Timely and individualized use of immunosuppression is associated with favourable outcomes in paediatric IgA vasculitis nephritis

2022 ◽  
Author(s):  
Matthew Hon-lam Lee ◽  
Eugene Yu-hin Chan ◽  
Alison Lap-tak Ma
Keyword(s):  
Iga Vasculitis

IgA Vasculitis with Multiple Large Ulcers and Protracted Healing in an Elderly Woman

2017 ◽  
Vol 79 (6) ◽  
pp. 547-551
Author(s):  
Daigo OKA ◽  
Hiroaki HAYASHI ◽  
Takenobu YAMAMOTO ◽  
Tamihiro KAWAKAMI ◽  
Wataru FUJIMOTO
Keyword(s):  
Elderly Woman ◽  
Iga Vasculitis

Characteristics of the course of nephritis associated with Iga-vasculitis Henoch-Schoenlein in children

2020 ◽  
Vol 24 (3) ◽  
pp. 64-71
Author(s):  
A. V. Sukalo ◽  
I. A. Kazyra
Keyword(s):  
Clinical Laboratory ◽  
Morphological Changes ◽  
Pediatric Nephrology ◽  
Morphological Characteristics ◽  
Lymphocyte Activation ◽  
Renal Tissue ◽  
Kidney Damage ◽  
Iga Vasculitis ◽  
Factors Affecting ◽  
Treatment Regimens

INTRODUCTION. Among systemic vasopathies in children, IgA vasculitis Henoch Schoenlein (HS) is the most common, according to various authors, kidney damage is noted in 25-80 % and usually determines the prognosis of the disease.THE AIM of the study was to analyze clinical, laboratory, immunological, morphological characteristics, features of the course and treatment of nephritis associated with IgA vasculitis HS in children, as well as factors affecting the prognosis.PATIENTS AND METHODS. The study included 31 patients with morphologically verified nephritis due to IgA vasculitis HS (18 – boys, 13 – girls) aged 3 to 17 years, who were monitored at the Nephrology Department of the "2nd Children's City Clinical Hospital" of the National Center for Pediatric Nephrology and Renal Replacement therapy in Minsk from 2010 to 2019 yrs.The following parameters were analyzed: the clinical variant of kidney damage, laboratory tests (including the study of BAFF, RANTES lymphocyte activation molecules, pro-inflammatory IL1β, caspase1, TNFα, growth factors VEGF, TGF), 24 hours monitoring and office blood pressure measurements, ECHO cardiography with indicescalculation, ultrasound of the carotid arteries with the thickness of intima-media complex, morphological changes in the renal tissue, as well as treatment regimens.RESULTS. The contribution of deGal-IgA1, markers of T and B lymphocytes activation, pro-inflammatory and profibrotic molecules in the development of the disease is shown. Arterial hypertension was registered in 42 % of children, signs of heart remodeling according to the calculated indices in 19,3 %. Decrease level of adiponectin, vitamin D, leptin, increase concentration of obestatin, Pro-BNP, hs-CRP, and TSAT indicator classify patients with nephritis due to IgA vasculitis HS at moderate risk for the developmentof cardio-vascular disorders, which suggests the need for timely correction.CONCLUSION. In most cases, nephritis with IgA vasculitis HS has a benign course with rare relapses and progression to the end stage of chronic kidney disease (6,5 %).

Management of IgA Vasculitis with Nephritis

2021 ◽  
Author(s):  
Jean-Daniel Delbet ◽  
Cyrielle Parmentier ◽  
Claire Herbez Rea ◽  
Antoine Mouche ◽  
Tim Ulinski
Keyword(s):  
Iga Vasculitis

scholarly journals IgA Vasculitis: Etiology, Treatment, Biomarkers and Epigenetic Changes

2021 ◽  
Vol 22 (14) ◽  
pp. 7538
Author(s):  
Hitomi Sugino ◽  
Yu Sawada ◽  
Motonobu Nakamura
Keyword(s):  
Immune Response ◽  
Autoimmune Diseases ◽  
Organ Dysfunction ◽  
Human Body ◽  
Therapeutic Approach ◽  
Viral Infections ◽  
External Environment ◽  
Iga Vasculitis ◽  
Epigenetic Changes ◽  
Flexible Response

