Acute glomerulonephritis case discussion

1. CASE DISCUSSION
Dr Lubna Aman
Fellow peads nephrology
SIUT.

2. A 12 year old boy resident of Karachi presented with history
of :
Fever 1 week.
Edema 4 days
Decreased urine out put 4 days.

3. • No h/o sore throat, cola color urine, skin rash,
photosensitivity, redness of eyes or blue discoloration of
fingers, joint pain, swelling, jaundice or any breathing
difficulty
• Past medical and Family history is unremarkable

4. Young boy of average height and weight.
• Temp = 98.60f
• R/R = 24 br/min.
• H/R = 87 b/min.
• B.P =147/92 mm Hg ( >95th centile).
• Anemic.
• Facial edema.
• Systemic Examination: unremarkable

5. Differential Diagnosis
Rapidly Progressive Glomerulonephritis
Diffuse proliferative glomerulonephritis

6. Investigations

8. Urine D/R
• Color : yellow
• Sp. Gravity: 1.025
• pH: 6.0
• Albumin: 3+
• Blood : 3+
• Leucocytes, sugar, ketones, bile : nil
• Pus cells, cast, epithelial cells: nil
• RBC casts.

10. U/S KUB
• Left kidney is 11.3 cm
• Right kidney is 10.7 cm.
• Both are echogenic .
• No calculus, no hydronephrosis.
• Minimally filled urinary bladder.

14. Final diagnosis
Mesangiocapillary GN with cellular crescents

15. Initial Management
• Calcium channel blockers (Amlodipine 5mg bid).
• Angiotensin converting enzyme inhibitors (Enalapril 10 mg
bid).

16. During Hospital StaySerumcreatinine
Days

17. Weeks
I/V Methylprednisolone + I/V Cyclophosphamide
I/V Cyclophosphamide
Aza+ Delta
Aza+Delta

18. On Follow Up
• Normal Renal functions
• Normotensive
• No urinary active sediment
• On maintenance immunosuppressive
• Plan is to continue immunosuppressive for 18
months

19. DISCUSSION

20. Definition
• Features of Acute glomerulonephritis (hematuria,
proteinuria, edema, hypertension, nephritic urinary
sediment)
• Progressive loss of kidney function over days or weeks (1)
• Histologically : glomerular crescent formation involving
50% or more glomeruli
1. Jennette JC. Rapidly progressive crescentic glomerulonephritis.
Kidney Int. 2003;63(3):1164–77.

21. • Presence of crescents histologic marker of severe
glomerular injury
• Circumferential crescents >80 % of glomeruli present
with advanced renal failure
• Crescents in less than 50 % of glomeruli, particularly if
these are non-circumferential, have an indolent course
• RPGN is a medical emergency, which if untreated might
rapidly progress to irreversible loss of renal function

22. Epidemiology
• The incidence of RPGN in children is not known.
• Crescentic GN comprises approximately 5% of unselected renal biopsies in
children.
• There are no population-based studies in children.
• The 2010 NAPRTCS Annual Transplant Report shows that
idiopathic crescentic GN contributes to 1.7 % of all transplanted patients [1]
1.Studies NAPRTaC. NAPRTCS 2010 Annual report.2010. https://web.emmes.com/study/ped/annlrept/
annlrept2006.

23. Pathogenesis

24. CRESCENT FORMATION

27. Cellular crescents
proliferation of
macrophages, epithelial
cells, and neutrophil.
Fibrocellular crescents
admixture of collagen fibers and
membrane proteins among the
cell.
Fibrous crescents
the cells are completely
replaced by collagen.
CRESCENTS

28. Immunofluorescence
Anti-GBM GN
linear deposition of
anti-GBM antibodies.
Immune-complex GN
granular deposits of immune
complexes along capillary wall
and mesangium.
Pauci-immune GN
scant or no immune
deposits, and associated
with systemic vasculitis.
An update on Glomerulonephritis-clinical and treatment aspects

30. RPGN without crescents
• Hemolytic uremic syndrome
• Acute interstitial nephritis
• Diffuse proliferative GN

31. Clinical Features
• Macroscopic hematuria (60–90 %)
• Oliguria (60–100 %),
• Hypertension(60–80 %)
• Edema (60–90 %)
• Hypertensive emergencies, pulmonary edema
and cardiac failure
• Systemic complaints, involving the upper
respiratory tract skin ,musculoskeletal and
nervous system

32. Diagnostic evaluation
• CBC; peripheral smear for anemia, retic count
• ESR
• Urea, creatinine, electrolytes
• Urinalysis: proteinuria; microscopy for erythrocytes
and leukocytes, casts
• Complement levels (C3, C4, CH50)
• ASOT, ANA, anti-dsDNA antibodies
• ANCA
• Indirect immunofluorescence, ELISA
• Renal biopsy (light microscopy, immunofluorescence,
• electron microscopy)

33. Required in specific instances
• Anti-GBM IgG antibodies
• Blood levels of cryoglobulin,
• hepatitis B and C serology
• Chest: radiograph, CT (patients with Goodpasture
Syndrome and vasculitides)
• Sinuses: radiograph, CT (patients with
granulomatosis with polyangiitis)

34. Diagnostic evaluation of Crescentic GN

35. Supportive management
• Fluid and electrolyte balance
• Providing adequate nutrition
• Control of infections
• Hypertension

36. I

37. Prognosis
• Almost 60% to 70% of patients recover renal function
• Patients with post streptococcal crescentic GN have
a better prognosis
• The outcome in patients with Pauci-immune
crescentic GN, MPGN, and idiopathic RPGN is less
favorable than immune mediated RPGN.
• Poor prognostic factors include fibrous crescents,
tubular atrophy, interstitial fibrosis, and
glomerulosclerosis.

38. Post-Transplant Recurrence
• Graft losses are uncommon and occur in less than 5% of
cases.
Conditions associated with a high risk of histological
recurrence include
• MPGN type II,
• IgA nephropathy,
• Henoch Schönlein purpura
• SLE.

41. The outcome at lastfollowup, at 34 (19-72) months