A challenging case: when Lupus and ANCA antibodies are positive with AKI and a catastrophic biopsy
Main Author: Nihal Mustafa Hamid Bashir
Al Ain, United Arab Emirates
SEHA Kidney Care
Purpose Statement: It is difficult to diagnose lupus nephritis and ANCA vasculitis based on positive antibodies alone, kidney biopsy is very important. The overlap of rheumatoid arthritis and systemic lupus erythematosus is well described, with a syndrome known as ‘rhupus’. ANCA-associated vasculitis, however, is uncommonly associated with other autoimmune conditions. It is rare to have overlap between lupus nephritis and ANCA vasculitis, many case reports mentioned about C- ANCA and lupus nephritis coexisting but nothing was mentioned about C- ANCA and P- ANCA with positive lupus markers
Method(s): A 69-year-old female, is known to have Diabetes Mellitus, Hypertension, and dyslipidemia. Of note, she had a history of covid 19 infection in 2019 and hip joint replacement in 2022. She was diagnosed as having Systemic lupus erythematosus and started on Mycophenolate mofetil 500 mg twice a day and oral Prednisolone with +ve cANCA & pANCA she underwent kidney biopsy complicated with massive bleeding required embolization, unfortunately, the biopsy was inadequate. the patient’s renal function continued to deteriorate reaching end-stage renal disease and she was started on hemodialysis.
Other medical problems included pancytopenia with high kappa/lamba, and polyclonal gammopathy, suggested to be related to viral and /or hydralazine despite that, Anti Histone AB was negative.
she was recently admitted with spinal abscess at T5-T6 level, with spinal cord compression requiring urgent evacuation, unfortunately, she is paraplegic now.
Result(s): the patient stabilized her creatinine to the range of 250-350 micromol/L however she is still dialysis dependent despite having urine production >500 ml/day. her immunosuppression medications initially included intravenous pulse steroids and mycophenolate mofetil dose of 1500 mg twice daily, and it was reduced gradually to 500 mg bid after her spinal abscess.
Conclusion(s): Complex cases require complex investigations, but when kidney biopsy is inadequate and complicated, treatment is very difficult and the balance between immunosuppression and prevention of opportunistic infections is very difficult.