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IgA vasculitis

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https://www.readbyqxmd.com/read/29672364/role-of-direct-immunofluorescence-in-cutaneous-small-vessel-vasculitis-experience-from-a-tertiary-center
#1
Khushboo Lath, Debajyoti Chatterjee, Uma Nahar Saikia, Biman Saikia, Ranjana Minz, Dipankar De, Sanjeev Handa, Bishan Radotra
Skin is commonly affected by vasculitic process and often subjected to biopsy. Cutaneous vasculitis can be either primary or part of a systemic vasculitic process. This study was conducted to evaluate the diagnostic utility of direct immunofluorescence (DIF) in determination of etiology of cutaneous vasculitis. All histologically proven cases of cutaneous vasculitis over the past two and half years were retrospectively analyzed along with their clinical and DIF findings (IgG, IgA, IgM, and C3). Within this study period, a total of 198 cases of small-vessel vasculitis were diagnosed based on skin biopsy and DIF findings...
April 17, 2018: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/29664511/bullous-henoch-sch%C3%A3-nlein-purpura-case-report
#2
Trinidad Hasbún, Ximena Chaparro, Viera Kaplan, Felipe Cavagnaro, Alex Castro
Henoch-Schönlein purpura (HSP) or IgA Vasculitis is the most common childhood vasculitis. The classic tetrad of signs and symptoms include palpable purpura, arthralgia, abdominal pain and renal disease. The occurrence of hemorrhagic bullae in children with HSP is rarely encountered. Objec tive: To report an unusual cutaneous manifestation of HSP in children. CASE REPORT: A 14-year-old girl complained about a 2-week painful bullous rash in both lower extremities and multiple arthral gias...
February 2018: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/29611051/iga-vasculitis-genetics-and-clinical-and-therapeutic-management
#3
REVIEW
Miguel A González-Gay, Raquel López-Mejías, Trinitario Pina, Ricardo Blanco, Santos Castañeda
PURPOSE OF REVIEW: The purpose of the study is to perform an update on the current knowledge on genetics, clinical manifestations, and therapy in immunoglobulin A vasculitis (IgAV) (Henoch-Schönlein purpura). RECENT FINDINGS: A strong genetic predisposition in individuals with IgAV was confirmed. It was due to the association with the HLA class II region that in people of European background is mainly related to HLA-DRB1*01 allele. Recent reports support the claim that kidney disease is more common in adults than in children with IgAV...
April 2, 2018: Current Rheumatology Reports
https://www.readbyqxmd.com/read/29599028/phenotypic-characterization-of-patients-with-rheumatologic-manifestations-of-common-variable-immunodeficiency
#4
Maria J Gutierrez, Kathleen E Sullivan, Ramsay Fuleihan, Clifton O Bingham
Patients with common variable immunodeficiency (CVID) have a higher incidence of rheumatologic disorders. To delineate this clinical association, we investigated the phenotypic features of patients with CVID affected by these conditions. METHODS: We conducted a retrospective analysis of 870 pediatric and adult patients with CVID included in the United States Immunodeficiency Network (USIDNET) registry. Outcomes included clinical characteristics (age, gender, ethnicity, rheumatologic diagnosis, and comorbidities), infectious history and basic immunophenotype (serum immunoglobulin levels, CD19+ B cells, and CD4/CD8 ratio) in patients with CVID and rheumatologic disorders compared to those with non-inflammatory CVID...
February 23, 2018: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29590463/diagnosing-iga-vasculitis-in-the-active-duty-population-the-importance-of-early-diagnosis-and-proper-biopsy-site-selection
#5
Daniel Flood, Briana Barber, Nathanial R Miletta
Immunoglobulin A associated vasculitis (IgAV), formerly called Henoch-Schönlein purpura, is a small vessel vasculitis which typically presents with upper and lower extremity palpable purpura and abdominal pain. It is the most common vasculitis in children, and is less common in adults. However, newer evidence suggests the incidence within the adult population is higher than previously reported. This case study demonstrates an adult military recruit presenting with new onset IgAV shortly after basic training...
March 26, 2018: Military Medicine
https://www.readbyqxmd.com/read/29532268/monoclonal-gammopathy-in-rheumatic-diseases
#6
Yue Yang, Long Chen, Yuan Jia, Yang Liu, Lei Wen, Yaoxian Liang, Yuan An, Shi Chen, Yin Su, Zhanguo Li
To analyze the clinical spectrum, laboratory characteristics, and outcomes of monoclonal gammopathy (MG) in patients with rheumatic diseases. Screening for the presence of MG was performed in 872 inpatients with rheumatic diseases from January 2010 to July 2017. A total of 41 patients were enrolled. Their clinical and biological features in addition to outcomes were described. For each patient with primary Sjögren syndrome (pSS), 2 age- and sex-matched pSS patients without MG were selected as controls. Risk factors for the presence of MG and malignant hematological neoplasias were assessed...
March 13, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29472804/henoch-sch%C3%A3-nlein-purpura-in-the-third-trimester-of-pregnancy
#7
Ivka Djakovic, Drazan Butorac, Zeljko Vucicevic, Vesna Kosec, Andrea Tesija Kuna, Liborija Lugović-Mihić
Henoch-Schönlein purpura (HSP) is an IgA-mediated small vessels' vasculitis that affects the skin, intestines and kidneys. Pregnancy has been reported as an exacerbating factor. We present the case of a 24-year-old primigravida with HSP that occurred in the third trimester and lasted up to the end of the successful delivery. She had pruritic maculopapular exanthema on her legs. Biopsy of a cutaneous lesion was performed for histopathologic features and direct immunofluorescence (DIF) for the presence of perivascular IgA deposition...
February 15, 2018: Biochemia Medica: časopis Hrvatskoga Društva Medicinskih Biokemičara
https://www.readbyqxmd.com/read/29438165/histologic-features-of-gastrointestinal-tract-biopsies-in-iga-vasculitis-henoch-sch%C3%A3-nlein-purpura
#8
Christine Y Louie, Adam J Gomez, Richard K Sibley, Dorsey Bass, Teri A Longacre
Immunoglobulin A (IgA) vasculitis or Henoch-Schönlein purpura (HSP) typically occurs in the pediatric population, although rare cases also occur in adults. Gastrointestinal (GI) involvement is common. The "classic" histologic finding in IgA vasculitis (HSP) is leukocytoclastic vasculitis (LCV); other histologic features in biopsies of IgA vasculitis (HSP) have only been rarely described. The pathology archival files at our institution were searched for GI biopsies from patients with IgA vasculitis (HSP)...
April 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29409373/epidemiology-of-primary-systemic-vasculitis-in-children-a-population-based-study-from-southern-sweden
#9
M Mossberg, M Segelmark, R Kahn, M Englund, A J Mohammad
OBJECTIVES: To estimate the annual incidence rate of paediatric primary systemic vasculitis (PSV) in a defined geographical area in southern Sweden. METHODS: Potential cases of PSV [IgA vasculitis (IgAV, Henoch-Schönlein purpura), Kawasaki disease (KD), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), polyarteritis nodosa (PAN), and Takayasu's arteritis (TAK)] were identified in a comprehensive regional healthcare register...
February 7, 2018: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/29374629/cross-phenotype-analysis-of-immunochip-data-identifies-kdm4c-as-a-relevant-locus-for-the-development-of-systemic-vasculitis
#10
Lourdes Ortiz-Fernández, Francisco David Carmona, Raquel López-Mejías, Maria Francisca González-Escribano, Paul A Lyons, Ann W Morgan, Amr H Sawalha, Kenneth G C Smith, Miguel A González-Gay, Javier Martín
OBJETIVE: Systemic vasculitides represent a heterogeneous group of rare complex diseases of the blood vessels with a poorly understood aetiology. To investigate the shared genetic component underlying their predisposition, we performed the first cross-phenotype meta-analysis of genetic data from different clinically distinct patterns of vasculitis. METHODS: Immunochip genotyping data from 2465 patients diagnosed with giant cell arteritis, Takayasu's arteritis , antineutrophil cytoplasmic antibody-associated vasculitis or IgA vasculitis as well as 4632 unaffected controls were analysed to identify common susceptibility loci for vasculitis development...
