Systemic lupus erythematosus (SLE) is a systemic connective tissue disorder involving multiple organs.We report a case of SLE with Posterior reversible encephalopathy syndrome (PRES) in the setting of diffuse proliferative nephropathy and Thrombotic thrombocytopenic purpura (TTP).
34 yr old asian female with no medical history presented with rash on face, and joint pains, for 1 week. Initial vitals signs were normal. Physical examination was normal except for oral ulcers and malar rash.Initial lab work up revealed normocytic anemia, renal failure with BUN/Cr –38/1.8, ESR –58 mm. A diagnosis of SLE was made based on high levels of ANA, dsDNA, Anti Smith antibodies and low complement levels. Renal biopsy revealed stage IV diffuse proliferative glomerulonephropathy. Patient was treated with high dose steroids. Clinical course—On hospital day 4, the patient developed severe headache, blurred vision which was followed by generalized tonic clonic seizures. Patient was treated with dilantin and transferred to the MICU. MRI of head revealed multiple areas of increased T2 signal in cortex and subcortical white matter in the temporal-occipital and posterior corpus callosum without evidence of vasculitis consistent with PRES. Blood pressure was tightly controlled with maintainance of systololic BP between 120–130 mmHg with the use of nicardipine infusion. Her symptoms gradually resolved over next 7 days. Her BP was maintained in stated range with the use of labetalol and hydralazine. MRI repeated with in 1 week revealed resolution of white matter changes. During her ICU stay, the patient also developed severe anemia and thrombocytopenia. Peripheral smear showed schistocytes consistent with hemolytic anemia and platelet count < 10K. A presumptive diagnosis of TTP was made and later confirmed with low ADAMTS-13 activity and patient was treated with plasmapheresis. Hematologic indices initially improved with daily plasmapheresis, but later relaped requiring 3 courses of Rituximab. Patient continued to have worsening renal function despite treatment with steroids and cyclophosphamide requiring hemodialysis. At present patient remains on hemodialysis for end stage renal disease due to glomerulonephropathy but with normal hematologic and CNS function.
This case highlights the unique association of PRES, TTP, and SLE. PRES is a clinical syndrome of headache, confusion, decreased level of consciousness, visual changes, and seizures, associated with characteristic radiological findings of posterior cerebral white matter edema. Well known risk factors for development of PRES are uncontrolled hypertension, eclampsia and immunosuppressive medications. A sudden rise in blood pressure with failure of auto-regulatory mechanisms leading to fluid extravasation has been the most accepted hypothesis for the development of PRES. Our patient did not have traditional risk factors for development of PRES. Treating clinicians must have high index of suspicion for this particular condition as prompt treatment results in rapid resolution of symptoms and neurological deficits in most cases. Treatment usually involves tight control of blood pressure and control of seizures. Plasmapheresis is an effective treatment in patients with TTP and we believe helped in the resolution of PRES. Unlike in some other forms of cerebral edema, use of steroids should be limited as it may worsen PRES.
SLE patients presenting with neuropshychiatric symptoms are often a diagnostic dilemma. Although they are more likely to have diagnosis such as vasculitis, cerebritis, venous thrombosis, and strokes, PRES should be considered in the differential. Characteristic findings on MR imaging help differentiate PRES from other diagnosis. Tight control of BP is critical.
Kavan Ramachandran, No Financial Disclosure Information; No Product/Research Disclosure Information