Dr S Chakradhar

IgA nephropathy (Berger's disease) : Is named for the deposits of IgA in the glomerulus (Mesangium) Commonly seen in children & young adults It often affects young males within days (24-48hrs) after an upper respiratory tract or gastrointestinal infection. Microscopic examination Shows increased number of mesangial cells with increased matrix Dr S Chakradhar

It usually presents as macroscopic haematuria (visibly bloody urine). Presentation It usually presents as macroscopic haematuria (visibly bloody urine). Gross hematuria often associated with URTI Others present with asymptomatic hematuria accompanied by mild to moderate proteinuria. Mild proteinuria of less than 1g/d is common, but the NS develops occasionally. Dr S Chakradhar

Serum IgA levels are increased in about 50% of cases but Serum complement is normal. Immuno-staining is positive for immunoglobulin A deposits within the matrix. Treatment Intermittent steroid therapy may reduce the frequency of episodes of gross of hematuria. Steroids may also result in remissions of proteinuria in those patients with NS. Dr S Chakradhar

Is variable, but tends to progress slowly. Prognosis Is variable, but tends to progress slowly. 50% of pts develop ESRF within 25 years of the time of diagnosis. Poor prognostic indicators include proteinuria, hypertension, and azotemia. Dr S Chakradhar

Henoch-Schönlein purpura : Is a systemic variant of IgA nephropathy which causes a small-vessel vasculitis and associated glomerulonephritis. Deposition of immune complexes containing the antibody IgA in the skin and kidney Dr S Chakradhar

Membranoproliferative (MPGN)/ Mesangiocapillary GN : There is thickening of GBM Splitting of BM occurs due to inclusion of processes of mesangial cells Proliferation of glomerular cells & mesangial cells Types Primary - Idiopathic Secondary to SLE, viral hepatitis Presents clinically as Nephrotic, Nephritic or mild proteinuria with inevitable progression to end stage renal failure Dr S Chakradhar

Minimal change GN : No changes are visible on simple light microscopy, but on electron microscopy there is fusion of podocytes This form of GN causes 80% of Nephrotic syndrome in children, but only 20% in adults. Dr S Chakradhar

Focal Segmental Glomerulo sclerosis (FSGS) : Sclerosis of some but not all glomeruli And only a segment of an individual glomerulus. May be Primary (Idiopathic) Secondary to reflux nephropathy, Alport syndrome, heroin abuse or HIV. Presents as a Nephrotic syndrome With varying degrees of impaired renal function (seen as a rising serum creatinine, hypertension). Dr S Chakradhar

Steroids are often tried but not shown to be effective. Treatment Steroids are often tried but not shown to be effective. 50% of people with FSGS continue to have progressive deterioration of kidney function, ending in renal failure. Normal Dr S Chakradhar

Membranous glomerulonephritis : Relatively common type of glomerulonephritis in adults, Microscopically, MGN is characterized by a thickened glomerular basement membrane without a hypercellular glomerulus. Immunofluorescence demonstrates diffuse granular uptake of IgG. Etiology Idiopathic (85%) – taken as autoimmune disease Secondary to systemic disease Malignant epithelial tumors – ca of lungs & colon SLE Infections – chronic nephritis malaria syphillis, schistosomiasis Drugs – captopril Dr S Chakradhar

Frequently produces a mixed nephrotic and nephritic picture. Prognosis follows the rule of thirds: 1/3rd remain with MGN indefinitely 1/3rd remit and 1/3rd progress to end-stage renal failure The kidney appears to shrink. Treatment with corticosteroids is attempted if the disease progresses. Dr S Chakradhar

RAPIDLY PROGRESSIVE (crescentic) GLOMERULONEPHRITIS (RPGN) Crescents are formed in Bowman’s space by proliferating parietal epithelial cells with infiltrating monocytes & macrophage & sometimes neutrophils & lymphocytes Reaches end stage renal failure within a period of days or weeks Dr S Chakradhar

Good Pasture’s (anti GMB) Disease Common Causes Systemic vasculitis SLE Good Pasture’s (anti GMB) Disease Aggressive phase of other inflammatory nephritis ( Ig A nephropathy, post infectious) Dr S Chakradhar

Patient presentation (Goodpasture’s syndrome) Adults, before the age of 30 years. A male predominance Onset generally preceded by upper respiratory infection Renal failure develop rapidly, Severe oliguria Symptoms like AGN – hematuria , proteinuria Hypertension is uncommon Pulmonary – haemoptysis & dyspnoe Diagnosis is confirmed by detecting anti – GBM antibodies Dr S Chakradhar

Prednisolone plus cyclophosphamise or azathiprin Treatment Prednisolone plus cyclophosphamise or azathiprin Plasma exchange to remove the Antiglomerular basement membrane antibodies in the plasma. Prognosis Prognosis depends on the degree of irreversible glomerular damage. Earlier diagnosis and treatment increases survival. Dr S Chakradhar

Chronic glomerulonephritis (CGN) Is the final stage of GN when sclerosis has limited many glomeruli & their associated tubules Persistent urinary abnormalities (proteinuria and/or hematuria) Hypertension Progressive loss of functioning Nephrons. These patients generally progress to ESRD. Dr S Chakradhar

Supportive and symptomatic. No specific treatment Hypertension and infections should be treated vigorously. Nephrotoxic agents should be avoided. Prognosis Depends upon the nature of the underlying disease and presence or absence of complications, especially hypertension. Ten, twenty, or more years may elapse from the first discovery of CGN until the development of ESRD. Dr S Chakradhar