9 - Lupus and connective tissue disease in pregnancy

Summary

There is an increasing understanding of the impact of the presence of lupus nephritis on pregnancy outcome. Until recently, relatively little was known about pregnancy in vasculitic diseases, which are less common in women of childbearing age. These include the primary systemic vasculitides with potential for renal involvement: small vessel vasculitides including Wegener's granulomatosis, microscopic polyangiitis, Henoch-Schönlein purpura and Churg—Strauss syndrome. Also included are the medium-large vessel vasculitides such as Churg—Strauss syndrome (which may involve small and medium vessels), polyarteritis nodosa and Takayasu's arteritis. Primary systemic vasculitis is now classified as antineutrophil cytoplasmic antibody (ANCA)-positive (typically Wegener's granulomatosis or microscopic polyangiitis) or ANCA-negative vasculitis, which may blur the distinctions between the classically defined disorders. In this chapter the traditional diagnostic terms are retained, as many believe these relate to typically distinct disease entities and these terms are used in the great majority of publications concerning pregnancy.

The issues relating to lupus nephritis and vasculitis are discussed, but a review of associated autoantibodies such as antiphospholipid antibodies and the anti-Ro (SSA) and anti-La (SSB) is not included. These autoantibodies need to be screened for and managed according to the separate role they may play. In particular, attention will need to be paid to antiphospholipid syndrome and the neonatal lupus syndromes.