Clinical course and predictors of death and esrd in patients with systemic lupus erythematosus and systemic vasculitis with kidney involvement

We studied case history, clinical, laboratory and histological data of 85 patients: 64 with SLE, 12 with ANCA-assosiated vasculitis and 9 with cryoglobulinemic vasculitis. Mean age 36,7 ± 14,2 years, M/F 23/62. The duration of the disease was 0,5-384,0 months; acute fase duration was 0,5-12,0 months. 55 (64,7%) patients were treated with prednisone and cyclophosphamide pulses, 9 (10,6%) patients with prednisone and asathioprine, and 21 (24,7%) patients with prednisone only. Patients were observed for 0,5-150,0 months. 2/3 of patient revealed CNS involvement, a half of them - pulmonary disturbances, 1/3 - antyphospholipid syndrome and carditis at the beginning of treatment. In patients with SLE more often were found cerebrovasculitis, carditis, leucopenia and antyphospholipid syndrome, in patients with ANCA-assosiated vasculitis - pulmonary infiltrates, leucocytosis and anemia, in patienys with cryoglobulinemic vasculitis - skin ulcers ( p < 0,05). Kidney involvement was characterised by proteinuria (nephrotic syndrome in more than 40% of cases), arterial hypertension (SAD mediana 150 mm Hg) and deterioration of renal function (serum creatinine mediana 2/6 mg/dl), in patients with cryoglobulinemic vasculitis more often were found nephrotic syndrome and arterial hypertension, in patients with ANCA-assosiated vasculitis - renal insufficiency ( p < 0,05). Combination of prednisone and cyclophosphamid pulses was more effective than prednisone and asathioprine and prednisone only as for renal (remission rate 83,6% versus 66,7%) and also extrarenal (remission rate 94,5% versus 70%) symptoms ( p < 0,05). 12-year survival was 65,2%, 12-year renal survivial - 64,6%, the disesase progressed to ESRD was more often in patients with systemic vasculitis than in SLE ( p < 0,05). Risk factors of death proved to be age >35 years and pulmonary hemorrhages, remission of extrarenal symtomes is a predictor of favourable outcome. Risk facrors of ESRD are serious deterioration of renal function, which demanded RRT at the begining of the treatment and the character of main disease - systemic vasculitis.

системная красная волчанка, системные васкулиты, иммунодепрессивная терапия, прогноз

1. Бажина О.В. Клинико-иммунологические аспекты поражения почек при системных васкулитах и системной красной волчанке. Дисс… к. м. н. 2000.

2. Бугрова О.В. Особенности функционального состояния почек у больных системной красной волчанкой и системной склеродермией. Дисс…д. м. н. 2001.

3. Игнатова Т.М., Апросина З.Г., Серов В.В. и др. Внепеченочные проявления хронической HCV-инфекции. Росс мед. журн. 2001; 2: 13-18.

4. Лучихина Е.Л. Причины неблагоприятных исходов при системной красной волчанке. Росс. мед. журн. 2001; 2: 19-23.

5. Мухин Н.А., Козловская Л.В., Малышко Е.Ю. Криоглобулинемический нефрит, ассоциированный с хронической инфекцией вируса гепатита С. Тер. арх. 2000; 6: 1-5.

6. Насонов Е.Л., Шилкина Н.П., Баранов А.А. Лечение больных с системными васкулитами. Тер. арх. 1998; 7; 11: 53-59.

7. Оттева Э.Н. Современное течение и лечение системной красной волчанки. Дисс…к. м. н. 2002.

8. Соловьев С.К. Эффективность интенсивной терапии у больных системной красной волчанкой с неблагоприятным жизненным прогнозом. Дисс…д. м. н. 2000.

9. Тареева И.Е., Шилов Е.М., Краснова Т.Н. и др. Волчаночный нефрит в середине XX века и в начале XXI века. Тер. арх. 2001; 6: 5-10.

10. Татарченко И.П., Комаров В.Т. Системная красная волчанки и инфекционный эндокардит: грани сходства и различия. Тер. арх. 2002; 4: 39-42.

11. Aasarod K., Bostad L., Hammerstorm J. et al. Renal histopathology and clinical course in 94 patients with Wegener’s granulomatosis. Nephrol Dial Transplant 2001; 16: 953-960.

12. Ball G.V., Bridges Jr.S.L. Ed Vasculitis. New York. Oxford University Press 2002; 1-4: 34-45.

13. Balow J.E., Austin III H.A. Maintenance Therapy for Lupus Nephritis - Something Old, Something New. N Engl J Med 2004; 350; 10: 1044-1046.

14. Bono L., Cameron J.S., Hicks J.A. The very long-term prognosis and complications of lupus nephritis and its treatment. Q J Med 1999; 92: 211-218.

15. Booth A.D., Almond M.K., Burns A. et al. Outcome of ANCA-associated renal vasculitis: a 5-year retrospective study. Am J Kidney Dis 2003; 41; 4: 776-784.

16. Cagnoli L. Instructions and implementations for precutaneous renal biopsy. Guidelines for the therapy of glomerular nephropathies. G Ital Nefrol 2003; 20 (suppl 24): 3-47.

17. Cameron J.S. Lupus Nephritis. J Am Soc Nephrol 1999; 10: 413-424.

18. Costenbader K.H., Karlson E.W., Liang M.H. et al. Defining Lupus Cases for Clinical Studies: The Boston Weighted Crireria for the Classification of Systemic Lupus Erythematosus. J Rheum 2002; 29; 12: 2545-2550.

19. de Groot K., Adu D., Savage C.O. The value of pulse cyclophosphamide in ANCA-associated vasculitis: metha-analysis and critical review. Nephrol Dial Transplant 2001; 16: 2018-2027.

20. Falk R.J., Nachman P.H., Hogan S.L. et al. ANCA glomerulonephritis and vasculitis: a Chapel Hill perspective. Semin Nephrol 2000; 20; 3: 233-243.

21. Fiehn C., Hajjar Y., Mueller K. et al. Improved clinical outcome of lupus nephritis during the past decade: importance of early diagnosis and treatment. Ann Rheun Dis 2003; 62: 435-439.

22. Galli M., Invernizzi F., Monteverde A. et al. Cryoglobulinemic Vasculitis. In Vasculitis New York. Oxford University Press 2002: 500-503.

23. Gupta R.K. Pauci-immune crescentic glomerulonephritis. Indian J Pathol Microbiol 2003; 46; 3: 357-366.

24. Igaki N., Nakaji M., Moriguchi R. et al. A case of hepatitis C virus-associated glomerulopathy presenting with MPO-ANCA-positive rapidly progressive glomerulonephritis. Nippon Jinzo Gakkai 2000; 42; 4: 353-358.

25. Jayne D.R., Rasmussen N., Andrassy K. et al. A Randomized Trial of Maintenance Therapy for Vasculitis Associated with Antineutrophil Cytoplasmic Autoantibodies. N Engl J Med 2003; 349: 36-44.

26. Jara L.J., Vera-Lastra O., Calleja M.C. Pulmonary-renal vasculitic disorders: differential diagnosis and management. Curr Rheumatol Rep 2003; 5; 2: 107-115.

27. Kallenberg C.G.M., Tervaert J.W.C. What is new in systemic vasculitis? (A report from the 9-th International Vasculitis/ANCA Workshop). Ann Rheum Dis 2000; 59; 11: 924-925.

28. Kapitsinou P.P., Ioannidis J.P.A., Boletis J.N. et al. Clinicopathologic Predictors of Death and ESRD in Patients With Pauci-Immune Necrotizing Glomerulonephritis. Am J Kidney Dis 2003; 41; 1: 29-37.

29. Lamprecht P., Schmitt W.H., Gross W.L. Mixed cryoglobulinaemia, glomerulonephritis and ANCA: essential cryoglobulinaemic vasculitis or ANCA-associated vasculitis? Нефрология и диализ 1999; 1; 3: 173-181.

30. Manger K., Manger B., Repp R. et al. Definition of risk factors for death, end stage renal disease, and tromboembolic events in a monocentric cohort of 338 patients with systemic lupus erythematosus. Ann Rheum Dis 2002; 61: 1065-1070.

31. Martins L., Rocha G., Rodriges A. et al. Lupus nephritis: a retrospective review of 78 cases from a single center. Clin Nephrol 2002; 57; 2: 114-119.

32. Mok C.C., Ho C.T., Sin Y.P. et al. Treatment of diffuse proliferative lupus nephritis: a comparison of two cyclophosphamide-containing regimens. Am J Kidney Dis 2001; 38: 256-264.

33. Mok C.C., Wong R.W.S., Lai K.N. Treatment of severe proliferative lupus nephritis: the current state. Ann Rheum Dis 2003; 62: 799-804.

34. Mok C.C., Ying K.Y., Lau C.S. et al. Treatment of Pure Membranous Lupus Nephropathy With Prednisone and Azathioprine: An Open-Label Trial. Am J Kidney Dis 2004; 43; 2: 269-276.

35. Moroni G., Ventura D., Riva P. et al. Antiphospholipid Antibodies Are Associated With an Increased Risk of Chronic Renal Insufficiency in Patients With Lupus Nephritis. Am J Kidney Dis 2004; 43; 1: 28-36.

36. Slot M.C., Tervaert J.W., Bootsma M.M. et al. Positive classic antineutrophil cytoplasmic antibody (C-ANCA) titer at switch to azathioprine with relapse in proteinase 3-related vasculitis. Arthr Rheum 2004; 51; 2: 269-273.

37. Vergunst C.E., van Gurp E., Hagen E.C. et al. An Index for Renal Outcome in ANCA-associated Glomerulonephritis. Am J kidney Dis 2003; 41; 3: 532-538.

38. Weening J.J., D’Agati V.D., Schwartz M.M. et al. The classification of glomerulonephritis in systemic lupus erythematosus revisited. Kidney Int 2002; 65: 521-530.

39. Weidner S., Geuss S., Hafezi-Rachti S. et al. ANCA-associated vasculitis with renal involvement: an outcome analysis. Nephrol Dial Transplant 2004; 19; 6: 1403-1411.