Rapidly progressive glomerulonephritis (RPGN) is characterized by a rapid loss of renal function over a short period of time. It is classified as primary or secondary RPGN based on its underlying causes. The pathogenesis involves crescent formation in glomeruli due to proliferation of cells and inflammation. Clinically, it presents with hematuria, proteinuria, hypertension, and declining kidney function. Diagnosis involves lab tests showing abnormalities in urine, blood counts, complement levels, and antibodies. Renal biopsy revealing crescentic lesions in glomeruli is diagnostic. Treatment depends on the specific type but often includes steroids, plasmapheresis and cyclophosphamide to slow kidney damage. Prognosis depends

1. RAPIDLY
PROGRESSIVE(CRESCENTIC)
GLOMERULONEPHRITIS
DR. DR.RASIKAPRIYA
MD PAEDIATRICS
FIRST YEAR

2. OBJECTIVES
•Case scenario
•Introduction
•Classification
•Pathogenesis
•Clinical manifestations
•Diagnosis
•Treatment and prognosis

3. CASE SCENARIO

4. INTRODUCTION
• RPGN is characterized clinically by a rapid
decrease in the glomerular filtration rate
of at least 50% over a short period , from
few days to 3 months.
• Crescent formation.

5. Definition: RPGN
• Clinical syndrome of glomerular disease
• Rapid loss of renal function > 50% decline
in GFR within 3 months.
• Nephrotic-nephritic urine sediment
• Extensive crescent formation

6. Classification
Primary :
Type 1- Anti GBM antibody disease, Goodpasture
syndrome (with pulmonary disease).
Type 2- Immune complex mediated.
Type 3- Pauciimmune(ANCA –positive)

7. Contd..
Secondary:
 Membranoproliferative GN
 Immmunoglobulin A nephropathy
 Henoch schonlein purpura
 Poststrptococcal GN
 SLE
 Polyarteritis nodosa
 Hypersensitivity angiitis

8. PATHOGENESIS
• Crescents in glomeruli - through proliferation of
parietal epithelial cells in Bowman’s space, may
be the final pathway of any severe inflammatory
glomerular injury.
• Podocytes and renal progenitor cells are
involved in the pathogenesis of CGN.

9. CONTD..
• The severity of the disease -degree of crescent
formation nonspecific response to severe injury
to the glomerular capillary wall .
• Rents are induced in the glomerular capillary
wall- movement of plasma products, including
fibrinogen, into Bowman's space with
subsequent
• Fibrin formation, the influx of macrophages and
T cells, release of proinflammatory cytokines-IL-1
and TNF a

11. CLINICAL MANIFESTATION
• Acute nephritis- hematuria, hypertension and renal
insufficiency
• Proteinuria
• Later- oliguric renal failure
• Extra renal manifestations.

12. History
• History of hematuria, frothing of urine, Oliguria/Anuria,
progressive renal failure
• SYSTEMIC FEATURES
 Hemoptysis, longstanding asthma or Petechiae is suggestive
of vasculitis
 Arthralgia, oral ulcers or photosensitivity indicates presence
of lupus.
 Recent Drug history, fluid loss, sepsis.
Joint n skin lesions.

13. Examination
• General examination
• Skin lesions
• Respiratory system

14. DIAGNOSIS
CBC :
Leukocytosis – Sepsis, vasculitis,
Eosinophilia – Churg Strauss
URINE ANALYSIS:
Dysmorphic RBCS, active sediments, Rbc casts, Sub
Nephrotic Proteinuria – Vasculitis, Lupus – RPGN
Isomorphic RBCs , Eosinophiluria – AIN
Nephrotic Range proteinuria – causes other than RPGN

15. • Low complements – Lupus Nephritis, Cryoglobulinemia,
PSGN(C4 normal)
• Raised ESR, CRP – Vasculitis, SLE
• HBsAg – MPGN , Vasculitis
• Hepatitis C – MPGN, Vasculitis
• Chest X ray – cavities/nodules – ANCA ass systemic
vasculitis

16. Laboratory approach
ANCA-veANCA+ve
Low C3Pauci immune GN
Immune complex
GN
Normal C3
Normal C3
Anti-GBM disease

17. SEROLOGIAL TESTS
• ANA, APLA – Lupus Nephritis,
• Anti GBM – Good pasture's Syndrome/Anti GBM dis
• ASLO, Anti DNAse- PSGN

18. Serum C3
normal
Low
IgA
Hereditary disease
Wegener granulomatosis
Good pasture’s syndrome
Henoch-Schonlein purpura
MPGN
Shunt
nephritis
IE
Hepatitis
B,C
Low
normal
Serum C4
SLEAPSGN
Normal Serum C3 83-177 mg/dL

19. • Rapid progressive renal failure
• Systemic features – Pulmonary renal/ rashes/ peripheral
neuropathy/ flu like syndrome
• Hematuria, sub Nephrotic proteinuria, active urinary sediment
• Low complement
• ANCA/ ANA/ Anti GBM/ ASLO – positive
RENAL BIOPSY

20. RENAL BIOSPY
LIGHT MICROSCOPY:
• Hallmark lesions – Crescents
• Cellular, fibro cellular, fibrous
• Lesions usually in various
stages of activity/ resolution
• Necrotizing inflammation-10%
• Fibrinoid necrosis, peri glomerular granulomas

23. ELECTRON MICROSCOPY
• RPGN I AND III- absence of electron dense
immune complex deposits
• RPGN II- multiple electron dense deposits

28. MANAGEMENT
• Plasmapheresis, steroids, and cyclophosphamide
are used in different combinations depending
upon the histological type of RPGN
• Prognosis depends upon the etiology and on the
extent of glomerular damage already present at
the initiation of therapy
• Circumferential fibrous crescents involving most
of the glomeruli bode a poor outcome

29. Take home message

30. THANK U