CONSULT ROUNDS

TMA in Lupus. This is a controversial topic and evidence is not really present on what to do.  The topic was about using plasmapheresis for such patients.

Key Points
1. Lupus Nephritis and plasmapheresis --> the evidence is negative for use of it.
2. TMA in Lupus( evidence of schistocytes, LDH , low haptoglobin, thrombocytopenia, renal failure, proteinuria, hemolytic anemia) ---> plasmapheresis has shown some benefit if given early to reverse the TMA process and or renal function improvement( its all based on case series and case reports)
3. In prior cases, acute renal failure and MAHA resolved with use of pheresis, steroids or heparin. The cases reported provided further evidence that a TMA can cause acute renal failure independent of lupus nephritis. TMA should be distinguished from other forms of renal vascular disease, particularly a noninflammatory lupus microangiopathy, which is probably mediated by subendothelial immune-complex deposits. The absence of immunoglobulin deposits in vessels involved by a TMA indicates that microvascular thrombosis is promoted by mechanisms other than those usually attributed to immune-complex disease. Phospholipid reactive antibodies may be pathogenetic in some cases. ADAMTS-13 Antibodies might play a role in lupus TMA or possible role of anti endothelial antibodies. Removal of these antibodies with pheresis is the goal.
Anti CD20 agents might be helpful as well given their role in decreasing production of those antibodies!