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Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome

J.W. Song, S-B. Hong, C-M. Lim, Y. Koh, D.S. Kim
European Respiratory Journal 2011 37: 356-363; DOI: 10.1183/09031936.00159709
J.W. Song
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S-B. Hong
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C-M. Lim
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Y. Koh
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D.S. Kim
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  • For correspondence: dskim@amc.seoul.kr
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    Figure 1–

    a) Comparison of survival curves (from rapid deterioration to death or last follow-up) between patients with acute exacerbation (AE) (—) and infection (---). b) Comparison of survival curves (from initial diagnosis to death or last follow-up) for patients with no rapid deterioration (RD) (—), AE (···), bilateral infection (----) and focal RD (– – –).

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  • Table 1– Incidence of acute exacerbation (AE) and rapid deterioration (RD)
    Incidence#AE¶RD
    1-yr58 (14.2)97 (23.0)
    2-yr71 (18.8)124 (31.2)
    3-yr75 (20.7)134 (35.4)
    • Data are presented as n (%). The cumulative incidences of AE, excluding patients first presented at the time of AE, are 11.6% (1-yr), 16.3% (2-yr) and 18.2% (3-yr). #: first event; ¶: 14 patients first presented at the time of AE.

  • Table 2– Aetiologies of rapid deterioration (RD; first episode)
    AetiologyCases n (%)#Documented organisms¶ (n)
    Total RD163 (35.4)
    Bilateral lesions140 (30.4)
     AE90 (19.5)
      Definite57 (12.4)
      Suspected33 (7.2)
     Infection37 (8.0)
      Definite21 (4.6)
       Bacterial9 (2.0)Streptococcus pneumoniae (2)MRSA (1)Haemophilus influenzae (4)Legionella spp. (1)Klebsiella pneumoniae (1)
       Viral7 (1.5)CMV (7; 2 mixed infections with RSV or Pneumocystis jiroveci)Influenza virus (1)RSV (1)
       Fungal2 (0.4)Candida spp. (1)Aspergillus spp. (1)
       Parasitic2 (0.4)Pneumocystis jiroveci (2)
       Mycobacterial1 (0.2)Mycobacterium tuberculosis (1)
      Suspected+16 (3.5)
     Heart failure5 (1.1)
     PTE2 (0.4)
     AEP1 (0.2)
     Uncertain§5 (1.1)
    Focal lesion23 (5.0)
     Pneumothorax9 (2.0)
     Infection14 (3.0)Klebsiella pneumoniae (1)Klebsiella oxytoca (1)Streptococcus pneumoniae (1)
    • AE: acute exacerbation; PTE: pulmonary thromboembolism; AEP: acute eosinophilic pneumonia; MRSA: methicillin-resistant Staphylococcus aureus; CMV: cytomegalovirus; RSV: respiratory syncytial virus. #: percentage of total subjects (n = 461); ¶: identified by culture, antigen test (for S. pneumoniae and Legionella), or direct fluorescence monoclonal antibody stain (for P. jiroveci); +: cases in which no organism was identified, but infection was clinically suspected due to symptoms such as grossly purulent sputum and rapid resolution of symptoms in response to antibiotics alone; §: cases in which no organism was identified, but the criteria for AE were not completely satisfied due to incomplete study.

  • Table 3– Comparison of the baseline characteristics between patients with and without acute exacerbation (AE)
    CharacteristicsNon-RDAEp-valueInfection#p-value¶
    Patients2989037
    Age yrs63.4±8.164.3±8.9ns63.7±8.6ns
    Males228 (76.5)69 (76.7)ns30 (81.1)ns
    Smoking0.107ns
     Never smoked76 (25.5)31 (34.4)10 (27.0)
     Smokers222 (74.5)59 (65.6)27 (73.0)
    Smoking exposure pack-yrs34.8±19.235.7±20.0ns34.0±16.2ns
    PFT % pred+
     FVC77.6±17.072.0±15.70.00575.5±18.5ns
     DL,CO66.4±19.062.2±19.3ns61.2±18.00.088
     TLC78.7±14.473.8±15.00.02175.9±18.5ns
     FEV188.5±18.086.0±18.3ns89.0±17.5ns
    BAL %+
     Macrophages71.0±18.467.8±16.4ns66.9±16.1ns
     Lymphocytes15.7±12.415.7±11.2ns18.2±14.2ns
     Neutrophils9.4±14.111.3±13.7ns10.8±9.4ns
     Eosinophils3.2±4.63.2±4.4ns1.9±2.3ns
    CRP mg·dL−1+0.5±0.70.4±0.3ns0.7±0.70.055
    Steroid with/without cytotoxic agent§184 (61.7)56 (62.2)ns20 (54.1)ns
    • Data are presented as n, mean±sd or n (%), unless otherwise stated. RD: rapid deterioration; PFT: pulmonary function test; % pred: % predicted; FVC: forced vital capacity; DL,CO: diffusing capacity of the lung for carbon monoxide; TLC: total lung capacity; FEV1: forced expiratory volume in 1 s; BAL: bronchoalveolar lavage; CRP: C-reactive protein; ns: nonsignificant. #: infection with bilateral lesions; ¶: No-RD versus infection; +: data from the patients who first presented with RD were excluded; §: within 30 days prior to RD.

