Inlammation and damage to blood vessels may be primary or caused by another disease. Immune-complex deposition is presumed to underlie most cases (Langford, 2015). Primary types include polyarteritis nodosa, temporal or giant-cell arteritis, T akayasu arteritis, Henoch-Schonlein purpura, Behyet syndrome, and cutaneous or hypersensitivity arteritis (Goodman, 2014). Small vessel vasculitides such as granuomatosis with poyangiitis and eosinophilic granuomatosis with poyangiitis have antibodies directed against proteins in the cytoplasmic granules of leukocytes-antineutrophil ytopsmic antibodies-NA (Pagnox, 201).
This necrotizing vasculitis of small and medium-sized arteries is characterized clinically by myalgia, neuropathy, gastrointestinal disorders, hypertension, and renal disease (Goodman, 2014). Of cases, approximately a third is associated with hepatitis B antigenemia (Langford, 2015). Symptoms are nonspecific, and fever, weight loss, and malaise are present in more than half of cases. Diagnosis is made by biopsy, and treatment consists of high-dose prednisone plus cyclophosphamide. Vasculitis due to hepatitis B antigenemia responds to antivirals, glucocorticosteroids, and plasma exchange (Chap. 55, p. 1063).
Only a few reports describe polyarteritis nodosa associated with pregnancy. Of 12 afected gravidas, polyarteritis first manifested during pregnancy in seven, and it was rapidly fatal by 6 weeks postpartum (Owen, 1989). he diagnosis was not made until autopsy in six of the seven women. Four women continued pregnancy, which resulted in one stillborn and three successful outcomes.
• Granulomatosis with Polyangiitis
Formerly Wegener granulomatosis, this is a small-vessel necrotizing granulomatous vasculitis afecting the upper and lower respiratory tract and kidney (Pagnoux, 2016). Disease frequently includes sinusitis and nasal disease-90 percent; pulmonary infiltrates, cavities, or nodules-85 percent; glomerulonephritis-75 percent; and musculoskeletalrlesions-65 percent (Sneller, 1995). At least 90 percent have polyangiitis (Langford, 2015). It is uncommon and usually encountered after age 50. Koukoura and associates (2008) reviewed 36 cases in association with pregnancy and found a higher preterm birth rate. In another report, a second woman had disease-related pneumonitis, but pregnancy did not appear to afect disease activity (Pagnoux, 2011). Because subglottic stenosis is found in up to a fourth of patients, the anesthesia team is ideally consulted antepartum (Engel, 2011).
Corticosteroids are standard treatment, but azathioprine, cyclosporine, and IVIG therapy may also be used. For severe disease in the late second or third trimester, cyclophosphamide in combination with prednisolone seems acceptable.