Abstract
In patients with interstitial lung disease (ILD), the diagnosis of idiopathic interstitial pneumonia is usually made after excluding, among other conditions, connective tissue diseases (CTDs). Although in most patients with a CTD and respiratory symptoms, the systemic nature of the disease is obvious, the ILD-related manifestations in CTDs may often dominate the clinical picture or precede systemic findings and thus mimic idiopathic interstitial pneumonia. With the exception of systemic lupus erythematosus, all CTDs may imitate chronic idiopathic interstitial pneumonias. In this setting, clues to an underlying CTD may be entirely absent or include subtle findings from various systems, including skin, vascular and musculoskeletal system or internal organs. Since nonspecific interstitial pneumonia is a relatively frequent histological pattern in CTDs, biopsy reports of nonspecific interstitial pneumonia should also prompt a search for an underlying CTD. Ultimately, diagnosis of a CTD requires confirmation with immunological testing; interpretation of the various laboratory tests should always be carried out in conjunction with clinical findings. The present article reviews specific clinical aspects of connective tissue disease-related interstitial lung disease that may help differentiate it from idiopathic interstitial pneumonia, especially when interstitial lung disease is the predominant or sole manifestation of an occult connective tissue disease.
Highlights
With the exception of systemic lupus erythematosus, all connective tissue diseases (CTDs) may imitate chronic idiopathic interstitial pneumonias
Findings favouring CTD-related interstitial lung disease (ILD) over interstitial pneumonias (IIPs) are the presence of pleural effusion or significant pleural thickening [26]
In patients presenting with ILD, prompt diagnosis of an occult CTD is crucially important for several reasons
Summary
In the absence of clinical or laboratory tests The most frequent types of ILD encountered in specific for IIPs, their diagnosis is critically patients with CTD are nonspecific interstitial dependent upon the exclusion of certain drug pneumonia (NSIP), usual interstitial pneumonia toxicities, environmental exposures and CTDs [1, (UIP), acute interstitial pneumonia, cryptogenic. An occult CTD may occasionally confuse and large, these entities exhibit similar radiolothe diagnostic process because the CTD-related gical and histological characteristics to their respiratory symptomatology may not always be idiopathic counterparts and are considered accompanied by systemic manifestations and indistinguishable [15, 17,18,19]. The present article focuses on specific CTDs that significant differences in survival between the European Respiratory Journal two groups. Patients with CTD-related ILD Print ISSN 0903-1936 c may present solely or predominantly with survive longer than those with IIP [20,21,22,23].
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