  • Table 4– Risk factors for acute exacerbation compared to no episodes of rapid deterioration (RD) at the time of initial diagnosis
    ParametersHR (95% CI)p-value
    Univariate Cox analysis
     Age1.021 (0.997–1.047)0.093
     Male sex0.921 (0.563–1.506)ns
     Smoking0.629 (0.407–0.972)0.037
     PFT % pred
      FVC0.975 (0.960–0.989)0.001
    DL,CO0.981 (0.967–0.994)0.005
      TLC0.970 (0.953–0.987)0.001
     BAL
      Macrophages0.991 (0.975–1.008)ns
      Lymphocytes1.001 (0.977–1.025)ns
      Neutrophils1.010 (0.989–1.031)ns
      Eosinophils0.994 (0.929–1.064)ns
     CRP0.786 (0.380–1.622)ns
     Steroids with/without cytotoxic agents#1.045 (0.682–1.602)ns
    Multivariate Cox analysis¶
     Smokers0.585 (0.342–1.001)0.050
     FVC % pred0.979 (0.964–0.995)0.011
    • HR: hazard ratio; PFT: pulmonary function test; FVC: forced vital capacity; % pred: % predicted; DL,CO: diffusing capacity of the lung for carbon monoxide; TLC: total lung capacity; BAL: bronchoalveolar lavage; CRP: C-reactive protein; ns: nonsignificant. #: within 30 days prior to RD; ¶: TLC % pred was excluded from the multivariate analysis due to the close correlation between FVC % pred and TLC % pred (r = 0.765; p<0.001).

  • Table 5– Comparison of the clinical features of the patients with acute exacerbation (AE) and the bilateral infection at the time of rapid deterioration (RD)
    CharacteristicsAEInfectionp-value
    Patients9037
    Age yrs65.3±7.966.1±7.6ns
    Males69 (76.7)30 (81.1)ns
    Disease duration months16.5±24.529.5±34.70.014
    Steroid with/without cytotoxic agent use#56 (62.2)20 (54.1)ns
     Duration¶ months3.4±3.84.5±5.2ns
     Last dose¶ mg18.8±13.218.4±15.0ns
    Duration of dyspnoea days11.2±12.56.1±7.40.030
    Initial symptoms
     Documented fever18 (20.0)19 (51.4)0.001
     Sputum production63 (70.0)31 (83.8)ns
    CRP mg·dL−18.3±6.814.7±9.8<0.001
    Pa,O2/FI,O2253±83228±91ns
    BAL
     Total cells·mm−3292±168349±342ns
     Lymphocytes %19.7± 19.68.1±7.50.041
     Neutrophils %22.9±19.955.2±28.90.001
    • Data are presented as n, mean±sd or n (%), unless otherwise stated. CRP: C-reactive protein; Pa,O2: arterial oxygen tension; FI,O2: inspiratory oxygen fraction; BAL: bronchoalveolar lavage; ns: nonsignificant. #: within 30 days prior to RD; ¶: steroid (prednisolone) treatment.

  • Table 6– Prognostic factors for in-hospital mortality of patients with acute exacerbation
    ParametersOR (95% CI)p-value
    Univariate logistic analysis
     Age1.022 (0.969–1.078)ns
     Male sex1.455 (0.543–3.895)ns
     Disease duration0.994 (0.977–1.011)ns
     Steroid with/without cytotoxic agent use#0.828 (0.353–1.943)ns
      Duration¶1.142 (0.932–1.399)ns
      Last dose¶1.034 (0.990–1.081)ns
     Duration of dyspnoea0.939 (0.902–0.978)0.003
     Documented fever2.364 (0.799–6.989)ns
     Sputum production1.705 (0.684–4.252)ns
     Pa,O2/FI,O20.989 (0.983–0.996)0.001
     CRP1.087 (1.009–1.172)0.029
     BAL
      Total cells0.998 (0.992–1.003)ns
      Lymphocytes0.905 (0.826–0.992)0.033
      Neutrophils1.055 (0.996–1.118)0.070
    Multivariate logistic analysis
     CRP2.467 (1.030–5.911)0.043
     BAL lymphocytes0.869 (0.737–1.024)0.093
    • Pa,O2: arterial oxygen tension; FI,O2: inspiratory oxygen fraction; CRP: C-reactive protein; BAL: bronchoalveolar lavage; ns: nonsignificant. #: within 30 days prior to rapid deterioration; ¶: steroid treatment.

