NP22 Nephrology

Parenchymal Kidney Diseases

Essential Med Notes 2015

Glomerular Syndromes
1. ASYMPTOMATIC URINARY ABNORMALITIES
Clinical/Lab Features
proteinuria (usually <2 g/d) and/or microscopic or macroscopic hematuria
isolated proteinuria
can be postural
occasionally can signal beginning of more serious GN (e.g. FSGS, IgA nephropathy,
amyloid, diabetic nephropathy)
hematuria with or without proteinuria
IgA nephropathy (Bergers disease): most common type of primary glomerular disease
worldwide, usually presents after viral URTI
hereditary nephritis (Alports disease): X-linked nephritis often associated with
sensorineural hearing loss; proteinuria <2 g/d
thin basement membrane disease: usually autosomal dominant, without proteinuria;
benign
benign recurrent hematuria: hematuria associated with febrile illness, exercise, or
immunization; a diagnosis of exclusion after other possibilities are ruled out

Features of Nephritic Syndrome

PHAROH
Proteinuria
Hematuria
Azotemia
RBC casts
Oliguria
Hypertension

IgA nephropathy is the most common

type of primary glomular disease
worldwide

2. NEPHRITIC SYNDROME
ACUTE NEPHRITIC SYNDROME
a subset of nephritic syndrome in which the clinical course proceeds over days
Etiology
etiology can be divided into low and normal complement levels (see Figure 14)
frequently immune-mediated, with Ig and C3 deposits found in GBM
outcome dependent on etiology
Clinical/Lab Features
proteinuria (but <3.5 g/1.73 m2/d), abrupt onset hematuria (microscopic or macroscopic,
azotemia (increased Cr and urea), RBC casts and/or dysmorphic RBCs in urine, oliguria, HTN
(due to salt and water retention), peripheral edema/puffy eyes, smoky urine
Glomerulonephritis with Nephritic Features

Anti-GBM Mediated
(RPGN Type I) (15%)

Immune Complex Mediated

(RPGN Type II) (24%)

Non-Immune Mediated
(RPGN Type III) (60%)

Double Antibody Positive Disease

(RPGN Type IV)

Linear IF pattern due to IgG and C3

deposition along capillary loops

Granular pattern due to subendothelial

or subepithelial deposits of IgG and C3

Pauci-immune: no immune staining

Has features of Type I and Type III

Double antibody positive

anti-GBM +ve

With lung
hemorrhage

Without lung
hemorrhage

Goodpastures
disease

Anti-GBM
disease

C3 normal

Decreased C3

IgA nephropathy
Henoch-Schnlein
purpura

Membranoproliferative
GN
SLE
Infective endocarditis
Post-infectious GN
Cryoglobulinemia

ANCA +ve

c-ANCA +ve

p-ANCA +ve

Granulomatosis
with
polyangiitis

Churg-Strauss
Microscopic
polyangiitis

Figure 14. Approach to nephritic syndrome

RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS/CRESCENTIC

GLOMERULONEPHRITIS
a subset of nephritic syndrome in which the clinical course proceeds over weeks to months
clinical diagnosis, not histopathological
any cause of GN can present as RPGN (except minimal change disease)
additional etiologies seen only as RPGN: Goodpastures syndrome and granulomatosis with
polyangiitis (previously called Wegener's granulomatosis)