53 yo female referred for elevated SCr (178 mol/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 mol/l

• was “severely nephrotic” at the time

• SCr now 149 mol/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.