January 27, 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29329643/iga-nephropathy-and-iga-vasculitis-with-nephritis-have-a-shared-feature-involving-galactose-deficient-iga1-oriented-pathogenesis
#11
Hitoshi Suzuki, Junichi Yasutake, Yuko Makita, Yuki Tanbo, Kohei Yamasaki, Tadashi Sofue, Toshiki Kano, Yusuke Suzuki
Galactose-deficient IgA1 has been proposed as an important effector molecule in IgA nephropathy (IgAN). We previously showed that the galactose-deficient IgA1-specific monoclonal antibody KM55 can detect circulating galactose-deficient IgA1 in patients with IgAN, enabling us to study the molecular roles of galactose-deficient IgA1. Herein, we further examined the pathophysiological significance of galactose-deficient IgA1 in glomerular deposits of patients with IgAN by immunohistochemistry using KM55. Immunostaining of galactose-deficient IgA1 with KM55 was performed in paraffin-embedded sections of renal biopsy specimens from 48 patients with IgAN and 49 patients with other renal diseases such as lupus nephritis, HCV-related nephropathy, IgA vasculitis with nephritis (IgA-VN), and membranous nephropathy...
March 2018: Kidney International
https://www.readbyqxmd.com/read/29319773/anca-positive-pauci-immune-crescentic-glomerulonephritis-in-a-patient-with-systemic-lupus-erythematosus
#12
Percy Herrera-Añazco, Percy Velásquez-Castillo, Josmel Pacheco-Mendoza, Germán Valenzuela-Rodriguez, Carmen Asato-Higa
The pauci-immune crescentic glomerulonephritis (PICGN) is generally associated with small-vessel vasculitis with a few reported cases associated with other autoimmune diseases such as Systemic Lupus Erythematosus (SLE). We present the case of a female 34-year-old patient with acute kidney injury symptoms with indication for renal replacement therapy in the context of clinical SLE diagnosis. A kidney biopsy was conducted and it was found that most glomeruli showed some segmental sclerosis with synechia to the Bowman's capsule...
October 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29304917/ultraviolet-purpura-in-iga-vasculitis
#13
Daisuke Tsukui, Hajime Kono
No abstract text is available yet for this article.
January 2018: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/29287933/-humoral-immune-diseases-cutaneous-vasculitis-and-auto-immune-bullous-dermatoses
#14
Janine Wechsler
Humoral immunity is the cause of multiple diseases related to antibodies (IgA, IgG, IgM) produced by the patient. Two groups of diseases are identified. The first group is related to circulating antigen-antibody complexes. The antigens are various. They are often unknown. These immune complexes cause a vascular inflammation due to the complement fixation. Consequently, this group is dominated by inflammatory vasculitis. In the second group, the pathology is due to the fixation in situ of antibodies to a target antigen of the skin that is no more recognized by the patient...
February 2018: Annales de Pathologie
https://www.readbyqxmd.com/read/29279496/a-case-of-paraneoplastic-iga-vasculitis-in-an-adult-with-lung-adenocarcinoma
#15
Shuji Ota, Terunobu Haruyama, Masashi Ishihara, Maika Natsume, Yoko Fukasawa, Takahiko Sakamoto, Shigeru Tanzawa, Ryo Usui, Takeshi Honda, Yasuko Ichikawa, Kiyotaka Watanabe, Yuko Sasajima, Nobuhiko Seki
A 50-year-old man with lung adenocarcinoma (c-T1aN2M1b) experienced reddish purpura mainly on the lower legs after receiving 12 cycles of second-line chemotherapy with docetaxel. There was tumor enlargement on computed tomography performed to assess the therapeutic response, so paraneoplastic IgA vasculitis was considered. IgA vasculitis was diagnosed based on a biopsy of the skin lesion and histology of an upper gastrointestinal hemorrhagic mucosal erosion. As IgA vasculitis can lead to serious gastrointestinal or systemic complications, IgA vasculitis should be considered as a differential diagnosis for rashes in patients with malignancy...
December 27, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29241772/location-of-skin-lesions-in-henoch-sch%C3%A3-nlein-purpura-and-its-association-with-significant-renal-involvement
#16
COMPARATIVE STUDY
Jessica St John, Priyanka Vedak, Anna Cristina Garza-Mayers, Mai P Hoang, Sagar U Nigwekar, Daniela Kroshinsky
BACKGROUND: Henoch-Schönlein purpura (HSP) is a small vessel IgA-predominant vasculitis. OBJECTIVE: To describe adult patients with HSP and determine if the distribution of skin lesions (ie, purpura above the waist or purpura below the waist only), is a predictor of significant renal involvement at the time of the skin biopsy and the months following. METHODS: A retrospective study on renal function from 72 adult patients with skin-biopsy proven HSP...
