Combination of glomerulonephritis and antiphospholipid syndrome nephropathy in systemic lupus erythematosus (review and case report)

Abstract

Combination of lupus nephritis and antiphospholipid syndrome nephropathy may occure in patients with SLE, complicated with antiphospholipid syndrome, in 32-68% of cases. Clinical manifestations of antiphospholipid syndrome nephropathy and proliferative lupus nephritis, such as arterial hypertension and progressive renal failure may be similar, and often only renal biopsy can help to make a proper diagnosis and choose adequate treatment strategy. Serial renal biopsies may help not only in assessing degree of activity and the progression of chronicity of lupus nephritis, but may also show possible class transformation. Transitions in lupus nephritis are not unusual and almost any class has been documented to go to any other class. Successful treatment of diffuse proliferative lupus nephritis may result in the transformation to membranous LN. The pediatric patient with biopsy proven LN class IV developed flare with dialysis-dependent renal failure due to superimposition of thrombotic microangiopathy. The case illustrates the difficulties of diagnostics, only the second biopsy, performed 7 months later and after 4 month of aggressive immunosupression with steroids and cyclophosphamide, indicated the presence of thrombotic microangiopathy with glomerular ischemia and transformation of the LN to class V. Interestingly, there were no other clinical symptoms of APS, anticardiolipin antybodies were in normal range, and only presence of lupus anticoagulant, found after second biopsy, supported diagnosis of APS. Addition of long-term anticoagulant therapy lead to partial restoration of renal function, so that patient became dialysis-free after 22 months of treatment. Supportive treatment with low-dose steroids, mycophenolates and warfarin allows to maintain remission over 18 months.