Long-Term Prognosis and Causes of Death in Systemic Lupus Erythematosus

Doria, Andrea; Iaccarino, Leonardo; Ghirardello, Anna; Zampieri, Sandra; Arienti, Silvia; Sarzi-Puttini, Piercarlo; Atzeni, Fabiola; Piccoli, Antônio; Todesco, S

doi:10.1016/j.amjmed.2005.11.034

Cited by 297 publications

(217 citation statements)

References 34 publications

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“…30 On the other hand, intrinsic immunodepression in SLE 1 Number of patients at risk MS 345 220 182 155 122 94 SSC 175 109 88 73 56 46 RA 89 73 51 36 32 29 SLE 85 68 41 32 19 16 JIA 65 52 47 43 37 34 HIC 37 28 21 20 16 15 Number of patients at risk MS 345 182 128 96 69 49 SSC 175 90 71 57 44 34 RA 89 26 14 13 12 12 SLE 85 50 27 19 12 10 JIA 65 36 30 26 21 19 HIC 37 19 10 8 7 7 with severe SLE or SSc significantly impair these patients' spontaneous survival, which was estimated to be between 30% and 50% at 5 years for SSc 31 and between 75% and 80% at 10 years for SLE. 32 In the overall EBMT registry population, the transplant-related mortality (5%) has clearly improved since the earlier reports on a smaller number of patients in 2001 (12%). 8 Among patients with SSc, there were more deaths from the original disease than Autologous HSCT for autoimmune diseases haematologica | 2010; 95 (2) 289 Table 4A.…”

Section: Discussionmentioning

confidence: 96%

Autologous hematopoietic stem cell transplantation for autoimmune diseases: an observational study on 12 years' experience from the European Group for Blood and Marrow Transplantation Working Party on Autoimmune Diseases

Farge¹,

Labopin²,

Tyndall³

et al. 2009

Haematologica

328

272

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BackgroundAutologous hematopoietic stem cell transplantation has been used since 1996 for the treatment of severe autoimmune diseases refractory to approved therapies. We evaluated the long-term outcomes of these transplants and aimed to identify potential prognostic factors. Design and MethodsIn this observational study we analyzed all first autologous hematopoietic stem cell transplants for autoimmune diseases reported to the European Group for Blood and Marrow Transplantation (EBMT) registry between 1996-2007. The primary end-points for analysis were overall survival, progression-free survival and transplant-related mortality at 100 days. ResultsNine hundred patients with autoimmune diseases (64% female; median age, 35 years) who underwent a first autologous hematopoietic stem cell transplant were included. The main diseases were multiple sclerosis (n=345), systemic sclerosis (n=175), systemic lupus erythematosus (n=85), rheumatoid arthritis (n=89), juvenile arthritis (n=65), and hematologic immune cytopenia (n=37). Among all patients, the 5-year survival was 85% and the progression-free survival 43%, although the rates varied widely according to the type of autoimmune disease. By multivariate analysis, the 100-day transplant-related mortality was associated with the transplant centers' experience (P=0.003) and type of autoimmune disease (P=0.03). No significant influence of transplant technique was identified. Age less than 35 years (P=0.004), transplantation after 2000 (P=0.0015) and diagnosis (P=0.0007) were associated with progression-free survival. ConclusionsThis largest cohort studied worldwide shows that autologous hematopoietic stem cell transplantation can induce sustained remissions for more than 5 years in patients with severe autoimmune diseases refractory to conventional therapy. The type of autoimmune disease, rather than transplant technique, was the most relevant determinant of outcome. Results improved with time and were associated with the transplant centers' experience. These data support ongoing and planned phase III trials to evaluate the place of autologous hematopoietic stem cell transplantation in the treatment strategy for severe autoimmune diseases.Key words: autologous hematopoietic stem cell transplantation, autoimmune diseases, multiple sclerosis, systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis, juvenile idiopathic arthritis, hematologic immune cytopenia, total body irradiation, antithymocyte globulins, cyclophosphamide. Citation: Farge D, Labopin M, Tyndall A, Fassas A, Mancardi GL, Van Laar J, Ouyang J, Kozak T, Moore J, Kötter I, Chesnel V, Marmont A, Gratwohl A, and Saccardi R. Autologous hematopoietic stem cell transplantation for autoimmune diseases: an observational study on 12 years' experience from the European Group for Blood and Marrow Transplantation Working Party onAutoimmune Diseases. Haematologica. 2010;95:284-292. doi:10.3324/haematol.2009

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Section: Discussionmentioning

confidence: 96%

Autologous hematopoietic stem cell transplantation for autoimmune diseases: an observational study on 12 years' experience from the European Group for Blood and Marrow Transplantation Working Party on Autoimmune Diseases

