Connective Tissue Disease-Related Interstitial Lung Disease: Prevalence, Patterns, Predictors, Prognosis, and Treatment
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STATE OF THE ART REVIEW
Connective Tissue Disease‑Related Interstitial Lung Disease: Prevalence, Patterns, Predictors, Prognosis, and Treatment Niranjan Jeganathan1 · Matheni Sathananthan2 Received: 4 May 2020 / Accepted: 26 July 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Interstitial lung disease is a common pulmonary manifestation of connective tissue diseases and results in significant morbidity and mortality. Although all connective tissue diseases are linked by underlying autoimmunity, there is significant variability in the presentation and clinical course of interstitial lung disease associated with the different types of connective tissue diseases. In this paper, we review the prevalence, patterns, predictors, and prognosis of interstitial lung disease secondary to the most common forms of connective tissue diseases. We have also highlighted the available evidence regarding treatment options for interstitial lung disease due to different connective tissue diseases. Keywords Interstitial lung disease · Connective tissue disease · Systemic sclerosis · Rheumatoid arthritis · Myositis · Sjögren’s syndrome Abbreviations ILD Interstitial lung disease CTD Connective tissue disease SSc Systemic sclerosis RA Rheumatoid arthritis PM Polymyositis DM Dermatomyositis SS Sjögren’s syndrome SLE Systemic lupus erythematosus MCTD Mixed connective tissue disease HRCT High-resolution computed tomography UIP Usual interstitial pneumonia NSIP Non-specific interstitial pneumonia OP Organizing pneumonia DAD Diffuse alveolar damage CPK Creatinine phosphokinase FVC Forced vital capacity DLCO Diffusion capacity for carbon monoxide BAL Bronchoalveolar lavage IPAF Interstitial pneumonia with autoimmune features
* Niranjan Jeganathan [email protected] 1
Loma Linda University Health, 11234 Anderson Street, Room 6424, Loma Linda, CA 92354, USA
Department of Internal Medicine, Loma Linda University Health, Loma Linda, CA 92354, USA
2
PH Pulmonary hypertension HSCT Hematopoietic stem cell transplantation
Introduction Connective tissue diseases (CTDs) refer to a group of autoimmune disorders which include systemic sclerosis (SSc), rheumatoid arthritis (RA), primary Sjögren’s syndrome (pSS), polymyositis (PM), dermatomyositis (DM), antisynthetase syndrome (ASS), systemic lupus erythematous (SLE), and mixed connective tissue disease (MCTD). These disorders have some common underlying mechanisms characterized by autoimmunity and immune-mediated organ dysfunction but also have unique features. There are numerous pulmonary manifestations of CTDs of which interstitial lung disease (ILD) is one of the most common, resulting in significant morbidity and mortality [1]. ILD affects the interstitium, the tissue layer around the alveoli, but could also cause alteration of the alveolar and airway architecture. ILD is commonly diagnosed simultaneously with or after the diagnosis of CTD is made. Occasionally ILD could be the initial manifestation of CTD, or present with findi
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