CC Nov 2015 IgA Nephropathy

echoecho's version from 2015-12-14 14:19


Question Answer
What is the most common cause of chronic glomurolnephritis world-wide?IgA nephropathy (IgAN)
Variable clinical features?microscopic hematuria to rapidly progressive GN
What symptoms do most young adults with IgAN present with? These symptoms were coincident with what infection?1) gross hematuria 2) upper respiratory or gastrointestinal infection
*** What is currently the only definitive way to diagnose IgAN?kidney biopsy
Is IgAN (along with related disorders such as Henoch-Schonlein purpura) an autoimmune disease?yes
In IgA Nephropathy, what are the offending antigens?circulating IgA1 antibodies with galactose-deficient carbohydrate side chains
REcognition of this abnormal galactose-deficient IgA1 by naturally occurring antibodies leads to formation of what?circulating immune complexes
These complexes are deposited where and cause what?1) glomeruli 2) inflammation
Why is kidney biopsy used for definitive diagnosis and not serum levels of galactose-deficient IgA1?because even though the galactose-deficient IgA1 levels are elevated in these patients, the sensitvity and specificity of theis test is diagnostically insufficient to replace kidney biopsy
Serum IgA1 autoantibody levels correlate with what? This level is useful for what two things?1) risk of progression to end-stage renal disease 2) useful for monitoring disease actvity and response to therapy
Define CKD (chronic kidney disease)?presence of abnormalities of kidney structure or function for > 3 months
How is CKD classified or staged?based on GFR and magnitude of albuminuria
IgAN patients with acute exacerbations and macroscopic hematuria often experience a transient decrease in what?GFR
Spontaneous recovery usually occurs within a ____ weeks?few
What should be repeated, together with an assessment of urinary excretion before determining a CKD stage?serum creatinine
*** The course of IgAN is variable, explain?some patients have prolonged clinical remissions, while others develop progressive renal failure
What can occur in 30% of patients by 20 years after dx?end-stage kidney disease
List the 3 patient characteristics which tailor treatment for patients? Which of these three best predict progressive disease?1) urinary protein excretion 2) HTN 3) severity of histologic lesions (best predicts progressive disease)
List symptoms of patients with mild disease? Treatment?1) normal BP, estimated GFR and urinary excretion 2) monitored periodically but do not require treatment
Those with HTN > 130/80 or proteinuria should be treated with what?ACEI or ARBs and possibly fish oil
Randomized trial results of use of fish oil?inconsistent
When is corticosteroid therapy inidicated?persistent proteinuria > 1 g/day
Measurement of what level is an important test for all forms of GN?serum C3
What is the result of serum C3 in IgAN?normal
List the diseases where the C3 is decreased?1) post-infectious 2) membranoproliferative GN 3) SLE associated GN
What characteristics are suggestive of Alport syndrome or hereditary nephritis rather than IgAN?family hx of GN (particularly if X-linked and associated with hearing loss)
Only a very small percentage of IgAN patients have a relative with biopsy-confirmed IgAN, true or false?true
What is the mode of inheritance?autosomal dominant with incomplete penetrance.
*** SUMMARY = What is type of nephropathy is the most common cause of chronic GN worldwide?IgA nephropathy (IgAN)
*** SUMMARY = List the patient characteristics that treatment is individualized to?1) urinary protein excretion 2) HTN 3) severity of histologic lesions (best predict progressive disease)
*** SUMMARY = What are the patient charactistics that should have patient be treated an ACEI or ARB?> 130/80 mmHg