Vasculitis ANCA and Systemic Scleroderma
PDF (Español)


Anti-neutrophil cytoplasmic antibody-associated vasculitis kidney diseases
kidney diseases.

How to Cite

Hurtado M, Contreras K, González CA, Bustamante A. Vasculitis ANCA and Systemic Scleroderma. Rev. Colomb. Nefrol. [Internet]. 2016 Nov. 18 [cited 2023 Dec. 9];3(2):137-42. Available from:


Systemic sclerosis (SSc) is an autoimmune disorder with microvascular endothelial cell apoptosis, excessive extracellular matrix protein and perivascular infiltration of mononuclear cells in the skin, producing damage and progressive fibrosis of the skin and visceral organs (lungs, heart and kidney). The most common renal diseases linked with SSc is renal scleroderma crisis, which is found in 5 to10% of the cases. ANCA associated vasculitis also has been described but less frequent. It can affect multiple organs and become life-threatening condition. The association between paucity-immune vasculitis and systemic sclerosis is uncommon 1.6% in a cohort of 35 patients. It could lead to severe renal dysfunction including renal replacement therapy and plasma exchange as part of the treatment. Early and timely treatment it is essential for the prognosis of the diseases. We present this rare association and our experiences highlighting the successful evolution due to early diagnosis and treatment. A 59 years old woman with systemic sclerosis who showed a rapidly progressive glomerulonephritis caused by to paucity-immune vasculitis associated with p-ANCA. We dismissed scleroderma crisis and renal biopsy confirmed the diagnosis. We start prednisolone and cyclophosphamide, with a favorable initial response, however, during the follow-up the patient had severe relapse, and therefore we decided to switch to Rituximab induction. After we performed the treatment the patient evolved favorably, included complete remission of the disease. We show you the rare association between paucity-immune vasculitis and systemic sclerosis, and the excellent response taking account an early diagnosis and therapy.
PDF (Español)


1. Bose N, Chiesa-Vottero A, Chatterjee S. Scleroderma renal crisis. Semin Arthritis Rheum. 2015; 44(6): 687-694. doi: 10.1016/j.semarthrit.2014.12.001.

2. Arad U, Balbir-Gurman A, Doenyas-Barak K, Amit-Vazina M, Caspi D, Elkayam O. Anti-Neutrophil Antibody Associated Vasculitis in Systemic Sclerosis. Semin Arthritis Rheum. 2011; 41(2): 223-229. doi:10.1016/j.semarthrit. 2010.11.001.

3. Omair MA, Mohamed N, Johnson SR, Ahmad Z, Lee P. ANCA-associated vasculitis in systemic sclerosis report of 3 cases. Rheumatol Int. 2013; 33(1): 139-143. doi: 10.1007/s00296-011-2359-z.

4. Denton CP, Lapadula G, Mouthon L, Müller-Ladner U. Renal complications and scleroderma renal crisis. Rheumatology. 2009; 48 Suppl 3: iii32-5. doi: 10.1093/rheumatology/ken483.

5. Denton CP, Black CM. Scleroderma--clinical and pathological advances. Best Pract Res Clin Rheumatol. 2004; 18(3): 271-290. doi:10.1016/j.berh.2004.03.001.

6. Trang G, Steele R, Baron M, Hudson M. Corticosteroids and the risk of scleroderma renal crisis: A systematic review. Rheumatol Int. 2012; 32(3): 645-653. doi: 10.1007/s00296-010-1697-6.

7. Greenhall GHB, Salama AD. What is new in the management of rapidly progressive glomerulonephritis? Clin Kidney J. 2015; 8(2): 143-150. doi:10.1093/ckj/sfv008.

8. Groot K De, Harper L, Jayne DRW, Felipe L, Suárez F, Gregorini G. Pulse Versus Daily Oral Cyclophosphamide for Induction of Remission. Ann Intern Med. 2009; 150(8): 670-680. doi:0.1007/s00393-009-0509-4.

9. Yates M, Watts RA, Bajema IM, et al. Eular/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016: 1-12. doi: 10.1136/.

10. Stone JH, Merkel PA, Spiera R, et al. Rituximab versus Cyclophosphamide for ANCA-Associated Vasculitis. N Engl J Med. 2010; 363(3): 221-232. doi:10.1056/NEJMoa0909905.

11. Guillevin L, Pagnoux C, Karras A, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med. 2014; 371(19): 1771-1780. doi: 10.1056/NEJMoa1404231.

12. Agnes H, Kalman P, Jozsef A, Henrik B, Mucsi I. The birmingham vasculitis activity score (BVAS) predicts all cause mortality in patients with ANCA associated vasculitis. Nephrol Dial Transplant. 2012; 27: ii378.

13. Derrett-Smith EC, Nihtyanova SI, Harvey J, Salama AD, Denton CP. Revisiting ANCA-associated vasculitis in systemic sclerosis: clinical, serological and immunogenetic factors. TL-52. Rheumatology (Oxford). 2013; 52 VN-r(10): 1824-1831. doi: 10.1093/rheumatology/ket213.

14. Zurita P, Martin S, Urrego C, et al. Vasculitis ANCA positivo en un paciente con esclerosis sistémica. Reum Clin. 2013; 9(1): 72-74. doi: 10.1007/s00296-011-2359-z.Pablo.

15. Bienaimé F, Clerbaux G, Plaisier E, Mougenot B, Ronco P, Rougier JP. d-Penicillamine-Induced ANCA-Associated Crescentic Glomerulonephritis in Wilson Disease. Am J Kidney Dis. 2007; 50(5): 821-825. doi:10.1053/j. ajkd.2007.05.026.

16. Alba MA, Flores-Suárez LF. Rituximab como terapia de mantenimiento en las vasculitis asociadas a ANCA: ¿cómo, cuándo y por qué? Reumatol Clínica. 2015; 12(1): 39-46. doi:10.1016/j.reuma.2015.06.002.

17. Cohen Tervaert JW. Rituximab in ANCA-associated vasculitis: a revolution? Nephrol Dial Transplant. 2011;26(10): 3077-3079. doi: 10.1093/ndt/gfr507.

18. Åhlström A, Kuitunen A, Peltonen S, et al. Comparison of 2 Acute Renal Failure Severity Scores to General Scoring Systems in the Critically Ill. Am J Kidney Dis. 2006; 48(2): 262-268. doi:10.1053/j.ajkd.2006.04.086.

19. Luqmani RA. State of the art in the treatment of systemic vasculitides. Front Immunol. 2014; 5(Oct): 1-9. doi: 10.3389/fimmu.2014.00471.

20. Guillevin L, Pagnoux C, Karras A, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. A prospective study in 117 patients. Presse Med. 2013; 42(4, part 2): 679 [abstract]. doi:10.1056/NEJMoa1404231.
No national or foreign publication may partially or totally reproduce or translate Revista Colombiana de Nefrología articles or abstracts without prior written permission from the journal’s Editorial Board.




Download data is not yet available.