rspНаучно-практическая ревматологияRheumatology Science and Practice1995-44841995-4492IMA-PRESS, LLC10.14412/1995-4484-2015-343-445rsp-2116Research ArticleОБЗОРREVIEWСовременные представления о гетерогенности поражений почек у больных склеродермиейCURRENT VIEWS ON THE HETEROGENEITY OF RENAL INVOLVEMENTS IN PATIENTS WITH SYSTEMIC SCLEROSISГордеевА. В.GordeevA. V.ЗахароваА. Ю.ZakharovaA. Yu.Znasy@mail.ruМутовинаЗ. Ю.MutovinaZ. Yu.АнаньеваЛ. П.AnanyevaL. P.ФГБНУ Научно-исследовательский институт ревматологии им. В.А. Насоновой, Москва, Россия 115522 Москва, Каширское шоссе, 34АРоссияV.A. Nasonova Research Institute of Rheumatology, Moscow, Russia; 2Training and Research Medical Center, Department for Presidential Affairs of the Russian Federation, Moscow, Russia 34A, Kashirskoe Shosse, Moscow 115522Russian FederationФГБУ ДПО «Учебно-научный медицинский центр» Управления делами Президента Российской Федерации, Москва, Россия 121359 Москва, ул. Маршала Тимошенко, 19, стр. 1АРоссияTraining and Research Medical Center, Department for Presidential Affairs of the Russian Federation, Moscow, Russia 19, Marshal Timoshenko St., Build 1A, Moscow 121359Russian Federation201510092015534343445Copyright © Гордеев А.В., Захарова А.Ю., Мутовина З.Ю., Ананьева Л.П., 20152015Гордеев А.В., Захарова А.Ю., Мутовина З.Ю., Ананьева Л.П.Gordeev A.V., Zakharova A.Y., Mutovina Z.Y., Ananyeva L.P.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://rsp.mediar-press.net/rsp/article/view/2116

Системный склероз, или системная склеродермия (ССД), – аутоиммунное заболевание соединительной ткани, основные клинические признаки которого обусловлены распространенными нарушениями микроциркуляции, фиброзом кожи и внутренних органов. По данным морфологических исследований, у 80% пациентов с ССД выявляются изменения почек, в том числе не связанные с ревматическими заболеваниями. В то время как распространенность склеродермического почечного криза в настоящее время оценивается в 2–5%, гораздо чаше отмечается асимптоматическое снижение почечной функции («немая уремия»), обусловленное наличием мультиморбидной и коморбидной патологии. Его частота у пациентов с ССД может достигать 55%. Факт наличия аутоиммунного заболевания соединительной ткани сам по себе может рассматриваться как фактор риска повреждения почек. Пятнадцатилетняя выживаемость больных ССД без поражения почек составляет 72%, при наличии поражения почек – не более 13%. Наличие протеинурии у пациентов с ССД является одним из наиболее значимых независимых факторов риска летальных исходов (относительный риск составляет 3,34), оставляя далеко позади такие канонические факторы риска, как легочная артериальная гипертензия, легочная рестрикция (соотношение объема форсированного выдоха за 1 с и форсированной жизненной емкости легких <80%), дыхательная недостаточность(III и IV класса по NYHA), а также снижение диффузионной способности легких и высокий кожный счет. Авторы впервые предлагают концепцию существования и патогенеза хронической склеродермической нефропатии, в основе которой лежит феномен сосудистой эндотелиальной дисфункции, формирующийся в различных структурных компонентах нефрона и почки в целом.

Systemic sclerosis (SS), is an autoimmune connective tissue disease, the main clinical signs of which are due to disseminated microcirculatory disorders and fibrosis of the skin and viscera. Morphological examinations showed that 80% of patients with SS had renal changes, including those unassociated with rheumatic diseases. Whereas the prevalence of scleroderma renal crisis is now estimated to be 2–5%, there is considerably often an asymptomatic reduction in renal function (silent uremia), which is caused by multimorbidity and comorbidity. Its incidence in patients with SS may be as high as 55%. The presence of autoimmune connective tissue disease may be itself regarded as a risk factor of renal involvement. Fifteen-year survival is 72% in SS patients with no renal involvement and not more than 13% in those having renal involvement. In patients with SS, proteinuria is one of the most important independent risk factors for fatal outcomes (relative risk, 3.34), leaving far behind canonical risk factors, such as pulmonary hypertension, restrictive lung disease (a forced expiratory volume in one second to forced vital capacity ratio of <80%), respiratory failure (NYHA Class III and IV), as well as decreased lung diffuse capacity and high skin scores. The authors first propose a concept of the existence and pathogenesis of chronic scleroderma nephropathy, the basis for which is the vascular endothelial dysfunction phenomenon developing in different structural components of the nephron and kidney as a whole.