IgA, previously called Henoch-Schönlein vasculitis, is an essential immune component that drives the host immune response to the external environment. As IgA has the unique characteristic of a flexible response to broad types of microorganisms, it sometimes causes an autoreactive response in the host human body. IgA vasculitis and related organ dysfunction are representative IgA-mediated autoimmune diseases; bacterial and viral infections often trigger IgA vasculitis. Recent drug developments and the presence of COVID-19 have revealed that these agents can also trigger IgA vasculitis. These findings provide a novel understanding of the pathogenesis of IgA vasculitis. In this review, we focus on the characteristics of IgA and symptoms of IgA vasculitis and other organ dysfunction. We also mention the therapeutic approach, biomarkers, novel triggers for IgA vasculitis, and epigenetic modifications in patients with IgA vasculitis.

scholarly journals POS0826 SKIN LIMITED IGA VASCULITIS IN ADULTS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 666.1-666
Author(s):  
A. Hočevar ◽  
J. Ostrovrsnik ◽  
K. Perdan-Pirkmajer ◽  
M. Tomsic ◽  
Z. Rotar
Keyword(s):  
Clinical Characteristics ◽  
Systemic Disease ◽  
Elevated Serum ◽  
Skin Lesions ◽  
Current Smoker ◽  
P Value ◽  
Symptom Duration ◽  
Iga Vasculitis ◽  
Reactive Protein

Background:IgA vasculitis (IgAV) could be limited to skin or evolve into a systemic disease, affecting characteristically joints, gastrointestinal tract and/or kidneys.Objectives:We aimed to look for differences between adult IgAV patients with disease limited to skin compared to systemic IgAV.Methods:Medical records of histologically proven adult IgAV cases, diagnosed between January 2010 and December 2020 at our secondary/tertiary rheumatology centre were analyzed.Results:During the 132-month observation period we identified 328 new IgAV cases (59.5% males, median (IQR) age 64.3 (45.1; 76.1) years). Ninety-four (40.2%) patients had skin limited disease, and the rest systemic IgAV.Clinical differences between skin limited and systemic adult IgAV are presented in table 1. Adults with IgAV limited to skin were significantly older, had less commonly skin lesions above the waistline and a lower level of C reactive protein compared to patients with a systemic disease. There were no differences in the frequency of skin necroses between the compared IgAV subgroups. The frequency of potential vasculitis triggers (prior infections, new medications, malignancy) was similar between the compared subgroups.Table 1.Clinical characteristics of IgA vasculitis patients with skin limited and systemic diseaseClinical characteristicsSkin limited IgAV (94)Systemic IgAV (234)P valueMale gender (%)54.361.50.263Age (years)*68.0 (55.0-80.5)61.5 (41.7-75.8)0.007Current smoker (%)13.821.80.123Antecedent infection (%)28.733.80.434New medication23.423.51.0History of cancer12.810.70.569Symptom duration (days)*7 (5-21)8 (5-14)0.756Purpura above waistline36.255.60.002Skin necroses (%)52.145.70.329ESR /mm/h) *32 (18-52)34 (17-53)0.873CRP (g/l) *13.5 (1-32)30 (11-68)<0.001Elevated serum IgA (%)50.649.10.892Legend: * median and IQR;Follow up data were available for 250 (76.2%) patients. During the follow up of median (IQR) 12.5 (6.8 – 22.4) months 35 patients relapsed (13/70 (18.6%) with skin limited IgAV and 22/180 (12.2%) with systemic IgAV, p= 0.224).Conclusion:Skin limited IgAV was associated with older age and less extensive skin puprura in adults. However, relapses of purpura were as common as in systemic IgAV.Disclosure of Interests:None declared

scholarly journals Gastrointestinal involvement and its association with the risk for nephritis in IgA vasculitis

2021 ◽  
Vol 13 ◽  
pp. 1759720X2110248
Author(s):  
Mario Sestan ◽  
Nastasia Kifer ◽  
Marijan Frkovic ◽  
Matej Sapina ◽  
Sasa Srsen ◽  
...  
Keyword(s):  
Logistic Regression ◽  
Odds Ratio ◽  
Biochemical Parameters ◽  
Upper Extremities ◽  
Iga Vasculitis ◽  
Older Children ◽  
Multivariate Logistic Regression ◽  
Gastrointestinal Involvement ◽  
Chronic Complications ◽  
Gi Symptoms

