<p>Interstitial Lung Disease in Systemic Sclerosis: Focus on Early Detection and Intervention</p>

Abstract

Systemic sclerosis (SSc) is a progressive and often devastating disease characterized by autoimmune dysfunction, vasculopathy, and fibrosis. Interstitial lung disease (ILD) is identified in the majority of patients with SSc and is the leading cause of SSc-related mortality. Although clinical manifestations and ILD severity vary among patients, lung function typically declines to the greatest extent during the first 3–4 years after disease onset. We aim to provide an overview of SSc-associated ILD (SSc-ILD) with a focus on current and emerging tools for early diagnosis of ILD and current and novel treatments under investigation. Early detection of ILD provides the opportunity for early therapeutic intervention, which could improve patient outcomes. Thoracic high-resolution computed tomography is the most effective method of identifying ILD in patients with SSc; it enables detection of mild lung abnormalities and plays an important role in monitoring disease progression. Cyclophosphamide and mycophenolate mofetil are the most commonly prescribed treatments for SSc-ILD. Recently, nintedanib (an antifibrotic) was approved by the Food and Drug Administration for patients with SSc-ILD; it is indicated for slowing the rate of decline in pulmonary function. However, there is a need for additional effective and well-tolerated disease-modifying therapy. Ongoing studies are evaluating other antifibrotics and novel agents. We envision that early detection of lung involvement, combined with the emergence and integration of novel therapies, will lead to improved outcomes in patients with SSc-ILD.

18F-FDG positron emission tomography scanning in systemic sclerosis-associated interstitial lung disease: a pilot study

“…The most common histopathologic findings are nonspecific interstitial pneumonia (NSIP; ~ 78%) [ 5 ]. Managing SSc-ILD is challenging because of the great heterogeneity of its courses, which can be stable or slowly or quickly deteriorating [ 6 – 8 ], and the modest benefit of immunosuppressants [ 9 – 12 ]. The initiation of immunosuppressants for SSc-ILD is usually recommended and more efficient in patients with active and/or progressing ILD, currently evaluated by the extension observed in high-resolution computed scans (HRCTs) and the deterioration in pulmonary function tests (PFTs; i.e., forced vital capacity [FVC] and diffusing capacity for the lung of carbon monoxide [D LCO ]) [ 12 ].…”

“…FDG PET/CT could therefore be more sensitive than HRCT alone in detecting early ILD and could allow early detection of therapeutic response. Together, these results suggest a need to provide more data on FDG PET/CT findings in SSc, especially in SSc-ILD [ 8 ]. Additionally, FDG PET/CT findings in extrapulmonary regions of interests (ROIs) linked to SSc have been scarce [ 24 – 26 ], and no study has reported systematic analysis of FDG extrapulmonary uptake related to SSc.…”

Risikostratifizierung ausgewählter schwerer Organbeteiligungen bei Systemischer Sklerose und bei Lupus-Nephritis

“…Als Risikofaktoren für die Entwicklung einer ILD konnten das Vorliegen einer dcSSc, männliches Geschlecht, Afro-karibische Eth nizität und der Nachweis von Topoisomerase I-Antikörpern (Scl-70-AK) sowie das Fehlen von Centromer-Antikörpern identifiziert wer den [1,7,8]. In der Lungenfunktionsdiagnostik sind eine Reduk tion der forcierten Vitalkapazität (FVC) und der Diffusionskapazität der Lunge für Kohlenmonoxid (DLCO) wegweisend für eine ILD [1].…”

Patient and Physician Perspectives on Systemic Sclerosis–Associated Interstitial Lung Disease

“…In patients with long-standing stable disease or early nonprogressive disease, treatment is not always indicated, and the best option may be supportive care. 28…”