How I Treat Complement-Mediated TMA

Sperati, C. John

doi:10.2215/cjn.13581021

Cited by 3 publications

(1 citation statement)

References 12 publications

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“…These include complement-mediated TMA, TTP, sepsis, lupus nephritis, and others ( 35 , 36 ). However, other reports show that sC5b-9 does not always correlate with TMA disease activity because it does not necessarily represent the amount of C5b-9 formed on the tissue ( 37 , 38 ). In our case, although the patient’s clinical symptoms improved rapidly after eculizumab administration, sC5b-9 remained at a high level for up to 50 days and there was a discrepancy between clinical symptoms and the sC5b-9 level ( Table 2 ).…”

Section: Discussionmentioning

confidence: 95%

Case report: Thrombotic microangiopathy concomitant with macrophage activation syndrome in systemic lupus erythematosus refractory to conventional treatment successfully treated with eculizumab

et al. 2023

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Thrombotic microangiopathy (TMA) is a rare but life-threatening complication of systemic lupus erythematosus (SLE). Macrophage activation syndrome (MAS) is also a rare, life-threatening hyperinflammatory condition that is comorbid with SLE. However, the association between TMA and MAS in patients with SLE has rarely been assessed, and the difficulty of diagnosing these conditions remains prevalent. The efficacy of eculizumab has been reported for SLE patients whose conditions are complicated with TMA. However, no study has investigated the therapeutic efficacy of eculizumab for TMA concomitant with SLE-associated MAS. Herein, we report the first case of TMA concomitant with SLE-associated MAS that was initially refractory to conventional immunosuppressive therapy but showed remarkable recovery after eculizumab treatment. Furthermore, we evaluated serum syndecan-1 and hyaluronan levels, which are biomarkers of endothelial damage. We found that these levels decreased after the administration of eculizumab, suggesting that TMA was the main pathology of the patient. This case illustrates that it is important to appropriately assess the possibility of TMA during the course of SLE-associated MAS and consider the use of eculizumab as necessary.

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Section: Discussionmentioning

confidence: 95%

Case report: Thrombotic microangiopathy concomitant with macrophage activation syndrome in systemic lupus erythematosus refractory to conventional treatment successfully treated with eculizumab

et al. 2023

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Onconephrology and Thrombotic Microangiopathy: Looking Beyond the Horizon

Gudsoorkar

Abudayyeh

Tchakarov

et al. 2022

Seminars in Nephrology

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How I treat thrombotic microangiopathy in the era of rapid genomics

Doreille

Rafat

Rondeau

et al. 2023

Blood

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Thrombotic microangiopathy (TMA) encompasses various genetically driven diseases. The emergence of ultra-fast genomic sequencing has recently opened up new avenues of research for genetic investigations in the setting of intensive care units. TMA is likely to be a suitable focus for fast-track genomic sequencing. By establishing an expeditious molecular diagnosis of patients with complement-dependent hemolytic uremic syndrome, fast-track genomic sequencing allows for the timely implementation or withdrawal of anti-C5 treatment while averting unnecessary, costly, and potentially harmful therapy in patients testing negative. Furthermore, genomics has the potential to reshape the taxonomic classification of TMA thanks to comprehensive genomic analysis. The most significant results arising from such analysis can be categorized as i) new descriptions of genetic diseases previously not recognized as associated with TMA, and ii) an enrichment of the phenotypic spectrum of diseases traditionally related to TMA. The latter draws on the concept of retrophenotyping, whereby genomic investigation precedes full clinical description. By taking precedence over a phenotypic approach, an unbiased genomic-focused analysis maximizes the chances of discovering new descriptions related to a given variant. Presented here are four cases of TMA which highlight these issues and substantiate the promise of fast-track genomic sequencing.

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How I Treat Complement-Mediated TMA

Cited by 3 publications

References 12 publications

Case report: Thrombotic microangiopathy concomitant with macrophage activation syndrome in systemic lupus erythematosus refractory to conventional treatment successfully treated with eculizumab

Case report: Thrombotic microangiopathy concomitant with macrophage activation syndrome in systemic lupus erythematosus refractory to conventional treatment successfully treated with eculizumab

Onconephrology and Thrombotic Microangiopathy: Looking Beyond the Horizon

How I treat thrombotic microangiopathy in the era of rapid genomics

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