Thrombotic microangiopathy in systemic lupus erythematosus: efficacy of eculizumab.

Thrombotic microangiopathy (TMA) is a severe disorder with poor outcomes. The cause is unknown for many patients, although TMA is associated with connective tissue disorders, including systemic lupus erythematosus (SLE). While uncommon, TMA is one of the most serious complications of SLE and in many cases may be resistant to therapy. We report a patient with SLE complicated by TMA that was refractory to standard therapy but responded well to eculizumab, with continued remission after 1 year of follow-up. Eculizumab might be useful in the management of resistant cases of TMA caused by SLE.

El-Husseini A ，Hannan S ，Awad A ，Jennings S ，Cornea V ，Sawaya BP ... -  《-》

A Rare Case of Lupus Nephritis Presenting as Thrombotic Microangiopathy with Diffuse Pseudotubulization Possibly Caused by Atypical Hemolytic Uremic Syndrome.

Ono M ，Ohashi N ，Namikawa A ，Katahashi N ，Ishigaki S ，Tsuji N ，Isobe S ，Iwakura T ，Sakao Y ，Tsuji T ，Kato A ，Fujigaki Y ，Shimizu A ，Yasuda H ... -  《-》

Use of Eculizumab in Atypical Hemolytic Uremic Syndrome, Complicating Systemic Lupus Erythematosus.

Bermea RS ，Sharma N ，Cohen K ，Liarski VM ... -  《-》

A systematic review of the role of eculizumab in systemic lupus erythematosus-associated thrombotic microangiopathy.

Wright RD ，Bannerman F ，Beresford MW ，Oni L ... -  《BMC Nephrology》

Insights from the use in clinical practice of eculizumab in adult patients with atypical hemolytic uremic syndrome affecting the native kidneys: an analysis of 19 cases.

Atypical hemolytic uremic syndrome (aHUS) is a devastating form of renal thrombotic microangiopathy. Despite plasma exchange, the standard treatment of aHUS for decades, the renal prognosis for patients with aHUS has remained poor. We assessed the off-trial use of eculizumab in adult patients with aHUS affecting the native kidneys. A retrospective study was conducted. aHUS was defined as the presence of 3 or more of the following: acute kidney injury (serum creatinine >1.4 mg/dL [120 μmol/L]), mechanical hemolytic anemia, thrombocytopenia, and the presence of thrombotic microangiopathy features in a kidney biopsy specimen. Patients who had received 4 or more weekly 900-mg infusions of eculizumab were included. 19 patients were identified through a query sent to all French nephrology centers. Evolution of kidney function, hemolysis, and thrombocytopenia after the initiation of eculizumab therapy. All patients had acute kidney injury (serum creatinine range, 2.2-17.0 mg/dL) and 12 required hemodialysis. Thirteen patients carried a mutation in 1 complement gene and 1 had anti-factor H antibodies. For first-line therapy, 16 patients underwent plasma exchange and 3 patients received eculizumab. Median time between aHUS onset and eculizumab therapy initiation was 6 (range, 1-60) days and median time to platelet count normalization after eculizumab therapy initiation was 6 (range, 2-42) days. At the 3-month follow-up, 4 patients still required dialysis, 8 had non-dialysis-dependent chronic kidney disease, and 7 had normalized kidney function. At last follow-up (range, 4-22 months), 3 patients remained dialysis dependent, 7 had non-dialysis-dependent chronic kidney disease (estimated glomerular filtration rate, 17-55 mL/min/1.73 m(2)), and 9 had normal kidney function. Risks of reaching end-stage renal disease within 3 months and 1 year of aHUS onset were reduced by half in eculizumab-treated patients compared with recent historical controls. Retrospective study and use of historical controls. Our data indicate that eculizumab improves kidney disease outcome in patients with aHUS.

Fakhouri F ，Delmas Y ，Provot F ，Barbet C ，Karras A ，Makdassi R ，Courivaud C ，Rifard K ，Servais A ，Allard C ，Besson V ，Cousin M ，Châtelet V ，Goujon JM ，Coindre JP ，Laurent G ，Loirat C ，Frémeaux-Bacchi V ... -  《-》