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Published ahead of print on June 7, 2007, doi:10.1164/rccm.200702-220OC
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American Journal of Respiratory and Critical Care Medicine Vol 176. pp. 691-697, (2007)
© 2007 American Thoracic Society
doi: 10.1164/rccm.200702-220OC

Original Article

Idiopathic Nonspecific Interstitial Pneumonia

Lung Manifestation of Undifferentiated Connective Tissue Disease?

Brent W. Kinder1, Harold R. Collard1, Laura Koth1, David I. Daikh1, Paul J. Wolters1, Brett Elicker2, Kirk D. Jones3 and Talmadge E. King, Jr.1

Departments of 1 Medicine, 2 Radiology, and 3 Pathology, University of California School of Medicine, San Francisco, California

Correspondence and requests for reprints should be addressed to Brent W. Kinder, M.D., 231 Albert Sabin Way, ML 0564, Cincinnati, OH 45267. E-mail: brent.kinder{at}uc.edu

Rationale: The American Thoracic Society/European Respiratory Society International Consensus Classification panel identified the clinical entity idiopathic nonspecific interstitial pneumonia (NSIP) as a provisional diagnosis and recommended further study.

Objectives: We hypothesized that idiopathic NSIP is an autoimmune disease and the lung manifestation of undifferentiated connective tissue disease (UCTD), a recently described, distinct entity.

Methods: We studied 28 consecutive patients with idiopathic interstitial pneumonia (IIP) enrolled in the University of California, San Francisco Interstitial Lung Disease Center who met prespecified criteria for UCTD, as follows: at least one clinical manifestation of connective tissue disease, serologic evidence of systemic inflammation in the absence of clinical infection, and absence of sufficient American College of Rheumatology criteria for another connective tissue disease. Medical record reviews, evaluation of radiographs, and scoring of lung biopsies were performed. The control group consisted of all other patients (n = 47) with IIP who did not meet the UCTD criteria.

Measurements and Main Results: The patients with UCTD were more likely to be women, younger, and nonsmokers than the IIP control subjects. Compared with the control group, patients with UCTD-ILD were significantly more likely to have ground-glass opacity on high-resolution computed tomography (HRCT) and NSIP pattern on biopsy, and less likely to have honeycombing on HRCT or usual interstitial pneumonia on biopsy. At our center, the majority of patients classified as idiopathic NSIP (88%) met the criteria for UCTD.

Conclusions: Most patients diagnosed with idiopathic NSIP meet the case definition of UCTD. Furthermore, these results show that the clinical entity idiopathic NSIP is different from idiopathic pulmonary fibrosis and appears to be an autoimmune disease.

Key Words: idiopathic interstitial pneumonia • systemic rheumatic disease • undifferentiated connective tissue • collagen vascular • autoimmune disease


AT A GLANCE COMMENTARY

Scientific Knowledge on the Subject
The clinical entity idiopathic nonspecific interstitial pneumonia is a provisional diagnosis and needs further study. Undifferentiated connective tissue disease is a recognized disease entity, but the pulmonary manifestations are not described. Their relationship is unknown.

What This Study Adds to the Field
Patients previously classified as having idiopathic nonspecific interstitial pneumonia have clinical, serologic, radiographic, and pathologic characteristics that are highly suggestive of autoimmune disease and meet criteria for undifferentiated connective tissue disease.

 

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AJRCCM 2007 176: 632-633. [Full Text]  



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