RPGN

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