Catastrophic antiphospholipid syndrome in a patient with systemic sclerosis and hereditary angioedema: case report and literature review

Abstract

Catastrophic antiphospholipid syndrome (CAPS) is a rare form of the antiphospholipid syndrome (APS) in which microvascular thrombotic events cause rapidly progressive multiorgan dysfunction. We describe a case of CAPS presenting in a patient with suspected systemic sclerosis (SSc) and hereditary angioedema (HAE), and conduct a literature review to examine the reported cases of CAPS in individuals with SSc. Two reported cases of APS occurring with HAE were also found. In CAPS, there may be multiple thrombi in the microvasculature of any organ, most commonly in the intra-abdominal viscera. Patients rapidly develop multiorgan dysfunction or failure. Diagnosis is partly based upon the presence of positive antiphospholipid antibodies. The recommended therapy is systemic anticoagulation, high-dose corticosteroids, and plasma exchange with or without the addition of intravenous immunoglobulin. Given the high mortality rate of CAPS, urgent diagnosis and treatment are necessary to attempt to halt the progression of multiorgan failure.

Keywords:

• Scleroderma
• systemic sclerosis
• antiphospholipid antibody syndrome
• catastrophic antiphospholipid antibody syndrome
• hereditary angioedema

Acknowledgments

The authors would like to thank Kellie Littlefield, DO, Department of Medicine, Oregon Health and Science University, for providing the images in Figure 1.

Patient consent

Consent for publication was obtained from the patient’s family following her death.

Conflicts of interest

None.