Background: We analysed clinical and biochemical parameters in predicting severe gastrointestinal (GI) manifestations in childhood IgA vasculitis (IgAV) and the risk of developing renal complications. Methods: A national multicentric retrospective study included children with IgAV reviewed in five Croatian University Centres for paediatric rheumatology in the period 2009–2019. Results: Out of 611 children, 281 (45.99%) had at least one GI manifestation, while 42 of 281 (14.95%) had the most severe GI manifestations. Using logistic regression several clinical risk factors for the severe GI manifestations were identified: generalized rash [odds ratio (OR) 2.09 (95% confidence interval (CI) 1.09–4.01)], rash extended on upper extremities (OR 2.77 (95% CI 1.43–5.34)] or face [OR 3.69 (95% CI 1.42–9.43)] and nephritis (IgAVN) [OR 4.35 (95% CI 2.23–8.50)], as well as lower values of prothrombin time (OR 0.05 (95% CI 0.01–0.62)], fibrinogen [OR 0.45 (95% CI 0.29–0.70)] and IgM [OR 0.10 (95% I 0.03–0.35)]] among the laboratory parameters. Patients with severe GI involvement more frequently had relapse of the disease [OR 2.14 (CI 1.04–4.39)] and recurrent rash [OR 2.61 (CI 1.27–5.38)]. Multivariate logistic regression found that the combination of age, GI symptoms at the beginning of IgAV and severity of GI symptoms were statistically significant predictors of IgAVN. Patients in whom IgAV has started with GI symptoms [OR 6.60 (95% CI 1.67–26.06)], older children [OR 1.22 (95% CI 1.02–1.46)] with severe GI form of IgAV (OR 5.90 (95% CI 1.12–31.15)] were particularly high-risk for developing IgAVN. Conclusion: We detected a group of older children with the onset of GI symptoms before other IgAV symptoms and severe GI form of the IgAV, with significantly higher risk for acute and chronic complications of IgAV.

scholarly journals AB0393 PRE-EXISTING MORBIDITY AS A RISK FACTORS FOR MORTALITY IN IgA VASCULITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1224.3-1225
Author(s):  
J. Nossent ◽  
D. Preen ◽  
W. Raymond ◽  
H. Keen ◽  
C. Inderjeeth
Keyword(s):  
Risk Factors ◽  
Cohort Study ◽  
Mortality Rate ◽  
Data Collection ◽  
Premature Death ◽  
Iga Vasculitis ◽  
Risk Of Death ◽  
Increased Risk ◽  
Serious Infections ◽  
Western Australian

Background:IgA vasculitis is generally considered to be a self-limiting condition, but this is at odds with the increased mortality observed in adult patients with IgA vasculitis (1).Objectives:With sparse data on prognostic factors in IgAV, we investigated whether pre-existing conditions are risk factors for mortality in adult IgAV patients.Methods:Observational population-based cohort study using state-wide linked longitudinal health data for adults with IgAV (n=267) and matched controls (n=1080) between 1980-2015. Charlson comorbidity index (CCI) and serious infections (SI) were recorded over an extensive lookback period prior to diagnosis. Date and causes of death were extracted from the WA Death Registry. Mortality rate (deaths/1000 person-years) ratios (MRR) and time dependent survival analysis assessed the risk of death. Age and gender specific mortality rate data were obtained from the Australian Bureau of Statistics.Results:During 9.9 (±9.8) years lookback IgAV patients accrued higher CCI scores (2.60 vs1.50 p<0.001) and had higher risk of SI (OR 8.4, p<0.001), not fully explained by CCI scores. During 19 years follow-up, the risk of death in IgAV patients (n=137) was higher than in controls (n=397) (MRR 2.06, CI 1.70-2.50, p<0.01) and the general population (SMRR 5.64, CI 4.25, 7.53, p<0.001). Survival in IgAV was reduced at five (72.7 vs. 89.7 %) and twenty years (45.2% vs. 65.6 %) (both p<0.05). CCI (HR1.88, CI:1.25 - 2.73, p=0.001), renal failure (HR 1.48, CI: 1.04 - 2.22, p=0.03) and prior SI (HR 1.48, CI:1.01 – 2.16, p=0.04) were independent risk factors. Death from infections (5.8 vs 1.8%, p=0.02) was significantly more frequent in IgAV patients.Conclusion:Premorbid accrual of comorbidity is increased and predicts premature death in IgAV patients. However, comorbidity does not fully explain the increased risk of serious infections prior to diagnosis or the increased mortality due to infections in IgAV.References:[1]Villatoro-Villar M, Crowson CS, Warrington KJ, Makol A, Ytterberg SR, Koster MJ. Clinical Characteristics of Biopsy-Proven IgA Vasculitis in Children and Adults: A Retrospective Cohort Study. Mayo Clin Proc. 2019;94(9):1769-80.Acknowledgements:The authors would like to acknowledge the support of the Arthritis Foundation of WA and acknowledge the Western Australian Data Linkage Branch, the Western Australian Department of Health, and the data custodians of, the Hospital and Morbidity Data Collection, the Emergency Department Data Collection the WA Cancer Register and the WA Death Register for their assistance with the study.Disclosure of Interests:None declared

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