Predicting gastrointestinal and renal involvement in adult IgA vasculitis (original) (raw)
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A Model Predicting Short Term Severity of IgA Vasculitis in Adults
Journal of Clinical & Experimental Dermatology Research
Background: Predictors of short term severity of adult immunoglobulin A vasculitis (IgAV) are unknown. We aimed to determine clinical features predicting the severity of acute adult IgAV and thus to aid the management of adult IgAV in daily practice. Methods: Medical records of adult, histologically proven IgAV cases, diagnosed between 01.01.2010 and 30.06.2016 at our secondary/tertiary rheumatology centre were reviewed. The disease activity was assessed using Birmingham vasculitis activity score-3. Renal disease was defined severe when nephrotic syndrome or nephritic syndrome with acute renal failure developed. Gastrointestinal (GI) disease was severe in case of bloody diarrhoea, ileus or bowel perforation. Results: During the 78-month observation period, we identified 184 new IgAV cases (57.1% male; 43.5% ever smokers). Skin, GI, renal and joint involvement were present in 184 (generalized purpura above the waist in 47.8%), 63 (severe in 16), 88 (severe in 23), and 81 patients, respectively. Four patients died during acute disease due to vasculitis. Patients with generalized skin lesions had more commonly GI tract, severe GI tract, and kidney involvement. Current smoking was strongly associated with severity of kidney disease. In logistic regression and classification tree model the history of new onset abdominal pain or the presence of generalized purpura in ever smoker emerged as the best predictors of severe GI or renal disease. Conclusions: Based on clinical characteristics only, the presence of abdominal pain, purpura above the waist and smoking history, seem to be good predictors of short term severity of adult IgAV.
Gastrointestinal involvement and its association with the risk for nephritis in IgA vasculitis
Therapeutic Advances in Musculoskeletal Disease, 2021
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...
Nephrology Dialysis Transplantation, 2017
Background. Henoch-Schönlein purpura, more recently renamed immunoglobulin A vasculitis (IgAV), is a systemic vasculitis characterized by IgA deposits. The current markers used to assess IgAV inaccurately evaluate the risk of nephritis occurrence and its long-term outcomes. The current study assessed biomarkers of nephritis outcomes. Methods. This French multicentre prospective study enrolled 85 adult patients at the time of disease onset. Patients were assessed for clinical and biological parameters and reexamined after 1 year. Immunoglobulins, cytokines, IgA glycosylation, IgA complexes and neutrophil gelatinaseassociated lipocalin (NGAL) concentrations were assessed in blood and urine. Results. We identified 60 patients with IgAV-related nephritis (IgAV-N) and 25 patients without nephritis (IgAV-woN). At the time of inclusion (Day 1), the serum levels of galactosedeficient IgA1 (Gd-IgA1) and urinary concentrations of IgA, IgG, IgM, NGAL, interleukin (IL)-1b, IL-6, IL-8, IL-10, IgA-IgG and IgA-sCD89 complexes were higher in the IgAV-N patients than in the IgAV-woN patients (P < 0.005 for all comparisons). After follow-up (1 year), 22 patients showed a poor outcome. Among the tested markers, urine IgA at disease onset adequately reclassified the risk of poor outcome over conventional clinical factors, including estimated glomerular filtration rate, proteinuria and age (continuous net reclassification improvement ¼ 0.72, P ¼ 0.001; integrated discrimination improvement ¼ 0.13, P ¼ 0.009) in IgAV patients.
IgA Vasculitis with Nephritis in Adults: Histological and Clinical Assessment
Journal of Clinical Medicine, 2021
Patients with IgA vasculitis (IgAV), an immune complex-mediated disease, may exhibit kidney involvement—IgAV with nephritis (IgAVN). The kidney-biopsy histopathologic features of IgAVN are similar to those of IgA nephropathy, but little is known about histopathologic disease severity based on the interval between purpura onset and diagnostic kidney biopsy. We assessed kidney histopathology and clinical and laboratory data in a cohort of adult patients with IgAVN (n = 110). The cases were grouped based on the interval between the onset of purpura and kidney biopsy: Group 1 (G1, <1 month, n = 14), Group 2 (G2, 1–6 months, n = 58), and Group 3 (G3, >6 months, n = 38). Glomerular leukocytes were more common in G1 than in the other groups (p = 0.0008). The proportion of neutrophils among peripheral-blood leukocytes was the highest in the patients biopsied within a month after onset of purpura (G1: 71 ± 8%). In the patients with an interval >6 months, the neutrophil proportion wa...
A Case of Adult-Onset IgA Vasculitis in a Cirrhotic Patient
Cureus
Immunoglobulin A vasculitis (IgAV; formerly called Henoch-Schönlein purpura) is a disease commonly seen in children as an immune reaction after a viral infection. It is a small vessel vasculitis characterized by immune complex deposits in various organs throughout the body. It mainly affects the skin, joints, abdomen and kidneys. This presentation is less likely to be seen in adults. In adults, IgAV can be seen due to decreased clearance of immune complexes through the liver. A damaged liver due to alcoholic liver cirrhosis can hinder the clearance of IgA complexes. We present an unusual case of a 42-year-old female who presented with alcoholic liver cirrhosis and ascites and later developed a purpuric rash in her lower extremities.
