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NEPHRITIC SYNDROME - This is characterised by
  1. Hypertension,
  2. Hematuria,
  3. Proteinuria
  4. Azotemia & Oliguria
  5. RBC cast are present in the urine.
Important Points

The five main primary glomerulopathies are-

  1. Acute nephritic syndrome
  2. Rapidly progressive glomerulonephritis (subacute glomerular inflammation)
  3. Nephrotic syndrome
  4. Asymptomatic abnormalities of urinary sediment (hematuria, proteinuria)
  5. Chronic glomerulonephritis.


A. Acute Poststreptococcal GN (Ref. Hari. 18th ed., pg - 2340)
  1. Postinfectious glomerulonephritis occur as a sequela of disease caused by bacteria, viruses, fungi, protozoa, and helminths. 
  2. The prototypical postinfectious glomerulonephritis is poststreptococcal glomerulonephritis.
  3. It is most common cause of GN in childhood. 
  4. Nephritis develops 1–3 weeks after pharyngeal (more common in winter) or cutaneous infection with (more common in summer) ‘nephritogenic’ strains of group A beta – hemolytic streptococci. 
  5. Diagnosis depends on a positive pharyngeal or skin culture, rising antibody titers and hypocomplementemia. 
  6. Renal biopsy reveals diffuse proliferative GN.
Pathology. Diffuse proliferative disease with mesangial and endothelial hypercellularity.
Electron microscopy-dense deposits are seen in the subepithelial area (LQ 2012) (humps), sub endothelial area (LQ 2012) and intra membranous.
Clinical features and diagnosis
  1. The typical clinical presentation is a sudden onset of hematuria and edema. 
  2. The characteristic, but not diagnostic, laboratory profile is azotemia, hypocomplementemia (LQ 2012), hematuria, leukocyturia, and proteinuria. Supporting data include elevated titers of antistreptolysin 0, antihyaluronidase, and anti-deoxyribonuclease B antibodies, all of which suggest preceding streptococcal infection.
Clinical course and diagnosis
  1. The typical course of acute disease is recovery, particularly among children. 
  2. The acute nephritis resolves with amelioration of edema and hypertension 1-3 weeks after onset. 
  3. Proteinuria may persist for several months, exacerbated by erect posture and exercise. 
  4. Microscopic hematuria similarly disappears slowly over a period of several months.
  5. Some patients advance to end-stage renal disease. 
Extra Edge
  1. Recurrence is not a feature of post streptococcal GN (LQ 2012)
  2. Immuno suppressive are not used in the treatment. 
  1. Therapy
    1. Hypertension must be treated aggressively, 
    2. Furosemide or bumetanide is required for the underlying edema-inducing disease. 
    3. Antibiotic - penicillin. 
    4. Prophylaxis following poststreptococcal glomerulonephritis is not indicated because recurrences are exceedingly rare. 
    5. Immunosuppressive agents or corticosteroids have no therapeutic role.

Extra Edge Early treatment of pharyngitis does not prevent development of acute GN.


RPGN is defined by 2 criteria.
  1. Presence of epithelial crescents in more than 70% of glomeruli
  2. Occurrence of rapidly progressive renal failure, End stage disease occurs within month
Crescentic glomerulonephritis
  1. Individuals typically present with rapid, progressive & irreversible deterioration of renal function.
  2. It may be primary (idiopathic) or secondary.
Important Points

The prognosis of RPGN depends upon Number of crescents.

  1. Crescents are produced by Proliferation of the parietal epithelial cells of Bowman 's capsule, Infiltration of monocytes and macrophages.
  2. RPGN is a syndrome associated with severe glomerular injury.
  3. It does not denote a specific etiologic form of glomerulonephritis.
Causes of RPGN
  1. Primary (idiopathic) diffuse crescentic glomerulonephritis
    1. Type I: anti-GBM antibody disease without pulmonary hemorrhage
    2. Membrane Proliferative glomerulonephritis (especially type II)
    3. Membranous glomerulonephritis with or without superimposed anti-GBM antibody disease,
    4. IgA nephropathy (Berger's disease)
  2. In Association with Infectious Diseases
    1. Poststreptococcal glomerulonephritis
    2. Infective endocarditis
    3. Hepatitis B
  3. In Association with Multisystem Diseases
    1. SLE
    2. Henoch-Schönlein purpura-
    3. Wegener's granulomatosis
    4. Microscopic polyarteritis
  4. Other variants
    1. Cryo immuno globulinemia (mixed, essential)
    2. Lung cancer, lymphoma
Important Points

RPGN does not occur in Alport syndrome & minimal change GN.

