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U06-16108

U06-16108. #652660020 2 month history of being unwell Vasculitic lesions on lower limbs ANCA positive Likely Wegener’s vs MPA. A 14 y/o male with 2 month history of intermittent purpuric lower limb rash. Seen by pediatrician and dx with HSP.

jeremy-butler
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U06-16108

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  1. U06-16108 #652660020 • 2 month history of being unwell • Vasculitic lesions on lower limbs • ANCA positive • Likely Wegener’s vs MPA

  2. A 14 y/o male with 2 month history of intermittent purpuric lower limb rash. Seen by pediatrician and dx with HSP. Other symptoms include: wt loss of 20 lbs, general malaise and fatigue. Denies any cough or hemoptysis, sinus or visual problems. Physical exam reveals a cachetic looking young teenager with normal vitals with normal bp, evidence of chronic papilledema (indicative of optic neuritis), and lower limb purpuric rash with arthritis in right ankle. Lab investigations shows : • normal Cr of 60, • presence of hematuria/proteinuria (urine pr/cr ratio of 95) • normal C3, C4, IgA. • Both p and c-ANCA were positive. • DsDNA normal at 2%. Patient was biopsied to r/o vasculitis - probably Wegener's Granulomatosis.

  3. IF • IgG- Mild mesangial staining. • IgA- Mild to moderate mesangial staining. • IgM- Mild mesangial staining. • C3- Mild to strong vascular staining. Trivial to mild punctate mesangial staining. • C1q- Negative. • Kappa- Mild mesangial staining. • Lambda- Mild to moderate mesangial staining. • Fibrin- Moderate interstitial staining. Moderate focal irregular staining of necrotizing foci within glomeruli. • Albumin- Moderate hyaline droplet change in tubular cytoplasm.

  4. IgG

  5. IgA

  6. IgM

  7. C3

  8. C3

  9. Kappa

  10. Lambda

  11. Fibrin

  12. Fibrin

  13. Albumin

  14. DiagnosisRenal Biopsy:Focal proliferative and necrotizing glomerulonephritis with crescent formation consistent with Henoch-Schönlein purpura.

  15. Pathologic differential diagnosis

  16. Underlying low grade IgA nephropathy with superimposed Wegener’s • « Although crescents and necrosis may be caused by severe IgA nephropathy alone, when they are conspicuous, the possibility of concurrent IgA nephropathy and ANCA disease should be considered. The likelihood of concurrent ANCA disease is highest when there are numerous crescents but only mild endocapillary hypercellularity. » Atlas of non tumor pathology, non-neoplastic kidney diseases, D’Agati et al. 2005.

  17. Underlying low grade IgA nephropathy with superimposed Wegener’s Elements in favor of the diagnosis • Relative paucity of IC deposits on EM • Absence of mesangial or endocapillary proliferation in remaining glomeruli

  18. Am J Kidney Dis, Oct 2000, vol 36(4):709-718.

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