Survival after lung transplantation in systemic sclerosis. A systematic review

Khan, Irfan Y.; Singer, Lianne G.; Perrot, Marc de; Granton, John; Keshavjee, Shaf; Chau, Cathy; Kron, Amie; Johnson, Sindhu R.

doi:10.1016/j.rmed.2013.09.015

Cited by 66 publications

(43 citation statements)

References 32 publications

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“…The short-and intermediate-term survival of SSc-ILD patients post-lung transplantation are similar to those seen in idiopathic pulmonary fibrosis [86]. Considering the main studies on lung and heart-lung transplantation an overall survival of 52-80% at 2 years is reported [87][88][89][90].…”

Section: Lung Transplantationsupporting

confidence: 53%

Interstitial lung disease in systemic sclerosis: where do we stand?

et al. 2015

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Interstitial lung disease (ILD) is common in systemic sclerosis (SSc) patients and despite recent advances in the treatment is, at present, the major cause of death. Today, an early diagnosis of ILD is possible, and is mandatory to improve the prognosis of the disease.Pulmonary function tests and high-resolution computed tomography remain the mainstay for the diagnosis of SSc-ILD, but there is a growing interest in lung ultrasound. Recently, the correlation between severity of fibrosis and some peripheral blood biomarkers has been described.Nonselective immunosuppressors are still the main treatment for ILD, with cyclophosphamide (CYC) most widely used to obtain remission. Novel therapies towards specific molecular and cellular targets have been suggested; in particular, rituximab (RTX) has shown promising results, but further research is needed. It is of paramount importance to define the severity of the disease and the risk of progression in order to define the need for treatment and the treatment intensity. We propose the division of the treatment strategies at our disposal to induce remission into three categories: high intensity (haematopoietic stem cell transplantation), medium intensity (CYC and RTX) and low intensity (azathioprine (AZA) and mycophenolate mofetil (MMF)). After obtaining remission, maintenance treatment with AZA or MMF should be started.In this review we explore new advances in the pathogenesis, diagnosis and treatment of SSc-ILD. @ERSpublications Early diagnosis of ILD is possible, and is mandatory to improve the prognosis of the disease http://ow.ly/P28JHThe relevance of interstitial lung disease in systemic sclerosisPulmonary disease in systemic sclerosis (SSc) mainly comprises interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). Over the past 40 years the SSc mortality rate has not changed significantly [1]. Nevertheless, while the frequency of deaths due to renal crisis has significantly decreased from 42% to 6%, the proportion of deaths due to ILD and PAH has increased [2]. In fact, ILD and PAH are the two main causes of death in SSc, accounting for 33% and 28% of deaths, respectively [2]. A European Scleroderma Trials and Research group (EUSTAR) analysis revealed, in a cohort of 3656 SSc patients, that ILD is present in 53% of cases with diffuse cutaneous SSc and in 35% of cases with limited

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Section: Lung Transplantationsupporting

confidence: 53%

Interstitial lung disease in systemic sclerosis: where do we stand?

et al. 2015

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“…Thus relatively fewer lung transplantations have been performed for these patients than for patients with other diseases (13). Many studies have indicated similar survival rates for these patient groups (13,14,16,(20)(21)(22)(23)(24)(25)(26). Among the studies comparing the survival rates between patients with CTD-ILD and those with other diseases who underwent lung transplantation, Takagishi et al reported survival outcomes for CTD patients and patients with chronic obstructive pulmonary disease (COPD) and IPF after lung transplantation.…”

Section: Discussionmentioning

confidence: 99%

“…Moreover, there have been few studies of CTD-ILD patients undergoing lung transplantation in Asia (13,(16)(17)(18).…”

Section: Original Articlementioning

confidence: 99%

Comparison of short-term outcomes for connective tissue disease-related interstitial lung disease and idiopathic pulmonary fibrosis after lung transplantation

