Review Series: Aspects of Interstitial lung disease: Connective tissue disease-associated interstitial lung disease: How does it differ from IPF? How should the clinical approach differ?

Abstract

The lung is frequently involved in connective tissue diseases (CTDs), although the frequency of lung manifestations varies according to the type of CTD. Interstitial lung diseases (ILD) are frequently seen in CTDs, particularly systemic sclerosis (SSc), polymyositis/dermatomyositis (PM/DM) and rheumatoid arthritis (RA), accounting for a significant proportion of deaths. A large percentage of patients with CTD-associated ILD has limited and stable disease, not requiring treatment. However, a significant minority has severe and/or progressive disease, necessitating prompt initiation of treatment. CTD-ILD histological patterns include non-specific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), organizing pneumonia (OP), diffuse alveolar damage (DAD) and lymphocytic interstitial pneumonia (LIP). NSIP is the most common pattern in all CTDs, except for RA, characterized by a higher frequency of UIP. ILD can present acutely or chronically, with acute presentations being more common in systemic lupus erythematosus and PM/DM. Idiopathic pulmonary fibrosis (IPF) is a progressively worsening ILD characterized by inflammation and fibrosis. The characteristic histological pattern of IPF is UIP. Interestingly, a UIP pattern is associated with a significantly better survival in CTD-related disease compared to the idiopathic variety. Prognosis in IPF is dismal, with a median survival since diagnosis of 2-3 years. No treatment regimen has been shown to improve survival in IPF. By contrast, although there have been only two randomized placebo-controlled trials investigating the effect of immunosuppressive treatment in SSc-associated ILD, clinical experience suggests that immunosuppressive drugs in CTD-related ILDs are capable of benefiting a significant proportion of patients, particularly those with certain histological patterns of disease. This review will essentially focus on CTD-associated ILD and will compare aspects of clinical presentation and management to those of IPF.

Phagocytic Activity of Neutrhophils in Patients With Systemic Sclerosis and Lung Injury

“…На сучасному етапі з використанням цитоме-тричних технологій є можливість оцінити на ранньому етапі ризик розвитку патології ле-гень у хворих на ССД і може бути використано для вчасного призначення адекватної базової терапії таким пацієнтам [9][10][11][12].…”

Interstitial Lung Diseases in Rheumatology Practice: A Single Center Experience

“…usual interstitial pneumonia (UIP) is more common than non-specific interstitial pneumonia (NSIP) in RA patients; however, NSIP is more common than UIP in other CTDs. LIP is a common pattern in pSS patients (29). The most common HRCT results in our cohort were ground-glass opacities and interlobular septal thickening (70.3%).…”

Interstitial Pneumonia Associated with Connective Tissue Disease: An Overview and an Insight

“…ILD may precede the extrapulmonary manifestations of CTD as a forme fruste of systemic disease, in some patients by years, while the rheumatic symptoms predate ILD in others [14][15][16]. Sometimes, it makes the distinction between idiopathic pulmonary fibrosis (IPF) and CTD-related ILD difficult [11,16]. Despite similarities in clinical and pathologic presentation, the prognosis and treatment of CTD-associated ILD can differ greatly from that of other forms of ILD such as IPF [13,16,17].…”

“…Certain HRCT features predict histopathologic patterns of the different forms of ILD. Table 2 outlines the classification of histological and corresponding radiological patterns defining each entity of IIP, which is also applicable to CTD-associated ILD, to be explained later [5,11].…”

“…Classification of histological and radiological patterns developed for IIPs is applied to CTD-ILD [1,5,11]. The radiological and corresponding histological patterns defining each entity of CTD-associated IP are summarized in Table 2 [11].…”