Glomerulonephritis - m.patient.media

1 View this article online at: includes a range of immune-mediated disorders that cause inflammation within the glomerulus and othercompartments of the kidney.[1] Glomerulonephritis results from a variety of immune and inflammatory mechanisms. It is often described as primary, when there is noassociated disease elsewhere, or secondary, when glomerular involvement is part of a systemic disease - eg, systemic lupuserythematosus (SLE), polyarteritis nodosa. Primary Glomerulonephritis may be classified according to the clinical syndrome produced,the histopathological appearance or the underlying aetiology. There is no direct correlation between the clinical syndrome produced andthe pathological description.

2 Glomerulonephritides may be:Minimal : affecting all : affecting only some of the : only affecting parts of an affected cases of Glomerulonephritis result in a mild, asymptomatic illness that remains undiagnosed.[1]Histological patternsThe commonly used pathological classification depends on light microscopy but immunofluorescence and electron microscopy provideadditional information and may give clues as to the change diseaseLight microscopy is virtually normal but electron microscopy shows widespread fusion of the epithelial cell foot processes onthe outside of the glomerular basement membrane. Immunofluorescence is usually often presents in children aged between 2 and 4 years.

3 Accounts for 90% of cases of nephrotic syndrome in childrenand about 20% of cases in features: nephrotic syndrome with selective proteinuria; normal renal function, normal blood pressure, normalcomplement levels; increased risk of infections, especially urinary tract infections and pneumococcal peritonitis (thereforegive prophylactic penicillin if oedematous).Associated with atopy in children, especially those who are also be related to underlying Hodgkin's disease in responds to a course of high-dose prednisolone but relapse is disease may go into remission following treatment with prednisolone and cyclophosphamide or third of patients have one episode, one third develop occasional relapses and one third have frequent relapses whichstop before change disease does not progress to end-stage chronic kidney segmental glomerulosclerosisSome of the glomeruli show segmental scarring.

4 Together with foot process fusion as in minimal change common cause of nephrotic syndrome in older children and younger adults; it may be associated with haematuria,hypertension and impaired renal 50% of patients may respond to a course of high-dose prednisolone, although treatment for up to four months is oftenrequired in adults. If this is unsuccessful, some patients may respond to the addition of cyclophosphamide; ciclosporin can beused to reduce to end-stage kidney disease over several years in up to 50% of patients but progression may be halted bytreatment with variant known as 'collapsing glomerulopathy' is associated with HIV nephropathyWidespread thickening of the glomerular basement membrane reveals granular deposits of immunoglobulin and most cases are idiopathic, it may also be secondary to SLE, hepatitis B, malignancy or the use of gold is more common in is the most common cause of nephrotic syndrome in adults.

5 It may present with proteinuria or nephritic syndrome,hypertension. Haematuria is idiopathic form may respond to a treatment regimen involving alternate months of corticosteroids with chlorambucil orcyclophosphamide, or to progresses to end-stage kidney disease in 30-50% of patients. The remainder with idiopathic membranous nephropathyhas a complete or partial spontaneous remission of nephrotic syndrome with stable renal Glomerulonephritis (MCGN)Page 1 of 4 This is also known as membranoproliferative is proliferation of mesangial cells, an increase in mesangial matrix and thickening of the glomerular can be subdivided according to the appearance on electron is uncommon.

6 It may present with nephrotic syndrome or nephritic syndrome in children and young is associated with low levels of forms of the disease are associated with hepatitis C with or without cryoglobulins, other chronic infections patients are often treated with corticosteroids but there is no treatment of proven 50% of patients will develop end-stage kidney disease within ten proliferative nephritisMesangial cell proliferation combined with matrix expansion occurs. It is most often seen in the context of IgA deposition,when it is known as IgA nephropathy. Other immunoglobulins and complement components may also be nephropathy (Berger's disease) often presents with macroscopic haematuria, which may be precipitated within a fewdays by an upper respiratory tract infection.

7 It is also detected as asymptomatic haematuria and/or proteinuria and canpresent with nephrotic is more common in is association with HLA B35 and D4, coeliac disease, alcoholic liver disease and studies suggest that a course of high-dose prednisolone can reduce proteinuria and delay renal impairment. In patientswith deteriorating renal function, immunosuppressive drugs are also often progression is slow, 20-30% of patients (unusual in children but more common in adults) may eventually developend-stage kidney renal lesion of Henoch-Sch nlein purpura is similar to that of IgA nephropathy and this may be a variant of the samedisease.

8 20% develop impaired renal failure and 5% develop end-stage kidney proliferative glomerulonephritisWidespread hypercellularity occurs, caused by both infiltrating inflammatory cells and proliferation of endothelial andmesangial cells. There is generally deposition of immunoglobulins and complement around the capillary generally presents with an acute nephritic syndrome two or more weeks after an is classically caused by streptococcal is rare in developed countries but post-streptococcal Glomerulonephritis remains common in the developing other bacterial and viral causes have now been described and it is also associated with all children will recover without treatment (other than antibiotics for the infection).

9 However, a small proportion of adultsmay develop renal 2 of 4 Focal segmental proliferative glomerulonephritisThis usually occurs secondary to systemic disease - eg, SLE, Alport's is often associated segmental necrosis of the capillary loops, which is followed by crescent term crescentic Glomerulonephritis is used when there is an accumulation of cells outside the capillary loops but withinBowman's glomerulonephritisThis may occur as part of the evolution of certain forms of primary Glomerulonephritis (eg, IgA nephropathy ormesangiocapillary Glomerulonephritis ); however, it is more often seen in conditions such as Goodpasture's syndrome andsystemic crescentic Glomerulonephritis is now regarded as a form of antineutrophil cytoplasmic antibody (ANCA)-positivevasculitis limited to the presents with the clinical syndrome of rapidly progressive treatment, the disease progresses to end-stage kidney disease within a few months.

10 Prednisolone andcyclophosphamide are generally effective in patients before severe renal damage exchange is recommended in patients with advanced renal 's syndrome:Due to autoantibodies directed against the alpha 3 chain of type IV collagen, which is a major structural componentof the glomerular basement of patients also have pulmonary syndrome presents with rapidly progressive Glomerulonephritis , usually leading to renal failure within sixmonths if with prednisolone, cyclophosphamide and plasma exchange is generally effective as long as it isstarted before renal disease is is very rare for patients to relapse and the long-term outcome is good following successful is a spectrum of disease, from asymptomatic urinary abnormalities to the nephritic and nephrotic syndromes.