Clinical Kidney Journal, 2017
Background: Rituximab (RTX), a B cell-depleting anti-CD20 monoclonal antibody, is approved for treatment of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Low immunoglobulin (Ig) levels have been observed surrounding RTX treatment. The association between the degree of Ig deficiency and infection risk is unclear in AAV patients. Methods: AAV patients treated with RTX for remission induction at a single center (2005-15) with serum Ig measurements were included. Patient characteristics; serum IgG, IgM and IgA levels and occurrence of infections were collected retrospectively. Low IgG was defined as mild (376-749 mg/dL) or severe (>375 mg/dL). Logistic regression models were adjusted for age at RTX administration, estimated glomerular filtration rate (eGFR) and race to examine the association of degree and type of Ig deficiency and infection risk. Results: Our cohort of 30 patients had a mean age of 63 (SD 7) years, 23 were women, 16 had granulomatosis with polyangiitis and 13 were PR3 ANCA positive. Nine patients received concomitant cyclophosphamide. The mean IgG level was 625 mg/ dL (SD 289), mean IgM level was 55 mg/dL (SD 41) and mean IgA level was 133 mg/dL (SD 79). In this cohort, 20 patients had low serum IgG levels (<750 mg/dL) following RTX treatment. During the follow-up period, four individuals developed infections requiring hospitalization. In unadjusted logistic regression analysis, an IgG level 375 mg/dL was associated with 23 times higher odds of hospitalized infection [95% confidence interval (CI) 1.8-298.4; P ¼ 0.02]. After adjustment for age, race and eGFR, results were similar [odds ratio (OR) 21.1 (95% CI 1.1-404.1) P ¼ 0.04]. Low IgA was also associated with an increased risk of infections requiring hospitalization after adjusting for age, race and eGFR [OR 24.6 (95% CI 1.5-799.5) P ¼ 0.03]. Low IgM was not associated with a higher risk of infections requiring hospitalization. Conclusions: Severe hypogammaglobulinemia was associated with increased odds of infection requiring hospitalization in this cohort. Further investigation is warranted given our study is limited by small sample size, concomitant cyclophosphamide use and variable timing of Ig measurement.
Long-Term Risk of Comorbidity after IgA Vasculitis in Childhood: A Population-Based Cohort Study
Rheumatology and Therapy
Introduction: Patients with IgA vasculitis (IgAV) may require aggressive treatment and are prone to disease relapses, and IgA deposition in tissues can persist. We investigated whether these factors predispose to long-term morbidity in children with IgAV. Methods: Observational cohort study comparing rates for comorbidity development by Charlson comorbidity index (CCI) and rates for hospitalization, procedures, and emergency department (ED) visits over a 20-year period for IgAV patients \ 20 years (n = 494) and matched hospital-based controls (n = 1385). Odds (OR) for events and rate ratios (RR) for event rates per 1000 person-years were derived from maximum likelihood estimates. Results: Patient survival (99.1 vs. 99.7%, p = 0.6) and overall comorbidity accrual CCI (0.21 vs. 0.23, p = 0.7) were similar for IgAV patients and hospital-based controls after 20 years. IgAV patients did not develop other rheumatic diseases, but more often were diagnosed with peptic ulcer and end-stage renal failure. Hospitalization rates were three times higher for IgAV patients (RR 3.41 CI 3.04-3.82) in the first year following diagnosis, while ED attendance rates were higher in subsequent years (RR 1.29; 1.02-1.04; p \ 0.01) for IgAV patients. Conclusions: Childhood IgAV patients have good long-term prognosis despite the occurrence of end-stage renal failure and compared to hospital-based controls are at not at increased risk for other comorbidity or rheumatic disease.
Massive Intestinal Bleeding in an Adult with IgA Vasculitis Treated with Intravenous Immunoglobulin
Case Reports in Rheumatology
We report the case of a 29-year-old adult presenting with severe IgA vasculitis, with cutaneous, urologic, and renal manifestations. The late appearance of severe gastrointestinal bleeding dominated the clinical picture, necessitating the administration of tens of units of packed cells and the augmentation of the immunosuppressive protocol. It was not until therapy with intravenous immunoglobulin (IVIG) was introduced that the massive bleeding was controlled. We herein discuss the patient’s presentation, the gastrointestinal manifestations of IgA vasculitis, the recommended treatments, and the existent evidence about IVIG therapy.
Kidney involvement in adults with IgA vasculitis: experiences from Clinical Hospital Dubrava, Zagreb
Reumatizam, 2021
IgA-vAskulItIs sA zAhvAćAnjem bubregA u odrAsloj dobI: IskustvA Iz klInIčke bolnIce dubrAvA, zAgreb ana gudelj gračanin 1 , tea mikula 2 , ivica horvatić 3 , luka torić 3 , majda golob 1 , Jasminka dobša 4 , matea liskij 2 , gabrijela buljan 2 , Karla draženović 2 , matej nedić 5 , danica galešić ljubanović 6 , Krešimir galešić 3 1 division of clinical Immunology, Allergology and rheumatology, department of Internal medicine, school of medicine university of zagreb, clinical hospital dubrava, zagreb, croatia / zavod za kliničku imunologiju, alergologiju i reumatologiju, klinika za unutarnje bolesti medicinskog fakulteta sveučilišta u zagrebu, klinička bolnica dubrava, zagreb, hrvatska; 2 school of medicine of the university of zagreb, zagreb, croatia / medicinski fakultet sveučilišta u zagrebu, zagreb, hrvatska; 3 division of nephrology and dialysis, department of Internal medicine, school of medicine university of zagreb, clinical hospital dubrava, zagreb, croatia / zavod za nefrologiju i dijalizu, klinika za unutarnje bolesti medicinskog fakulteta sveučilišta u zagrebu, klinička bolnica dubrava, zagreb, hrvatska;