Case 25: Young female with SLE (class IV lupus nephritis, dx 2007) with Evans syndrome and active urinary sediment
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
Liz LightstoneNephrology Discussant | Imperial College, London, UK | No conflict of interest reported
Patient is a 20-year-old female with history of systemic lupus and a diagnosis of class IV lupus nephritis at age 11, who presents with SOB, and chest pain.
On her first presentation many years ago, she received induction therapy with cyclophosphamide and prednisone and ultimately transitioned to mycophenolate. She self-discontinued this treatment and prednisone and hydroxychloroquine a couple years later.
Approximately one year ago, she restarted hydroxychloroquine for arthralgias, and 6 months ago, she started oral prednisone 40 mg daily for arthralgia and sulfamethoxazoletrimethoprim for prophylaxis. She also was found to have new proteinuria (0.5 g/g) and was referred for nephrology outpatient evaluation.
She is now presenting with shortness of breath and pleuritic chest pain. She is found to be anemic, with Hb 5 g/dl, thrombocytopenic with platelets of 46 k/microliter.
She is started on pred 60 mg b.i.d. for presumed Evans syndrome with immune mediated hemolytic anemia and immune thrombocytopenia. Within the first week, she showed response in Hb/plt levels, but also rapid weight gain, ongoing arthralgias, and insomnia.
Medical history: Recent Strep. Pneumo after starting prednisone Obesity Asthma Hypertension
Medications: Albuterol Inhaler Atovaquone Suspension Folic Acid Hydroxychloroquine Omeprazole Prescription pain medication for severe pain Acetaminophen PredniSONE 60 mg PO BID Calcium Carbonate Vitamin D
Physical Exam: BP 138/97, heart rate 98, O2 98 RA Severe obesity, abdominal striae No rash. No cardiac rub, and otherwise unremarkable
Laboratory and other data: CRP 68 mg/l C3 60 mg/dl (90-180 mg/dl) C4 4 mg/dl (10-40 mg/dl) dsDNA titer 1:1280 Urine: protein to creatinine ratio 0.9 g/g Urine sediment with numerous RBCs, few acanthocytes, 2 WBC casts
Pathology images pending
Class IV lupus nephritis, diagnosed 10 years ago (full report not available).
Questions posed & summary of key discussion points
1. What are the therapeutic options for this patient given the current renal findings in the context of overall presentation and past tissue diagnosis? 2. How would you treat initially, and how would you manage long term? 3. Is a renal biopsy crucial at this point, and if so, at what point would you consider pursuing?
Author(s) of case summary:
Case summary pending
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