Tags

Type your tag names separated by a space and hit enter

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?
Chron Respir Dis. 2011; 8(1):53-82.CR

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.

Authors+Show Affiliations

Department of Respiratory Medicine, Catholic University of the Sacred Heart, Rome, Italy.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

21339375

Citation

de Lauretis, Angelo, et al. "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?" Chronic Respiratory Disease, vol. 8, no. 1, 2011, pp. 53-82.
de Lauretis A, Veeraraghavan S, Renzoni E. 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? Chron Respir Dis. 2011;8(1):53-82.
de Lauretis, A., Veeraraghavan, S., & Renzoni, E. (2011). 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? Chronic Respiratory Disease, 8(1), 53-82. https://doi.org/10.1177/1479972310393758
de Lauretis A, Veeraraghavan S, Renzoni E. 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. Chron Respir Dis. 2011;8(1):53-82. PubMed PMID: 21339375.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - 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? AU - de Lauretis,Angelo, AU - Veeraraghavan,Srihari, AU - Renzoni,Elisabetta, PY - 2011/2/23/entrez PY - 2011/2/23/pubmed PY - 2011/6/18/medline SP - 53 EP - 82 JF - Chronic respiratory disease JO - Chron Respir Dis VL - 8 IS - 1 N2 - 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. SN - 1479-9731 UR - https://www.unboundmedicine.com/medline/citation/21339375/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 L2 - http://journals.sagepub.com/doi/full/10.1177/1479972310393758?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -