Microscopic polyangiitis and polyarteritis nodosa: How and when do they start?

Authors

  • Christian Agard,

    1. Hôpital Avicenne, Assistance Publique-Hôpitaux de Paris, and Université Paris-Nord, UPRES EA 3409, Bobigny Cedex, France
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    • Drs. Agard and Mouthon contributed equally to this work.

  • Luc Mouthon,

    Corresponding author
    1. Hôpital Avicenne, Assistance Publique-Hôpitaux de Paris, and Université Paris-Nord, UPRES EA 3409, Bobigny Cedex, France
    • Department of Internal Medicine, Hôpital Avicenne, 125, route de Stalingrad, 93009 Bobigny Cedex, France
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    • Drs. Agard and Mouthon contributed equally to this work.

  • Alfred Mahr,

    1. Hôpital Avicenne, Assistance Publique-Hôpitaux de Paris, and Université Paris-Nord, UPRES EA 3409, Bobigny Cedex, France
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  • Loïc Guillevin

    1. Hôpital Avicenne, Assistance Publique-Hôpitaux de Paris, and Université Paris-Nord, UPRES EA 3409, Bobigny Cedex, France
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Abstract

Objective

To describe initial clinical symptoms attributable to microscopic polyangiitis (MPA) or polyarteritis nodosa (PAN).

Methods

We retrospectively reviewed the medical files of 72 patients (mean followup 6.7 years) with biopsy-proven MPA (n = 36) or PAN (n = 36).

Results

Initial manifestations were similar in both entities except for peripheral neuropathy (P = 0.02) and gastrointestinal tract involvement (P = 0.006), which were significantly more frequent in PAN, and general signs alone in MPA (8%; P = 0.02). The mean time to diagnosis was 9.8 ± 19.4 months; 35% of the patients died and 26% relapsed; significantly more MPA than PAN patients relapsed (P = 0.03). Time to diagnosis ≥90 days was associated with a trend toward more patients relapsing (P = 0.12), but not with an increased risk of mortality.

Conclusion

Initial symptoms of MPA and PAN are usually nonspecific and last for several months before the diagnosis is made. A longer time to diagnosis is associated with a tendency to a higher relapse rate.

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