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.
- Weight loss.
- Mononeuritis multiplex.
- Purpura necrotic.
- Kidney failure.
- Crusted rhinitis.
- Inflammatory syndrome.
Confirmation of the diagnosis
- To differentiate necrotising vasculitis from other diseases (cancer, lymphoma, syndrome hypereosinophilic...), it is recommended:
- To carry out a biopsy
- If the biopsy is normal or not feasible: to undertake an ANCA detection (immunofluorescence, Elisa)
- An angiography (highlight a microaneurysm).
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:
- Immunosuppressants ,
- For the PAN, PAM and CSS: azathioprine or cyclophosphamide (if signs of gravity);
- For the WG: cyclophosphamide or methotrexate.
- Plasma exchanges.
- Intravenous immunoglobulins.
- Monoclonal antibodies.
- Treatments associated with a corticosteroid therapy:
- Hygiene and dietetic rules,
- 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);
- Pharmacological treatments :
- Antihypertensives ,
- Antiplatelet .
- Others :
- 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)
- Every month
- 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.
Photographic copyright: Wd2007 | Dreamstime.com
Les vascularites nécrosantes systémiques (HAS)
Las vasculitis necrosantes sistémicas