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: 13). 11/2007; 176(7):691-7. DOI: 10.1164/rccm.200702-220OC
Source: PubMed


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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    • "Overall, the ATS/ERS workshop committee has used the logical diagnosis of NSIP for middle aged women and nonsmokers without clinical and serologic evidence for collagen vascular disease (2). Even in some situations, NSIP has clinically been used in cases of undifferentiated connective tissue disorder (20). "
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    ABSTRACT: Background: In many cases of ILD (interstitial lung disease), overlap diagnosis is considered. Here, a few cases with diagnosis of a variety of ILDs, where eventual open lung biopsy has been performed are selected. Reference will be made to reliable sources to show that NSIP can still be a variant of UIP (Usual interstitial pneumonia) with better treatment response and prognosis. Case Presentation: In case 1, there is a difference between the HRCT(High Resolution Computed Tomography) result (NSIP pattern without fibrosis) and pathologic result (which includes fibrosing NSIP more closely related to UIP).Case 2 shows obvious discord between HRCT result (UIP pattern) and pathologic result (NSIP pattern). In case 3, there is again a discrepancy between HRCT report (very mild architectural distortion suggestive for ILD like NSIP) and pathology report (destructed lung tissue with interstitial fibrosis suggestive of HP (Hypersesitivity Pneumonitis) and not NSIP. Conclusion: In this paper, we demonstrate that although NSIP can be a distinct diagnosis in most cases, but in rare cases the distinction between the other kinds of ILD especially UIP and NSIP in spite of full workup including tissue assessment can be very difficult.
    Full-text · Article · Apr 2014 · Caspian Journal of Internal Medicine
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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.
    Full-text · Article · Jan 2014
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    • "Anti-nuclear antibodies are found in patients not only with autoimmune diseases but also those with various non-rheumatological conditions associated with tissue damage such as infections, cancer, and hormonal or blood diseases. Recent studies have shown that even idiopathic NSIP might be associated with an autoimmune background that later reveals itself as an organspecific or a systemic autoimmune disease (20, 21). Thus, we presumed that the presence of ANA might be an early manifestation of this systemic background and that simultaneous systemic manifestations might be related to a poor prognosis. "
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    ABSTRACT: The purpose of this study was to investigate the long-term clinical course of non-specific interstitial pneumonia (NSIP) and to determine which factors are associated with a response to steroid therapy and relapse. Thirty-five patients with pathologically proven NSIP were included. Clinical, radiological, and laboratory data were reviewed retrospectively. The male-to-female ratio was 7:28 (median age, 52 yr). Thirty (86%) patients responded to steroid therapy, and the median follow-up was 55.2 months (range, 15.9-102.0 months). Five patients (14%) showed sustained disease progression and three died despite treatment. In the five with sustained disease progression, NSIP was associated with various systemic conditions, and the seropositivity of fluorescent antinuclear antibody was significantly associated with a poor response to steroids (P = 0.028). The rate of relapse was 25%, but all relapsed patients improved after re-treatment. The initial dose of steroids was significantly low in the relapse group (P = 0.020). In conclusion, progression is associated with various systemic conditions in patients who show progression. A low dose of initial steroids is significantly associated with relapse.
    Full-text · Article · Jun 2012 · Journal of Korean medical science
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