Mycophenolate Versus Cyclophosphamide for Progressive Interstitial Lung Disease Associated with Systemic Sclerosis: A 2-Year Case Control Study

Panopoulos, Stylianos; Bournia, Vassiliki-Kalliopi; Trakada, Georgia; Giavri, I.; Kostopoulos, Charalampos; Sfikakis, Petros P.

doi:10.1007/s00408-013-9499-8

Cited by 59 publications

(43 citation statements)

References 24 publications

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“…The results of this meta-analysis show that mycophenolate is a safe therapeutic agent and its administration has been linked with stabilisation of lung functional parameters. A prospective study in which patients treated with MMF were matched 1:1 with patients treated with CYC, showed a deterioration of lung HRCT at 2 years after MMF treatment but not after CYC, despite the fact that patients treated with CYC had more extensive ILD at baseline [60]. While awaiting the results of a multicentre randomised clinical trial, the Scleroderma Lung Study II, which aims to compare the functional effect of MMF with oral CYC in patients with SSc-ILD, MMF is generally used as a maintenance treatment after CYC and is only occasionally employed as an induction treatment [61].…”

Section: Nonselective Immunosuppressorsmentioning

confidence: 99%

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: Nonselective Immunosuppressorsmentioning

confidence: 99%

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

et al. 2015

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“…Nevertheless, several reports from small case series describe a positive outcome in SSc-ILD patients treated with MMF, with stabilization or mild improvement of PFTs and HRCT fibrosis after 6 up to 24 months of therapy [84][85][86][87][88]. Stabilization of lung function has also been observed in a recent, uncontrolled, prospective study including early, immunosuppressive therapy-naive dcSSc patients, with a median treatment duration of 18 months [89].…”

Section: Mycophenolatementioning

confidence: 84%

Management of Scleroderma-Associated Pulmonary Involvement

Dobrotă

Distler

Wells

et al. 2015

Curr Treat Options in Rheum

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“…A case-control study of patients with SSc-ILD treated with MMF was compared with cyclophosphamide-treated patients (matched for age, gender, baseline FVC) [54]. No change in PFTs was seen, but decline in HRCT was seen in the MMF-treated group.…”

Section: Mycophenolate Mofetil (Mmf)mentioning

confidence: 99%

Update on Systemic Sclerosis

McCray

Mayes

2015

Curr Allergy Asthma Rep

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Systemic sclerosis (SSc) is a rare autoimmune disease characterized by autoantibody production, small-vessel vasculopathy, and skin and other organ fibrosis. The disease is clinically heterogeneous with most patients having some degree of skin sclerosis with varying organ system involvement. Early disease can be difficult to diagnose, especially with minimal skin sclerosis and absence of anti-nuclear antibody (ANA) positivity; however, studies have demonstrated early diagnosis is important as early treatment could potentially lead to better outcomes. New classification criteria have recently been published that have higher sensitivity for detecting early disease thus allowing for a broader spectrum of patients to be represented in clinical trials. Treatment guidelines have been published based on a limited number of randomized-controlled clinical trials; however, there are ongoing phase II and III clinical trials with novel agents that are promising and will change the treatment landscape over the next decade.

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Mycophenolate Versus Cyclophosphamide for Progressive Interstitial Lung Disease Associated with Systemic Sclerosis: A 2-Year Case Control Study

Abstract: Although these results derive from patients selected for receiving at least 1 year of treatment and therefore they do not represent an intention-to-treat cohort, an eagerness to replace cyclophosphamide by mycophenolate in SSc-associated ILD treatment is not supported.

Cited by 59 publications

References 24 publications

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

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

Management of Scleroderma-Associated Pulmonary Involvement

Update on Systemic Sclerosis

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