Case Presentation:

We report a unique case of a patient with SLE, manifesting as acute renal failure, thrombocytopenia, and altered mental status. TTP–Like Syndrome may present with a myriad of symptoms and laboratory abnormalities. This case highlights the diagnostic challenges raised by the occurrence of microangiopathic hemolysis in the setting of multi–organ involvement in a patient with underlying SLE.

Discussion:

A 42–year–old African American female with a history of hypertension and systemic lupus erythematous presented with altered mental status, renal failure, and thrombocytopenia. Vitals signs revealed blood pressure of 161/113, pulse rate 153, respiratory rate 36, saturation 92% on 4 liters of nasal cannula. On examination, crackles present at both lung bases bilaterally, 3+ pitting edema in lower extremities. Notable laboratory studies were hemoglobin 9.2, wbc 11.2, platelets 138, BUN 37, creatinine of 2.6. CT of the head revealed no acute processes. Echocardiography revealed an EF 10–15% with global LV–hypokinesis. Lab studies showed low complement levels, a positive dsDNA antibody screen. Dialysis was initiated in addition to 5 rounds of plasmapharesis, 1 gm of methylprednisone/day for 3 days, mycophenolate mofetil, and plaquenel. Renal biopsy showed thrombotic microangiopathy with secondary segmental sclerosis and mild interstitial fibrosis and no evidence of lupus nephritis. On day 8, repeat echo showed ejection fraction of 35%, a lower serum creatinine, urine output improved. Subsequently dialysis was ceased.

Conclusions:

This case highlights the diagnostic challenge raised by the occurrence of microangiopathic changes in the setting of multiorgan involvement in patients with underlying SLE. Microangiopathic changes can be caused by active SLE, malignant hypertension, antiphopholipid syndrome, TTP; differentiation between these syndromes is difficult given their numerous overlapping clinical and laboratory features. Prompt institution of aggressive blood pressure control, plasma exchange, corticosteroids should be instituted within 24hrs of presentation as delay in treatment initiation may increase treatment failure.