22 year old lady with muscle weakness, renal insufficiency and proteinuria

freecme000
Published on Dec 12, 2022
CC 22-yr-old Hispanic lady presents with proximal muscle weakness, arthralgia, low-grade fevers, and dry mouth, eyes and difficulty swallowing.

EXAM - BP 142/92 mmHg , thick and tight skin on the fingers, oral ulcers. Rest WNL

LABS
>creatinine of 2.1 mg/dl,
>24-h urine protein of 2.6 g,
>UA - positive for RBC, no casts
>ANA is positive, with a speckled pattern at a titer of 1:1100.
>Anti– Dss DNA, Sm, Cardiolipin Ab /Hep/Anti GBM / ANCA/Cryoglobulin negative
>RNP-ENA antibodies and U1RNP antibodies are strongly positive,
>complement -mild decrease in C3/normal C4

RENAL BIOPSY
-Diffuse proliferative GN changes
-EM - extensive sub endothelial deposits.
-IF positive for C3 ,C1q, IgG

BEST NEXT STEP IN MANAGEMENT OF THIS PATIENT IS

A. Voclosporin plus MMF plus tapering steroids for 6 months followed by MMF maintenance
B.500 mg IV cyclophosphamide every 2 weeks for 6 doses followed by MMF maintenance
C. 3 gm per day MMF daily plus tapering oral prednisone for 6 Mo followed by MMF maintenance
D. Belimumab plus MMF for 6 Mo followed by MMF maintenance

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