[Pregnancy and vasculitides]

Systemic vasculitides, like Takayasu's arteritis, polyarteritis nodosa, Wegener's granulomatosis, Churg-Strauss syndrome, Henoch-Schönlein purpura, or Behçet's disease can affect women of child-bearing years. The rarity of these vasculitides, their frequent fatal outcomes until recent years, and the use of toxic immunosuppressants to treat patients, contra-indicating pregnancy and/or potentially inducing hypofertility or sterility, explain the few pregnancies reported in the literature so far. Notably, it does not seem that pregnancy has a major impact on vasculitis outcome, in contrast with systemic lupus erythematosus, but a specialized management of these pregnant patients is mandatory. There are some reported cases of vasculitis revealed during pregnancy. Even though some of these pregnant patients had a severe disease and died, most of them had a favourable outcome, and a living inborn, providing prompt care and adequate treatment. When vasculitis is already known and treated, pregnancy should at best be planned, when the disease is in sustained remission and all toxic immunosuppressants have been stopped for months. Vasculitis sequella, like hypertension, renal insufficiency, or asthma, must also be taken into account, monitored and appropriately managed throughout the pregnancy and a few weeks following delivery. In case of vasculitis' flare during pregnancy, potential treatments include corticosteroids, intravenous immunoglobulins, azathioprine, plasma exchanges, and, for limited skin manifestations or Behçet's disease, hydroxychloroquine or colchicine. Importantly, when the disease is severe, a delay in the prescription of a stronger, immunosuppressant, chiefly intravenous cyclophosphamide, can be more detrimental, although being potentially toxic, for both the mother and the foetus than an ineffective and/or inappropriate regimen with less active drugs. Safety data on biologics, like rituximab, for pregnant women are very sparse to date and their use is therefore not recommended, unless confronted with a severe and refractory disease, and after referring to a specialized center for vasculitides.