  1. Clinical features.
    1. Patients present with abrupt -onset renal failure, with rapid loss of renal function (in less than 3 months); frequently normal blood pressure; and normal kidney size.
    2. Nonspecific symptoms (e.g., weakness, nausea, cough, weight loss, fever, myalgia, arthralgia).
    3. Extrarenal involvement, with the exception of lung involvement, is rare.
    4. Renal manifestations. oliguria and azotemia
    5. Pulmonary manifestations. Transient, mild pulmonary infiltrates or hemoptysis
  2. Diagnosis.
    1. There are no diagnostic laboratory findings.
    2. When intrarenal vasculitis (i.e., pauci-immune glomerulonephritis) is the underlying cause, the antineutrophilic cytoplasmic antibody (ANCA) test is positive.
    3. The diagnosis is based on the discovery of epithelial crescents in a majority of glomeruli in the renal biopsy specimen.
  3. Therapy.
    1. In anti-GBM disease, plasmapheresis and immunosuppressive.
    2. In immune complex glomerulonephritis, treatment depends on the individual causative disorder.
    3. In pauci-immune deposit disease, treatment involves pulse methylprednisolone and cyclophosphamide.
C. Goodpasture’s Syndrome:
Good pasture's syndrome is an autoimmune disease characterized by formation of Anti GBM antibodies that attack both pulmonary capillaries and the Glomerulus (GBM)
It refers to a group of illnesses defined by triad of:
  1. Glomerulonephritis (usually crescentic)
  2. Pulmonary hemorrhage (Hemoptysis may precede nephritis).
  3. Anti-GBM antibody in serum.
Association of hematuria and hemoptysis suggest a diagnosis of Goodpasture's syndrome.
  1. Circulating Anti GBM antibodies (lgG): Positive   
  2. ANCA antibodies: Typically Negative (ANCA negative vasculitis) 
  3. ANA antibodies: usually normal
  4. C3 levels: Usually normal
  5. Antibodies against Non collagenous Domain (NCL) of a3 chain of collagen type IV
(Found in Glomerular Basement membrane & Pulmonary capillaries) 
Antibodies against glomerular Basement membrane Antibodies against pulmonary capillaries
Glomerulonephritis / Renal Disease Pulmonary / Lung Manifestations
Two Age Groups
  1. Young men in late 20's (Men>>females)
  2. Men and women in 60-70 years
  1. Hemoptysis (Frank)
  2. Dyspnea
  3. Hematuria
  4. Proteinuria
  5. Edema
  1. Diffuse crescentic Glomerulonephritis
  2. Linear IgG staining along basement membrane on immunofluorescence
X-ray finding
- Diffuse bilateral pulmonary infiltrates


Important Points
  1. The renal and pulmonary components may be severe or clinically silent.
  2. The presence of the anti-GBM antibody, is the essential feature of the diagnosis.
  3. Rapidly progressive renal failure is typical
  4. Circulating anti – glomerular basement membrane (GBM) antibody and linear immuno – fluorescence on renal biopsy establishes the diagnosis
  1. Plasma exchange may produce remission.
  2. Prednisolone
  3. Immunosuppressive therapy
D. IgA nephropathy
IgA Nephropathy (Berger’s Disease) (Ref. Hari. 18th ed., Pg - 2342)
  1. Typically hematuria occur with in 1 to 3 days after URTI.
  2. This is the most common form of primary glomerular disease in the world
  3. This is most common form of primary glomerular disease causing hematuria in children in the world. (MCQ)
  4. It progresses to end stage renal disease in 20 to 40% of patients affected over a 20 years period.
  5. Gross, intermittent hematuria, which is glomerular, is the presenting symptom.
  6. There is presence of abnormal proteinuria
  1. IgA nephropathy may show mesangial widening and focal and segmental inflammation.
  2. Diffuse mesangial proliferation or crescentic glomerulonephritis may also be present. Immunofluorescence shows mesangial deposition of IgA often with C3 and properdin.
  3. Early components of the classical complement pathway (C1q or C4) are usually not seen.
  4. Electron microscopy confirms electron-dense deposits in the mesangium that may extend to the subendothelial area of adjacent capillary walls in a small subset of cases, usually those with focal proliferation.

