Abstract
The aims of this study were to analyze the clinical and pathological features of lupus nephritis (LN) and examine the association between these features and pathological condition, treatment, and prognosis. Of the 177 systemic lupus erythematosus patients who died while receiving inpatient care at Juntendo University Hospital between 1960 and 2001, we investigated the clinical features, treatment, and pathological features of 73 of these who underwent pathological autopsy and had a clear medical history. We divided these cases into two groups, i.e., those up to 1979 (Group A) and those during and after 1980 (Group B) in order to investigate changes in tendencies by age. We also divided the cases into three groups by time interval between diagnosis and death to investigate long-term prognosis. Uremia was the direct cause of death in 38.9% of cases in Group A and only 10.8% of cases in Group B. Pathological features showed a tendency to change to a sclerotic lesion as the duration of the disorder became longer. Uremia attributable to LN was the direct cause of death in relatively fewer cases, although it is still found in the majority of LN cases and remains a problem requiring stringent management. The treatment of sclerotic lesions may be an issue that needs further attention.
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Arao T, Fujiki T, Kosumi H, Hatono C, Numata T. 4 autopsy cases of systemic lupus erythematosus, especially on lupus nephritis. Nippon Hifuka Gakkai Zasshi. 1961;71:524–33.
Grishman E, Gerber MA, Churg J. Patterns of renal injury in systemic lupus erythematosus: light and immunofluorescence microscopic observations. Am J Kidney Dis. 1982;2[Suppl 1]:135–41.
Grishman E, Venkataseshan VS. Vascular lesions in lupus nephritis. Mod Pathol. 1988;1(3):235–41.
Tan Eng M, Cohen Alan S, Fries James F, Masi Alfonse T, McShane Dennis J, Rothfield Naomi F, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982;25:1271–7.
Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1997;40:1725.
Austin HA 3rd, Muenz LR, Joyce KM, Antonovych TT, Balow JE. Diffuse proliferative lupus nephritis: identification of specific pathologic features affecting renal outcome. Kidney Int. 1984;25:689–95.
Weening JJ, D’Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB, et al. The classification of glomerulonephritis in systemic lupus erythematosus revisited. Kidney Int. 2004;65:521–30.
Churg J, Sobin LH. Renal disease: classification and atlas of glomerular disease. Tokyo: Igaku-Shoin; 1982. p. 127–31.
Churg J, Bernstein J, Glassok RJ. Renal disease: classification and atlas of glomerular disease. 2nd ed. New York: Igaku-Shoin; 1995. p. 151–6.
Merrel M, Shulman LE. Determination of prognosis in chronic disease, illustrated by systemic lupus erythematosus. J Chronic Dis. 1955;1:12–32.
Cervera R, Khamashta MA, Font J, Sebastiani GD, Gil A, Lavilla P, et al. Morbidity and mortality in systemic lupus erythematosus during a 10-year period: a comparison of early and late manifestation in a cohort of 1000 patients. Medicine. 2003;82:299–308.
Mok CC, Lee KW, Ho CT, Lau CS, Wong RW. A prospective study of survival and prognostic indicators of systemic lupus erythematosus in a southern Chinese population. Rheumatology (Oxford). 2000;39:399–406.
Mok CC, Wong RW, Lau CS. Lupus nephritis in southern Chinese patients: clinicopathologic findings and long-term outcome. Am J Kidney Dis. 1999;34:315–23.
Bono L, Cameron JS, Hicks JA. The very long-term prognosis and complications of lupus nephritis and its treatment. QJM. 1999;92:211–8.
Mok CC, Mak A, Chu WP, To CH, Wong SN. Long-term survival of southern Chinese patients with systemic lupus erythematosus. Medicine. 2005;84:218–24.
Kang I, Park SH. Infectious complications in SLE after immunosuppressive therapies. Curr Opin Rheumatol. 2003;15:528–34.
Fiehn C, Hajiar Y, Mueller K, Waldherr R, Ho AD, Andrassy K. Improved clinical outcome of lupus nephritis during the past decade: importance of early diagnosis and treatment. Ann Rheum Dis. 2003;62:435–9.
Nagasawa T. Collagen disease and dialysis—overview. Jpn J Clin Dial. 1995;11:917–22.
Doria A, Iaccarino L, Ghirardello A, Zampieri S, Arienti S, Sarzi-Puttini P, et al. Long-term prognosis and causes of death in systemic lupus erythematosus. Am J Med. 2006;119:1497–9.
Bernatsky S, Clarke A, Ramsey-Goldman R. Malignancy and systemic lupus erythematosus. Curr Rheumatol Rep. 2002;4:351–8.
Abu-Shakra M, Ehrenfeld M, Shoenfeld Y. Systemic lupus erythematosus and cancer: associated or not? Lupus. 2002;11:137–44.