системная склеродермиясклеродермический почечный кризсосудистая эндотелиальная дисфункцияхроническая склеродермическая нефропатиянеиммунные факторы прогрессирования нефропатийхроническая болезнь почекsystemic sclerosisscleroderma renal crisisvascular endothelial dysfunctionchronic scleroderma nephropathynonimmune factors of progressive nephropathieschronic kidney diseaseReferencesГусева НГ. Системная склеродермия. Москва: Медицина; 1975. 270 с. [Guseva NG. Sistemnaya sklerodermiya [Systemic scleroderma]. Moscow: Meditsina; 1975. 270 p.].Гусева НГ. Системная склеродермия. Москва: Медицина; 1975. 270 с. [Guseva NG. Sistemnaya sklerodermiya [Systemic scleroderma]. Moscow: Meditsina; 1975. 270 p.].Auspitz H. Ein beitrag zur lehre vom haut-sklerem der erwachsenen. Wien Med Wochenschr. 1863;xiii:755.Auspitz H. Ein beitrag zur lehre vom haut-sklerem der erwachsenen. Wien Med Wochenschr. 1863;xiii:755.Lynch B, Burns A. Rheumatological Conditions and the Kidney. In: Harber M, editor. Practical Nephrology. Springer; 2014. P. 291–5.Lynch B, Burns A. Rheumatological Conditions and the Kidney. In: Harber M, editor. Practical Nephrology. Springer; 2014. P. 291–5.Trostle DS, Bedetti CD, Steen VD, et. al. Renal vascular histology and morphometry in systemic sclerosis. A case-control autopsy study. Arhritis Rheum. 1988;31:393–400. doi: 10.1002/art.1780310311Trostle DS, Bedetti CD, Steen VD, et. al. Renal vascular histology and morphometry in systemic sclerosis. A case-control autopsy study. Arhritis Rheum. 1988;31:393–400. doi: 10.1002/art.1780310311Steen VD, Medsger TA. Change in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis. 2007;66:940–4. doi: 10.1136/ard.2006.066068Steen VD, Medsger TA. Change in causes of death in systemic sclerosis, 1972–2002. Ann Rheum Dis. 2007;66:940–4. doi: 10.1136/ard.2006.066068Поражение почек при системной склеродермии. В кн.: Тареев ЕМ, редактор. Основы нефрологии. Москва: Медицина; 1972. С. 635–44 [Renal disease in systemic sclerosis. In: Tareev EM, editor. Osnovy nefrologii [Fundamentals of Nephrology]. Moscow: Meditsina; 1972. P. 635–44].Поражение почек при системной склеродермии. В кн.: Тареев ЕМ, редактор. Основы нефрологии. Москва: Медицина; 1972. С. 635–44 [Renal disease in systemic sclerosis. In: Tareev EM, editor. Osnovy nefrologii [Fundamentals of Nephrology]. Moscow: Meditsina; 1972. P. 635–44].Shanmugam VK, Steen VD. Renal manifestations in scleroderma: evidence for subclinical renal disease as a marker of vasculopathy. Reumatol Int. 2010;2010:ID-538589.Shanmugam VK, Steen VD. Renal manifestations in scleroderma: evidence for subclinical renal disease as a marker of vasculopathy. Reumatol Int. 2010;2010:ID-538589.Yang Y, Liu T, Zhao J, et al. Warfarin-related nephropathy: prevalence, risk factors and prognosis. Int J Cardiol. 2014;176:1297–8. doi: 10.1016/j.ijcard.2014.07.166Yang Y, Liu T, Zhao J, et al. Warfarin-related nephropathy: prevalence, risk factors and prognosis. Int J Cardiol. 2014;176:1297–8. doi: 10.1016/j.ijcard.2014.07.166Bosch X, Poch E, Josep MG. Rhabdomyolysis and acute kidney injury. N Engl J Med. 2009;361:62–72. doi: 10.1056/NEJMra0801327Bosch X, Poch E, Josep MG. Rhabdomyolysis and acute kidney injury. N Engl J Med. 2009;361:62–72. doi: 10.1056/NEJMra0801327Steen VD. Scleroderma renal crisis. Rheum Dis Clin North Am. 2003;29:315. doi: 10.1016/S0889-857X(03)00016-4Steen VD. Scleroderma renal crisis. Rheum Dis Clin North Am. 2003;29:315. doi: 10.1016/S0889-857X(03)00016-4Steen VD, Costantino JP, Shapiro AP, et al. Outcome of renal crisis in system sclerosis: relation to availability of angiotensin converting enzyme (ACE) inhibitors. Ann Intern Med. 1990;113:352–67. doi: 10.7326/0003-4819-113-5-352Steen VD, Costantino JP, Shapiro AP, et al. Outcome of renal crisis in system sclerosis: relation to availability of angiotensin converting enzyme (ACE) inhibitors. Ann Intern Med. 1990;113:352–67. doi: 10.7326/0003-4819-113-5-352Walker UA, 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;66:754–63. doi: 10.1136/ard.2006.062901Walker UA, 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;66:754–63. doi: 10.1136/ard.2006.062901Moore HC, Sheehan HL. The kidney of scleroderma. Lancet. 1952;259(6698):68–70. doi: 10.1016/S0140-6736(52)91798-4Moore HC, Sheehan HL. The kidney of scleroderma. Lancet. 1952;259(6698):68–70. doi: 10.1016/S0140-6736(52)91798-4Shanmugam VK, Steen VD. Renal disease in scleroderma: an update on evaluation, risk stratification, pathogenesis and management. Curr Opin Rheumatol. 2012 November;24(6):669–76. doi: 10.1097/BOR.0b013e3283588dcfShanmugam VK, Steen VD. Renal disease in scleroderma: an update on evaluation, risk stratification, pathogenesis and management. Curr Opin Rheumatol. 