Published ahead of print on June 7, 2007, doi:10.1164/rccm.200702-220OC
Am. J. Respir. Crit. Care Med., Volume 176, Number 7, October 2007, 691-697
A more recent version of this article appeared on October 1, 2007
Submitted on February 8, 2007
Accepted on June 7, 2007
Idiopathic NSIP: 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
1 Department of Medicine, University of California San Francisco School of Medicine, San Francisco, CA, USA,
2 Department of Radiology, University of California San Francisco School of Medicine, San Francisco, CA, USA,
3 Department of Pathology, University of California San Francisco School of Medicine, San Francisco, CA, USA
* To whom correspondence should be addressed. E-mail: bkinder{at}medsfgh.ucsf.edu.
RATIONALE: The ATS/ERS international consensus classification panel identified the clinical entity idiopathic nonspecific interstitial pneumonia (NSIP) as a provisional diagnosis and recommended further study. 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 enrolled in the UCSF ILD center who met prespecified criteria for UCTD: at least 1 clinical manifestation of CTD, serologic evidence of systemic inflammation in the absence of clinical infection, and absence of sufficient American College of Rheumatology criteria for another CTD. Medical record reviews, evaluation of radiographs, and scoring of lung biopsies were performed. The control group consisted of all other patients (n = 47) with idiopathic interstitial pneumonia that did not meet the UCTD criteria.
RESULTS: The UCTD patients were more likely to be women, younger and non-smokers than the IIP controls. Compared with the control group, UCTD-ILD patients were significantly more likely to have ground-glass opacity on HRCT and NSIP pattern on biopsy, and less likely to have honeycombing on HRCT or UIP 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. Further, these results show that the clinical entity idiopathic NSIP is different from IPF and appears to be an autoimmune disease.
Key words: idiopathic interstitial pneumonia, systemic rheumatic disease, undifferentiated connective tissue, collagen vascular, autoimmune disease
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