Najafi CC, Korbet SM, Lewis EJ, Schwartz MM, Reichlin M, Evans J. Significance of histologic patterns of glomerular injury upon long-term prognosis in severe lupus glomerulonephritis. Kidney Int. 2001;59:2156–63.
Contreras G, Lenz O, Pardo V, Borja E, Cely C, Iqbal K, et al. Outcomes in African American s and Hispanics with lupus nephritis. Kidney Int. 2006;69:1846–51.
Terai C, Koseki Y, Uesato M, Moriguchi M. Lupus nephritis. Intern Med. 1999;83:78–81.
Gonzalez-Crespo MR, Lopez-Fernandez JI, Usera G, Poveda MJ, Gomez-Reino JJ. Outcome of silent lupus nephritis. Semin Arthritis Rheum. 1996;26:468–76.
Zabaleta-Lanz M, Vargas-Arenas RE, Tapanes F, Daboin I, Atahualpa Pinto J, Bianco NE. Silent nephritis in systemic lupus erythematosus. Lupus. 2003;12:26–30.
Valeri A, Radhakrishnan J, Estes D, D’Agati V, Kopelman R, Pernis A, et al. Intravenous pulse cyclophosphamide treatment of severe lupus nephritis: a prospective five-year study. Clin Nephrol. 1994;42:71–8.
Kunis CL, Kiss B, Williams G, et al. Intravenous “pulse” cyclophosphamide therapy of crescentic glomerulonephritis. Clin Nephrol. 1992;37(1):1–7.
Tse KC, Li FK, Tang CS, Lai KN, Chan TM. Angiotensin inhibition or blockade for the treatment of patients with quiescent lupus nephritis and persistent proteinurea. Lupus. 2005;14:947–52.
Honma M, Ichikawa Y, Akizuki M, Kashiwazaki S, Kondo H, Hashimoto H, et al. Double blind trial of pulse methylprednisolone versus conventional oral prednisolone in lupus nephritis. Ryumachi. 1994;34(3):616–27.
Gourley MF, Austin HA 3rd, Scott D, Yarboro CH, Vaughan EM, Muir J, et al. Methylprednisolone and cyclophosphamide, alone or in combination, in patients with lupus nephritis. A randomized, controlled tiral. Ann Intern Med. 1996;125(7):549–57.
Sesso R, Monteiro M, Sato E, Kirsztajn G, Silva L, Ajzen H. A controlled trial of pulse cyclophosphamide versus pulse methylprednisolone in severe lupus nephritis. Lupus. 1994;3(2):107–12.
Radharkrishnan J, Kunis CL, D’Agati V, Appel GB. Cyclosporin treatment of lupus membranous nephropathy. Clin Nephrol. 1994;42:147–54.
Rihova Z, Vankova Z, Maixnerova D, Dostal C, Jancova E, Honsova E, et al. Treatment of lupus nephritis with cyclosporine—an outcome analysis. Kidney Blood Press Res. 2007;30(2):124–8.
Jonsdottir T, Gunnarsson I, Risselada A, Henriksson EW, Klareskog L, van Vollenhoven RF. Treatment of refractory SLE with rituximab plus cyclophosphamide: clinical effects, serological changes, and predictors of response. Ann Rheum Dis. 2008;67(3):330–4.
Mok CC, Tong KH, To CH, Siu YP, Au TC. Tacrolimus for induction therapy of diffuse proliferative lupus nephritis: an open-labeled pilot study. Kidney Int. 2005;68:813–7.
Cordeiro AC, Isenberg DA. Novel therapies in lupus—focus on nephritis. Acta Reumatol Port. 2008;33(2):157–69.
Abu-Shakra M, Urowitz MB, Gladman DD, Gough J. Mortality studies in systemic lupus erythematosus. Results from a single center. I. Causes of death. J Rheumatol. 1995;22:1259–64.
Cervera R, Khamashta MA, Font J, Sebastiani GD, Gil A, Lavilla P, et al. Morbidity and mortality in systemic lupus erythematosus during a 10-year period: a comparison of early and late manifestation in a cohort of 1000 patients. Medicine (Baltimore). 2003;82:299–308.
Doria A, Iaccarino L, Ghirardello A, Zampieri S, Arienti S, Sarzi-Puttini P, et al. Long-term prognosis and causes of death in systemic lupus erythematosus. Am J Med. 2006;119:700–6.
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Kon, T., Yamaji, K., Sugimoto, K. et al. Investigation of pathological and clinical features of lupus nephritis in 73 autopsied cases with systemic lupus erythematosus. Mod Rheumatol 20, 168–177 (2010). https://doi.org/10.1007/s10165-009-0260-3
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DOI: https://doi.org/10.1007/s10165-009-0260-3