High incidence of pulmonary arterial hypertension in systemic sclerosis patients with anti-centriole autoantibodies

Abstract

Systemic sclerosis (SSc)-related autoantibodies are useful tools in identifying clinically homogenous subsets of patients and predicting their prognosis. In this report, we described five SSc patients with anti-centriole antibodies. All five patients were females and had digital ulcers/gangrene. Four of five (80%) patients had pulmonary arterial hypertension (PAH). None of the five patients had active pulmonary fibrosis or developed renal crisis. Anti-centriole antibodies may be a marker for PAH and digital ulcers/gangrene.

Keywords:

• Anti-centriole antibody
• Pulmonary arterial hypertension
• Peripheral vasculopathy
• Systemic sclerosis

Acknowledgements

We thank Ms. Masako Matsubara and Yuko Yamada for technical assistance.

Funding/Support

This study was supported in part by a Grant-in-Aid from the Ministry of Education, Science and Culture of Japan.

Conflict of interest

None.