2012 November;24(6):669–76. doi: 10.1097/BOR.0b013e3283588dcfPenn H, Howie AJ, Kingdon EJ, et al. Scleroderma renal crisis: patient characteristics and long-term outcomes. QJM. 2007;100:485–94. doi: 10.1093/qjmed/hcm052Penn H, Howie AJ, Kingdon EJ, et al. Scleroderma renal crisis: patient characteristics and long-term outcomes. QJM. 2007;100:485–94. doi: 10.1093/qjmed/hcm052Steen VD. Kidney involvement in systemic sclerosis. La Presse Medicale. 2014;43(10):e305-14. doi: 10.1016/j.lpm.2014.02.031Steen VD. Kidney involvement in systemic sclerosis. La Presse Medicale. 2014;43(10):e305-14. doi: 10.1016/j.lpm.2014.02.031Clements PJ, Lachenbruch PA, Furs DE, et al. Abnormalitis of renal physiology in systemic sclerosis. A prospective study with 10-year follow-up. Arthritis Rheum. 1994;37:67–74. doi: 10.1002/art.1780370110Clements PJ, Lachenbruch PA, Furs DE, et al. Abnormalitis of renal physiology in systemic sclerosis. A prospective study with 10-year follow-up. Arthritis Rheum. 1994;37:67–74. doi: 10.1002/art.1780370110Teixeira 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;67(1):110–6. doi: 10.1136/ard.2006.066985Teixeira 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;67(1):110–6. doi: 10.1136/ard.2006.066985Qian Q. Thrombotic microangiopathy not associated with the classic/idiopathic TTP-HUS. Core concepts in parenchymal kidney disease. New York: Springer; 2014. P. 249–62.Qian Q. Thrombotic microangiopathy not associated with the classic/idiopathic TTP-HUS. Core concepts in parenchymal kidney disease. New York: Springer; 2014. P. 249–62.Sachin SS, Ronco C, Pophale R. Cardio-renal syndrome type 5: epidemiology, pathophysiology and treatment. Semin Nephrol. 2012;32:49–56. doi: 10.1016/j.semnephrol.2011.11.007Sachin SS, Ronco C, Pophale R. Cardio-renal syndrome type 5: epidemiology, pathophysiology and treatment. Semin Nephrol. 2012;32:49–56. doi: 10.1016/j.semnephrol.2011.11.007Naniwa 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;17(1):37–44. doi: 10.3109/s10165- 006-0540-0Naniwa 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;17(1):37–44. doi: 10.3109/s10165- 006-0540-0Vesely SK, George JN, 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;102:60–8. doi: 10.1182/blood-2003-01-0193Vesely SK, George JN, 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;102:60–8. doi: 10.1182/blood-2003-01-0193Batal I, Domsic RT, Shafer A, et al. Scleroderma renal crisis: a pathology perspective. Int J Rheumatol. 2010;2010:543705. doi: 10.1155/2010/543704Batal I, Domsic RT, Shafer A, et al. Scleroderma renal crisis: a pathology perspective. Int J Rheumatol. 2010;2010:543705. doi: 10.1155/2010/543704Hudson 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. Semin Arthritis Rheum. 2014;43:666–72. doi: 10.1016/j.semarthrit.2013.09.008Hudson 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. Semin Arthritis Rheum. 2014;43:666–72. doi: 10.1016/j.semarthrit.2013.09.008Hudson 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 ssociated with worse outcomes-methodology and preliminary results. Int J Rheumatol. 2010;2010.pii:347402. doi: 10.1155/2010/347402. Epub 2010 Sep 14.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 ssociated with worse outcomes-methodology and preliminary results. Int J Rheumatol. 2010;2010.pii:347402. doi: 10.1155/2010/347402. Epub 2010 Sep 14.Denton CP, Lapadula G, Mouthon L, et al. Renal complication and scleroderma renal crisis. Rhematology. 2009;48:iii32-5. doi: 10.1093/rheumatology/ken483Denton CP, Lapadula G, Mouthon L, et al. Renal complication and scleroderma renal crisis. Rhematology. 2009;48:iii32-5. doi: 10.1093/rheumatology/ken483Борисов ИА, Голованова ОЕ, Гордеев АВ и др. Хроническая почечная недостаточность у людей пожилого и старческого возраста. Терапевтический архив. 1988;(6):111–6 [Borisov IA, Golovanova OE, Gordeev AV, et al. Chronic renal failure in elderly and senile. Terapevticheskii arkhiv. 1988;(6):111–6 (In Russ.)].Борисов ИА, Голованова ОЕ, Гордеев АВ и др. Хроническая почечная недостаточность у людей пожилого и старческого возраста. Терапевтический архив. 1988;(6):111–6 [Borisov IA, Golovanova OE, Gordeev AV, et al. Chronic renal failure in elderly and senile. Terapevticheskii arkhiv. 1988;(6):111–6 (In Russ.)].Сура ВВ, Борисов ИА, Гордеев АВ, Камаева ОИ. О факторах прогрессирования патологии почек и принципиальных возможностях воздействия на них. Терапевтический архив. 1998;(12):5–8 [Sura VV, Borisov IA, Gordeev AV, Kamaeva OI. Factors progression of renal disease and the fundamental possibility of exposure to them. Terapevticheskii arkhiv. 1998;(12):5–8 (In Russ.)].Сура ВВ, Борисов ИА, Гордеев АВ, Камаева ОИ. О факторах прогрессирования патологии почек и принципиальных возможностях воздействия на них. Терапевтический архив. 1998;(12):5–8 [Sura VV, Borisov IA, Gordeev AV, Kamaeva OI. Factors progression of renal disease and the fundamental possibility of exposure to them. Terapevticheskii arkhiv. 