This document provides an overview of glomerulonephritis and associated diseases. It defines glomerulonephritis as inflammatory conditions involving the glomeruli. It discusses the pathogenesis, clinical presentation, screening, outcomes, definitions of common renal syndromes, and causes of nephrotic syndrome. It also provides detailed descriptions of specific glomerulonephritis types including minimal change disease, membranous glomerulonephritis, focal segmental glomerulosclerosis, IgA nephropathy, mesangiocapillary glomerulonephritis, proliferative glomerulonephritis, diabetic nephropathy, crescentic glomerulonephritis, and glomerul

1. Glomerulonephritis-
Associated Diseases
Simplified Overview
Dr./ Sahar H. Mostafa
Elmataria Teaching Hospital-Cairo-Egypt

2. Abbreviations
• ACE-Is: angiotensin converting enzyme
inhibitors
• ACR: albumin/creatinine ratio
• ANCA: anti-neutrophilic cytoplasmic
antibody
• ARBs: angiotensin-receptor blockers
• CS: corticosteroids
• FSGS: focal and segmental
glomerulosclerosis
• GBM: glomerular basement membrane
• GN: glomerulonephritis
• HTN: hypertension
• MCD: minimal change disease
• MCGN: mesangiocappillary GN
• MGN: membranous GN
• MPGN: membranoproliferative GN
• PSGN: poststreptococcal GN
• RA: rheumatoid arthritis
• RVD: renovascular disease
• RVT: renal vein thrombosis
• UAER: urinary albumin excretion rate
• UFH: unfractionated heparin
• VUR: vesicoureteric reflux

3. Clinical background
• Glomerulonephritis is broadly defined as inflammatory;
however:
o No inflammation in MCD
o Other diseases exist which do not involve GN(ex: diabetic
nephropathy)
• Inflammation leads to proliferation of cellular
structures(mesangial/endothelial/epithelial) &/or
scarring)
• It may be:
o Idiopathic (1ry)
o 2ry to systemic diseases, drugs, ..

4. Pathogenesis
• Immune dysregulation:
a. Inappropriate response to “self”-Ag (ex: anti-GBM disease,
ANCA +ve vasculitis)
b. Ineffectual response to “foreign”-Ag (ex: MGN 2ry to HBV)
• Response:
a. Circulating immune complexes are deposited in glomeruli
(ex: SLE)
b. In-situ formation of immune complexes within glomeruli (ex:
anti-GBM disease)

5. Screening
• Dipstick for proteinuria (± 24-H quantification)
• Dipstick for hematuria (with urine microscopy for red
cells and casts)
• Assess for hypertension

6. Clinical Outcome
• It depends upon severity of tubulo-interstitial
damage rather than the extent of glomerular injury
• Control of BP (target < 130/80)
• ACE-Is / ARBs:
i. Decrease proteinuria
ii. Ameliorate progressive scarring

7. Definition of Common Renal Syndromes:
Asymptomatic proteinuria (< 3 gm/d)
Nephritic Syndrome (C.C. by HTN, hematuria and edema)
HTN
Nephrotic syndrome (> 3 gm proteinuria/d, S. albumin <2.5 gm/dl,
edema and hypercholestrolemia)
Hematuria (Microscopic/Macroscopic)
Acute and Chronic Renal Failure

8. CAUSES Of NEPHROTIC SYNDROME
Common Rare
 1ry GN
 DM
 BM nephropathy (e.g. Alport’s)
 Infections (e.g. Leprosy, malaria,
hepatitis B)
 Preeclampsia
 Accelerated HTN
 Myeloma
 Amyloidosis
 Drugs: Gold, penicillamine,
captopril, NSAIDs, Hg
 C.T. Diseases (e.g. SLE)
 VUR
 Constrictive pericarditis
 Sickle cell disease
 Allergies (e.g. Bee-sting,
penicillin)
 Hereditary GN (e.g.
Finnish-type nephrotic
syndrome)

9. Classification of GN / Syndrome Presentation
Proteinuria Nephrotic Nephritic Hematuria ARF CRF
MCD + +++ - - - -
MGN +++ ++ - ± - ++
FSGS ++ ++ ± - ± ++
Mesangial
IgA
+ + + +++ ± ++
MCGN ++ ++ + + + +
Diffuse
proliferati
ve GN
+ ± +++ ++ ++ +
Diabetic
glomerulo
-sclerosis
+++ ++ - - - +++
Crescentic
nephritis
+ ± +++ ++ +++ +
FS
proliferati
ve GN
+ ++ ++ ++ ++ +

