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Peripheral neuropathy in Wegener’s granulomatosis, Churg-Strauss syndrome and microscopic polyangiitis
  1. Luigi Cattaneo (luigi.cattaneo{at}nemo.unipr.it)
  1. Dipartimento di Neuroscienze, Università degli Studi di Parma, Italy
    1. Elisabetta Chierici (bettachierici{at}hotmail.com)
    1. Dipartimento di Neuroscienze, Università degli Studi di Parma, Italy
      1. Laura Pavone (laurapavone{at}hotmail.com)
      1. Dipartimento di Clinica Medica e Scienze della Prevenzione, Università degli Studi di Parma, Italy
        1. Chiara Grasselli (grasselli{at}unipr.it)
        1. Dipartimento di Clinica Medica e Scienze della Prevenzione, Università degli Studi di Parma, Italy
          1. Paolo Manganelli (farnese15{at}libero.it)
          1. Unità Operativa di Reumatologia e Medicina Interna, Azienda Ospedaliero-Universitaria di Parma, Italy
            1. Carlo Buzio (carlo.buzio{at}unipr.it)
            1. Dipartimento di Clinica Medica e Scienze della Prevenzione, Università degli Studi di Parma, Italy
              1. Giovanni Pavesi (neurophy{at}unipr.it)
              1. Dipartimento di Neuroscienze, Università degli Studi di Parma, Italy

                Abstract

                Objective: To compare the clinical aspects of peripheral neuropathy associated with Wegener’s granulomatosis (WG), Churg-Strauss syndrome (CSS) and microscopic polyangiitis (MP).

                Methods: Cohort study conducted in a single university hospital. Patients were included when a definite diagnosis of WG, CSS or MP was made according to the current classification criteria in our hospital between 1999 and 2006. All patients underwent periodically a clinical and electrophysiological screening for peripheral neuropathy, assessment of disability and clinical and laboratory evaluation during a mean follow-up of 38 months.

                Results: Sixty-four consecutive patients diagnosed with WG (26 patients), CSS (26 patients) and MP (12 patients) were recruited. Peripheral neuropathy occurred in 27/64 patients: 6 with WG, 15 with CSS, and 6 with MP. Neuropathy occurred earlier in the disease history in CSS and MP compared to WG. Among patients with WG those who developed peripheral neuropathy during follow-up were older than those without neuropathy both at the time of onset and of diagnosis of vasculitis. Distal symmetric polyneuropathy was present in 11 patients, and single or multiple mononeuropathy in 16. Patients with WG had a less severe form of mononeuritis multiplex than CSS or MPA patients. Disability and pain were greater in patients with mononeuropathy, though 1/3 of them were painless. Relapses of neuropathy were extremely infrequent.

                Conclusions: Peripheral neuropathy in WG occurs less frequently, later in the disease course and in a milder form than in CSS and in MP. Single or multiple mononeuropathy associated with these subsets of vasculitis can often be painless.

                • Churg-Strauss syndrome
                • Peripheral neuropathy
                • Wegener's granulomatosis
                • mononeuritis
                • vasculitis

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