Rapidly progressive lupus nephritis and concomitant thrombotic microangiopathy.
Although uncommon, thrombotic microangiopathy (TMA) is one of the most serious complications in patients with systemic lupus erythematosus. A 30-year-old black woman admitted to our hospital because of fever, fatigue, 'dark' urine and rapidly progressive renal failure was found to have systemic lupus erythematous and atypical hemolytic uremic syndrome. Kidney biopsy showed WHO class IV lupus nephritis with crescents and TMA. Hemodialysis was initiated for worsening renal failure. The patient was treated with corticosteroids, monthly pulse intravenous Cyclophosphamide, plasmapheresis and Rituximab on a weekly basis for 4 weeks. The patient's blood pressure was aggressively controlled using antihypertensive agents. Despite this extensive therapy, she remained dialysis dependent although hematological parameters returned to normal values.
Department of Nephrology, Pitié-Salpêtriere Hospital, Paris, France.
Chems
C
Gharbi
Edward
E
Bourry
Philippe
P
Rouvier
Sabria
S
Hacini
Ahmed
A
Letaief
Alain
A
Baumelou
Hassane
H
Izzedine
D058186:Acute Kidney Injury / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000473:pathology / Q000628:therapy; D008181:Lupus Nephritis / Q000209:etiology* / Q000473:pathology* / Q000628:therapy; D006435:Renal Dialysis; D057049:Thrombotic Microangiopathies / Q000209:etiology* / Q000473:pathology* / Q000628:therapy; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones