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

Abstract

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

High Prevalence of Acute Exacerbation of Interstitial Lung Disease in Japanese Patients with Systemic Sclerosis

“…Moreover, acute exacerbation of ILD (AE-ILD) occurs at a 1-year frequency of 1.25%-3.3% and at a lifetime incidence of 7.2%, with a high mortality rate in patients with connective tissue disease (Park et al 2007;Suda et al 2009). Given the chronic nature of the disease, it has been considered that AE-ILD is not common in patients with SSc (Fan et al 2014;Cappelli et al 2015), but recent papers suggest that AE-ILD may occur even in patients with SSc (Park et al 2007;Bussone and Mouthon 2011;Tachikawa et al 2012;Ando et al 2013;Yasuoka 2016). However, little data are available regarding the prevalence of AE-ILD in Japanese patients with SSc.…”

Lung ultrasound—a new diagnostic modality in persistent tachypnea of infancy

“…They found that the number of B-lines seen on LUS increased consistently with the growing extent of parenchymal changes on CT. 36 Increased number of B-lines was also shown to be a sensitive marker of ILD and to correlate significantly with HRCT scores in rheumatoid arthritis and patients with SSc. 37,38 In addition, the distance between each of two adjacent B-lines was shown to correlate with the extent of the ILD on chest HRCT Note: Data are presented as medians (IQR). P adj , P-value adjusted with the use of the Benjamini-Hochberg procedure.…”

Treatment of connective tissue disease-associated interstitial lung disease: the pulmonologist’s point of view

“…However, patients undergoing HSCT showed a significant improvement in FVC and total lung capacity. HSCT treatment in scleroderma shows promise with respect to long term survival benefits in patients with SSc-ILD [81]. …”