1998;(12):5–8 (In Russ.)].Bose N, Chiesa-Vottero A, Chatterjee S, et al. Scleroderma renal crisis. Semin Arthritis Rheum. 2015 Jun;44(6):687–94. doi: 10.1016/j.semarthrit.2014.12.001. Epub 2014 Dec 11.Bose N, Chiesa-Vottero A, Chatterjee S, et al. Scleroderma renal crisis. Semin Arthritis Rheum. 2015 Jun;44(6):687–94. doi: 10.1016/j.semarthrit.2014.12.001. Epub 2014 Dec 11.Redd D, Frech TM, Murthaugh MA. Informatics can identify systemic sclerosis patients at risk for scleroderma renal crisis. Comp Biol Med. 2014;53:203–5. doi: 10.1016/j.compbiomed.2014.07.022 31. Helfrich DJ, Banner B, Steen VD, et al. Normotensive renal failure in systemic sclerosis. Arthritis Rheum. 1989;32:1128–34. doi: 10.1002/anr.1780320911Redd D, Frech TM, Murthaugh MA. Informatics can identify systemic sclerosis patients at risk for scleroderma renal crisis. Comp Biol Med. 2014;53:203–5. doi: 10.1016/j.compbiomed.2014.07.022 31. Helfrich DJ, Banner B, Steen VD, et al. Normotensive renal failure in systemic sclerosis. Arthritis Rheum. 1989;32:1128–34. doi: 10.1002/anr.1780320911Steen VD, Medsger TA. Case-control study of corticosteroids and other drugs that either precipitate or protect from the development of scleroderma renal crisis. Arthritis Rheum. 1998;41:1613–19. doi: 10.1002/1529-0131(199809)41:9<1613::AID-ART11>3.0.CO;2-OSteen VD, Medsger TA. Case-control study of corticosteroids and other drugs that either precipitate or protect from the development of scleroderma renal crisis. Arthritis Rheum. 1998;41:1613–19. doi: 10.1002/1529-0131(199809)41:9<1613::AID-ART11>3.0.CO;2-ODeMarco PJ, Weisman MH, Seibold JR, et al. Predictors and outcomes of scleroderma renal crisis: the high-dose versus low-dose D-penicillamine in early diffuse systemic sclerosis trial. Arthritis Rheum. 2002;46:2983–9. doi: 10.1002/art.10589DeMarco PJ, Weisman MH, Seibold JR, et al. Predictors and outcomes of scleroderma renal crisis: the high-dose versus low-dose D-penicillamine in early diffuse systemic sclerosis trial. Arthritis Rheum. 2002;46:2983–9. doi: 10.1002/art.10589Teixeira L, Mouthon L, Mahr A, et al. Scleroderma renal crisis: presentation, outcome and risk factors based on a retrospective multicenter study of 50 patients. Arthritis Rheum. 2006;54 Suppl:s743.Teixeira L, Mouthon L, Mahr A, et al. Scleroderma renal crisis: presentation, outcome and risk factors based on a retrospective multicenter study of 50 patients. Arthritis Rheum. 2006;54 Suppl:s743.Okano Y, Steen VD, Medsger TA. Auto-antibody reactive with RNA polymerase III in systemic sclerosis. Ann Intern Med. 1993;119:1005–13. doi: 10.7326/0003-4819-119-10-199311150- 00007Okano Y, Steen VD, Medsger TA. Auto-antibody reactive with RNA polymerase III in systemic sclerosis. Ann Intern Med. 1993;119:1005–13. doi: 10.7326/0003-4819-119-10-199311150- 00007Bunn CC, Denton CP, Shi-Wen X, et al. Anti-RNA polymerases and other autoantibody specificities in systemic sclerosis. Br J Rheumatol. 1998;37:15–20. doi: 10.1093/rheumatology/37.1.15Bunn CC, Denton CP, Shi-Wen X, et al. Anti-RNA polymerases and other autoantibody specificities in systemic sclerosis. Br J Rheumatol. 1998;37:15–20. doi: 10.1093/rheumatology/37.1.15Yanaba K, Asano Y, Tada Y, et al. Increased serum soluble CD147 levels in patients with systemic sclerosis: association with scleroderma renal crisis. Clin Rheum. 2012:31(5):835–9. doi: 10.1007/s10067-012-1949-9Yanaba K, Asano Y, Tada Y, et al. Increased serum soluble CD147 levels in patients with systemic sclerosis: association with scleroderma renal crisis. Clin Rheum. 2012:31(5):835–9. doi: 10.1007/s10067-012-1949-9Nguyen B, Mayes MD, Arnett FC, et al. HLA-DRB1*0407 and *1304 are risk factors for scleroderma renal ctisis. Arthritis Rheum. 2011;63:530–4. doi: 10.1002/art.30111Nguyen B, Mayes MD, Arnett FC, et al. HLA-DRB1*0407 and *1304 are risk factors for scleroderma renal ctisis. Arthritis Rheum. 2011;63:530–4. doi: 10.1002/art.30111Гордеев АВ, Савицкий СН, Сура ВВ. Старческий пиелонефрит: патофизиология гипертензивного синдрома. Терапевтический архив. 1993;(6):30–4 [Gordeev AV, Savitskii SN, Sura VV. Senile pyelonephritis: pathophysiology of hypertensive syndrome. Terapevticheskii arkhiv. 1993;(6):30–4 (In Russ.)].Гордеев АВ, Савицкий СН, Сура ВВ. Старческий пиелонефрит: патофизиология гипертензивного синдрома. Терапевтический архив. 1993;(6):30–4 [Gordeev AV, Savitskii SN, Sura VV. Senile pyelonephritis: pathophysiology of hypertensive syndrome. Terapevticheskii arkhiv. 1993;(6):30–4 (In Russ.)].Kowal-Bielecka O, Landewe R, Avouac J, et al. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis. 2009;68(5):620–8. doi: 10.1136/ard.2008.096677Kowal-Bielecka O, Landewe R, Avouac J, et al. