Predicting mortality in systemic Wegener's granulomatosis: A survival analysis based on 93 patients

Authors

  • Dominique Bligny,

    1. UPRES EA–3409 Recherche Clinique et Thérapeutique, Hôpital Avicenne, Assistance Publique des Hôpitaux de Paris, Université Paris-Nord, Bobigny, France
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    • Drs. Bligny and Mahr contributed equally to this work.

  • Alfred Mahr,

    Corresponding author
    1. UPRES EA–3409 Recherche Clinique et Thérapeutique, Hôpital Avicenne, Assistance Publique des Hôpitaux de Paris, Université Paris-Nord, Bobigny, France
    • Department of Internal Medicine, Hôpital Avicenne, 125, route de Stalingrad, 93009 Bobigny Cedex, France===

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    • Drs. Bligny and Mahr contributed equally to this work.

  • Philippe Le Toumelin,

    1. UPRES EA–3409 Recherche Clinique et Thérapeutique, Hôpital Avicenne, Assistance Publique des Hôpitaux de Paris, Université Paris-Nord, Bobigny, France
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  • Luc Mouthon,

    1. UPRES EA–3409 Recherche Clinique et Thérapeutique, Hôpital Avicenne, Assistance Publique des Hôpitaux de Paris, Université Paris-Nord, Bobigny, France
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  • Loïc Guillevin

    1. UPRES EA–3409 Recherche Clinique et Thérapeutique, Hôpital Avicenne, Assistance Publique des Hôpitaux de Paris, Université Paris-Nord, Bobigny, France
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Abstract

Objective

To determine predictors of survival of patients with systemic Wegener's granulomatosis (WG).

Methods

We retrospectively studied 93 patients (median age 52 years, male/female ratio 1.7) with systemic WG. All subjects received cytotoxic drugs. Survival was evaluated as a function of the main clinical and laboratory parameters and 2 disease activity scores assessed at diagnosis. Statistical analyses used the multivariate Cox proportional hazards regression model.

Results

The mean followup was 4.5 years; 25 (27%) patients died. According to univariate analysis, a pejorative prognostic value was attributed to serum creatinine >160 μmole/liter (P < 0.001); age >52 years (P < 0.002); absence of ear, nose, and throat (ENT) involvement (P < 0.001); and hemoglobin ≤11.8 gm/dl (P = 0.02). Multivariate analysis retained age >52 years (hazard ratio [HR] = 3.40, P = 0.04) as an independent predictor of poor outcome, whereas the presence of ENT involvement was associated with a longer survival (HR = 0.31, P = 0.02).

Conclusion

Our results suggest that an older age and the absence of ENT involvement at diagnosis independently predict an increased risk of mortality for WG patients. These findings could indicate that the prognosis of WG might be governed by the balance between the granulomatosis as opposed to the vasculitic disease process.

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