A.V. GORDEEV¹, A.YU. ZAKHAROVA¹, Z.YU. MUTOVINA², L.P. ANANIEVA¹

¹Nasonova Research Institute of Rheumatology, 34 A Kashirskoye highway, Moscow, Russian Federation, 155221

²Educational and Scientific Medical Center of the Department of Presidential Affairs of the Russian Federation, 19 Marshala Timoshenko St., building 1a, Moscow, Russian Federation, 121359
Gordeev A.V. — D. Med. Sc., Professor, Senior Research Scientist, tel. (495) 614-44-55, e-mail: Andrewgordeew@gmail.com
Zakharova A.Yu. — Junior Research Scientist of the Laboratory of microcirculation and inflammation, tel. (499) 615-93-94 e-mail: Znasy@mail.ru

Mutovina Z. Yu. — Cand. Med. Sc., Associate Professor of the Department of therapy, cardiology and functional diagnostics with the course of nephrology and rheumatology, tel. (495) 530-05-25 e-mail: unmc@yandex.ru

Ananieva L.P. — D. Med. Sc., Professor, Head of the Laboratory of microcirculation and inflammation, tel. (499) 615-93-94, e-mail: ananieva@irramn.ru

Acute scleroderma nephropathy or scleroderma renal crisis (SRC) — the most serious manifestation of systemic sclerosis (SSc). SRC is characterized by the development of acute renal failure (ARF), and as usual by moderate to severe arterial hypertension with hyperreninemia. SRC pathogenesis is associated with damage to endothelial cells, vascular endothelial dysfunction, hypercoagulation and activation of the renin-angiotensin system. Until the end of the 1970s, the SRC is the leading cause of death in patients with SSc. Use of angiotensin-converting enzyme (ACE) inhibitors made real changes, reduced the overall level of the annual death rate of patients with SSc from 76% to less than 15. However, until now the development of the SRC is associated with a poor prognosis and a high death risk. Detection and monitoring of patients with high risk of SRC development is the main factor for improving survival in patients with the development of SRC. Patients with SSc may have other forms of acute kidney damage. In case of «untypical» cases of SRC it may be reasonable to conduct morphological study of kidney biopsy.

Key words: systemic sclerosis, scleroderma renal crisis, normotensive renal crisis, risk factors.