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR). Ann Rheum Dis. 2009;68(5):620–8. doi: 10.1136/ard.2008.096677Cheung WY, Gibson IW, Rush D, et al. Late recurrence of scleroderma renal crisis: poor outcome despite angiotensin II blockade. Am J Kidney Dis. 2005;45:930–4. doi: 10.1053/j.ajkd.2005.01.007Cheung WY, Gibson IW, Rush D, et al. Late recurrence of scleroderma renal crisis: poor outcome despite angiotensin II blockade. Am J Kidney Dis. 2005;45:930–4. doi: 10.1053/j.ajkd.2005.01.007Izzedine H, Rouvier P, Deray G. Endotelin receptor antagonismbased treatment for scleroderma renal crisis. Am J Kidney Dis. 2013;62(2):394–5. doi: 10.1053/j.ajkd.2013.04.016Izzedine H, Rouvier P, Deray G. Endotelin receptor antagonismbased treatment for scleroderma renal crisis. Am J Kidney Dis. 2013;62(2):394–5. doi: 10.1053/j.ajkd.2013.04.016Chighizola CB, Penn H, Meroni PL, et al. The N-terminal fragment of brain natriuretic peptide as an outcome predictor in scleroderma renal crisis. Arthritis Rheum. 2011;63:S573.Chighizola CB, Penn H, Meroni PL, et al. The N-terminal fragment of brain natriuretic peptide as an outcome predictor in scleroderma renal crisis. Arthritis Rheum. 2011;63:S573.Foneseca C, Renzoni E, Sestini P, et al. Endotehelin axis polymorphismism in patients with scleroderms. Arthriris Rheum. 2006;54:3034–42. doi: 10.1002/art.22036Foneseca C, Renzoni E, Sestini P, et al. Endotehelin axis polymorphismism in patients with scleroderms. Arthriris Rheum. 2006;54:3034–42. doi: 10.1002/art.22036Siva B, McDonald SP, Hawley CM, et al. End-stage kidney disease due to scleroderma – outcomes in 127 consecutive ANZDATA registry cases. Nephrol Dial Transplant. 2011;26:3165–71. doi: 10.1093/ndt/gfq861Siva B, McDonald SP, Hawley CM, et al. End-stage kidney disease due to scleroderma – outcomes in 127 consecutive ANZDATA registry cases. Nephrol Dial Transplant. 2011;26:3165–71. doi: 10.1093/ndt/gfq861Reem HA, Hania SZ, Amr A. Renal disease in systemic sclerosis with normal serum creatinine. Clin Rheumatol. 2010;29:729–37. doi: 10.1007/s10067-010-1389-3Reem HA, Hania SZ, Amr A. Renal disease in systemic sclerosis with normal serum creatinine. Clin Rheumatol. 2010;29:729–37. doi: 10.1007/s10067-010-1389-3Gigante A, Rosato E, Massa R, et al. Evalution of Chronic Kidney Disease Epidemiology Collaboration equation to estimate glomerular filtration rate in scleroderma patients. Rheumatology. 2010;51:1426–31. doi: 10.1093/rheumatology/kes049Gigante A, Rosato E, Massa R, et al. Evalution of Chronic Kidney Disease Epidemiology Collaboration equation to estimate glomerular filtration rate in scleroderma patients. Rheumatology. 2010;51:1426–31. doi: 10.1093/rheumatology/kes049Scheja A, Bartosik I, Wuttge DM, et al. Renal function is mostly preserved in patients with systemic sclerosis. Scand J Rheumatol. 2009;38:295–8. doi: 10.1080/03009740802629424Scheja A, Bartosik I, Wuttge DM, et al. Renal function is mostly preserved in patients with systemic sclerosis. Scand J Rheumatol. 2009;38:295–8. doi: 10.1080/03009740802629424Campo A, Mathai SC, Le Pavec J, et al. Hemodynamic predictors of survival in scleroderma-related pulmonary arterial hypertension. Am J Respir Crit Care Med. 2010;182:252–60. doi: 10.1164/rccm.200912-1820OCCampo A, Mathai SC, Le Pavec J, et al. Hemodynamic predictors of survival in scleroderma-related pulmonary arterial hypertension. Am J Respir Crit Care Med. 2010;182:252–60. doi: 10.1164/rccm.200912-1820OCLivi R, Teghini L, Pignone A, et al. Renal functional reserve is impaired in patients with systemic sclerosis without clinical signs of kidney involvement. Ann Rheum Dis. 2002;61:682–6. doi: 10.1136/ard.61.8.682Livi R, Teghini L, Pignone A, et al. Renal functional reserve is impaired in patients with systemic sclerosis without clinical signs of kidney involvement. Ann Rheum Dis. 2002;61:682–6. doi: 10.1136/ard.61.8.682Livi R, Giuducci S, Perfetto F, et al. Lack of activation of renal functional reserve predicts the risk of significant renal involvement in systemic sclerosis. Ann Rheum Dis. 2011;70:1963–7. doi: 10.1136/ard.2011.152892Livi R, Giuducci S, Perfetto F, et al. Lack of activation of renal functional reserve predicts the risk of significant renal involvement in systemic sclerosis. Ann Rheum Dis. 2011;70:1963–7. doi: 10.1136/ard.2011.152892Meier FMP, Frommer KW, Dinser R, et al. Update on the profile of the EUSTAR cohort: an analysis of the EULAR Scleroderma Trials and Research group database. Ann Rheum Dis. 2012;71:1355–60. doi: 10.1136/annrheumdis-2011-200742Meier FMP, Frommer KW, Dinser R, et al. Update on the profile of the EUSTAR cohort: an analysis of the EULAR Scleroderma Trials and Research group database. Ann Rheum Dis. 2012;71:1355–60. doi: 10.1136/annrheumdis-2011-200742Tyndall AJ, Bannert B, Vonk M, et al. Cases and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69:1809–15. doi: 10.1136/ard.2009.114264Tyndall AJ, Bannert B, Vonk M, et al. Cases and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69:1809–15. doi: 10.1136/ard.2009.114264Sieberlich L, Hunzelmann N, Krieg T, et al. Intermediate molecular weight proteinuria and albuminuria identify scleroderma patients with increased morbidity. Clin Nephrology. 