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Crescentic Glomerulonephritis Diagnosis in 54-Year-Old Male with Nephritic Urine

A 54-year-old Caucasian male presented with leg edema, skin rash, leg pain, and numbness. Eight weeks prior, he experienced leg swelling and rash, misdiagnosed initially as cellulitis. His lab tests revealed elevated creatinine (247) and proteinuria (314). A kidney biopsy on 25-OCT-06 indicated focal proliferative glomerulonephritis (GN) with unusual IgA staining, raising suspicion for Henoch-Schönlein purpura. Although the immunofluorescence pattern is atypical, it aligns with reported pediatric cases. Further analysis of subsequent biopsies indicated variable IgA deposition.

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Crescentic Glomerulonephritis Diagnosis in 54-Year-Old Male with Nephritic Urine

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  1. U06-20274 #324676110 • Creat 250 • Nephritic urine • ? Crescentic GN

  2. 54-year old Caucasian male • CC: Leg Edema, Skin Rash, Leg Pain and hands numbness. • HPI: 8 Wks prior to admission he noticed leg edema and rash. Treated with Cephazolin and cephalexin for few days for cellulitis. No URI, No Fever, No gross hematuria, No Sore throat. • PMH: Asthma (using combivent PRN)

  3. Labs: (on admission) • Hgb: 132 • Cr 247 (Baseline Cr: 110) • U/A: SG of >1.030 • Pro/Crea: 314.00 • ALT, AST and Alk.Pho: NL • dsDNA: Neg, • RF(Sep2006): 22 • ANA: NEG • p-ANCA and c-ANCA: NEG X 2 • C3 and C4: 1.33 and .91

  4. Kidney Biopsy on 25-OCT-06 ?

  5. IF • IgG-Negative. • IgA- Moderate irregular peripheral lobular finely granular staining. • IgM-Negative. • C3- Moderate irregular peripheral lobular staining. Moderate vascular staining. • C1q-Negative. • Kappa-Negative. • Lambda-Negative. • Fibrin- Mild interstitial staining. • Albumin-Negative.

  6. IgA

  7. IgA

  8. IgA

  9. IgA

  10. C3

  11. C3

  12. Fibrin

  13. EM • Will be ready in the coming weeks.

  14. DiagnosisRenal Biopsy: • Focal proliferative GN with peripheral lobular granular staining for IgA and possible arteriolitis with necrotizing changes. • Rule out Henoch-Schönlein purpura. Comment: the IF pattern is unusual but has been described in Henoch-Schönlein purpura.

  15. Series of 10 paediatric cases • A/a focal and segmental hypercellularity with # monocytes • IgA deposits at periphery of lobules, not in mesangium • Early phase of disease ? • 3/6 subsequent biopsy was normal (with no IgA deposition) • 3/6 subsequent biopsy with mesangial IgA

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