Dx of hypocomplementemic urticarial vasculitis ~ 15 yrs ago renal Bx same time  MPGN

1. 53 yo female referred for elevated SCr (178 mmol/l, 28 ml/min) and change in symptoms….? connective tissue disease • Dx of hypocomplementemic urticarial vasculitis ~ 15 yrs ago • renal Bx same time  MPGN • ? cryoglobulins (+) • C1 esterase inhibitor low • given steroids+cyclophosphamide twice between 1995-97 • on dapsone since 1999 • SCr since 2002 130-180 mmol/l • was “severely nephrotic” at the time • SCr now 149 mmol/l • urine now • 3+ hematuria, 1+ proteinuria, • P:C ratio 42 mg/mmol • Hgb 85-95 x 10+yrs • microcytic, low retics • C3 0.42 (), ANA neg, anti dsDNA Ab neg, ENA neg • PTT, PT normal

2. U05-309#015007112881 Previous Dx of hypocomplementemic urticarial vasculitis. Now ?Lupus. (Pre kidney Bx ~ 10 yrs ago → not definitive). Native L kidney.

9. IF • IgG- Moderate coarsely granular capillary loop staining. Moderate granular peritubular staining • IgA- Negative • IgM- Negative • C3- Mild to moderate granular capillary loop staining. Mild to granular peritubular staining • C1q- Moderate granular capillary loop staining. Moderate granular peritubular staining • Kappa- Moderate coarsely granular capillary loop staining. Mild granular peritubular staining. • Lambda- Trivial to mild granular capillary loop staining • Fibrin- Trivial to mild interstitial staining • Albumin- Mild hyaline droplet change in tubular cytoplasm

10. IgG

11. IgG

12. C3

13. Clq

14. Clq

15. Kappa

16. Lambda

17. Fibrin

18. Albumin

26. Diagnosis:Renal Biopsy: Biopsy of marginal adequacy (only two glomeruli by light microscopy) with changes compatible with diffuse proliferative lupus nephritis of mild activity and moderate chronicity with extensive peritubular capillary deposits with fingerprint-like substructure diagnostic of SLE.