Systemic sclerosis & Raynaud's

Studying the Role of C5-Inhibition Therapy in Scleroderma Renal Crisis-Induced Thrombotic Microangiopathy – A Review of Literature

Semin Arthritis RheumPublished Systematic review of case reports, case series and observational studies

Study context

Population
Patients with SSc and scleroderma renal crisis (SRC) (including SRC-TMA) treated with Eculizumaball treated with Eculizumab
Participants
17 patients
Outcome
Clinical (renal or hematologic) improvement with Eculizumab
Methodological detailsliterature search from inception to December 2022, Concomitant therapy: Angiotensin-converting enzyme inhibitors (ACE-I) 11/17 (64.7%), plasmapheresis 9/17 (52.9%), steroids 5/17 (29.4%), CYC 3/17 (17.6%), calcium channel blockers 3/17 (17.6%), RTX 3/17 (17.6%)
  • Literature search from inception to December 2022
  • Concomitant therapy: ACE-I 11/17 (64.7%), plasmapheresis 9/17 (52.9%), steroids 5/17 (29.4%), CYC 3/17 (17.6%), calcium channel blockers 3/17 (17.6%), RTX 3/17 (17.6%)

Pooled result

As reported in the abstract

Eculizumab

82.3%

14 of 17

Further pooled results

RRT required

Patients requiring renal replacement therapy
11/17 (64.7%)

Concomitant therapies reported

ACE-I
11/17 (64.7%)
Plasmapheresis
9/17 (52.9%)
Steroids
5/17 (29.4%)
CYC
3/17 (17.6%)
Calcium channel blockers
3/17 (17.6%)
RTX
3/17 (17.6%)

Original publication

Studying the Role of C5-Inhibition Therapy in Scleroderma Renal Crisis-Induced Thrombotic Microangiopathy – A Review of Literature. Semin Arthritis Rheum. Published 22 August 2023. doi:10.1016/j.semarthrit.2023.152256

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