Systemic necrotising vasculitis

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The following long-term disease guide aims at optimising how to deal both in the diagnosis and the care of patients affected by systemic necrotising vasculitis by health professionals.


  • Systemic necrotising vasculitis (SNV) gathers a set of inflammatory diseases concerning the capillary, venous and arterial blood vessels, among which:
    • Polyarteritis nodosa (PAN),
    • Vasculitis associated with the Anti-neutrophil cytoplasmic antibodies (ANCA)
      • Wegener's granulomatosis (WG),
      • Churg-Strauss syndrome (CSS),
      • Microscopic polyangiitis (MPA).


Because of their heterogeneous symptoms, a "multidisciplinary" assessment is recommended.

This diagnosis also determines the degree of severity of the disease.

Clinical signs

  • Arthralgias.
  • Myalgia.
  • Fever.
  • Weight loss.
  • Mononeuritis multiplex.
  • Purpura necrotic.
  • Kidney failure.
  • Crusted rhinitis.
  • Inflammatory syndrome.
  • Hypereosinophilia.

Confirmation of the diagnosis

  • To differentiate necrotising vasculitis from other diseases (cancer, lymphoma, syndrome hypereosinophilic...), it is recommended:
    • To carry out a biopsy
      • of an organ
      • of a tissue
    • If the biopsy is normal or not feasible: to undertake an ANCA detection (immunofluorescence, Elisa)
    • An angiography (highlight a microaneurysm).

Further examinations

  • Check-ups
    • pulmonary,
    • renal,
    • cardiac,
    • neurological,
    • hepatic.


Dealing with a patient affected by VNS is multidisciplinary.


  • Treatment is composed of a phase of attack (3-6 month) then a phase of maintenance (12-24 month) to limit relapses:
    • Corticoïdes,
    • Immunosuppressants ,
      • For the PAN, PAM and CSS: azathioprine or cyclophosphamide (if signs of gravity);
      • For the WG: cyclophosphamide or methotrexate.

Further treatments

  • Plasma exchanges.
  • Intravenous immunoglobulins.
  • Monoclonal antibodies.
  • Treatments associated with a corticosteroid therapy:
    • Hygiene and dietetic rules,
    • Potassium,
    • IPP,
    • Osteoporosis prevention (bisphosphonates).
  • In order to prevent any possible infections:
    • Trimethoprim and Sulfamethoxazole ;
    • Rifampicin and isoniazid (patients having antecedents of untreated tuberculosis, contact with tuberculosis);
    • Antifungal.
    • Vaccination.

Other treatments

  • Pharmacological treatments :
    • Analgesics,
    • Antihypertensives ,
    • Benzodiazepines,
    • Antiplatelet .
  • Others :
    • Dialyse,
    • Surgery,
    • Physical therapy.

Follow-up of the patient

  • A follow-up is necessary
    • To prevent complications related to the disease,
    • To ensure a follow-up of the treatment in order to,
      • Avoid a relapse,
      • Deal with the failures of treatment,
      • Prevent complications related to treatment.

It consists of tests
  • Clinical every 3 months (similar to the initial diagnosis)
  • Paraclinical
    • Every month
      • NFS
      • Hepatic check-up
    • Every 2 months
      • TP TCA
      • Proteinuria, leucocytes and red corpuscles, nitrites
      • Bacteriological examinations of the urines
      • Serum EPP
      • Every 3 months: control of the ANCA.


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