2008;7(2):110–7. doi: 10.5414/CNP70110Sieberlich L, Hunzelmann N, Krieg T, et al. Intermediate molecular weight proteinuria and albuminuria identify scleroderma patients with increased morbidity. Clin Nephrology. 2008;7(2):110–7. doi: 10.5414/CNP70110Dawnay A, Wilson AGT, Lamb E, et al. Microalbuminuria in systemic sclerosis. Ann Rhem Dis. 1992;51(3):384–8. doi: 10.1136/ard.51.3.384Dawnay A, Wilson AGT, Lamb E, et al. Microalbuminuria in systemic sclerosis. Ann Rhem Dis. 1992;51(3):384–8. doi: 10.1136/ard.51.3.384Rubel D, Stock J, Ciner A, et al. Antifibrotic, nephroprotective effects of paricalcitol versus calcitol on top of ACE-inhibitor therapy in the COL4A3 knockout mouse model for progressive renal fibrosis. Nephrol Dial Transplant. 2014;29(5):1012–9. doi: 10.1093/ndt/gft434Rubel D, Stock J, Ciner A, et al. Antifibrotic, nephroprotective effects of paricalcitol versus calcitol on top of ACE-inhibitor therapy in the COL4A3 knockout mouse model for progressive renal fibrosis. Nephrol Dial Transplant. 2014;29(5):1012–9. doi: 10.1093/ndt/gft434Rivolta R, Mascagni B, Berruti V, et al. Renal vascular damage in systemic sclerosis patients without clinical evidence of nephropathy. Arthritis Rheum. 1996;24:1944–8. doi: 10.1002/art.1780390622Rivolta R, Mascagni B, Berruti V, et al. Renal vascular damage in systemic sclerosis patients without clinical evidence of nephropathy. Arthritis Rheum. 1996;24:1944–8. doi: 10.1002/art.1780390622Nishijima C, Sato S, Hasegawa M, et al. Renal vascular damage in Japanese patients with systemic sclerosis. Rheumatology. 2001;40:406–9. doi: 10.1093/rheumatology/40.4.406Nishijima C, Sato S, Hasegawa M, et al. Renal vascular damage in Japanese patients with systemic sclerosis. Rheumatology. 2001;40:406–9. doi: 10.1093/rheumatology/40.4.406Rosato E, Gigante A, Barbano B, et al. Intrarenal hemodynamic parameters correlate with glomerular filtration rate and digital microvascular damage in patients with systemic sclerosis. Semin Arthritis Rheum. 2012;41:815–21. doi: 10.1016/j.semarthrit.2011.11.005Rosato E, Gigante A, Barbano B, et al. Intrarenal hemodynamic parameters correlate with glomerular filtration rate and digital microvascular damage in patients with systemic sclerosis. Semin Arthritis Rheum. 2012;41:815–21. doi: 10.1016/j.semarthrit.2011.11.005Rosato E, Cianci R, Barbano B, et al. N-acetylcycteine infusion reduces the resistance index of renal artery in the early stage of systemic sclerosis. Acta Pharmacol Sin. 2009;30:1283–8. doi: 10.1038/aps.2009.128Rosato E, Cianci R, Barbano B, et al. N-acetylcycteine infusion reduces the resistance index of renal artery in the early stage of systemic sclerosis. Acta Pharmacol Sin. 2009;30:1283–8. doi: 10.1038/aps.2009.128Balakumar P, Chakkarwar VA, Krishan P, et al. Vascular endothelial dysfunction: a tug of war in diabetic nephropathy? Biomed Pharmacother. 2009;63:171–9. doi: 10.1016/j.biopha.2008.08.008Balakumar P, Chakkarwar VA, Krishan P, et al. Vascular endothelial dysfunction: a tug of war in diabetic nephropathy? Biomed Pharmacother. 2009;63:171–9. doi: 10.1016/j.biopha.2008.08.008Shah R. Endothelin in health and disease. Eur J Intern Med. 2007;18:272–82. doi: 10.1016/j.ejim.2007.04.002Shah R. Endothelin in health and disease. Eur J Intern Med. 2007;18:272–82. doi: 10.1016/j.ejim.2007.04.002Dhaun N, Goddard J, Webb DJ. The endothelin system and its antagonism in chronic kidney disease. J Am Soc Nephrol. 2006;17(4):943–55. doi: 10.1681/ASN.2005121256Dhaun N, Goddard J, Webb DJ. The endothelin system and its antagonism in chronic kidney disease. J Am Soc Nephrol. 2006;17(4):943–55. doi: 10.1681/ASN.2005121256Liakouli V, Capriani P, Marelli A, et al. Angiogenic cytokines and growth factors in systemic sclerosis. Autoimmun Rev. 2011;10:590–4. doi: 10.1016/j.autrev.2011.04.019Liakouli V, Capriani P, Marelli A, et al. Angiogenic cytokines and growth factors in systemic sclerosis. Autoimmun Rev. 2011;10:590–4. doi: 10.1016/j.autrev.2011.04.019Ruffatti A, Sinico RA, Radice A, et al. Autoantibodies to proteinase 3 and myeloperoxidase in systemic sclerosis. J Rheumatol. 2002;29(5):918–23.Ruffatti A, Sinico RA, Radice A, et al. Autoantibodies to proteinase 3 and myeloperoxidase in systemic sclerosis. J Rheumatol. 2002;29(5):918–23.Arad U, Balbir-Gurman A, Doenyas-Barak K, et al. Anti-neutrophil antibody associated vasculitis in systemic sclerosis. Semin Arthritis Rheum. 2011;41:223–9. doi: 10.1016/j.semarthrit.2010.11.001Arad U, Balbir-Gurman A, Doenyas-Barak K, et al. Anti-neutrophil antibody associated vasculitis in systemic sclerosis. Semin Arthritis Rheum. 2011;41:223–9. doi: 10.1016/j.semarthrit.2010.11.001Derrett-Smith EC, Nihtyanova SI, Harvey J, et al. Revisiting ANCA-associated vasculitis in systemic sclerosis: clinical, serological and immunogenetic factors. Rheumatology. 