53 yo female referred for elevated SCr (178 mmol/l, 28 ml/min) and change in symptoms….? connective tissue disease Dx of hypocomplementemic urticarial.

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Presentation transcript:

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

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

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

IgG

IgG

C3

Clq

Clq

Kappa

Lambda

Fibrin

Albumin

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.