Pulmonary Complications of Connective Tissue Diseases

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Lung involvement is common in patients who have complications of connective tissue disease (CTDs) and causes considerable morbidity and mortality. High resolution CT (HRCT) has a pivotal role in the detection of lung fibrosis. In patients who have coexistent pathologic processes, HRCT often allows the predominant process to be identified. HRCT has an important role in detecting possible complications such as opportunistic infection or the development of malignancy. However, the limitations of HRCT should not be overlooked. In many cases, HRCT appearances are nonspecific and may or may not be related to an underlying CTD. Thus, radiologic findings should never be interpreted without knowledge of the clinical picture.

Section snippets

Interstitial lung disease

The spectrum of lung disease in CTDs includes all of the disease processes sub-classified in the idiopathic interstitial pneumonias (IIPs) [1], but the prevalence of individual processes differs greatly between the CTDs and the IIPs. The most prevalent form of interstitial lung disease in the IIPs is usual interstitial pneumonia (UIP), which is characterized by a patchy distribution often affecting the periphery of the acinus or lobule, the presence of fibroblastic foci, and often honeycombing

Chest radiography

Historically, plain chest radiography has been used to detect and monitor interstitial lung disease. Chest radiography is relatively cheap, readily available, and associated with a low radiation burden. However, conventional radiography is insensitive [24], has a low diagnostic accuracy [25], and interpretation is often difficult, as judged by the high level of interobserver variability reported in several studies [26], [27], [28]. Furthermore, the severity of disease often is difficult to

Rhematoid arthritis

In RA, the most common of CTDs, lung involvement can take the form of a variety of interstitial lung processes, including UIP, NSIP, organizing pneumonia, acute interstitial pneumonia, LIP, and intra-parenchymal rheumatoid nodule formation. Bronchiectasis and the various bronchiolitides outlined earlier also can complicate RA. Pleural disease is common and is usually self-limited. Pulmonary vasculopathy is rare.

In RA, alone among the CTDs, UIP appears to be at least as common as NSIP; even in

Key clinical problems in connective tissue disease: the deconstruction of coexisting disease processes

Among CTDs, the variety of underlying lung pathologies is greatest in RA. Reversible interstitial lung processes, irreversible interstitial disease, variably reversible airway-centered disease, and pleural involvement seldom all coexist in the same patient. However, combinations of two or even three disorders are not infrequent. Furthermore, it is widely believed (although not yet proven) that smoking promotes the onset and progression of interstitial lung disease in RA—many patients who have

Key clinical problem in connective tissue disease: is lung disease clinically significant?

As discussed earlier, HRCT consistently is more sensitive than chest radiography in interstitial lung disease, and this applies equally to CTD. In SSc, HRCT is much more sensitive than chest radiography [93], and in SS [94], [95] and SLE [96], HRCT discloses parenchymal abnormalities in a majority or large minority of patients. In RA, the prevalence of interstitial abnormalities on HRCT is approximately 25% [97]. It is now widely accepted that HRCT is the most accurate routine noninvasive means

Key clinical problem in connective tissue disease: prognostic evaluation

Among CTDs, outcomes from interstitial disease in PM/DM appear to be the most diverse. In principle, the prognostic use of HRCT has much to offer the clinician in PM/DM, but the prognostic value of HRCT in IIPs should not be extrapolated uncritically to PM/DM and needs to be nuanced carefully in RA and SSc. Other CTDs are insufficiently studied in this regard.

The subgroup of patients who have reversible disease are contained mostly within the subgroup that has consolidation or predominant

Key clinical problem in connective tissue disease: differential diagnosis of lung disease

A frequent challenge in SLE (in particular), and in CTDs (in general), is to distinguish between expected pulmonary processes caused by CTDs and other disorders. Radiologists often are asked whether an abnormality is caused by the underlying rheumatologic disorder, is a complication of its treatment (ie, opportunistic infection caused by immunosuppression or drug induced lung disease), or is entirely unrelated. This problem often is difficult, as illustrated by the scenario of “new” air space

Key clinical problem in Sjögren's syndrome: the detection of proliferative disease

The radiologic patterns seen in SS reflect the diverse range of pathology [94], [95], [132], [133], [134]. There is nonspecific ground-glass opacification and centrilobular nodules [45] in patients who have lymphocytic interstitial infiltration. In some patients who have SS, there is intrapulmonary cyst formation, occasionally which is associated with the deposition of amyloid nodules [132], [134]. The pathophysiology of cyst formation is not entirely clear, but it seems likely that these are

Summary

Lung involvement is common in patients who have CTDs and causes considerable morbidity and mortality. Imaging tests and HRCT have a variety of roles in diagnosis and management. HRCT has a pivotal role in the detection of lung fibrosis. For patients who have coexistent pathologic processes, HRCT often allows the predominant process to be identified. The extent of interstitial disease also can be informally evaluated with HRCT, which provides insights into the likely pathophysiologic and

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