GlomerulonephritisGlomerulonephritis

Michael PakdamanMichael Pakdaman

MS - 3MS - 3

Glomerulus – Anato / HistoGlomerulus – Anato / Histo

Nephr I / O tic ????Nephr I / O tic ????

Nephr I / O tic ????Nephr I / O tic ???? NephrOtic (PrOtein)NephrOtic (PrOtein) 3 Systemic Diseases3 Systemic Diseases

– DiabetesDiabetes– SLESLE– AmyloidosisAmyloidosis

1 “membrane”1 “membrane”– Membranous GNMembranous GN

2 others2 others– Minimal ChangeMinimal Change– Focal SegmentalFocal Segmental

NephrItic (RBC +/-casts)NephrItic (RBC +/-casts) 3 Autoimmune3 Autoimmune

– Poststrep GN (Type III)Poststrep GN (Type III)– IgA Nephropathy (Type IgA Nephropathy (Type

III)III)– Goospasture’s (Type II)Goospasture’s (Type II)

1 “membrane”1 “membrane”– MembranoproliferativeMembranoproliferative

2 others2 others– CrescenticCrescentic– Alport’s (collagen IV defect)Alport’s (collagen IV defect)

HypersensitivityHypersensitivityEssentials of GNEssentials of GN

Type I – IgE cross-linking on presensitizes mast cells Type I – IgE cross-linking on presensitizes mast cells inflammatory mediators releasedinflammatory mediators released

Type II – Antibodies directed against Type II – Antibodies directed against specific “enemies.” Damage cells via specific “enemies.” Damage cells via complement mediated “MAC” complement mediated “MAC” Inflammatory response NOT necessarily Inflammatory response NOT necessarily presentpresent

Type III – Immune complex deposits (eg Type III – Immune complex deposits (eg SLE) SLE) activates complement activates complement C5a C5a chemotactic to neutrophils chemotactic to neutrophils damage damage

Type IV – T-cell mediatedType IV – T-cell mediated

NephrOticNephrOtic Membranous (#1)– Type III HSMembranous (#1)– Type III HS

– Immune deposits IN the GBMImmune deposits IN the GBM– Assoc w/ hepatitis B antigenemia, autoimmune diseases, Assoc w/ hepatitis B antigenemia, autoimmune diseases,

thyroiditis, malignancies, pharm (gold, thyroiditis, malignancies, pharm (gold, penicillaminepenicillamine, , captoprilcaptopril, NSAIDs). , NSAIDs).

Minimal Change Disease (#1 Kids)Minimal Change Disease (#1 Kids)– ““lipoid nephrosis (why???)”lipoid nephrosis (why???)”– Tx w/ steroidsTx w/ steroids

Focal Segmental GlomerulosclerosisFocal Segmental Glomerulosclerosis– Presents w/ acute onset; Glomerular “scarring” Presents w/ acute onset; Glomerular “scarring” – Mutliple etiology: Podocyte injury, Nephron loss, Renal Mutliple etiology: Podocyte injury, Nephron loss, Renal

vasodilatation (diabetic nephropathy, sickle cell, obesity, vasodilatation (diabetic nephropathy, sickle cell, obesity, Von Gierke’s, Pregnancy, Obesity, Healing of prior Von Gierke’s, Pregnancy, Obesity, Healing of prior inflammatory injury)inflammatory injury)

NephrOtic – Systemic DiseaseNephrOtic – Systemic Disease

Diabetes – (1) GBM thickening, (2) Diabetes – (1) GBM thickening, (2) KW Nodules, (3) DIFFUSE glomerular KW Nodules, (3) DIFFUSE glomerular sclerosissclerosis

SLE (Type III) – SubENDOthelial SLE (Type III) – SubENDOthelial depositsdeposits

Amyloidosis Amyloidosis

“ “apple green”apple green”

NephrNephrIItic – Autoimmunetic – Autoimmune

Poststrep GN (#1 acute) – type III Poststrep GN (#1 acute) – type III (“small” – sub(“small” – subepiepithelial “humps”) – thelial “humps”) – follows sore throat or cellulitis follows sore throat or cellulitis – Peripheral & periorbital edema Peripheral & periorbital edema

(autoimmune)(autoimmune) IgA – post-infectious – type IIIIgA – post-infectious – type III

– Mild, self-limiting, assoc w/ Henoch-SchMild, self-limiting, assoc w/ Henoch-Sch Goodpastures – type IIGoodpastures – type II

– Men in mid 20’sMen in mid 20’s

NephrNephrIItic – Other 3tic – Other 3

Membranoproliferative – MESANGIAL Membranoproliferative – MESANGIAL CELLS proliferate. Assoc w/ Hep C, CELLS proliferate. Assoc w/ Hep C, SLE, a1-antitrypsin.SLE, a1-antitrypsin.

Crescentic GN – Fibrin deposition in Crescentic GN – Fibrin deposition in Bowman’s. Assoc w/ post-strep & Bowman’s. Assoc w/ post-strep & membranous GN.membranous GN.

Alport’s – Hereditary, type IV Alport’s – Hereditary, type IV collagen defect, CN VIII defectivecollagen defect, CN VIII defective

MembranoproliferativeMembranoproliferative“tram-tracking”“tram-tracking”

Linear – Type IILinear – Type II

Goodpasture’sGoodpasture’s(anti-GBM)(anti-GBM)

Capillary BM of glomerulus & alveolar wallsCapillary BM of glomerulus & alveolar walls

Granular – Type IIIGranular – Type IIIIgA NephropathyIgA Nephropathy

(mesangial (mesangial deposits)deposits)

Post-Strep GNPost-Strep GN(Sub(Subepiepithelial)thelial)

Membranous GNMembranous GN(deposits are (deposits are inin the the

GBM)GBM)

SLE GNSLE GN(Sub(Subendoendothelial)thelial)

Colon CA (anti-CEA Colon CA (anti-CEA deposits)deposits)