10. 1- MCD
• Nephrotic presentation (80% in children & 28% in adults)
• Highly selective proteinuria (Ig G/transferrin <0.1)
• Renal function: Normal
• L/M: Normal
• E/M: Podocytes fusion
• Steroid-sensitive (short courses of high-dose prednisolone)
• Excellent prognosis
• Relapses (10%):
i. Cyclophosphamide (oral in children and pulsed iv in adults)
ii. If > 2-4 times/year: Cyclosporin (to avoid long-term steroid side
effects)

11. 2- MGN
• Commonest in adults (mid 20s & >60Ys)
• Presentation: nephrotic/asymptomatic proteinuria/CRF
• Histology: Granular Ig G deposits + Complement on GBM
(Sub-epithelial “spikes” with silver stain)
• Progression:
i. 1/3: CRF  ESRD
ii. 1/3: response to cytotoxic regimes (Ponticelli regime: chlorambucil alternating
with CS)
iii. 1/3: spontaneous remission
• RVT: in 5%, if s. albumin is < 2gm/dl, give prophylactic dose of
UFH or enoxaparin
• It may be:
i. 1ry
ii. 2ry (Malignancy/Chronic infections/CT diseases/Drugs)

12. 3- FSGS
1ry 2ry
<10% in children & elderly
Up to 20% in young adults
Presentation: proteinuria &/or
CRF
In children: identical to MCD
Ig M deposits
Ttt: moderate-dose steroids
for 3-6 Ms
? relapses
Heroin abuse
HIV
Obesity
Reduced functioning renal
mass (ex: after nephrectomy)
RVD (hemodynamic stress /
ischemic changes)
Ttt: optimal BP control with
ACE-inhibitors, or ARBs (no
immunosuppressives)

13. 4- Ig A nephropathy
• Commonest 1ry GN in adults
• Presentation: Hematuria
o Macroscopic or recurrent microscopic
o Synpharyngitic
• ↑ S. Ig A (50%)
• ? Associations: cirrhosis/dermatitis herpetiformis/coeliac disease/mycosis
fungoides
• Mesangial proliferation with Ig A deposits ± Crescents (similar to Henoch-
Schonlein nephritis)
• Ttt: Optimal BP control with ACE-Is or ARBs / Alternate-Day steroid for 6
Ms / omega-3 FAs
• Outcome:
o 25 %: progression to ESRD over 20 Ys
o Better prognosis: if < 1 gm proteinuria/day at time of presentation

14. PATHOLOGY ICGN Anti-GBM
Disease
PAUCI-IMMUNE GN OTHERS
CLINICAL
DISEASES
 Idiopathic
 PSGN
 SLE
 Cryoglob
ulinemia
 SBE
 Shunt
nephritis
 Ig A
nephropa
thy
 HSP
Goodpasture's
Syndrome
 Wegner’s
granulomatosis
 Microscopic PAN
 Malignant HTN
 HUS
 TTP
 IN
 Sclerosis crisis
 Atheroembolic disease
 Toxemia
C3 LOW Normal Normal Normal Normal
ANCA Negative Negative Negative POSITIVE Negative
Anti-GBM Abs Negative Negative POSITIVE Negative Negative
I/F Granular Ig and C3 Linear Ig G and
C3
Sparse/Absent Ig and C3
D.D. of nephritic syndromes

15. 5- MCGN (or MPGN)
• Presentation:
i. microscopic hematuria + low grade proteinuria
ii. Nephrotic syndrome (35%)
iii. CRF
iv. Rapidly deteriorating renal function (10%)
• 3 Types:
i. Type I and III: subendothelial & mesangial deposits ± mixed cryoglobulinemia
/ ? Association: HCV, HBV, SBE, SLE, Sjogren's, SCA, CLL, ..
ii. Type II: dense deposit in mesangium: double-contour of GBM / usually
familial and ? Associated with lipodystrophy or with factor H deficiency /↓
serum Complement, presence of circulating C3-nephritic factor
• Prognosis: Poor with 50 % progression to ESRD
• Ttt: steroids ? effective

16. 6- diffuse proliferative GN (or PSGN)
• Children/young adults are most often affected
• Presentation: nephritic syndrome/ARF
• Diffuse proliferation within glomeruli
• ? Preceeded by sore throat / impetigo
• Can be seen in SLE or Goodpasture's syndrome
• Serum C3 is low
• Spontaneous recovery, with restoration of full renal function, in
post-streptococcal cases

17. 7- Diabetic Nephropathy
• Higher prevalence in type II > type I
• Persistent albuminuria > 300 mg/24-Hs. = Total proteinuria >
500 mg/24-Hs)
• Screening: ACR of > 2.5 is taken as the Cut-off for
microalbuminuria in an early morning specimen
• Excess risk of mortality (microalbuminuria represents the renal
manifestation of generalized vascular endothelial dysfunction)
• Association with retinopathy (common basement membrane
pathology)
• Prevention:
o Tight glycemic control
o ACE-Is (Type I) / ARBs (Type II): renoprotective, independent
of HTN control