  • Table 7– Treatment during acute exacerbation (AE) and in-hospital outcome of patients with AE according to treatment
    Treatment regimenCasesSurvivalp-value
    Steroid pulse#13 (14.4)7 (53.8)0.933
    Steroid pulse# plus cytotoxic agent¶8 (8.9)4 (50.0)
    High-dose steroid+46 (51.1)19 (41.3)
    High-dose steroid+ plus cytotoxic agent¶14 (15.6)11 (78.6)
    Low-dose steroid§6 (6.7)3 (50.0)
    Low-dose steroid§ plus cytotoxic agent¶1 (1.1)1 (100.0)
    No treatment2 (2.2)0
    Total90 (100)45 (50)
    • Data are presented as n (%) unless otherwise stated. #: steroid pulse was ≥500 mg·day−1 methylprednisolone for 3 days, followed by high-dose steroid; ¶: cytotoxic agents were azathioprine, cyclosporine or cyclophosphamide; +: high-dose steroid was ≥0.5 mg·kg−1·day−1 prednisolone; §: low-dose steroid was ≤0.5 mg·kg−1·day−1 prednisolone.

  • Table 8– Prognostic factors for the overall survival from the initial diagnosis of idiopathic pulmonary fibrosis
    ParametersHR (95% CI)p-value
    Univariate Cox analysis
     Age1.016 (1.001–1.031)0.032
     Male sex0.910 (0.685–1.209)NS
     Smoking0.737 (0.568–0.956)0.021
     PFT % pred
      FVC0.976 (0.968–0.984)<0.001
      DL,CO0.978 (0.971–0.986)<0.001
      TLC0.975 (0.965–0.984)<0.001
     BAL
      Macrophages1.003 (0.993–1.014)NS
      Lymphocytes0.998 (0.985–1.012)NS
      Neutrophils0.996 (0.980–1.012)NS
      Eosinophils0.979 (0.941–1.018)NS
     CRP1.073 (0.808–1.423)NS
     Steroid with/without cytotoxic agent1.529 (1.177–1.986)0.001
     Occurrence of AE#2.770 (2.132–3.599)<0.001
    Multivariate Cox analysis¶
     Age1.017 (1.001–1.033)0.032
     PFT % pred
      FVC0.987 (0.977–0.997)0.009
      DL,CO0.985 (0.976–0.994)0.001
     Steroid with/without cytotoxic agent1.552 (1.113–2.164)0.010
     Occurrence of AE2.592 (1.888–3.560)<0.001
    • HR: hazard ratio; PFT: pulmonary function test; % pred: % predicted; FVC: forced vital capacity; DL,CO: carbon monoxide diffusing capacity of the lung; TLC: total lung capacity; BAL: bronchoalveolar lavage; CRP: C-reactive protein; AE: acute exacerbation; NS: not significant. #: overall occurrence during follow up; ¶: TLC % pred was excluded from the multivariate analysis due to the close correlation between FVC and TLC % pred (r = 0.765; p<0.001).

Additional Files

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    Files in this Data Supplement:

    • Supplementary tables -
      Table E1. Diagnostic criteria for acute exacerbation by Collard et al.
      Table E2. Investigation protocol for the patients with suspected acute exacerbation (AE) of IPF
      Table E3. Immunosuppressive therapy before rapid deterioration in patients with opportunistic infection
      Table E4. Discriminating factors for acute exacerbation compared to infection at the time of rapid deterioration
      Table E5. Immediate outcomes of rapid deterioration from the onset of rapid deterioration
      Table E6. Comparison of clinical features between survivors and non-survivors of acute exacerbation
      Table E7. Impact of rapid deterioration: comparison of the overall survival after the initial diagnosis between patients with and without rapid deterioration
      Table E8. Multiple episodes of rapid deterioration
      Table E9. Etiologies of multiple episodes of rapid deterioration
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Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome
J.W. Song, S-B. Hong, C-M. Lim, Y. Koh, D.S. Kim
European Respiratory Journal Feb 2011, 37 (2) 356-363; DOI: 10.1183/09031936.00159709

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Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome
J.W. Song, S-B. Hong, C-M. Lim, Y. Koh, D.S. Kim
European Respiratory Journal Feb 2011, 37 (2) 356-363; DOI: 10.1183/09031936.00159709
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