antoine G Sreih, Kenneth Warrington, Michael E Wechsler, Peter A Merkel, Nader A Khalidi
OBJECTIVES: ANCA-associated vasculitis (AAV) is a group of multisystem diseases that can have several ocular manifestations. There are published data on ocular manifestations of granulomatosis with polyangiitis (GPA), but few for eosinophilic granulomatosis with polyangiitis (EGPA) or microscopic polyangiitis (MPA). There is little information concerning chronicity, complications, and association with other cranial manifestations of AAV. METHODS: This study retrospectively analyzed longitudinal multicentre cohorts of individuals with AAV followed between 2006 and 2022...
November 28, 2022: Rheumatology
João Cardoso, Ana Gaspar, Cristina Esteves
Leptospirosis is an infection caused by Leptospira . Leptospirosis causes disease in humans mainly in developing countries and also in countries with poor housing and sanitation, due to animals (mainly rats) that are potential sources of contamination. The clinical manifestations and the severity of leptospirosis are highly variable. We present the case of a 56-year-old female that was admitted to the emergency department with a three-week history of fever (38.4ºC), headache, dyspnea, and cough...
October 2022: Curēus
Eva Baier, Désirée Tampe, Ingmar Alexander Kluge, Samy Hakroush, Björn Tampe
INTRODUCTION: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a potentially life-threatening systemic small-vessel vasculitis that is characterized by pauci-immune glomerulonephritis, depicting in turn a major denominator of AAV mortality. It is well established that AAV patients feature an increased risk of developing thrombotic events, and platelets are activated in AAV patients being triggered by the alternative complement pathway. Platelets guard vessels integrity and initiate thrombus formation in response to endothelial damage, further constituting a triangular interconnection with the activation of neutrophils and the complement system...
2022: Frontiers in Immunology
Jingting Wang, Qin Zhang, Weiyun Shi, Yilin Pang, Suxia Li
BACKGROUND: Rheumatoid vasculitis (RV) is a rare but potentially devastating complication of rheumatoid arthritis (RA). It typically occurs in patients with extra-articular manifestations. Here we reported a case of PUK with nodular episcleritis and pulmonary nodules that occurred in the same patient without joint involvement. CASE PRESENTATION: A 43-year-old Chinese woman, exhibited a partial crescent-shaped marginal corneal ulcer in the right eye at admission and the ulcer developed rapidly into nearly 360-degree ulcers in both eyes within one week...
2022: Frontiers in Immunology
Romain Muller, Mikael Ebbo, Paul Habert, Laurent Daniel, Antoine Briantais, Pascal Chanez, Jean Yves Gaubert, Nicolas Schleinitz
Immunoglobulin G4-related disease (IgG4-RD) is a recently described rare systemic fibroinflammatory disease with an estimated incidence of less than 1 in 100,000 persons per year. The disease can affect virtually any organ and is characterized by unifying histopathological findings. Recently, four subgroups of patients have been characterized: hepatobiliary, head and neck, Mikulicz syndrome and retroperitoneal fibrosis, who illustrate the mainly abdominal and ENT tropism of the disease. Yet, thoracic involvement is not uncommon...
November 27, 2022: Respirology: Official Journal of the Asian Pacific Society of Respirology
Kubra Ozdemir Yalcinsoy, Pinar Cakar Ozdal, Merve Inanc Tekin, Mustafa Salih Karatepe, Yasemin Ozdamar Erol
PURPOSE: To evaluate the clinical features, treatment, and visual outcome of patients with acute retinal necrosis (ARN). METHODS: The data of patients were retrospectively reviewed. Factors associated with visual loss and factors affecting the risk for retinal detachment (RD) development were evaluated. RESULTS: Twenty-four eyes of 24 patients (7 female/17 male, mean age 43.7 years, mean follow-up period 31.0 months) were included...
November 27, 2022: International Ophthalmology
M Fornaro, S Stano, D Goletti, A Semeraro, F P Cantatore, N Maruotti, S Colella, A Abbruzzese, L Santo, E Praino, F Iannone
INTRODUCTION: The objective of this study was to assess the 10-year prevalence of latent tuberculosis infection (LTBI) among Apulian patients with rheumatic diseases (RDs). Secondary endpoint was to record new cases of active TB disease and LTBI among patients treated with biologic disease-modifying antirheumatic drugs (bDMARDs). METHODS: We analysed the results from the patients included in the BIOPURE registry from 2009 to 2018, who underwent QuantiFERON-TB Gold In-tube (QFT-GIT) test as screening before biologic treatment...
November 27, 2022: European Journal of Clinical Investigation
Irena Pintea, Ioana Adriana Muntean, Carmen Teodora Dobrican, Nicolae Miron, Diana Deleanu
Benralizumab is a humanized recombinant mAb that binds to the interleukin 5 receptor (IL-5R) expressed on eosinophils and is approved for the treatment of severe eosinophilic asthma. There are a series of severe eosinophilic disorders that may benefit from this treatment, and it could be a life-saving therapy. In this paper, we present two severe patients with eosinophil-induced diseases that had a good resolution after one dose of Benralizumab 30 mg. The first case is a severe non-necrotizing eosinophilic vasculitis following critical COVID-19 disease and the second case is a DRESS (Drug Rash with Eosinophilia and Systemic Symptoms Syndrome) due to allopurinol...
