WEGENER,S GRANULOMATOSIS

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Hematuria

Glomerular

arly-morning periorbital puffiness

•weight gain

•oliguria,

• the presence of dark-colored urine, and edema or hypertension

•painless

•joint pains

•skin  rashes

prolonged fever

Non glomerural

•history of passage of clots in urine

•fever

• abdominal pain

• dysuria, frequency, and  enuresis

•recent trauma to the abdomen

GLOMERULAR HEMATURIA

Isolated Renal Disease

•IgA nephropathy (Berger disease)  

 •Alport  syndrome (hereditary nephritis)

•Thin glomerular basement membrane nephropathy   

•Postinfectious GN (poststreptococcal GN)  

•Membranous nephropathy  

• Membranoproliferative GN  

• Focal segmental glomerulosclerosis  

• Antiglomerular basement membrane disease

GLOMERULAR HEMATURIA

Multisystem Disease

•Systemic lupus erythematosus nephritis

• Henoch-Schönlein purpura nephritis  

• Wegener granulomatosis   

•Polyarteritis nodosa   

•Goodpasture syndrome   

•Hemolytic-uremic syndrome   

•Sickle cell glomerulopathy  

• HIV nephropathy

Hematuria with familial association

Glomerular

- Benign Familial Hematuria ( Thin  Membrane

Disease )           

        

 - Alport  syndrome

 

 Non-glomerular

 - Idiopathic Hypercalciuria

 

 - Polycystic Kidney Disease

 

 - Urolithiasis

 

 - Tumors

 

Alport Syndrome

Its classically X-linked form, suggested by hematuria in a male.

Positive family history of hematuria, deafness, and renal failure.

Abnormal collagen IV composition.

 

Alport syndrome

- hereditary disorder of GBM

X-linked dominant

Autosomal recessive

Autosomal dominant

Hearing defects

Sensorineural bilateral

Never congenital

Boys- 85% . Girls – 18% < 15y

Progression of hearing loss parallels renal impairment

Diagnosis of Alport syndrome

Hematuria with or without  proteinuria

Hypertension

Renal failure

Ocular defects – anterior lenticonus

Familial hematuria

Sensorineural hearing loss

Progression to renal failure occurring in at least one affected subject

ANCA-associated” Disorders

•Wegener granulomatosis

• Microscopic polyangiitis

• Churg-Strauss syndrome

Wegener's granulomatosis (WG) and

microscopic polyangiitis (MPA)

•WG and MPA are related systemic vasculitides

•Both are associated with  (ANCA) and have similar

 features on renal  histology(a focal necrotizing, pauci-immuneglomerulonephritis that is characterized by necrotizing glomerulonephritis with little or no deposition of immunoreactants (IgG, IgM, IgA, and complement components).

•Approximately 90 to 95 percent of patients with active, generalized WG are ANCA-positive

 

•WG patients with ANCA, 80 to 90 percent, have PR3-ANCA(C-ANCA),and the remaining patients have MPO-ANCA(P-ANCA)

•Dual positivity for both PR3- and MPO-ANCA may rarely

•Approximately 70 percent of patients with MPA are ANCA-positive. In contrast to WG, most ANCA-positive MPA patients have P-ANCA, with a minority having C-ANCA.