Case 19: Progressive renal failure in middle aged female with SLE, seropositive rheumatoid arthritis, limited scleroderma and Sjogren’s syndrome
To preserve patient privacy and for didactic purposes, case descriptions and pathology reports have been anonymized and partially fictionalized. The pathology images are representative images from a mixture of similar cases.
Ali Poyan MehrCase Presenter | Beth Israel Deaconess Medical Center, Boston, MA, USA | Conflict of interest reported: Mallinckrodt - Glomerular Disease Consultant and Research Grant
Mihran NaljayanNephrology Discussant | Louisiana State University Health Sciences Center, New Orleans, LA, USA | No conflict of interest reported
Patient is a 55-year-old female with a history of SLE and seropositive rheumatoid arthritis, limited scleroderma and Sjogren’s syndrome and idiopathic interstitial lung disease. She was diagnosed with lupus in her 20s, primarily in form of arthropathy. In 2004, she had a renal biopsy and reportedly diagnosed as class V lupus nephritis. She was not treated at that time (patient moved, and did not initiate follow up. Her care has been scattered across several institutions and changes in providers).
Her baseline creatinine was around 0.6-0.9 over the years, stable. She had a moderate degree proteinuria of around 2 g throughout 2002 all the way until 2013 when it slowly improved.
Physical Exam: BP: 123/81. Heart Rate: 106. O2 Saturation%: 98. Perioral wrinkling of her lips, Conjunctivae pale. Chest, mild telangiectasias; fingers unremarkable. No lower extremity edema, no rashes. No oropharyngeal ulcers. Nails benign. Heart: Tachycardic with a 2/6 systolic murmur. Lungs: Clear.
Laboratory and other data: On initial presentation in 2002: Anti-Cardiolipin IgG 43 Anti-MI2 Positive Anti-SM 40 Anti-RNP 182 Anti-Centromer 34</span Anti-scl.70 21 RF 1:256 Actin Ab 24 Date – Serum creatinine – 24-hour urinary protein – C3/C4 – Anti-dsDNA – ANA
2/2002 – 0.7 mg/dl – 2.3 g – 79/8 – 66 – n/a
8/2004 – 0.9 mg/dl – 2.7 g – 144/19 – 0 – n/a
8/2006 – 0.9 mg/dl – n/a – n/a – 86 – 995
3/2007 – 0.8 mg/dl – n/a – n/a – 127 – n/a
7/2008 – 0.7 mg/dl – n/a – n/a – 58 – n/a She was started sometime in 2009/2010 on mycophenolate and prednisone but stopped on her own after couple weeks
9/2010 – 0.8 mg/dl – 0.28 g – 144/14 – 2 – n/a
10/2013 – n/a – 0.3 g – n/a – 2 – n/a
1/2015 – 1.6 mg/dl – 0.8 g – 113/17 – 2 – n/a
3/2015 – 1.9 mg/dl – n/a – 146/22 – 2 – n/a 4/2015: She underwent a renal biopsy:
Class III lupus nephritis: 8/55 glomeruli globally sclerotic. Also, showed mild focal interstitial inflammatory infiltrates composed of lymphocytes, plasma cells, and few neutrophils.
She was started on mycophenolate 1500 mg BID and prednisone 60 mg daily
6/2015: Serum creatinine 2.1 mg/dl
11/2015: Serum creatinine 1.6 mg/dl 12/2015: She once again completely discontinued meds on her own due to steroid side effects and fear of immunosuppression.
4/2016: Serum creatinine 2.1 mg/dl, C3/C4 97/15 Anti-dsDNA 21
8/2016: Serum creatinine 2.7 mg/dl, Anti-dsDNA 39
9/2016: Serum creatinine 3 mg/dl She was started again on mycophenolate 1500 mg BID and prednisone 40 mg daily.
Urinary exam notable for numerous white blood cells, 1-3 non-dysmorphic RBCs. Dipstick, trace heme, positive for 5-10 leukocytes phpf.
Short term follow up: Creatinine improved, malaise improved, but significant Cushing’s syndrome and diarrhea and fatigue remained persistent issues. Change to mycophenolate sodium 720mg twice daily and down titration of prednisone to 20 mg daily only, marginally improved her complaints. She feels chronically unwell and does not look “well”.
Pathology images pending
04/2015: Class III lupus nephritis: 8/55 glomeruli globally sclerotic. Also, showed mild focal interstitial inflammatory infiltrates composed of lymphocytes, plasma cells, and few neutrophils.She was started on mycophenolate 1500 mg BID and prednisone 60 mg daily.
09/2016: Re-biopsied: Classification Class IV-G Diffuse Lupus Nephritis (C). 18 glomeruli: 12 are globally sclerotic and 2 are segmentally sclerotic. Of the remainder, 0 show fibrinoid necrosis and/or cellular crescents, and 0 show endocapillary proliferation. Jones stain reveals that less than 50% of the glomeruli show the majority of their capillaries with membranous type changes.
Questions posed & summary of key discussion points
1. What alternative regimen if any would you choose for her current presentation (also consider her advanced sclerosis)?
Author(s) of case summary:
Case summary pending
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