November 9, 2022: Journal of Clinical Medicine
Samy Hakroush, Ingmar Alexander Kluge, Eva Baier, Désirée Tampe, Björn Tampe
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a small-vessel vasculitis affecting multiple organ systems, including the kidney. Small vessels in the kidney include small-sized arteries, capillaries, and venules. Intrarenal C4 deposits are now increasingly recognized as a potential marker and pathogenic mechanism of autoantibody-mediated tissue damage in ANCA-associated renal vasculitis. We here describe the relevance of complement C4 deposits localized to distinct vascular compartments in a cohort of biopsy-proven ANCA-associated renal vasculitis...
November 18, 2022: International Journal of Molecular Sciences
Caterina Giannessi, Olga Smorchkova, Diletta Cozzi, Giulia Zantonelli, Elena Bertelli, Chiara Moroni, Edoardo Cavigli, Vittorio Miele
Behcet's disease (BD) is a chronic systemic inflammatory disorder characterized by underlying chronic vasculitis of both large- and small-caliber vessels. Thoracic involvement in BD can occur with various types of manifestations, which can be detected with contrast-enhanced MSCT scanning. In addition, MR can be useful in diagnosis. Characteristic features are aneurysms of the pulmonary arteries that can cause severe hemoptysis and SVC thrombosis that manifests as SVC syndrome. Other manifestations are aortic and bronchial artery aneurysms, alveolar hemorrhage, pulmonary infarction, and rarely pleural effusion...
November 19, 2022: Diagnostics
Ludovica R M Lanzafame, Maria Ludovica Carerj, Giovanna Rizzo, Fabio Minutoli, Giuseppe M Bucolo, Natasha Irrera, Giuseppe Muscogiuri, Sandro Sironi, Alfredo Blandino, Tommaso D'Angelo
Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated fibro-inflammatory disorder. Coronary IgG4-RD has been scarcely reported and may present as "tumor-like" lesions. These pseudo-masses may be underdiagnosed mainly due to a vague clinical picture that can vary from complete lack of symptoms to acute coronary syndrome or sudden cardiac death. Early recognition of coronary IgG4-RD is essential to monitor disease activity and prevent life-threatening complications. We report a comprehensive non-invasive imaging evaluation of a patient affected by coronary IgG4-RD, which was diagnosed as an incidental finding during routine pre-laparoscopic cholecystectomy checkup...
November 16, 2022: Diagnostics
Maria Prendecki, Kavita Gulati, Noelle Pisacano, Damilola Pinheiro, Tejal Bhatt, Marie-Anne Mawhin, Frederic Toulza, Esteban S Masuda, Andrew Cowburn, Katharine M Lodge, Frederick W K Tam, Candice Roufosse, Charles D Pusey, Stephen P McAdoo
OBJECTIVE: Syk is a cytoplasmic protein tyrosine kinase that plays a role in signaling via B cell and Fc receptors (FcR). FcR engagement and signaling via Syk is thought to be important in antineutrophil cytoplasm antibody (ANCA) IgG-mediated neutrophil activation. This study was undertaken to investigate the role of Syk in ANCA-induced myeloid cell activation and vasculitis pathogenesis. METHODS: Phosphorylation of Syk in myeloid cells from healthy controls and ANCA-associated vasculitis (AAV) patients was analyzed using flow cytometry...
July 31, 2022: Arthritis & Rheumatology
Jon Stampa Ferro, Beatriz Fernández González, Teresa Arzallus Marco, Arántzazu Izagirre Arostegi, Beatriz Azcue Prieto, Laura Cavero Barreras, Nerea Miren Segués Merino, Horacio Alonso-Galán
We present the case of a patient who was admitted due to jejunitis in the context of an IgA vasculitis, previously known as Schönlein-Henoch vasculitis.
November 25, 2022: Revista Española de Enfermedades Digestivas
Ngoc Trong Tuong Than, Çigdem Yaşar, Brandon Huy Pham, Brandon Chau Lam, Huy Luong Doan, Amir Akhavanrezayat, Muhammad Sohail Halim, David Joseph Iberri, Doan Luong Hien, Quan Dong Nguyen
PURPOSE: To describe the clinical course and management of a patient with bilateral retinal vasculitis associated with cold agglutinin disease (CAD) treated with obinutuzumab and infliximab. OBSERVATIONS: A 69-year-old Hispanic woman was referred to a tertiary Uveitis Clinic with progressively worsening blurry vision, right eye (OD) worse than left eye (OS). Past ocular history was significant for epiretinal membranes in both eyes (OU). Past medical history was notable for non-specific joint disease, primarily affecting her knees bilaterally, and pulmonary symptoms (e...
December 2022: American Journal of Ophthalmology Case Reports
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