An 8 year old child is brought with c/o severe abdominal pain. O/E palpable purpura is noted. There is history of recent diarrhea which is completely resolved. What is the expected pathological finding in kidney?
Correct Answer: Mesangial deposits of IgA on electron microscopy
Description: HENOCH SCHONLEIN (ANAPHYLACTOID) PURPURA • Most common systemic vasculitis in children
• 90% cases occur in the age group 3 to 10 years • Immune complex deposition causing small vessel vasculitis
• Many cases of HSP follow a documented upper respiratory infection Characterized by the tetrad of
• Palpable purpura (due to vasculitis, not due to thrombocytopenia) - occur in gravitydependent areas (lower extremities) or on pressure points (buttocks)
• Polyarthralgia in the absence of arthritis (non-deforming, oligoarticular, involves large joints, migratory)
• Gl symptoms (colicky abdominal pain, passage of blood and mucus per rectum, intussusception) - in 80% children
• Mild glomerulonephritis (IgA deposition in the mesangium, microscopic hematuria) - in 50% children
• Chronic renal disease develops in 1-2% of children with HSP • Approximately 8% of those with HSP nephritis go on to have end-stage renal disease
• Myocardial involvement can occur in adults but is rare in children Laboratory features • ANCA negative
• Leukocytoclastic vasculitis with IgA deposition in the small vessels in the skin, joints, gastrointestinal tract & kidney
• Deposition of C3, fibrin, and IgM are also seen to a lesser extent • Elevated ESR • Normal platelet count
• Normal complement levels Diagnosis, prognosis and treatment • No laboratory finding is diagnostic of HSP; The diagnosis of HSP is a clinical one
• Treatment for mild and self-limited HSP is supportive • Steroids are most often used to treat significant gastrointestinal involvement or other lifethreatening manifestations
• Predisone: useful in decreasing tissue edema, arthralgias, and abdominal discomfort; but does not alter overall prognosis nor prevent renal disease.
• The prognosis is excellent ❖ Deposits of IgA are also found in the glomerular mesangium in a variety of systemic diseases, including chronic liver disease,
Crohn's disease, gastrointestinal adenocarcinoma, chronic bronchiectasis, idiopathic interstitial pneumonia, dermatitis herpetiformis, mycosis fungoides, leprosy, ankylosing spondylitis,
relapsing polychondritis, and Sjogren's syndrome.
Category:
Pathology
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