GLOMERULONEPHRITIS

Ali aL-ahrash, MD, PhD

Azzawyia kidney center

DEFINITION

A group of disorders in which:

Symmetrical involvement of both kidneys

Glomerular injury caused by immune depositin

Renal involvement as part of systemic diseases

characterized by inflammation either of the

glomeruli or small blood vessels in the kidneys,

but not all diseases necessarily have an

inflammatory component.

CLASSIFICATIONS

Affected kidney

Focal

Some glomeruli

Diffuse

All glomeruli

Affected glomerulus

Global

The whole glomerulus

Segmental

Part of the glomerulus

CAUSES

Unknown

Antigen derived from:

bacteria, B, HS, staph, salmonella

virus, measles, mumps, HBV

parasite, malaria, schistosoma

drugs, penicellamin

from the host it self, SLE,malignant tumor

If the body failed to clear the circulating immune

complexes or dose not have the ability to produce

appropriate antibodies these mechanisms are

triggered by:

Complement activation

Fibrin deposition

Platlet aggregation

Inflammation with neutrophil-dependent mechanism

Activation of kinin system

Primary GN are intrinsic to the kidney.

Secondary GN are associated with certain

infections (bacterial, viral or parasitic

pathogens), drugs, systemic disorders (SLE,

vasculitis), or diabetes.

PATHOLOGY

The pathological mechanisms are:

Immune complex deposition or

insitu immune complex formation,

the antigens are :

Exogenous, e.g bacteria, virus

Endogenous, antibody against host DNA

Deposition of the anti basement membrane

antibodies, e.g GPS

SITES OF IC DEPOSITIONS

IMMUNE COMPLEX DEPOSITION

NO

MC GN

FSGS

YES

MGN

IGA

MPGN

RPGN

HISTOLOGICAL CLASSIFICATIONS

1 Thin Basement Membrane Disease

2 Non Proliferative 2.1 Minimal change GN (also known as Minimal

Change Disease)

2.2 Focal Segmental Glomerulosclerosis (FSGS)

2.3 Membranous glomerulonephritis

3 Proliferative 3.1 IgA nephropathy (Berger's disease)

3.2 Post-infectious

3.3 Membranoproliferative/mesangiocapillary GN

3.4 Rapidly progressive glomerulonephritis

THIN BASEMENT MEMBRANE DISEASE

BENIGN FAMILIAL HEMATURIA

WHAT HAPPENED AFTER IMMUNE RESPONSE?

Cellular changes

proliferative

Glomerular cells enlargment

Inflammatory cells infiltration(exudative)

Non proliferative

MINIMAL CHANGE DISEASE

90% OF CHILDREN, 10% OF ADULT cause of NS

Absence of histologic abnormality on LM, fusion

of foot process of podocytes on EM

T cell mediated immunity against glomerular

epithelium (NO immune complex deposition)

FOCAL SEGMENTAL GLOMERULOSCLEROSIS

(FSGS)

Increase incidence in both child & adult

More in males

More in blacks

In US the most common cause of GN leading to End stage CKD

HTN is found in 30-50% of child &adult

Presence of some permeability factors are responsible for recurrence of the disease after transplant

Majority will proceed for End stage ,5-25% got spontaneous remission

MEMBRANOUS GLOMERULONEPHRITIS

Thickning of glomerular basal membrane

Characterized histological by deposition of IgG &IC on subepithelial space as a spikes

70-80% primary, 20-30% secondary

Uncommon in chlidren less than 5%

Leading cause of NS (30-50%)

30% spontnous remission, 30% progress to end stage, 30% stable, 10% died due to non renal causes

MEMBRANOPROLIFERATIVE/

MESANGIOCAPILLARY GN

Diffuse proliferation of capillary loops

Hypocomplementimia GN

Idiopathic or secondary to chronic infection, cryoglobulinemia or systemic immune disorder

Type I; subendothelial depostion

Type II (DDD); mesangial and glomerular basement membrane deposition

Type III; subendo & subepithelial deposition with marked disruption of basement membrane

Idiopathic MPGN carry poor prognosis in both

untreated child &adullt, it progress to end stage

Has high recurrence rate after tx

IgA nephropathy (Berger's disease)

Mesangial proliferation due to massive IgAdeposition

10-40% of renal biopsies

Disease of men between 20-40 ys

Diagonosis by immunoflurecent microscope

More prevalent in westren &Asia

Benign recurrent hematuria was used befor

Its an important cause of end stage

Macroscopic Hematuria &HTN following URTI

Reccure after tx

POST-INFECTIOUS GN

Charactarized by diffuse exudative

endocapillary GN after bacterial infection

sub epithelial electron dense deposition in a

form of humps

The AG is not well recognized, only

streptoccocal AG

It’s a disease of children

Ac. NS after 2 wks after throat or skin infection

RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS

Acute GN, followed by rapid drop of GFR in days

presence of intra &extra capillary proliferation in more than 50% of the glomerlulus in a form of crescent formation

Usually associated with system immune disease like, ANCA, ANA, Anti GBM Ab, SLE

Poor remission

Preservation of kidney size

CLINICAL PRESENTATION OF GN

it may present with;

isolated hematuria

and/or proteinuria

or as a nephrotic syndrome,

or nephritic syndrome,

or acute renal failure,

or chronic renal failure.

ACUTE NEPHRITIC SYNDROME

is a collection of signs (known as a syndrome) associated with disorders affecting the glomeruli.

It is characterized by having a thin glomerular basement membrane and small pores in the podocytes of the glomerulus,

leading to proteins and red blood cells to pass into the urine.

nephrotic syndrome is characterized by only proteins moving into the urine.

Both nephritic syndrome and nephrotic syndrome result in hypoalbuminemia due to protein albumin moving from the blood to the urine.

hematuria, with red blood cell (RBC) casts in

the urine

Proteinuria (<3.5 g/day)

Hypertension - mild

Uremia

oliguria (low urine output <400 mL/day)

CLINICAL PRESENTATION

Usually children with H/O 1-3 weeks URTI, otitis

media or cellulitis

Usually infection with group A beta HSC

INVESTIGATIONS

Urine for; RBC, Red cell castes, proteins

RFT

Throat swab for C/S

ASOT

ANA

C3

24 hrs urinary proteins

CXR

Renal biopsy

TREATMENT

Hospital management Daily in put /out put

Daily weighing

BP monitoring

Dietary control; protein in case of azotemia

Salt restriction

Fluid restriction

Diuretics therapy for HPN, fluid over load

Manag the life threatining complications like hypertensive encephalopathy, pulmonary oedema, uraemia accordingly

Prophylaxis therapy for individual at risk with penicillin

Needs follow up

BOOK SOURCES