Extra Edge IgA nephropathy is a type of mesangioproliferative glomerulonephritis with IgA deposition in the mesangium.


Important Points

Example of Mesangioproliferative glomerulonephritis.

Causes Typical pathological findings Typical clinical presentation
IgA nephropathy
Proliferation of mesangial cells and deposition of IgA in matrix Hematuria (LQ 2012)

Diseases Associated with IgA NephropathyQ

  1. Idiopathic (majority)
  2. Renal – limited or as component of Henoch – Schönlein purpura
  3. In associated with systemic diseases or drugs
    1. Liver → Chronic liver disease with involvement of biliary tree
    2. GIT → Celiac disease, Crohn’s disease, adenocarcinoma
    3. Respiratory → Idiopathic interstitial pneumonitis, obstructive bronchiolitis, adenocarcinoma
    4. Skin → Dermatitis herpetiformis, mycosis fungoides, leprosy
    5. Eyes → Episcleritis, anterior uveitis
    6. Miscellaneous → Ankylosing spondylitis, relapsing polychondritis, Sjögren’s syndrome, monoclonal IgA gammopathy, schistosomiasis


Henoch – Schönlein Purpura (HSP)
  1. It is generalized vasculitis causing GN, purpura, arthralgias and abdominal pain, occurring mainly in children. 
  2. Renal involvement is manifested by hematuria and proteinuria. 
  3. Serum IgA is normal or raised, platelet count is normal. (LQ 2012)
  4. Treatment is symptomatic. 
Laboratory Feature of Renal vasculitis in children due to HSP
1. Serum IgA levels Normal or Elevated (LQ 2012)
2. Antinuclear antibody (ANA) Negative
3. Antineutrophil cytoplasmic Negative
4. Antibodies (ANCA) Negative
5. Complement (C3, C4) Normal
6. Cryoglobulins Negative


Important Points
  1. Recurrent episodes of painless gross hematuria represent the classic clinical presentation of IgA nephropathy,
  2. The most common systemic vasculitis in children is Henoch Schönlein Purpura, and the most common primary glomerular disease is IgA Nephropathy. H.S purpura and IgA nephropathy may actually represent spectra of same disease and both are associated with normal or elevated levels of IgA.
  3. ANA and ANCA are characteristically Negative and serum complement levels are typically normal.
Characteristic Causes of Recurrent Gross Hematuria (Ref. Hari. 18th ed., Pg - 2169)
  1. IgA Nephropathy
  2. Alport’s syndrome - Isolated Glomerular diseases with recurrent Gross hematuria
  3. Thin Glomerular basement membrane disease
  4. Hypercalciuria
  5. Urolithiasis
TABLE 264-8 Drug-Induced Glomerular Disease (Ref. Hari. 18th ed., pg - 1692)
Morphologic Lesion Causative Agent
1. Minimal change diseases NSAID, IF-α, Rifampin, Ampicillin
2. Membranous nephropathy Penicillamine, Gold, Mercury, Trimethadione, Captopril, Chlomethiazole
3. Focal segmental glomerulosclerosis Heroin
4. Pauci-immune necrotizing GN Ciprofloxacin, Hydralazine
5. Proliferative GN with vasculitis Allopurinol, Penicillin, Sulfonamides, Thiazides, Intravenous amphetamines
6. RPGN Rifampin, Warfarin, Carbimazole, Amoxicillin, Penicillamine

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