18. 8- RPGN (or Crescentic GN)
• RPGN: clinical description
• Crescentic GN: histological counterpart
• All age groups are affected
• Presentation: ARF / acute nephritic syndrome
• Causes:
I. Idiopathic (1ry)
II. 2ry: Goodpasture’s / ANCA +ve vasculitis / SLE
• Ttt: Aggressive chemotherapy ± plasma exchange
I. Induction therapy: pulsed iv methylprednisolone 1 gm/d/3-consecutive Ds;
followed by 1 mg/kg/d to be tappered over 6 Ms to 10-20 mg/d + 7 x 4 L.
plasma exchanges (in 1st 10-14 Ds) + iv pulsed cyclophosphamide 0.5-1
g/month for 6 Ms
II. Maintenance therapy: low-dose steroid + azathioprine, for 12-18 Ms

19. GN With Hypocomplementemia
§ SLE membranous/proliferative/crescentic)
§ Shunt nephritis (FSGS/MCGN), associated with coagulase-negative
staph. infection of ventriculo-atrial shunt
§ 1ry Complement deficiency (C1q / C2/ C4 deficiency)
§ Endocarditis (FS proliferative GN)
§ PSGN
§ MCGN
§ Cryoglobulinemia, especially Type II

20. PLASMA EXCHANGE IN RENAL DISEASES
AGREED BENEFIT UNCERTAIN BENEFIT
 Good-Pasture Syndrome
 ANCA-positive diseases;
mandatory if severe
pulmonary hemorrhage
 Dialysis-requiring ARF
 Idiopathic crescentic GN
 Cryoglobulinemia
 Myeloma; cases with
hyperviscosity
 HUS
 TTP
o SLE-nephritis (severe)
o Henoch-Schonlein
nephritis
o Myeloma; cases with
ARF due to cast
nephropathy

21. Self assessment

22. Question 1:
Which one of the following is true of renal involvement in
HIV infection?
a. Antiviral therapy is of little benefit in HIV-associated
nephropathy
b. HIV-associated nephropathy is indistinguishable
histologically from FSGS
c. HIV-associated nephropathy typically presents with
nephrotic-range proteinuria
d. Hypernatremia is common in HIV infection
e. Renal involvement is common in AIDS

23. Answer 1:
Which one of the following is true of renal involvement in
HIV infection
a. Antiviral therapy is of little benefit in HIV-associated
nephropathy
b. HIV-associated nephropathy is indistinguishable
histologically from FSGS .. (focal/global collapse of
capillaries)
c. HIV-associated nephropathy typically presents with
nephrotic-range proteinuria
d. Hypernatremia is common in HIV infection ..(hypo)
e. Renal involvement is common in AIDS .. (rare)

24. Question 2:
A 32-Y-old man has a 15-Y-history of diabetes; serum
creatinine is 2.3 mg/dl. A diagnosis of diabetic
nephropathy is established by renal biopsy
Which of the following is/are true of this patient?
a. ACE-Inhibitors are unlikely to help this condition at
this time
b. He has a less than 30% chance of having HTN at this
time
c. He has a 50% chance of having the nephrotic
syndrome at this time
d. Because of his age, nephropathy is likely to be his
only major organ system diabetic complication to
date
e. He is likely to experience ESRD requiring dialysis
within the next 4-Ys

25. Answer 2:
A 32-Y-old man has a 15-Y-history of diabetes; serum
creatinine is 2.3 mg/dl. A diagnosis of diabetic
nephropathy is established by renal biopsy
Which of the following is/are true of this patient?
a. ACE-Inhibitors are unlikely to help this condition at
this time
b. He has a less than 30% chance of having HTN at this
time .. (almost all)
c. He has a 50% chance of having the nephrotic
syndrome at this time .. (most)
d. Because of his age, nephropathy is likely to be his
only major organ system diabetic complication to
date .. (also retinopathy and other macrovascular
complications)
e. He is likely to experience ESRD requiring dialysis
within the next 4-Ys

26. Question 3:
A 23-Y-old medical student develops a viral URT infection
and notices that his urine becomes very dark. Dipstick
urinalysis shows blood+3, but no protein. BP is 124/74.
Examination is unremarkable and all routine
investigations are normal.
What is the most likely diagnosis?
a. Henoch-Schonlein syndrome
b. Mesangial IgA disease
c. ATN
d. Rhabdomyolysis
e. Membranous GN

27. Answer 3:
A 23-Y-old medical student develops a viral URT infection
and notices that his urine becomes very dark. Dipstick
urinalysis shows blood+3, but no protein. BP is 124/74.
Examination is unremarkable and all routine
investigations are normal.
What is the most likely diagnosis?
a. Henoch-Schonlein syndrome … (purpura preceeded by
abdominal pain, ..)
b. Mesangial IgA disease
c. ATN … (no hematuria)
d. Rhabdomyolysis … (myoglobinuria, impaired KFTs)
e. Membranous GN … (proteinuria, almost never hematuria)