Farge¹,

Labopin²,

Tyndall³

et al. 2009

Haematologica

328

272

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“…Approximately 50% of SLE patients develop LN, which increases the risks for renal failure, cardiovascular disease, and death. Clinical presentation of LN varies from mild asymptomatic proteinuria to severe nephrotic syndrome (NS), hematuria, and renal failure [1, 2]. The pathogenesis of LN has not been clarified so far; however, among a huge variety of autoantibodies involved in SLE tissue damage, LN retains the most extensive group and is triggered by complex autoantibody interactions.…”

Section: Introductionmentioning

confidence: 99%

Immunosuppressive Treatment for Lupus Nephritis: Long-Term Results in 178 Patients

Zakharova

Makarova

Zvonova

et al. 2016

BioMed Research International

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Lupus nephritis is one of the most severe Systemic Lupus Erythematosus features, defining treatment modality and prognosis. Our retrospective study, including 178 patients treated for lupus nephritis during 23 years with mostly cyclophosphamide-based initial regimens followed by azathioprine or mycophenolic acid, demonstrates 84.8% of renal response with 19.2% of flares, 15-year patient survival 78.7% and kidney survival 76.3%, and low damage accrual. Both patient and kidney survival significantly differ for subgroups that achieved complete or partial renal response and nonresponders: patient 15-year survival 95% versus 65% versus 35%; kidney 15-year survival 100% versus 58% versus 0%, respectively. 51% (24 out of 47) of patients evaluated at the end of the study period sustained complete renal response; however, only 9 of them had 0 disease activity according to SELENA SLEDAI scale, while 13 patients had scores 2–4 due to the serological abnormalities only. We conclude that (1) initial treatment with cyclophosphamide followed by azathioprine is effective and can be used in agreement with International Guidelines until the evidence for biological treatments benefits becomes available; (2) complete and even partial renal response have positive prognostic value, and failure to achieve renal response negatively influences kidney and patient survival; (3) the validity of complete renal response in SLE is questioned by the absence of conventional definition of SLE remission.

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“…En contraste, el uso de fármacos modificantes, (los que por definición no incluyen a los corticosteroides) disminuye el riesgo de hospitalización insinuando que el control de la enfermedad es beneficioso y logra antagonizar el impacto de las infecciones 105 . De la misma manera, en pacientes con LES, cerca de dos tercios de las causas de muerte obedecen a complicaciones de la enfermedad o su tratamiento, tales como infecciones, eventos cardiovasculares, cáncer y hemorragia gastrointestinal 106 . Estas causas tienden a ocurrir varios años después del diagnóstico y contribuyen a una sobrevida acortada a largo plazo 106 .…”

Section: Otros Riesgos En Pacientes Reumatológicosunclassified

“…De la misma manera, en pacientes con LES, cerca de dos tercios de las causas de muerte obedecen a complicaciones de la enfermedad o su tratamiento, tales como infecciones, eventos cardiovasculares, cáncer y hemorragia gastrointestinal 106 . Estas causas tienden a ocurrir varios años después del diagnóstico y contribuyen a una sobrevida acortada a largo plazo 106 . …”

Section: Otros Riesgos En Pacientes Reumatológicosunclassified

Infecciones en pacientes reumatológicos asociadas a corticosteroides y antagonistas del factor de necrosis tumoral α

Fíca

2014

Rev. chil. infectol.

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Long-Term Prognosis and Causes of Death in Systemic Lupus Erythematosus

Cited by 297 publications

References 34 publications

Autologous hematopoietic stem cell transplantation for autoimmune diseases: an observational study on 12 years' experience from the European Group for Blood and Marrow Transplantation Working Party on Autoimmune Diseases

Autologous hematopoietic stem cell transplantation for autoimmune diseases: an observational study on 12 years' experience from the European Group for Blood and Marrow Transplantation Working Party on Autoimmune Diseases

Immunosuppressive Treatment for Lupus Nephritis: Long-Term Results in 178 Patients

Infecciones en pacientes reumatológicos asociadas a corticosteroides y antagonistas del factor de necrosis tumoral α

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Long-Term Prognosis and Causes of Death in Systemic Lupus Erythematosus

Cited by 297 publications

References 34 publications

Autologous hematopoietic stem cell transplantation for autoimmune diseases: an observational study on 12 years' experience from the European Group for Blood and Marrow Transplantation Working Party on Autoimmune Diseases

Autologous hematopoietic stem cell transplantation for autoimmune diseases: an observational study on 12 years' experience from the European Group for Blood and Marrow Transplantation Working Party on Autoimmune Diseases

Immunosuppressive Treatment for Lupus Nephritis: Long-Term Results in 178 Patients

Infecciones en pacientes reumatológicos asociadas a corticosteroides y antagonistas del factor de necrosis tumoral &#945;

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Infecciones en pacientes reumatológicos asociadas a corticosteroides y antagonistas del factor de necrosis tumoral α