Detection and Monitoring of Interstitial Lung Disease in Patients with Systemic Sclerosis

Khanna, Surabhi; Nance, John W.; Suliman, Sally

doi:10.1007/s11926-022-01067-5

Cited by 17 publications

(27 citation statements)

References 50 publications

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“…In the EULAR Scleroderma Trials and Research group database, the largest registry of SSc patients worldwide, the mean mRSS of dcSSc and lcSSc were 19 and 8.1, respectively [29]. These findings suggest substantial differences in the mRSS among different ethnic groups, as pointed out by Khanna et al [24] 3) Interstitial lung disease ILD is associated with significant morbidity and mortality in patients with SSc [30]. The prevalence of ILD in SSc varies between 20% to 60% according to the definition of ILD, study design, and ethnicity [5,31].…”

Section: ) Skin Fibrosismentioning

confidence: 94%

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Epidemiology and Treatment of Systemic Sclerosis in Korea

Lee

Moon

2022

J Rheum Dis

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Systemic sclerosis (SSc), a rare, chronic progressive systemic autoimmune disease of unknown etiology, is characterized by autoimmunity, tissue fibrosis, and obliterative vasculopathy. SSc can affect all major organs including the skin, blood vessels, lung, heart, kidneys, and gastrointestinal tract. Our understanding of its pathogenesis has increased over the past few decades, leading to improved diagnosis and treatment. However, the mortality rate of SSc remains considerable, mainly due to cardiopulmonary causes. A growing body of evidence suggests that geographical, regional, and ethnic differences could affect the epidemiology, clinical characteristics and prognosis of SSc. Although Korean data of this issue are lacking, a considerable amount of research has been published by many Korean researchers. To establish treatment strategies for Korean patients, extensive Korean research data are needed. This review summarizes the prevalence, incidence, mortality, and clinical and laboratory manifestations of Korean patients with SSc and discusses the current trends in evidence-based treatment and recommendations.

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Section: ) Skin Fibrosismentioning

confidence: 94%

“…ILD is associated with significant morbidity and mortality in patients with SSc [ 30 ]. The prevalence of ILD in SSc varies between 20% to 60% according to the definition of ILD, study design, and ethnicity [ 5 , 31 ].…”

Section: Main Subjectsmentioning

confidence: 99%

Epidemiology and Treatment of Systemic Sclerosis in Korea

Lee

Moon

2022

J Rheum Dis

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“…The extent of fibrosis is often associated with an adverse prognosis [ 20 ]. The most frequent HRCT pattern seen in patients with SSc-ILD is NSIP, characterized by imaging findings of GGO, reticulation, and traction bronchiectasis primarily in the lower lobes of the lungs ( Figure 4 ) [ 21 ]. UIP patterns are occasionally observed, and some patients may exhibit an unspecific pattern that does not fulfill the criteria of any defined classification [ 21 ].…”

Section: Main Subjectsmentioning

confidence: 99%

Radiologic approach and progressive exploration of connective tissue disease-related interstitial lung disease: meeting the curiosity of rheumatologists

Jeon,

Nam,

Yoon

et al. 2023

J Rheum Dis

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Interstitial lung disease (ILD) is often observed in connective tissue diseases (CTDs), frequently in rheumatoid arthritis, systemic sclerosis, primary Sjögren’s syndrome, and inflammatory myositis. Early detection of ILDs secondary to rheumatic diseases is important as timely initiation of proper management affects the prognosis. Among many imaging modalities, high-resuloution computed tomography (HRCT) serves the gold standard for finding early lung inflammatory and fibrotic changes as well as monitoring afterwards because of its superior spatial resolution. Additionally, lung ultrasound (LUS) and magnetic resonance imaging (MRI) are the rising free-radiation imaging tools that can get images of lungs of CTD-ILD. In this review article, we present the subtypes of ILD images found in each CTD acquired by HRCT as well as some images taken by LUS and MRI with comparative HRCT scans. It is expected that this discussion would be helpful in discussing recent advances in imaging modalities for CTD-ILD and raising critical points for diagnosis and tracing of the images from the perspective of rheumatologists.

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“…Although PFTs represent a cornerstone for non-invasive monitoring of ILD, HRCT more sensitively detects early ILD and should be performed in all patients with a definite diagnosis of SSc regardless of PFT results. 35 , 49 In early SSc-ILD, radiographic features include ground glass opacity with possible subtle reticulations, in the bilateral lower lobes. The predominant radiographic pattern of SSc-ILD is non-specific interstitial pneumonia (NSIP), characterized by bilateral reticulation, ground glass opacities and traction bronchiectasis.…”

Section: Risk Stratificationmentioning

confidence: 99%

Predicting the Progression of Very Early Systemic Sclerosis: Current Insights

Bellocchi¹,

Chung²,

Volkmann³

2022

OARRR

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Systemic sclerosis (SSc) is a complex autoimmune connective tissue disease with distinct pathological hallmarks (ie, inflammation, vasculopathy, fibrosis) that may predominate at different stages in the disease course with varying severity. Initial efforts to classify patients with SSc identified a subset of patients with very early SSc. These patients possessed signs of SSc (eg, Raynaud phenomenon, SSc specific autoantibodies and/or nailfold capillary abnormalities) without fulfilling complete SSc classification criteria. Recognizing the inherent value in early diagnosis and intervention in SSc, researchers have endeavored to identify risk factors for progression from very early SSc to definite SSc. The present review summarizes the clinical phenotype of patients with very early and early SSc. Through a scoping review of recent literature, this review also describes risk factors for progression to definite SSc with a focus on the specific clinical features that arise early in the SSc disease course (eg, diffuse cutaneous sclerosis, interstitial lung disease, esophageal dysfunction, renal crisis, cardiac involvement). In addition to clinical risk factors, this review provides evidence for how biological data (ie, serological, genomic, proteomic profiles, skin bioengineering methods) can be integrated into risk assessment models in the future. Furthering our understanding of biological features of very early SSc will undoubtedly provide novel insights into SSc pathogenesis and may illuminate new therapeutic targets to prevent progression of SSc.

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Detection and Monitoring of Interstitial Lung Disease in Patients with Systemic Sclerosis

Cited by 17 publications

References 50 publications

Epidemiology and Treatment of Systemic Sclerosis in Korea

Epidemiology and Treatment of Systemic Sclerosis in Korea

Radiologic approach and progressive exploration of connective tissue disease-related interstitial lung disease: meeting the curiosity of rheumatologists

Predicting the Progression of Very Early Systemic Sclerosis: Current Insights

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