Available online 25 August 2022, 102887

Severe thrombocytopenia is a poor prognostic factor for SLE and is associated with a high mortality rate.

Current treatment for SLE-ITP relies heavily on immunosuppressants as for primary ITP.

Approximately 75% of SLE-ITP cases received treatments with GCs and antimalaria. 1/3 of the cases fail to respond to them.

Repeated high-dose GCs and aggressive immunosuppressants, which predispose patients to an increased risk of infection.

Optimal treatment regimen based on the mechanism can help to avoid ineffective treatment, thereby achieving early remission.

Immune thrombocytopenia (ITP) is a common hematological manifestation of systemic lupus erythematosus (SLE). The heterogeneity of its clinical characteristics and therapeutic responses reflects a complex pathogenesis. A better understanding of its pathophysiological mechanisms and employing an optimal treatment regimen is therefore important to improve the response rate and prognosis, and avoid unwanted outcomes. Besides glucocorticoids, traditional immunosuppressants (i.e. cyclosporine, mycophenolate mofetil) and intravenous immunoglobulins, new therapies are emerging and promising for the treatment of intractable SLE-ITP, such as thrombopoietin receptor agonists (TPO-RAs), platelet desialylation inhibitors(i.e. oseltamivir), B-cell targeting therapy(i.e. rituximab, belimumab), neonatal Fc receptor(FcRn) inhibitor, spleen tyrosine kinase(Syk) inhibitor and Bruton tyrosine kinase(BTK) inhibitor et al., although more rigorous randomized controlled trials are needed to substantiate their efficacy. In this review, we update our current knowledge on the pathogenesis and treatment of SLE-ITP.

Systemic lupus erythematosus

Immune thrombocytopenia

Thrombopoietin

Neonatal fc receptor

Spleen tyrosine kinase

Bruton tyrosine kinase

Immune thrombocytopenia

Systemic lupus erythematosus

Thrombopoietin receptor agonist

Ashwell Morrell receptors

Spleen tyrosine kinase

Bruton tyrosine kinase

All-trans retinoic acid

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