Clinical and Pathophysiological Insights Into Immunological Mediated Glomerular Diseases in Childhood.

Marisa Giani,Antonio Mastrangelo,Jessica Serafinelli,Giovanni Montini

doi:10.3389/fped.2020.00205

Marisa Giani, Antonio Mastrangelo + Show 2 more

Open Access

https://doi.org/10.3389/fped.2020.00205

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Abstract

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.

Highlights

The glomerulus represents the anatomical entity of the kidney most frequently affected by autoimmune and inflammatory diseases
Glomerulonephritis (GN) is the major clinical and pathological expression of this involvement, which sometimes occurs in the context of systemic diseases, such as lupus nephritis and vasculitis
Major advances have been made as regards our understanding of the pathogenesis of some pediatric forms of GN (Table 1). n Hypocomplementemic forms represent a wide spectrum of diseases ranging from acute to severe forms of C3 glomerulopathies

Summary

INTRODUCTION

The glomerulus represents the anatomical entity of the kidney most frequently affected by autoimmune and inflammatory diseases. Treatment Post infectious GN can be prevented by the early onset of antibiotic therapy, in which case the respiratory or skin infections may not be so clinically evident For this reason, in the context of epidemic infection, patients with clinical evidence of PIGN have to be treated as though they have an active infection [15]. The important prognostic factors, reported in both adults and children, include deterioration of renal function, overt nephrotic syndrome (NS), severe glomerular hematuria (>50 red blood cells per high power field) and the presence of crescents or chronic lesions on kidney histology. Treatment Patients with IC-MPGN secondary to chronic infection or other autoimmune/myeloproliferative disorders should be treated for the underlying disease (Table 3) [16]. It is rarely necessary to add an immunosuppressive agent (e.g., MMF) as a first-line drug, though this may be due to the lack of experience in the literature regarding the use of this drug

Glomerulopathies

Findings

53. Kidney Disease