Interstitial Lung Disease in the Connective Tissue Diseases
Section snippets
Respiratory Symptoms
Patients with CTD-ILD are often asymptomatic early in the disease course and symptoms are usually nonspecific. Many patients present with dyspnea on exertion, fatigue, or cough. However, CTD-ILD in an asymptomatic patient may be discovered incidentally through radiographic abnormalities. Once lung function is significantly impaired, progressive dyspnea often develops. In time, diffusion defects lead to exertional hypoxemia. Increased dead space ventilation may also contribute to breathlessness.
RA
RA is a chronic inflammatory disease affecting the synovial lined joints and symmetrically involves the small joints of the hands and feet.6 The diagnosis of RA has typically been made with the use of criteria proposed by the American Rheumatism Association in 1987.6 However, the use of newer molecular markers such as anti-CCP antibodies has led to earlier diagnosis, reflected in the criteria proposed in 2010 by the American College of Rheumatology (ACR) and European League Against Rheumatism
Systemic sclerosis (scleroderma)
SSc is a multisystem disorder characterized by endothelial and epithelial cell injury, fibroblast dysregulation, and immune system abnormalities that ultimately lead to systemic inflammation, fibrosis, and vascular injury.138, 139 Clinically, the disease is heterogeneous and may involve multiple organ systems, most commonly the respiratory system, the skin, and the digestive system. Pulmonary involvement is the leading cause of morbidity and mortality among patients with SSc.140 ILD is
Idiopathic inflammatory myopathies
The idiopathic inflammatory myopathies (IIMs) are autoimmune disorders typically affecting the skeletal muscle, leading to inflammation and proximal muscle weakness.189, 190 Systemic involvement, including inflammation of the skin, lung, joints, and GI tract may be present.191 In particular, the presence of ILD has long been recognized and contributes significantly to morbidity and mortality.192, 193, 194 There are several subtypes of the IIMs, all of which may be complicated by ILD, including
Sjögren syndrome
Sjögren syndrome is characterized by lymphocytic infiltration of the exocrine glands and marked B-cell hyperreactivity.236 In particular, the salivary and lacrimal glands are affected, leading to the sicca syndrome characterized by dry eye (keratoconjunctivitis sicca) and dry mouth (xerostomia), often accompanied by arthritis.237 When Sjögren syndrome is seen in isolation, it is called primary Sjögren syndrome. Secondary Sjögren syndrome may accompany other CTDs such as RA, SSc, SLE, and PM/DM.
SLE
SLE is an immune-mediated disease that most commonly occurs in younger women.260 It typically presents with malar, discoid, and photosensitivity rashes, oral ulcers, nonerosive arthritis, glomerulonephritis, and hematologic abnormalities.261 Autoantibodies including ANA, anti–double-stranded DNA (dsDNA), and anti-Smith are commonly detected and are part of the diagnostic criteria.261 The most common form of pulmonary involvement in SLE is pleuritis, but parenchymal lung disease, pulmonary
Summary
Lung disease is a common manifestation of the CTDs and may be a presenting feature. Subclinical disease is common. Clinically apparent disease is often slowly progressive but may present in an acute fashion, contributing to high morbidity and mortality. Infection and drug reaction may share clinical features with CTD-ILD and should be considered when evaluating the patient with dyspnea and abnormal radiographic findings. Treatment of CTD-ILD is not well studied but typically includes
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Cited by (32)
Development of a risk indicator score for the identification of interstitial lung disease in patients with rheumatoid arthritis
2021, Reumatologia ClinicaCitation Excerpt :There are few situations in which the screening of ILD is well established. Clear guidelines for screening of ILD in scleroderma has had a great impact on patient management and outcomes, given that ILD is one of the leading causes of death in that population.26–28 ILD may have similar implications in RA patients.
The new concept of interstitial pneumonia with auto-immune features (IPAF)
2018, Revue du Rhumatisme MonographiesSynthesis no. 2: The concept of interstitial lung disease with autoimmune manifestations
2018, Revue des Maladies Respiratoires ActualitesSurvival and outcomes after lung transplantation for non-scleroderma connective tissue–related interstitial lung disease
2017, Journal of Heart and Lung TransplantationInterstitial lung disease in anti-synthetase syndrome: Initial and follow-up CT findings
2015, European Journal of RadiologyCitation Excerpt :CT features were consistent with OP or mixed NSIP–OP patterns in most of the remaining cases, in view of the high prevalence of consolidations, present in 45% of cases. A high prevalence of consolidations has been previously reported, both in patients with anti-Jo1 antibody [3,5] and patients with myositis [19], which is not the case for other connective-tissue-disease associated ILD [20]. Therefore, we believe searching for the presence of anti-synthetase antibody should be included in the diagnostic work-up of ILD when showing OP or mixed NSIP–OP patterns on CT.
Prevalence of venous thromboembolic events and diagnostic performance of the wells score and revised geneva scores for pulmonary embolism in patients with interstitial lung disease: A prospective study
2014, Heart Lung and CirculationCitation Excerpt :Idiopathic interstitial pneumonia (IIP) and connective tissue disease-associated ILD (CTD-ILD) are the most common forms of ILDs. Although the two conditions share similar clinical manifestations, radiographic findings and histopathologic appearance [1,2], they may have distinct outcomes, depending on the organs involved and complications [3]. Venous thromboembolism, which includes deep-vein thrombosis (DVT) and pulmonary embolism (PE), is initiated by endothelial injury of the venous system, and occurs as a result of disequilibrium between coagulation and fibrinolysis [4].