False about Henoch – SchönleinPurpura ? (LQ)
|A||It is generalized vasculitis causing GN, purpura, arthralgias and abdominal pain, occurring mainly in children.|
|B||Renal involvement is manifested by hematuria and proteinuria.|
|C||Serum IgA is normal or raised|
|D||Platelet count is reduced|
1. IgA Nephropathy (Berger’s Disease) (Ref. Hari- 18th ed., Pg. 2342)
a. Typically hematuria occur with in 1 to 3 days after URTI.
b. This is the most common form of primary glomerular disease in the world
c. This is most common form of primary glomerular disease causing hematuria in children in the world. (MCQ)
d. It progresses to end stage renal disease in 20 to 40% of patients affected over a 20 years period.
e. Gross, intermittent hematuria, which is glomerular, is the presenting symptom.
There is presence of abnormal proteinuria
1. IgA nephropathy may show mesangial widening and focal and segmental inflammation.
4. Electron microscopy confirms electron-dense deposits in the mesangium that may extend to the subendothelial area of adjacent capillary walls in a small subset of cases, usually those with focal proliferation.
Extra Edge: IgA nephropathy is a type of mesangioproliferative glomerulonephritis with IgA deposition in the mesangium.
Extra edge: Example of Mesangioproliferative glomerulonephritis.
Typical pathological findings
Typical clinical presentation
Proliferation of mesangial cells and deposition of IgA in matrix
Diseases Associated with IgA Nephropathy Q
i. Idiopathic (majority)
ii. Renal – limited or as component of Henoch – Schönleinpurpura
iii. In associated with systemic diseases or drugs
a. Liver Chronic liver disease with involvement of biliary tree
b. GIT Celiac disease, Crohn’s disease, adenocarcinoma
c. Respiratory Idiopathic interstitial pneumonitis, obstructive bronchiolitis, adenocarcinoma
d. Skin Dermatitis herpetiformis, mycosis fungoides, leprosy
e. Eyes Episcleritis, anterior uveitis
f. Miscellaneous Ankylosing spondylitis, relapsing polychondritis, Sjögren’s syndrome,monoclonal IgA gammopathy, schistosomiasis