Prevalence, imaging patterns and risk factors of interstitial lung disease in connective tissue disease: a systematic review and meta-analysis

Abstract

IntroductionInterstitial lung disease (ILD) is a frequent manifestation of connective tissue disease (CTD) with substantial variability in prevalence and outcomes reported across CTD subtypes. This systematic review summarises the prevalence, risk factors and ILD patterns on chest computed tomography of CTD-ILD.MethodsA comprehensive search was performed in Medline and Embase to identify eligible studies. Meta-analyses were completed using a random effects model to determine the pooled prevalence of CTD-ILD and ILD patterns.Results11 582 unique citations were identified with 237 articles included. Pooled prevalence of ILD was 11% in rheumatoid arthritis (95% CI 7–15%), 47% in systemic sclerosis (44–50%), 41% in idiopathic inflammatory myositis (33–50%), 17% in primary Sjögren's syndrome (12–21%), 56% in mixed connective tissue disease (39–72%) and 6% in systemic lupus erythematosus (3–10%). Usual interstitial pneumonia was the most prevalent ILD pattern in rheumatoid arthritis (pooled prevalence of 46%), while nonspecific interstitial pneumonia was the most common ILD pattern in all other CTD subtypes (pooled prevalence range 27–76%). Across all CTDs with available data, positive serology and higher inflammatory markers were risk factors for development of ILD.DiscussionWe identified substantial variability in ILD across CTD subtypes suggesting that CTD-ILD is too heterogenous to be considered a single entity.

Novel Therapeutic Approaches in Connective Tissue Disease-Associated Interstitial Lung Disease

“…119 Prevalence of lung involvement in pSS is estimated at 15 to 20%. 2,[120][121][122][123] Mortality is increased in patients with lung involvement with one study showing 5-year survival of 84%. 121 A second study showed 4-fold increase in mortality over 10 years in patients with pSS and lung involvement compared with patients with no lung involvement.…”

“…Interstitial lung disease (ILD) is one of the most common pulmonary manifestations of multiple CTD subtypes, including systemic sclerosis (SSc), rheumatoid arthritis (RA), idiopathic inflammatory myopathy (IIM), and primary Sjögren's syndrome (pSS). 1,2 Although all CTDs can develop ILD, the prevalence of ILD varies by subtype. 1,2 Regardless of the specific CTD, development of ILD is associated with increased mortality.…”

Lung regeneration: diverse cell types and the therapeutic potential

“…Many respiratory diseases, especially IPF, are accompanied by varying degrees of fibrotic response, characterized by progressive scarring of lung tissue, which reduces gas exchange and leads to high disability and mortality worldwide. 7,8 The lungs are in direct contact with the outside world, and adults are susceptible to lung injury as they inhale more than 10,000 L of unsterile air daily. 9 Many factors, such as environmental pollution, smoking, viral infections, and autoimmune diseases, contribute to lung injury.…”

“…With socialization and industrialization, the burden of disease has transitioned from acute and subacute infectious diseases to a chronic noncommunicable disease pattern, and even with the recent coronavirus disease 2019 (COVID‐19) pandemic, a significant proportion of patients recovering from acute infectious diseases have moved to chronic noncommunicable clinical syndromes. Many respiratory diseases, especially IPF, are accompanied by varying degrees of fibrotic response, characterized by progressive scarring of lung tissue, which reduces gas exchange and leads to high disability and mortality worldwide 7,8 …”

Imaging Features of Autoimmune Disease-Related Interstitial Lung Diseases

“…Interstitial lung disease (ILD) may develop as a consequence of an autoimmune disease such as rheumatoid arthritis (RA), systemic sclerosis (SSc), or myositis and is associated with morbidity and premature mortality 1–5 . Although most cases of autoimmune disease-related ILD are observed in patients who have already been diagnosed with an autoimmune disease, in some cases, ILD is the first sign of an autoimmune disease to be identified 1,2,6–8 .…”