2013;52:1824–31. doi: 10.1093/rheumatology/ket213Derrett-Smith EC, Nihtyanova SI, Harvey J, et al. Revisiting ANCA-associated vasculitis in systemic sclerosis: clinical, serological and immunogenetic factors. Rheumatology. 2013;52:1824–31. doi: 10.1093/rheumatology/ket213Akkara Veetil BM, Schimmer BM. A case of limited systemic sclerosis with p-ANCA, complicated by multiple cerebral hemorrhages. Rheumatol Int. 2009;29:325–9. doi: 10.1007/s00296-008-0675-8Akkara Veetil BM, Schimmer BM. A case of limited systemic sclerosis with p-ANCA, complicated by multiple cerebral hemorrhages. Rheumatol Int. 2009;29:325–9. doi: 10.1007/s00296-008-0675-8Jones RB, Ferraro AJ, Chaudhry AN, et al. A multicenter survey of rituximab therapy for refractory antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2009;60(7):2156–68. doi: 10.1002/art.24637Jones RB, Ferraro AJ, Chaudhry AN, et al. A multicenter survey of rituximab therapy for refractory antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2009;60(7):2156–68. doi: 10.1002/art.24637Merkel PA, Chang Y, Pierangeli SS, et al. The prevalence and clinical associations of anticardiolipin antibodies in a large inception cohort of patients with connective tissue diseases. Am J Med. 1996;101(6):576–83. doi: 10.1016/S0002-9343(96)00335-XMerkel PA, Chang Y, Pierangeli SS, et al. The prevalence and clinical associations of anticardiolipin antibodies in a large inception cohort of patients with connective tissue diseases. Am J Med. 1996;101(6):576–83. doi: 10.1016/S0002-9343(96)00335-XParodi A, Drosera M, Barbieri L, Rebora A. Antiphospholipid antibody system in systemic sclerosis. Rheumatology. 2001;40(1):111–2. doi: 10.1093/rheumatology/40.1.111Parodi A, Drosera M, Barbieri L, Rebora A. Antiphospholipid antibody system in systemic sclerosis. Rheumatology. 2001;40(1):111–2. doi: 10.1093/rheumatology/40.1.111Schoenroth L, Fritzler M, Lonzetti L, Senecal J-L. Antibodies to β2 glycoprotein I and cardiolipin in SSc. Ann Rheum Dis. 2002;61(2):183a-4a. doi: 10.1136/ard.61.2.183-aSchoenroth L, Fritzler M, Lonzetti L, Senecal J-L. Antibodies to β2 glycoprotein I and cardiolipin in SSc. Ann Rheum Dis. 2002;61(2):183a-4a. doi: 10.1136/ard.61.2.183-aAssous N, Allanore Y, Batteux F, et al. Prevalence of antiphospholipid antibodies in systemic sclerosis and association with primitive pulmonary arterial hypertension and endothelial injury. Clin xp Rheumatol. 2005;23:199–204.Assous N, Allanore Y, Batteux F, et al. Prevalence of antiphospholipid antibodies in systemic sclerosis and association with primitive pulmonary arterial hypertension and endothelial injury. Clin xp Rheumatol. 2005;23:199–204.Toure AO, Ly F, Diatta A, et al. Antiphospholipid antibodies and systemic scleroderma. Turk J Haematol. 2013;30(1):32–6. doi: 10.4274/tjh.2012.0059Toure AO, Ly F, Diatta A, et al. Antiphospholipid antibodies and systemic scleroderma. Turk J Haematol. 2013;30(1):32–6. doi: 10.4274/tjh.2012.0059Sulik A, Kowal-Bielecka O, Domyslawska I, et al. The prevalence and clinical significance of antiphospholipid antibodies in patients with systemic sclerosis – preliminary report. Rocz Akad Med Bialymst. 2005;50:228–31.Sulik A, Kowal-Bielecka O, Domyslawska I, et al. The prevalence and clinical significance of antiphospholipid antibodies in patients with systemic sclerosis – preliminary report. Rocz Akad Med Bialymst. 2005;50:228–31.Wielosz E, Dryglewska M, Majdan M. Antiphospholipid antibodies and kidney involvement in patients with systemic sclerosis. Clin Rheumatol. 2009;28:955–9. doi: 10.1007/s10067-009-1188-xWielosz E, Dryglewska M, Majdan M. Antiphospholipid antibodies and kidney involvement in patients with systemic sclerosis. Clin Rheumatol. 2009;28:955–9. doi: 10.1007/s10067-009-1188-xСавицкий СН, Гордеев АВ. Гемостаз и почки. Терапевтический архив. 1992;(6):97–100 [Savitskii SN, Gordeev AV. Hemostasis and kidneys. Terapevticheskii arkhiv. 1992;(6):97–100 (In Russ.)].Савицкий СН, Гордеев АВ. Гемостаз и почки. Терапевтический архив. 1992;(6):97–100 [Savitskii SN, Gordeev AV. Hemostasis and kidneys. Terapevticheskii arkhiv. 1992;(6):97–100 (In Russ.)].Ребров АП, Гайдукова ИЗ, Патрикеева ДА. Хроническая болезнь почек у пациентов с системной склеродермией: частота встречаемости и факторы, ассоциирующиеся с нарушением функции почек. Клиническая медицина. 2013;(6):34–8 [Rebrov AP, Gaidukova IZ, Patrikeeva DA. Chronic kidney disease in patients with systemic sclerosis: the incidence of and factors associated with impaired renal function. Klinicheskaya meditsina. 2013;(6):34–8 (In Russ.)].Ребров АП, Гайдукова ИЗ, Патрикеева ДА. Хроническая болезнь почек у пациентов с системной склеродермией: частота встречаемости и факторы, ассоциирующиеся с нарушением функции почек. Клиническая медицина. 