January 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29236760/distribution-of-circulating-t-follicular-helper-cell-subsets-is-altered-in-immunoglobulin-a-vasculitis-in-children
#17
Deying Liu, Jinxiang Liu, Jinghua Wang, Lishuang Guo, Congcong Liu, Yanfang Jiang, Haifeng Wang, Sirui Yang
BACKGROUND: Immunoglobulin A vasculitis (IgAV) is the most common vasculitis in children. Previously, we demonstrated that patients with IgAV show abnormal proliferation of cluster of differentiation (CD)4+C-X-C chemokine receptor type (CXCR)5+ follicular helper T (Tfh) cells. Here, we explored the status of Tfh cell subsets and plasma cytokine levels in patients with IgAV. METHODS: CD4+CXCR5+CD45RA-, CD45RA-CXCR3+CCR6-, CD45RA-CXCR3-CCR6-, CD45RA-CXCR3-CCR6+, and CD45RA-CXCR3+CCR6+ Tfh cell fractions and plasma concentrations of interferon-γ, interleukin (IL)-4, and IL-17A were evaluated by flow cytometry and a flow cytometric bead array, respectively, in 30 patients with IgAV and 15 healthy controls (HCs)...
2017: PloS One
https://www.readbyqxmd.com/read/29225270/iga-vasculitis-with-simultaneous-cardiopulmonary-involvement-a-case-report
#18
Kazunori Bando, Hirofumi Maeba, Ichiro Shiojima
A 60-year-old man with a history of hypertension, type 2 diabetes, and reflux esophagitis was admitted to our hospital with hemoptysis, dyspnea, and leg edema. We diagnosed him with adult IgA vasculitis based on the presence of purpura, elevated serum IgA fibronectin complexes, pathophysiological findings, a skin biopsy showing leukocytoclastic vasculitis, and immunofluorescence studies demonstrating granular IgA and C3 deposits in the blood vessel wall. He showed concurrent cardiopulmonary involvement without involvement of the gastrointestinal system and kidneys, which are commonly affected in IgA vasculitis patients...
December 8, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29219653/characteristics-and-outcome-of-intractable-vasculitis-syndrome-in-children-nation-wide-survey-in-japan
#19
Naoko Nakano, Masaaki Mori, Hiroaki Umebayashi, Naomi Iwata, Norimoto Kobayashi, Kenji Masunaga, Tomoyuki Imagawa, Takuji Murata, Noriko Kinjo, Kazushige Nagai, Mari Miyoshi, Syuji Takei, Shumpei Yokota, Eiichi Ishii
OBJECTIVE: Primary systemic vasculitis (PSV) is a rare disorder in children and difficult to distinguish from other diseases. However, appropriate diagnosis and prompt treatment will affect on the morbidity and mortality of intractable PSV. In this study, we conducted a nationwide survey in Japan, to clarify epidemiology and clinical outcome of PSV. METHODS: We had sent survey questionnaires to most of the Japanese institutions that employed pediatricians, requesting the number of patients with refractory PSV who were diagnosed and treated between 2007 and 2011...
December 8, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/29204761/clinical-value-of-systemic-symptoms-in-iga-nephropathy-with-anca-positivity
#20
Lijiao Xie, Jianghua He, Xing Liu, Sha Tang, Weili Wang, Furong Li, Ying Zhang, Jun Zhang, Yunjian Huang, Jinghong Zhao, Yafei Li, Jingbo Zhang
Our aim was to evaluate the pathogenic role of anti-neutrophil cytoplasmic antibodies (ANCAs) in patients with IgA nephropathy (IgAN). A total of 2390 patients with biopsy-confirmed IgAN were analyzed retrospectively. Thirty-five IgAN patients with ANCA and 40 IgAN patients without ANCA were enrolled. According to the Birmingham Vasculitis Activity Score (BVAS) items, the ANCA-positive patients were further divided into two subgroups which with or without systemic symptoms. The cumulative renal survival rate was calculated using Kaplan-Meier analysis...
December 4, 2017: Clinical Rheumatology
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