Idiopathic nonspecific interstitial pneumonia: Lung manifestation of undifferentiated connective tissue disease?

Department of Medicine, University of California School of Medicine, San Francisco, California, USA.
American Journal of Respiratory and Critical Care Medicine (Impact Factor: 11.99). 11/2007; 176(7):691-7. DOI: 10.1164/rccm.200702-220OC
Source: PubMed

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

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    • "Patients with a connective tissue disorder (CTD) are susceptible to lung involvement and some histopathological patterns of interstitial lung disease (ILD), including usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), organizing pneumonia (OP), and diffuse alveolar damage, which occasionally occur in rheumatoid arthritis (RA) [1] [2] [3] [4]. Joint manifestations of RA usually precede lung involvements by several years; however, in less than 10% of cases of RA associated UIP or NSIP, ILD may be the initial manifestation of RA [5] [6]. "
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    ABSTRACT: Rheumatoid arthritis patients are susceptible to interstitial lung disease, and joint manifestations of rheumatoid arthritis usually precede lung involvements by several years. Organizing pneumonia, as the first manifestation of rheumatoid arthritis, is extremely rare, and its clinical features remain currently unknown. We present a case and a literature review of patients who were pathologically diagnosed with organizing pneumonia first and met the diagnostic criteria of rheumatoid arthritis later. In this review, we observed the following: (1) patients with organizing pneumonia preceding rheumatoid arthritis have a high prevalence of rheumatoid factor or anticyclic citrullinated peptide antibodies; (2) almost all patients developed rheumatoid arthritis within one year after the diagnosis of organizing pneumonia. We suggest that patients with organizing pneumonia and positive for either rheumatoid factor or anticyclic citrullinated peptide antibody should be cautiously followed up regarding the development of rheumatoid arthritis, particularly during the first year after the diagnosis of organizing pneumonia.
    01/2014; 2014:758619. DOI:10.1155/2014/758619
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    ABSTRACT: ObjectivesWe evaluated the performance of high-resolution computed tomography (HRCT) to differentiate chronic diffuse interstitial lung diseases (CDILD) with predominant ground-glass pattern by using logical analysis of data (LAD). MethodsA total of 162 patients were classified into seven categories: sarcoidosis (n = 38), connective tissue disease (n = 32), hypersensitivity pneumonitis (n = 18), drug-induced lung disease (n = 15), alveolar proteinosis (n = 12), idiopathic non-specific interstitial pneumonia (n = 10) and miscellaneous (n = 37). First, 40 CT attributes were investigated by the LAD to build up patterns characterising a category. From the association of patterns, LAD determined models specific to each CDILD. Second, data were recomputed by adding eight clinical attributes to the analysis. The 20 × 5 cross-folding method was used for validation. ResultsModels could be individualised for sarcoidosis, hypersensitivity pneumonitis, connective tissue disease and alveolar proteinosis. An additional model was individualised for drug-induced lung disease by adding clinical data. No model was demonstrated for idiopathic non-specific interstitial pneumonia and the miscellaneous category. The results showed that HRCT had a good sensitivity (≥64%) and specificity (≥78%) and a high negative predictive value (≥93%) for diseases with a model. Higher sensitivity (≥78%) and specificity (≥89%) were achieved by adding clinical data. ConclusionThe diagnostic performance of HRCT is high and can be increased by adding clinical data. KeywordsInterstitial lung disease-Ground-glass opacity-High-resolution computed tomography-Logical analysis of data-Medical informatics
    European Radiology 06/2010; 20(6):1297-1310. DOI:10.1007/s00330-009-1671-4 · 4.34 Impact Factor
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