Steroid therapy in children with IgA nephropathy

Cambier, Alexandra; Boyer, Olivia; Deschênes, Georges; Gleeson, James; Couderc, Anne; Hogan, Julien; Robert, Thomas

doi:10.1007/s00467-018-4189-7

Cited by 18 publications

(14 citation statements)

References 53 publications

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“…As shown in this study, immunosuppressant treatment was more common among patients who progressed to CKD or among those with unfavorable outcomes, most likely because of non-responsiveness to RAS blockers. However, considering that remission is the most important prognostic factor and the time intervals from onset of disease to progression to CKD or ESRD is relatively short (4-6 years), immunosuppressant therapy might need to be initiated at an earlier stage of IgAN [40].…”

Section: Discussionmentioning

confidence: 99%

Remission of Proteinuria May Protect against Progression to Chronic Kidney Disease in Pediatric-Onset IgA Nephropathy

Suh

Jang

Hyun

et al. 2020

JCM

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Immunoglobulin A nephropathy (IgAN) is one of the most common primary glomerulopathies diagnosed in children and adolescents. This study aimed to evaluate the clinical features in and outcomes of pediatric IgAN over the last 30 years. Patients who were diagnosed before age of 18 at 20 centers in Korea were evaluated retrospectively. Of the 1154 patients (768 males, 386 females) with a median follow-up of 5 years, 5.6% (n = 65) progressed to stage 3–5 chronic kidney disease (CKD). The 10- and 20-year CKD-free survival rates were 91.2% and 75.6%, respectively. Outcomes did not differ when comparing those in Korea who were diagnosed prior to versus after the year 2000. On multivariate analysis, combined asymptomatic hematuria and proteinuria as presenting symptoms and decreased renal function at the time of biopsy were associated with progression to CKD, while remission of proteinuria was negatively associated with this outcome. Patients who presented with gross hematuria or nephrotic syndrome tended toward positive outcomes, especially if they ultimately achieved remission. While remission of proteinuria might imply that the disease is inherently less aggressive, it also can be achieved by management. Therefore, more aggressive management might be required for pediatric-onset IgAN.

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Section: Discussionmentioning

confidence: 99%

Remission of Proteinuria May Protect against Progression to Chronic Kidney Disease in Pediatric-Onset IgA Nephropathy

Suh

Jang

Hyun

et al. 2020

JCM

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“…Immunoglobulin A nephropathy or Berger's disease, first described in 1968 by Berger and Hinglais, is the most common type of primary GN throughout the world in adolescents and young adults, with a male predominance (109,110). It has a higher incidence in Asia and Australia (24-30% of all GN) and Europe (18%) than in the United States (2-10%): these differences are in part related to a different "biopsy policy" (110), as regards microscopic hematuria.…”

Section: Iga Nephropathymentioning

confidence: 99%

“…IgAN has recently been redefined as an immune complexmediated disease with a multi-hit pathogenesis, in which genetic and epigenetic factors play a role in association with the intestinal microbiota (gut-kidney axis) and the mucosalassociated lymphoid tissue (50,(109)(110)(111).…”

Section: Pathogenesismentioning

confidence: 99%

“…Following antigen exposure (environmental or dietary antigens, mucosal infections), the innate immune system is excessively activated by the Toll-like receptors, B-cell activating factor and proliferation-inducing ligand signaling (49). As a result of mis-homing of a proportion of mucosal B cells to systemic sites, poorly O-galactosylated-IgA1 (Gd-IgA1) is secreted into the circulation by gut lymphocytes with autoantibody production against the Gd-IgA1 hinge region and nephritogenic immune complex formation (49,50,(109)(110)(111). The glomerular deposition of these ICs is favored by the dysregulation of CD71, a receptor on mesangial cells stimulated by soluble CD89, which is involved in IgA complex amplification, the activation of mesangial cells and pro-inflammatory cytokine (IL-6, IL-8, IL-1β, TGF-β) release.…”

Section: Pathogenesismentioning

confidence: 99%

“…We suggest performing a urinary dipstick weekly for 1 month, every 2 weeks for 2 months and monthly for 3-6 months. A renal biopsy is suggested in patients with persistent proteinuria (>1 mg/mg for 4 weeks) or impaired GFR at onset (110,135).…”

Section: Clinical and Laboratory Featuresmentioning

confidence: 99%

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Clinical and Pathophysiological Insights Into Immunological Mediated Glomerular Diseases in Childhood

et al. 2020

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The kidney is often the target of immune system dysregulation in the context of primary or systemic disease. In particular, the glomerulus represents the anatomical entity most frequently involved, generally as the expression of inflammatory cell invasion or circulant or in situ immune-complex deposition. Glomerulonephritis is the most common clinical and pathological manifestation of this involvement. There are no universally accepted classifications for glomerulonephritis. However, recent advances in our understanding of the pathophysiological mechanisms suggest the assessment of immunological features, biomarkers, and genetic analysis. At the same time, more accurate and targeted therapies have been developed. Data on pediatric glomerulonephritis are scarce and often derived from adult studies. In this review, we update the current understanding of the etiologic events and genetic factors involved in the pathogenesis of pediatric immunologically mediated primitive forms of glomerulonephritis, together with the clinical spectrum and prognosis. Possible new therapeutic targets are also briefly discussed.

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