Connective Tissue Disease–Associated Interstitial Pneumonia and Idiopathic Interstitial Pneumonia: Similarity and Difference
Section snippets
ILD as the Initial Manifestation of CTD
Up to 15% of patients presenting with a diagnosis of ILD were diagnosed with CTD during their initial evaluation,3 and up to 25% of ILDs occur in patients with an undiagnosed CTD, sometimes making the distinction between idiopathic pulmonary fibrosis (IPF) and CTD-related ILD difficult.4 There are patients who present ILD and extrapulmonary features that do not meet the diagnostic criteria put forward for a systemic connective disease. It has been suggested that these patients may be
Systemic Sclerosis
SSc is a systemic autoimmune disease of uncertain etiology characterized by cellular and humoral autoimmunity, vascular injury, and tissue fibrosis. Its peak incidence is between 45 and 64 years, and it is more common in women (3:1 to 8:1 female predominance) and African Americans.5 It is subdivided into a limited cutaneous form, previously known as CREST (Calcinosis, Raynaud's syndrome, Esophageal dysmotility, Sclerodactyly, Telangiectasia) syndrome, and a diffuse cutaneous form, with varying
Rheumatoid Arthritis
RA is a relatively common collagen vascular disease, occurring in 1%-2% of the population, more frequently in women (3:1 female predominance), and typically between 25 and 50 years of age.22, 23 Although RA is primarily characterized by synovial inflammation, extra-articular manifestations are seen in approximately half of patients with RA, and evidence of lung disease is seen in up to 80% of patients who are clinically suspected to have RA-associated ILD.24 A recent HRCT study demonstrated a
Systemic Lupus Erythematosus
SLE is a complex rheumatologic condition with a broad spectrum of systemic involvement. It has an overall incidence of 2-11 persons per 100,000 a year; it is higher in women, who make up approximately 90% of patients with SLE, multiracial populations. In the African American population, the diagnosis is common among women of childbearing age; however, in other populations, it occurs more commonly in women older than 40 years.37 Respiratory symptoms and abnormal lung function are relatively
Inflammatory Myopathies
Idiopathic inflammatory myopathies are a heterogeneous group of disorders characterized by subacute or chronic skeletal muscle inflammation and weakness. This group includes inclusion-body myositis, PM, and DM. PM and DM are frequently considered together as their signs and symptoms are similar, with the exception of cutaneous manifestations such as heliotrope rash on the upper eyelids and raised Gottron papules on the dorsal surface of the hands seen in DM. Pathologically, these are distinct
Mixed Connective Tissue Disease
MCTD is characterized by antiribonucleoprotein autoantibodies (specifically anti-U1-RNP) and a spectrum of clinical features such as Raynaud phenomenon, swollen hands, arthritis, serositis (with pleural and pericardial involvement), myositis, esophageal dysmotility, pulmonary hypertension, and ILD. It is debated whether this constellation of symptoms defines a unique CTD or represents an overlap between SLE, SSc, and PM-DM. In a recent study, the prevalence of MCTD was 3.8 per 100,000, with a
Sjögren Syndrome
SS is a relatively common autoimmune condition occurring in 0.1% of the general population and up to 3% of older adults, almost exclusively in women (up to 13:1 female:male ratio). It is characterized by lymphocytic infiltration of exocrine glands resulting in typical sicca symptoms such as dry eyes and mouth, with focal lymphocytic sialoadenitis on salivary gland pathology or positive results for the presence of antinuclear antibodies against ribonucleoproteins, Ro/SSA, or La/SSB.4, 67, 68 SS
Idiopathic ILD vs ILD in CTD
Some clinical features can be used to discriminate between the ILD of CTD and idiopathic ILD. These generally include the demographics of the affected population, presence of serum antibodies, and to some degree, the findings on HRCT.75 IPF is defined as a chronic progressive fibrosing ILD of unknown origin characterized by UIP histology on surgical lung biopsy or findings typical of UIP on HRCT of the chest. The pattern of lower lung subpleural-predominant reticulation with honeycombing that
Conclusions
CTDs represent a heterogeneous group of disorders that can produce a broad spectrum of thoracic pathologies on imaging. In patients with CTD with subacute or chronic thoracic symptoms, HRCT is an important diagnostic tool to perform a thorough evaluation of ILD. These patients may show a variety of findings, from stigmata of ILD to pleural disease, airway disease, or pulmonary hypertension. Although most patients with CTD-related ILD have NSIP pathology and associated nonspecific imaging
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Cited by (29)
The phenotype of mixed connective tissue disease patients having associated interstitial lung disease
2023, Seminars in Arthritis and RheumatismMultimodality imaging in connective tissue disease-related interstitial lung disease
2021, Clinical RadiologyCitation Excerpt :Serum antibodies, extra-pulmonary manifestations (such as Raynaud phenomenon and articular involvement) and multicompartment thoracic involvement (lungs, airways, pleura, cardiovascular system, and oesophagus) may aid the diagnosis (Electronic Supplementary Material Fig. S1). Demographic features should also be taken into account, as idiopathic pulmonary fibrosis (IPF) is more frequent in male patients in their sixth or seventh decade of life, while patients with CTD-ILD are usually younger, of female gender, and less likely to have a smoking history.5,6 The most common patterns of CTD-related ILD are non-specific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), organizing pneumonia (OP), and lymphoid interstitial pneumonia (LIP).
Thoracic Manifestations of Connective Tissue Diseases
2019, Seminars in Ultrasound, CT and MRIInterstitial Lung Disease and Other Pulmonary Manifestations in Connective Tissue Diseases
2019, Mayo Clinic ProceedingsImaging of Pulmonary Manifestations of Connective Tissue Diseases
2016, Radiologic Clinics of North AmericaCitation Excerpt :For example, arthropathy suggests RA; esophageal dilation and pulmonary artery enlargement out of proportion to lung fibrosis suggest SSc; soft tissue calcification suggests DM or SSc; and pleural or pericardial effusion or thickening suggests SLE. Extrapulmonary manifestations, demographic features, and serology can help distinguish CTD-ILD from idiopathic interstitial pneumonia.5,7 Mediastinal lymphadenopathy is frequent in CTD and should not be considered malignant in the absence of known neoplasm.
Grant Support: Dr Khanna receives Grant support from the National Institute of Health and Scleroderma foundation. He is also a consultant for Actelion, Bayer, Celgene, DIGNA, Genentech, Gilead, Intermune, Pfizer, and United Therapeutics.