CYCLOPHOSPHAMIDE FOR LUPUS NEPHRITIS CAUSING PRES (REVERSIBLE POSTERIOR ENCEPHALOPATHY SYNDROME): CASE REPORT

CYCLOPHOSPHAMIDE FOR LUPUS NEPHRITIS CAUSING PRES (REVERSIBLE POSTERIOR ENCEPHALOPATHY SYNDROME): CASE REPORT

PETRY, ANDRÉ ADALBERTO; COLOMBO, DEBORAH; ANDRADE, MARIANA; DELFINO, JULIANA; SANTOS, THIAGO ALBERTO FERNANDES GOMES DOS; SKARE, THELMA LAROCA

Pôster:

Reversible posterior encephalopathy syndrome (PRES) is a clinical radiographic syndrome of heterogeneous etiologies that are gathered together because of similar findings on neuroimaging studies, predominantly involving the bilateral parieto-occipital regions. PRES has been described in hypertensive encephalopathy, eclampsia, and with the use of cytotoxic drugs. Prompt recognition and treatment is important to avoid the permanent damage that can occur in this condition. Its pathogenesis remains uncertain, but it seems to be related to altered cerebral autoregulation and endothelial dysfunction. Death and neurological disability resulting from progressive cerebral edema and/or intracerebral hemorrhage have been described as consequences of PRES. In a patient with lupus, a differential diagnosis of PRES with central nervous system involvement of this disease should be done.

Case report

A 35 years old female patient had diagnosis of systemic lupus erythematosus (SLE) in 2012 when she developed class V glomerulonephritis, positive ANA, anti-dsDNA, anti-Ro, anti-Sm and hypocomplementemia. At that time, she was treated with cyclophosphamide, glucocorticoid and hydroxychloroquine with complete remission. She remained well until March, 2019, when her nephritis recurred (proteinuria of 7g/24hr, creatinine 0.74 mg/dL and a second renal biopsy with class V). She was treated, again, with intravenous cyclophosphamide and steroids. Twenty-three days after the first cyclophosphamide dose she developed severe headache, uncontrolled hypertension, loss of consciousness and repeated convulsions needing tracheal intubation to preserve ventilation. A cerebral angio resonance was performed and showed hypersignal areas in T2/Flair involving the posterior subcortical white matter in the occipital lobes. The diagnosis of PRES was done and the patient was treated with anticonvulsants, dexamethasone and sodium nitro prussiate. An infection for CMV complicated the situation and was treated with ganciclovir. Cyclophosphamide was discontinued. She gradually recovered and her nephritis was handled with mophetyl mycophenolate.

Conclusion

The prompt recognition and the removal of the inciting factor are fundamental for the success of treatment of patients with PRES.

Reversible posterior encephalopathy syndrome (PRES) is a clinical radiographic syndrome of heterogeneous etiologies that are gathered together because of similar findings on neuroimaging studies, predominantly involving the bilateral parieto-occipital regions. PRES has been described in hypertensive encephalopathy, eclampsia, and with the use of cytotoxic drugs. Prompt recognition and treatment is important to avoid the permanent damage that can occur in this condition. Its pathogenesis remains uncertain, but it seems to be related to altered cerebral autoregulation and endothelial dysfunction. Death and neurological disability resulting from progressive cerebral edema and/or intracerebral hemorrhage have been described as consequences of PRES. In a patient with lupus, a differential diagnosis of PRES with central nervous system involvement of this disease should be done.

Case report

A 35 years old female patient had diagnosis of systemic lupus erythematosus (SLE) in 2012 when she developed class V glomerulonephritis, positive ANA, anti-dsDNA, anti-Ro, anti-Sm and hypocomplementemia. At that time, she was treated with cyclophosphamide, glucocorticoid and hydroxychloroquine with complete remission. She remained well until March, 2019, when her nephritis recurred (proteinuria of 7g/24hr, creatinine 0.74 mg/dL and a second renal biopsy with class V). She was treated, again, with intravenous cyclophosphamide and steroids. Twenty-three days after the first cyclophosphamide dose she developed severe headache, uncontrolled hypertension, loss of consciousness and repeated convulsions needing tracheal intubation to preserve ventilation. A cerebral angio resonance was performed and showed hypersignal areas in T2/Flair involving the posterior subcortical white matter in the occipital lobes. The diagnosis of PRES was done and the patient was treated with anticonvulsants, dexamethasone and sodium nitro prussiate. An infection for CMV complicated the situation and was treated with ganciclovir. Cyclophosphamide was discontinued. She gradually recovered and her nephritis was handled with mophetyl mycophenolate.

Conclusion

The prompt recognition and the removal of the inciting factor are fundamental for the success of treatment of patients with PRES.

Palavras-chave:

DOI: 10.5151/sbr2019-073

Referências bibliográficas

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Como citar:

PETRY, ANDRÉ ADALBERTO; COLOMBO, DEBORAH; ANDRADE, MARIANA; DELFINO, JULIANA; SANTOS, THIAGO ALBERTO FERNANDES GOMES DOS; SKARE, THELMA LAROCA; "CYCLOPHOSPHAMIDE FOR LUPUS NEPHRITIS CAUSING PRES (REVERSIBLE POSTERIOR ENCEPHALOPATHY SYNDROME): CASE REPORT", p-73-73. In: Anais do 36º Congresso Brasileiro de Reumatologia. [ISBN 978-85-212-1892-0]. São Paulo: Blucher, 2019.
ISSN 23577282, DOI 10.5151/sbr2019-073