Abstract
BackgroundAutoimmune serologies are often obtained in the initial evaluation of uncharacterized interstitial lung disease (ILD). Whether this practice is helpful in delineating connective-tissue disease related ILD (CTD-ILD) is not well known. We assessed the frequency of incident CTD-ILD as detected by autoimmune serology testing and presenting clinical signs and symptoms.MethodsConsecutive patients seen at our institution over a four year period with newly diagnosed uncharacterized ILD and autoimmune serologic testing were included. Serologic assessment was performed as a standardized order set of 13 laboratory tests. Presenting demographics and clinical signs or symptoms suggestive of autoimmune disease were correlated with the presence or absence of positive serology studies and final CTD-ILD diagnoses.ResultsOverall prevalence of newly diagnosed CTD-ILD was 6.9% (42 of 605). Positive serology was seen in 35.2% (213 of 605) of screened ILD. CTD-ILD was diagnosed in 19.2% of those with positive serology, and 52.8% of those with both positive serology and suggestive clinical signs or symptoms. Only 1.4% of those with positive serology and negative review of systems were diagnosed with CTD-ILD. CTD-ILD diagnoses were made more frequently in younger patients ≤60 years with no diagnoses made after the age of 80 (P = 0.009). Positive serology in non-CTD-ILD cases did not appear to confer any survival advantage.ConclusionsThe yield of autoimmune serology testing in uncharacterized ILD appears greatest in those with suggestive clinical signs or symptoms on presentation for CTD-ILD.
Highlights
Autoimmune serologies are often obtained in the initial evaluation of uncharacterized interstitial lung disease (ILD)
Connective-tissue disease (CTD)-ILD was diagnosed in only 19.2% of those with positive serology, but found in 52.8% of those with both positive serology and suggestive clinical signs or symptoms of autoimmune disease
Remaining subjects with positive serology and review of systems (ROS) were diagnosed as interstitial pneumonia with autoimmune features (IPAF) in 7, vasculitis in 4, Idiopathic pulmonary fibrosis (IPF) in 4, other interstitial pneumonias (IIP) in 6, unclassifiable ILD in 8, hypersensitivity pneumonitis in 2, sarcoid in 1, and rare lung diseases in 2
Summary
Autoimmune serologies are often obtained in the initial evaluation of uncharacterized interstitial lung disease (ILD). Whether this practice is helpful in delineating connective-tissue disease related ILD (CTD-ILD) is not well known. In a screening autoimmune serologies are often routinely obtained in initially uncharacterized ILD, with or without clinical suspicion of CTD [10]. The utility of such screening practices (which specific laboratory tests to obtain and in which patients) and its implications for diagnosis and treatment have not been well studied. Positive serologic findings have been reported in idiopathic pulmonary fibrosis
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