2013;(6):34–8 [Rebrov AP, Gaidukova IZ, Patrikeeva DA. Chronic kidney disease in patients with systemic sclerosis: the incidence of and factors associated with impaired renal function. Klinicheskaya meditsina. 2013;(6):34–8 (In Russ.)].Anders H-J, Vielhauer V. Renal co-morbidity in patients with rheumatic disease Arthritis Res Ther. 2011;13:222. doi: 10.1186/ar3256Anders H-J, Vielhauer V. Renal co-morbidity in patients with rheumatic disease Arthritis Res Ther. 2011;13:222. doi: 10.1186/ar3256Моисеев BC, Мухин НА, редакторы. Сердечно-сосудистый риск и хроническая болезнь почек: стратегии кардио-нефропротекции. Национальные рекомендации. Москва; 2013 [Moiseev BC, Mukhin NA, editors. Serdechno-sosudistyi risk i khronicheskaya bolezn' pochek: strategii kardio-nefroprotektsii. Natsional'nye rekomendatsii [Cardiovascular risk and chronic kidney disease: strategies of cardionephroprotection. National Guidelance]. Moscow; 2013].Моисеев BC, Мухин НА, редакторы. Сердечно-сосудистый риск и хроническая болезнь почек: стратегии кардио-нефропротекции. Национальные рекомендации. Москва; 2013 [Moiseev BC, Mukhin NA, editors. Serdechno-sosudistyi risk i khronicheskaya bolezn' pochek: strategii kardio-nefroprotektsii. Natsional'nye rekomendatsii [Cardiovascular risk and chronic kidney disease: strategies of cardionephroprotection. National Guidelance]. Moscow; 2013].Levey AS, Coresh J. Chronic kidney disease. Lancet. 2012;379:165–80. doi: 10.1016/S0140-6736(11)60178-5Levey AS, Coresh J. Chronic kidney disease. Lancet. 2012;379:165–80. doi: 10.1016/S0140-6736(11)60178-5Гордеев АВ, Галушко ЕА, Насонов ЕЛ. Концепция мультиморбидности в ревматологической практике. Научно-практическая ревматология. 2014;52(4):362–5 [Gordeev AV, Galushko EA, Nasonov EL. The concept of multimorbidity in rheumatologic practice. Nauchno-prakticheskaya revmatologiya = Rheumatology Science and Practice. 2014;52(4):362–5 (In Russ.)]. doi: 10.14412/1995-4484-2014-362-365Гордеев АВ, Галушко ЕА, Насонов ЕЛ. Концепция мультиморбидности в ревматологической практике. Научно-практическая ревматология. 2014;52(4):362–5 [Gordeev AV, Galushko EA, Nasonov EL. The concept of multimorbidity in rheumatologic practice. Nauchno-prakticheskaya revmatologiya = Rheumatology Science and Practice. 2014;52(4):362–5 (In Russ.)]. doi: 10.14412/1995-4484-2014-362-365Balbir-Gurman A, Braun-Moscovici Y. Scleroderma – New aspects in pathogenesis and treatment. Best Pract Res Clin Rheumatol. 2012;26:13–24. doi: 10.1016/j.berh.2012.01.011Balbir-Gurman A, Braun-Moscovici Y. Scleroderma – New aspects in pathogenesis and treatment. Best Pract Res Clin Rheumatol. 2012;26:13–24. doi: 10.1016/j.berh.2012.01.011Rodrigeuez-Itrurbe B, Ferrebuz A,Vanegas V, et al. Early treatment with cGMP phospodiesterase inhibitor ameliorates progression of renal damage. Kidney Int. 2005;68:2131–42. doi: 10.1111/j.1523-1755.2005.00669.xRodrigeuez-Itrurbe B, Ferrebuz A,Vanegas V, et al. Early treatment with cGMP phospodiesterase inhibitor ameliorates progression of renal damage. Kidney Int. 2005;68:2131–42. doi: 10.1111/j.1523-1755.2005.00669.xJaffe IA. Induction of auto-immune syndromes by penicillamine therapy in rheumatoid arthritis and other diseases. Semin Immunopathol. 1981;4:193–207. doi: 10.1007/BF01857095Jaffe IA. Induction of auto-immune syndromes by penicillamine therapy in rheumatoid arthritis and other diseases. Semin Immunopathol. 1981;4:193–207. doi: 10.1007/BF01857095Radic M, Martinovic DK, Radic J. Drug-induced vasculitis: a clinical and pathological review. Neth J Med. 2012;70:16–21.Radic M, Martinovic DK, Radic J. Drug-induced vasculitis: a clinical and pathological review. Neth J Med. 2012;70:16–21.Vedove CD, Del Giglio M, Schena D, et al. Drug-induced lupus erythematosus. Arch Dermatol Res. 2009;301:99–105. doi: 10.1007/s00403-008-0895-5Vedove CD, Del Giglio M, Schena D, et al. Drug-induced lupus erythematosus. Arch Dermatol Res. 2009;301:99–105. doi: 10.1007/s00403-008-0895-5Derk CT, Jimenez SA. Goodpasture-like syndrome induced by Dpenicillamine in patient with systemic sclerosis: report and review of literature. J Rheumatol. 2003;30(7):1616–20.Derk CT, Jimenez SA. Goodpasture-like syndrome induced by Dpenicillamine in patient with systemic sclerosis: report and review of literature. J Rheumatol. 2003;30(7):1616–20.Гусева НГ. Системная склеродермия и псевдосклеродермические синдромы. Москва: Медицина; 1993 [Guseva NG. Sistemnaya sklerodermiya i psevdosklerodermicheskie sindromy [Systemic scleroderma and psevdosclerodermic syndromes]. Moscow: Meditsina; 1993].Гусева НГ. Системная склеродермия и псевдосклеродермические синдромы. Москва: Медицина; 1993 [Guseva NG. Sistemnaya sklerodermiya i psevdosklerodermicheskie sindromy [Systemic scleroderma and psevdosclerodermic syndromes]. Moscow: Meditsina; 1993].

The authors declare that there are no conflicts of interest present.