Intravenous immunoglobulin modulates cutaneous involvement and reduces skin fibrosis in systemic sclerosis: An open‐label study

Levy, Yair; Amital, Howard; Langevitz, Pnina; Nacci, Francesca; Righi, Anna; Conforti, Letizia; Generini, Sergio; Cerinic, Marco Matucci; Shoenfeld, Yehuda

doi:10.1002/art.20195

Cited by 99 publications

(48 citation statements)

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“…The use of intravenous immunoglobulins has been evaluated in a randomised double-blind placebo-controlled trial and in some open-label studies, in which an improvement of skin involvement was observed [79][80][81][82]. In these studies the effects on ILD were not evaluated.…”

Section: Immunoglobulinsmentioning

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: Immunoglobulinsmentioning

confidence: 99%

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

et al. 2015

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“…In 2004, an open-label study on ten patients with dcSSc and five subjects with lcSSc treated with monthly IVIG infusions found that the mean degree of skin involvement evaluated with the modified Rodnan skin score decreased by 35 % during the time of the study, while a significant improvement in patients' well-being and quality of life was identified by means of a decrease in the Health Assessment Questionnaire (HAQ) score. Notably, although differences did not reach statistical significance, patients with a longer disease duration showed a more pronounced Rodnan skin score improvement when compared with SSc patients with a shorter disease duration (44 vs. 21 %) [78]. Another 60-year-old Japanese woman with SSc successfully treated with IVIG was reported one year later: The modified Rodnan skin score and histological skin biopsies performed before and 4 weeks after IVIG administration showed that skin thickness was dramatically reduced [79].…”

Section: Management Of Systemic Scleroderma With Intravenous Immunoglmentioning

confidence: 97%

Intravenous immunoglobulins (IVIG) in systemic sclerosis: a challenging yet promising future

et al. 2014

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The etiology and pathogenesis of systemic sclerosis are still largely unknown, but a variety of humoral and cellular autoimmune phenomena have been documented. In addition, the rarity of the disease, the broad spectrum of clinical manifestations, and the relevant risk of severe complications as well as the highly variable disease course render its management a major challenge. Some immunomodulatory agents have been used, but no single agent has given a convincing proof of effectiveness, and treatment has remained largely symptomatic through recent years. Novel therapies are currently being tested and may have the potential of modifying the disease process and overall clinical outcome. Efficacy of intravenous immunoglobulins (IVIG) in different regimens (1-2 g/kg of body weight, administered over 2-5 consecutive days) has been described in a limited number of trials and small case series, showing benefits in skin, articular, and lung interstitial disease symptoms. However, studies on IVIG in systemic sclerosis still remain few, and further randomized controlled trials should be undertaken to assess their clinical effectiveness or define the optimal dosage and times of administration.

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“…The success of this therapy prompted a number of us to use IVIG in other fibrotic disorders, [3][4][5][6][7][8][9][10][11][12][13][14][15][16] e.g. systemic sclerosis.…”

Section: Sirmentioning

confidence: 99%