Send to

Choose Destination
Autoimmun Rev. 2018 Sep;17(9):882-889. doi: 10.1016/j.autrev.2018.03.012. Epub 2018 Jul 10.

Therapy of scleroderma renal crisis: State of the art.

Author information

Division of Rheumatology, Department of Medicine (DIMED), University of Padova, Via Giustiniani 2, 35128 Padova, Italy.
Apheresis Unit, Department of Transfusion Medicine, University Hospital of Padova, Via Giustiniani 2, 35128 Padova, Italy.
Division of Rheumatology, Department of Medicine (DIMED), University of Padova, Via Giustiniani 2, 35128 Padova, Italy. Electronic address:


Scleroderma renal crisis (SRC) is an uncommon but still life-threatening manifestation of systemic sclerosis (SSc). The incidence of SRC has decreased in the last few decades, probably due to a widespread use of vasodilators in SSc patients. It is well-recognized that exposure to different drugs can trigger SRC (corticosteroids, cyclosporine) or might prevent its occurrence (iloprost, calcium channel blockers). The prognosis of this life-threatening manifestation has not substantially improved since 1980s, when ACE-inhibitors were introduced in its treatment. ACE-inhibitors remain the mainstay in the therapy of SRC due to their efficacy in controlling malignant hypertension; indeed, the prognosis largely depends on the rapid improvement of the ongoing renal ischemia. Calcium-channel blockers and in third line diuretics and alpha-blockers should be used as additional therapy if blood pressure control remains suboptimal despite maximum tolerated doses of ACE-inhibitors. Given the growing evidence on the role of complement activation and endothelin-1 in the pathogenesis of SRC, recent case-series and case reports have suggested the use of C5-inhibitors and endothelin receptor antagonists in the therapy of SRC, mainly in the refractory cases. Plasma-exchange seems to give some benefits in patients with SRC and microangiopathy or intolerant to ACE-inhibitors. Renal transplantation is the last treatment option and its outcome is similar to that reported in other connective tissue disorders, with a 5-year patient survival rate of about 82%. In this review we summarize the current knowledge in the treatment of SRC.


Angiotensin-converting enzyme inhibitors; C5-inhibitors; Endothelin receptor antagonists; Plasma-exchange; Renal transplantation; Scleroderma renal crisis

[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Elsevier Science
Loading ...
Support Center