28. Question 4:
A 36-Y-old man on polychemotherapy for a relapse of stage IIIb
Hodgkin presents with increasing shortness of breath. O/E:
Bilateral basal crackles and pitting edema of lower legs and
hepatosplenomegaly were found.
Na=132 mmol/l, K=5.1 mmol/l, Albumin=28 gm/l, Urea=13.6
mmol/l, Creatinine=197 µmol/l
CXR: Widened mediastinum, cardiomegaly and interstitial
pulmonary edema
Abdominal U/S: Hepatosplenomegaly and large kidneys without
evidence of obstruction
What is the likely diagnosis?
a. Renal lymphoma
b. Membranous GN
c. Cytostatic-induced Cardiomyopathy
d. Reactive amyloidosis
e. Renal vein thrombosis

29. Answer 4:
A 36-Y-old man on polychemotherapy for a relapse of stage IIIb
Hodgkin presents with increasing shortness of breath. O/E:
Bilateral basal crackles and pitting edema of lower legs and
hepatosplenomegaly were found.
Na=132 mmol/l, K=5.1 mmol/l, Albumin=28 gm/l, Urea=13.6
mmol/l, Creatinine=197 µmol/l
CXR: Widened mediastinum, cardiomegaly and interstitial
pulmonary edema
Abdominal U/S: Hepatosplenomegaly and large kidneys without
evidence of obstruction
What is the likely diagnosis?
a. Renal lymphoma
b. Membranous GN
c. Cytostatic-induced Cardiomyopathy
d. Reactive amyloidosis
e. Renal vein thrombosis

30. Question 5:
A 15-Y-old boy presented with arthralgia, skin rash and
hematuria. Renal biopsy showed focal necrotizing GN with
diffuse Mesangial IgA deposits
The most likely diagnosis is:
a. SLE
b. Henoch-Schonlein Purpura
c. Juvenile RA
d. Post-streptococcal GN
e. Goodpasture’s syndrome

31. Answer 5:
A 15-Y-old boy presented with arthralgia, skin rash and
hematuria. Renal biopsy showed focal necrotizing GN with
diffuse Mesangial IgA deposits
The most likely diagnosis is:
a. SLE
b. Henoch-Schonlein Purpura
c. Juvenile RA
d. Post-streptococcal GN
e. Goodpasture’s syndrome

32. Question 6:
A 20-Y-old hairdresser complains of having seen “blood in
his urine”. He relates that with an URT infection
Which one of the following findings is most likely to suggest
a non-glomerular source of bleeding?
a. Crenated (dysmorphic) RBCs
b. Red blood casts
c. Lipiduria
d. Proteinuria > 1 gm/24 Hs with hematuria
e. Eosinophils in the urine

33. Answer 6:
A 20-Y-old hairdresser complains of having seen “blood in
his urine”. He relates that with an URT infection
Which one of the following findings is most likely to suggest
a non-glomerular source of bleeding?
a. Crenated (dysmorphic) RBCs
b. Red blood casts
c. Lipiduria
d. Proteinuria > 1 gm/24 Hs with hematuria
e. Eosinophils in the urine

34. Question 7:
A 32-Y-old man presents with marked ankle swelling. BP is
168/90. Examination is otherwise unremarkable. Dipstick
testing of urine demonstrates: protein +++, with no
hematuria. Investigations demonstrate: plasma creatinine
of 164 µmol/l, albumin 16 gm/l and 24-H urinary protein
excretion of 8 gm.
Which one of the following statements is correct?
a. Barium enema should be performed to exclude carcinoma
of the colon
b. The renal lesion is characterized by subendothelial deposits
on biopsy
c. Male sex, hypertension and renal impairment at
presentation are poor prognostic indicators
d. There is a recognized association with hepatitis C
e. The natural history of this condition is characterized by a
rapid decline of renal function

35. Answer 7:
A 32-Y-old man presents with marked ankle swelling. BP is
168/90. Examination is otherwise unremarkable. Dipstick
testing of urine demonstrates: protein +++, with no
hematuria. Investigations demonstrate: plasma creatinine
of 164 µmol/l, albumin 16 gm/l and 24-H urinary protein
excretion of 8 gm.
Which one of the following statements is correct?
a. Barium enema should be performed to exclude carcinoma
of the colon .. (patient is young)
b. The renal lesion is characterized by subendothelial deposits
on biopsy .. (subepithelial)
c. Male sex, hypertension and renal impairment at
presentation are poor prognostic indicators
d. There is a recognized association with hepatitis C .. (HBV)
e. The natural history of this condition is characterized by a
rapid decline of renal function