ANCA-Associated Vasculitis in Systemic Sclerosis

She improved progressively and currently has no peripheral neuropathy or myopathy. Her pulmonary involvement has been stable. However, over time, there has been more extensive cutaneous sclerosis and the development of more digital ulcers.

A large, well-demarcated ulcer along the medial malleolar area is shown in a patient with systemic sclerosis. Such ulcers are associated with small-vessel vasculitis.

Discussion

The development of vasculitis in patients with SS is highly uncommon.¹ Whereas the histopathology of SS vasculopathy demonstrates a myofibroblast proliferation and matrix deposition in the subendothelial layer leading to occlusion, the histopathology of vasculitis is characterized by the presence of mononuclear infiltrates and destruction of the vessel wall.

A recent publication by the French Vasculitis Study Group identified nine patients with SS and vasculitis.² Another study described the characteristics of a series of 40 patients with SS and associated systemic vasculitis, finding that the main clinical manifestation was a rapidly progressive glomerulonephritis (83%), followed by alveolar hemorrhage (28%), limb ischemia (13%) and skin vasculitis (10%).³

The presence of a positive ANCA in patients with SS is estimated to be in the range of 7–13%, but its clinical relevance is unknown. MPO-ANCA is more frequent than PR3-ANCA in patients with SS.⁴

Conclusion

The patient improved after immunosuppressive therapy. She has not experienced a new outbreak of vasculitis, although her cutaneous disease related to scleroderma has progressed.

Carmen Busca Arenzana is a medical resident in the internal medicine department of the Hospital La Paz in Madrid, Spain.
Ander Mayor-Ibarguren is a medical resident in the dermatology department of the Hospital La Paz in Madrid, Spain.
Amara Pieren-Salazar is a medical resident in the rheumatology department of the Hospital La Paz in Madrid, Spain.
Natalia Martin-Suñe, MD, works in the internal medicine department of the Hospital La Paz in Madrid, Spain.
Isabel Esteban-Rodríguez, MD, works in the pathology department of the Hospital La Paz in Madrid. Spain.
Juan José Ríos-Blanco, MD, works in the internal medicine department of the Hospital La Paz in Madrid, Spain.
Francisco Arnalich-Fernandez, MD, works in the internal medicine department of the Hospital La Paz in Madrid, Spain.

References

1. Quéméneur T, Mouthon L, Cacoub P, et al. Systemic vasculitis during the course of systemic sclerosis. Medicine (Baltimore). 2013 Jan;92(1):1–9.
2. Arad U, Balbir-Gurman A, Doenyas-Barak K, et al. Anti-neutrophil associated vasculitis in systemic sclerosis. Semin Arthritis Rheum. 2011 Oct;41(2):223–229.
3. Rho YH, Choi SJ, Lee YH, et al. Scleroderma associated with ANCA-associated vasculitis. Rheumatol Int. 2006 Mar;26(5):369–375.
4. Mouthon L, Millet A, Régent A, et al. Physiopathologie des vascularites ANCA-positives. Presse Med. 2012 Oct;41(10):996–1003.

Acknowledgments: The authors would like to thank Martin J. Smyth for his help in correcting their English.