Pregnancy and Active Wegener Granulomatosis

Abstract

EDITORIAL COMMENT: We accepted this case report not only because readers could encounter a patient with Wegener granulomatosis, but also because the use of cyclophosphamide from 22 weeks gestation did not harm the fetus. We have asked the authors to provide readers with a 100–200 word summary of the aetiology, clinical features and complications of Wegener granulomatosis. Author's response: Wegener granulomatosis is a systemic necrotizing granulomatous vasculitis of unknown aetiology with a predilection for the kidney and upper respiratory tract, especially the nose and sinuses. It classically causes acute renal failure, epistaxis, and pulmonary haemorrhage. It can however affect any organ and untreated has an 82% 1‐year mortality. It can occur at any age but is more common in elderly patients. It is very strongly associated with cytoplasmic antineutrophil cytoplasmic antibody (cANCA) which is directed against a serine proteinase 3 antigen and can be used to follow the course of the disease. Unless the patient has very localized disease, treatment always involves cyclophosphamide and high dose steroids ± plasmapheresis to obtain remission. This would then be followed by a prolonged course of azathioprine and steroid treatment.

Pregnancy in patients with Wegener's granulomatosis: report of five cases in three women

“…Of 15 pregnancies beginning in inactive WG, 14 ended in live births (one unrelated WG therapeutic abortion) (our patients and those reported in the following references) 6-1014 15 18 …”

“…Four relapses occurred while pregnancy had begun during active disease7 8 12 16 and four while WG was in remission 68 14 15 WG activity at pregnancy onset seemed to be the main indicator of poor maternal and fetal prognosis as four of 15 pregnancies beginning during active WG ended in two spontaneous fetal deaths, one therapeutic abortion, and one maternal death despite therapeutic abortion 78 12 16Clinical activity correlated with positive ANCA at pregnancy onset in two cases 7…”

“…Cyclophosphamide was given orally in four cases with various lengths of treatment: 6,6 8 12,9 or 18 months 16. It was given intravenously in four cases with varied cumulative doses: 1 g,7 15 3 g,7 and 23 g 18. Of note, two of our patients became pregnant after at least 16 cyclophosphamide pulses.…”

“…8 14 15 In two cases, ANCA remained positive despite clinical remission 815 ANCA monitoring was useful for individual cases: persistently reduced ANCA titres associated with clinical remission suggest inactive disease and help to authorise pregnancy. Nevertheless, apart from in the context of pregnancy, ANCA titres do not have an absolute predictive value for WG activity.…”

Pregnancy complicating Wegener's granulomatosis

“…3 Those who fared the best had been treated with cyclophosphamide after the first trimester or had received additional immunosuppression other than steroids alone. 14,17,19 Postpartum flares were more common in this group with three of the seven patients affected. 3,18,19 Wegener's diagnosed during pregnancy Thirteen cases of Wegener's presented for the first time during pregnancy.…”