Connective Tissue Disease–Associated Interstitial Pneumonia and Idiopathic Interstitial Pneumonia: Similarity and Difference

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Interstitial lung diseases (ILDs) are increasingly recognized in patients with systemic diseases. Patients with early ILD changes may be asymptomatic. Features of ILD overlap among systemic diseases and with idiopathic variety. High-resolution computed tomography plays a central role in diagnosing ILDs. Imaging features are often nonspecific. Therapy- and complication-related lung changes would pose difficulty in diagnosing and classifying an ILD. Biology and prognosis of secondary ILDs may differ between different disease-related ILDs and idiopathic variety. Combination of clinical features, serological tests, pulmonary and extrapulmonary imaging findings, and pathology findings may help to diagnose ILDs.

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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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    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.

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