Park¹,

Kim²,

Song³

et al. 2018

J. Thorac. Dis.

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Background: Pulmonary involvement is common in connective tissue disease (CTD), and respiratory failure is a major cause of morbidity and mortality in CTD-related interstitial lung disease (CTD-ILD).Lung transplantation is thus important for these patients. However, survival, outcomes, and management of these patients after transplantation have been debated. The aim of this study was to evaluate the outcomes for CTD-ILD compared to those for idiopathic pulmonary fibrosis (IPF) after lung transplantation.Methods: We performed a single-centre retrospective study of 62 patients with CTD-ILD or IPF who underwent lung transplantation at a tertiary hospital in South Korea between October 2012 and October 2016. Results: Patients with CTD-ILD (n=15) were younger (46 vs. 60 years, P=0.001) and were less likely to be male (33.3% vs. 76.6%, P=0.004) than were patients with IPF (n=47). The 1-year cumulative survival rate was 80.0% for CTD-ILD and 59.6% for IPF (log-rank P=0.394). There was no difference in the cumulative survival rate (log-rank P=0.613) of age-and sex-matched patients with CTD-ILD (n=15) and IPF (n=15).The incidence of primary graft dysfunction was similar (P=0.154), and 2 (18.2%) patients developed possible CTD flare. Conclusions:Patients with CTD-ILD and those with IPF who underwent lung transplantation had similar survival rates.

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“…No hay estudios controlados con asignación aleatoria, pero un metaanálisis reciente muestra que la supervivencia de estos pacientes puede ser igual a la de los trasplantados por otras causas siempre y cuando sean seleccionados cuidadosamente (52).…”

Section: Nuevos Tratamientosunclassified

Enfermedad pulmonar intersticial en pacientes con esclerosis sistémica. Revisión narrativa de la literatura

Álvarez-Barreneche

Velásquez-Franco

Navas

2017

iatreia

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RESUMENIntroducción: la esclerosis sistémica es una enfermedad autoinmune crónica, caracterizada por la tríada de vasculopatía de pequeños vasos, activación del sistema inmune y aumento de los depósitos de matriz extracelular tanto en la piel como en los órganos internos. En los últimos años, el compromiso pulmonar ha cobrado gran importancia, pasando a ser la primera causa de muerte, dada la disminución de la mortalidad por crisis renales con el advenimiento de los inhibidores de la enzima convertidora de angiotensina. La afección pulmonar puede ocurrir como hipertensión o enfermedad intersticial difusa; esta última es más común en pacientes con la variedad generalizada, en los primeros tres años de la enfermedad. Tiene mal pronóstico si no se inicia el tratamiento, cuya meta es detener el deterioro de la función pulmonar. Entre los tratamientos disponibles, la ciclofosfamida cuenta con la mejor evidencia, pero se están estudiando, con resultados preliminares satisfactorios, otros tratamientos como micofenolato mofetil, rituximab, trasplante autólogo de células hematopoyéticas y trasplante de pulmón.Objetivo: describir, de acuerdo con lo reportado en la literatura, la epidemiología, la fisiopatología, los métodos diagnósticos y el tratamiento de la enfermedad pulmonar intersticial en pacientes con esclerosis sistémica.Métodos: revisión estructurada, no sistemática de la literatura, enfocada en los aspectos de interés, mencionados y que incluyó 52 artículos.

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Survival after lung transplantation in systemic sclerosis. A systematic review

Abstract: The short-term and intermediate-term survival post-lung transplantation are similar to IPAH and ILD patients requiring lung transplantation.

Cited by 66 publications

References 32 publications

Interstitial lung disease in systemic sclerosis: where do we stand?

Interstitial lung disease in systemic sclerosis: where do we stand?

Comparison of short-term outcomes for connective tissue disease-related interstitial lung disease and idiopathic pulmonary fibrosis after lung transplantation

Enfermedad pulmonar intersticial en pacientes con esclerosis sistémica. Revisión narrativa de la literatura

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