Survival of patients with biopsy-proven usual interstitial pneumonia and nonspecific interstitial pneumonia

Thoracic and Radiology Dept, The Prince Charles Hospital, Brisbane, Australia.
European Respiratory Journal (Impact Factor: 7.64). 07/2002; 19(6):1114-8. DOI: 10.1183/09031936.02.00244002
Source: PubMed


This is the first Australian study to examine survival and clinical characteristics in biopsy-proven idiopathic interstitial pneumonia. A cohort of 70 patients from a single institution between January 1990 and December 1999 was reviewed. All patients were Caucasian, 23 (33%) female. Mean age+/-SD at diagnosis was 60+/-12 yrs for males and 54+/-14 yrs for females. A total 24% of patients had never smoked. The histopathological diagnoses were usual interstitial pneumonia (UIP) (n=59), nonspecific interstitial pneumonia (NSIP) (n=7), desquamative interstitial pneumonia (n=3) and acute interstitial pneumonia (n=11). Clinical and functional characteristics of the two main histological subgroups of UIP and NSIP showed significantly older patients in the UIP group and a significantly lower mean forced expiratory volume in one second (FEV1) in the NSIP group. Median survival for UIP was 78 months compared with 178 months for NSIP. No survival difference between treated and untreated patients with UIP was found. Multivariate analysis revealed smoking alone to be predictive of poorer survival. This study demonstrates the best median survival for usual interstitial pneumonia of available series and confirms a survival difference between usual interstitial pneumonia and nonspecific interstitial pneumonia. Furthermore, the reported results may have implications for treatment timing using conventional protocols currently recommended.

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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.
    Journal of Korean medical science 06/2012; 27(6):661-7. DOI:10.3346/jkms.2012.27.6.661 · 1.27 Impact Factor
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    • "Some of them were retrospective analyses or case series only. Among the drugs tried or on trial are Etanercept, Imatinib, Prednisone (Daniil et al., 1999; Douglas et al., 1997; Douglas, Ryu, & Schroeder, 2000; Douglas et al., 1998; Nicholson AG, 2000; Riha et al., 2002; Ziesche, Hofbauer, Wittmann, Petkov, & Block, 1999), N-Acetylcysteine (Demedts et al., 2005), TGF-β antibody (Genzyme, 2007), Interferon-γ (Antoniou et al., 2006; Raghu et al., 2004; Raghu R, 2001), Interferon-β (Raghu, Bozic, & Brown, 2001), Pirfenidone (Azuma, Nukiwa et al., 2005; S. Nagai et al., 2002; Raghu, Johnson, Lockhart, & Mageto, 1999), Colchicine (Douglas, Ryu, & Schroeder, 2000; Douglas et al., 1998; Selman et al., 1998), Bosentan, Cyclosporin-A (Alton, Johnson, & Turner-Warwick, 1989; Moolman, Bardin, Rossouw, & Joubert, 1991), D-Penicillamin (Chapela, Zuniga, & Selman, 1986; Selman et al., 1998), Heparin (Kubo et al., 2005), Relaxin (ATS, 2002), Angiotensin converting enzyme (ACE) inhibitors (Nadrous, Ryu, Douglas, Decker, & Olson, 2004), and CTGF antibodies (Mageto Y, 2004). Interestingly, azathioprine and cyclophosphamide, two drugs that are still in the current ATS/ERS guidelines for IPF treatment (ATS, 2002), have never been evaluated in the bleomycin model as far as we know. "
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    The International Journal of Biochemistry & Cell Biology 12/2008; 40(3):362-82. DOI:10.1016/j.biocel.2007.08.011 · 4.05 Impact Factor
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    ABSTRACT: Fibrosis is the accumulation of fibroblasts and the connective tissue products secreted by these cells, usually subsequent to tissue injury. While fibrosis can be useful in preserving the general structural integrity of a tissue, it often alters cell-cell and cell-connective tissue interactions, which leads to loss of tissue function. On the basis of the concept that mononuclear phagocytes can direct the development of fibrosis through the release of specific mediators that stimulate fibroblast proliferation, we propose a therapeutic strategy to prevent fibrosis by preventing the release of these specific mediators. The present study demonstrated that colchicine, a widely used and well-tolerated drug, can block alveolar macrophage release of 2 mediators associated with the development of fibrosis in interstitial lung diseases, fibronectin, and the alveolar-macrophage-derived growth factor (AMDGF). Colchicine blocked the spontaneous release of fibronectin by alveolar macrophages obtained from patients with fibrotic lung disease by 23 +/- 4% after 24 h and by greater than 90% after 72 h. AMDGF release was blocked by 68 +/- 10% after 4 h (p less than 0.01, all comparisons). The effect of colchicine was not due to nonspecific toxicity since [14C]proline tracer studies demonstrated that macrophages treated with colchicine were capable of de novo protein synthesis and the secretion of several protein products, despite the fact that fibronectin and AMDGF release were suppressed. The effect of colchicine on the spontaneous release of both fibronectin and AMDGF could be observed at concentrations less than 10 ng/ml, levels that can be achieved in vivo.(ABSTRACT TRUNCATED AT 250 WORDS)
    The American review of respiratory disease 02/1988; 137(1):181-5. DOI:10.1164/ajrccm/137.1.181 · 10.19 Impact Factor
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