REFERENCES

1. Guseva N.G. Sistemnaya sklerodermiya [Systemic scleroderma]. Moscow: Meditsina, 1975. 270 p.
2. Moore H.C. and Sheehan H.L. The kidney of scleroderma. The Lancet. 259.6698, 1952, rr. 68-70.
3. Steen V.D., Medsger T.A. Change in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis., 2007, vol. 66, rr. 940-944.
4. Steen V.D., Costantino J.P., Shapiro A.P. et al. Outcome of renal crisis in system sclerosis: relation to availability of angiotensin converting enzyme (ACE) inhibitors. Ann Intern Med., 1990, vol. 113, rr. 352-367.
5. Walker U.A., Tyndall A., Czijak L. et al. Clinical risk assessment of organ manifestation in systemic sclerosis: a report from the EULAR Scleroderm Trial and Research group database. Ann Rheum Dis., 2007, vol. 66, rr. 754-763.
6. Shanmugam V.K., Steen V.D. Renal disease in scleroderma: an update on evaluation, risk stratification, pathogenesis and management. Curr Opin Rheumatol., 2012 November, vol. 24(6), rr. 669-676.
7. Penn H., Howie A.J., Kingdon E.J. et al. Scleroderma renal crisis: patient characteristics and long-term outcomes. QJM, 2007, vol. 100, rr. 485-494.
8. Penn N., Quillinan N., Khan K. et al. Targeting the endothelin axis in scleroderma renal crisis: rational and feasibility. QJ Med, 2013, vol. 106, rr. 839-848.
9. Stratton R.J. et al. Different patterns of endothelial cell activation in renal and pulmonary vascular disease in scleroderma. QJM: An International Journal of Medicine, 1998, vol. 91(8), pp. 561-566.
10. Hudson M., Baron M., Lo E. at al. An international, web-based, prospective cohort study to determine whether the use of ACE inhibitors prior to the onset of scleroderma renal crisis is associated with worse outcomes-methodology and preliminary results. Intern J Rheum, 2010.
11. Clements P.J., Lachenbruch P.A., Furs D.E. et al. Abnormalitis of renal physiology in systemic sclerosis. A prospective study with 10-year followup. Arthritis Rheum, 1994, vol. 37, rr. 67-74.
12. Teixeira L., Mouthon L., Mahr A. et al. Mortality and risk factors of scleroderma renal crisis: a French retrospective study of 50 patients. Ann Rheum Dis, 2008, vol. 67(1), rr. 110-6.
13. Qian Q. Thrombotic Microangiopathy Not Associated with the Classic/Idiopathic TTP-HUS. Core Concepts in Parenchymal Kidney Disease. Springer New York, 2014, rr. 249-262.
14. Sachin S.S., Ronco C., Pophale R. Cardio-renal syndrome type 5: epidemiology, pathophysiology and treatment. Semin Nephrol, 2012, vol. 32, rr. 49-56.
15. Naniwa T., Banno S., Sugiura Y. et al. Pulmonary-renal syndrome in systemic sclerosis: a report of three cases and review of the literature. Mod Rheumatol, 2007, Vol. 17(1), R. 37-44.
16. Vesely S.K., George J.N., Lammle B. et al. ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood, 2003, vol. 102, rr. 60-8.
17. Batal I., Domsic RT, Shafer A. et al. Scleroderma renal crisis: a pathology perspective. Int J Rheumatol, 2010: 543705.
18. Steen V.D. Kidney involvement in systemic sclerosis. La Presse Médicale, 43.10, 2014, rr. e305-e314.
19. Hudson M., Baron M., Tatibouet S. et al. Exposure to ACE inhibitors prior to onset of scleroderma renal crisis – Results from the International Scleroderma Renal Crisis Survey. Seminaes in Arthritis and Rheumatism 43, 2014, R. 666-672.
20. Denton C.P., Lapadula G., Mouthon L. et al. Renal complication and scleroderma renal crisis. Rhematol, 2009, vol. 48, rr. iii32-iii35.
21. Bose N., Chiesa-Vottero A., Chatterjee S. et al. Scleroderma renal crisis. Semm in Arthritis and Rheum, in press, 2015.
22. Stern E.P., Steen V.D., Denton C.P. Management of Renal Involvement in Scleroderma. Current Treatment Options in Rheumatology, 2015, rr. 1-13.
23. Trang G., Steele R., Baron M. et al. Corticosteroids and the risk of scleroderma renal crisis: a systematic review. Rheumatol Int, 2012, vol. 32(3), rr. 645-653.
24. Steen V.D., Medsger T.A. Case-control study of corticosteroids and other drugs that either precipitate or protect from the development of scleroderma renal crisis. Arthritis Rheum, 1998, vol. 41, rr. 1613-19.
25. Okano Y., Steen V.D., Medsger T.A. Auto-antibody reactive with RNA polymerase III in systemic sclerosis. Ann Intern Med, 1993, vol. 119, rr. 1005-13.
26. Bunn C.C., Denton C.P., Shi-Wen X. et al. Anti-RNA polymerases and other autoantibody specificities in systemic sclerosis. Br J Rheumatol, 1998, vol. 37, rr. 15-20.
27. Yanaba K., Asano Y., Tada Y., et al. Increased serum soluble CD147 levels in patients with systemic sclerosis: association with scleroderma renal crisis. Clin Rheum, 2012, vol. 31(5), rr. 835-839.
28. Nguyen B., Mayes M.D., Arnett F.C. et al. HLA-DRB1*0407 and *1304 are risk factors for scleroderma renal ctisis. Arthritis Rheum, 2011, vol. 63, rr. 530-534.
29. Gordeev A.V., Savitskiy S.N., Sura V.V. Senile pyelonephritis. The pathophysiology of hypertensive syndrome. Terapevticheskiy arkhiv, 1993, no. 6, pp. 30-34 (in Russ.).
30. Kowal-Bielecka O. et al. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). Annals of the Rheumatic Diseases, 2009, vol. 68(5), pp. 620-628.
31. Cheung W.Y., Gibson I.W., Rush D. et al. Late recurrence of scleroderma renal crisis: poor outcome despite angiotensin II blockade. Am J Kidney Dis, 2005, vol. 45, rr. 930-934.
32. Izzedine H., Rouvier P., Deray G. Endotelin receptor antagonism-based treatment for scleroderma renal crisis. Am J Kidney Dis, 2013, vol. 62(2), rr. 394-395.
33. Chighizola C.B., Penn H., Meroni P.L. et al. The N-terminal fragment of brain natriuretic peptide as an outcome predictor in scleroderma renal crisis. Arthritis Rheum, 2011, vol. 63, rr. S573.
34. Siva B., McDonald S.P., Hawley C.M. et al. End-stage kidney disease due to scleroderma-outcomes in 127 consecutive ANZDATA registry cases. Nephrol Dial Transplant, 2011, vol. 26, rr. 3165-3171.
35. Cheung W.Y., Gibson I.W., Rush D. et al. Late recurrence of scleroderma renal crisis: poor outcome despite angiotensin II blockade. Am J Kidney Dis, 2005, vol. 45, rr. 930-934.