Pregnancy and Active Wegener Granulomatosis

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<ul><li>Amt. NZ J Ohsret Gwue</li><li><p>Figure I . Chest X-ray showing right upper lobe shadowing suggestive of alveolar haemorrhage. </p><p>Septrin was ceased during week 9 of the pregnancy. She received no active treatment from week 9 to week 21. Week 18 she represented with blocked sinuses and increasing epistaxis. Her cANCA was 5, the haemo- globin level had dropped from 13 to 9.6 g/dL. ESR was 76; she had no systemic symptoms. </p><p>Week 21 she was tired, mildly breathless and com- plained of haemoptysis. Examination was normal apart from bilateral erythematous oedematous, nasal mucosa. The haemoglobin value was 6.9 g/dL, white cell count 16x1OV/L, 75% neutrophils. Urea, elect- rolytes and liver function tests were normal. ESR 101. cANCA 7, MSU microscopy revealed 3+ blood, 0.6 g/day protein with a few cellular and granular casts. Chest radiograph (figure I ) confirmed pulmonary haemorrhage in the right upper lobe. DLCONA had gone up by 50% to 6.3. Renal biopsy showed only low grade mesangial proliferation; immunofluorescence was compatible with pauci-immune glomerulonephritis. </p><p>She was admitted and transfused. One gramme of methyl-prednisolone was administered daily for 3 days followed by 75 mg daily orally. Cyclophospha- mide 150 mg (2 m a g ) daily was given orally beginning on week 22. </p><p>Haemoptysis and chest radiograph cleared in the first week. She had no more epistaxis. DLVONA returned to 4.3 by week 28. Week 31, prednisolone was decreased to 50 mg because of steroid myopathy. Week 35, because of a wish to breast-feed her baby, she was switched to azathioprine 150 mg (2 m g k g ) daily, and prednisolone was decreased by 5 mg per week. </p><p>She was induced at 37 weeks because of mild oligohydramnios. A female infant, birth-weight 2,870 g, without anatomical defect was born vagi- nally. The infants full blood count was normal apart from ?, transiently depressed platelet count of 130xIO/L. The child was breast-fed and is well. She had normal 6-week and 9-month checks, an episode of mild bronchiolitis and later a presumed viral gastroenteritis; both recovered without specific treatment. </p><p>The patient had no flare of the disease postpartum as has been previously described (5). Nine months postpartum she is well without epistaxis. Her current medication is azathioprine 150 mg daily. Her cANCA is 4. </p><p>DISCUSSION Pregnancy is rare in patients with WG. The course </p><p>of the disease and safety of immunosuppressive therapy have only rarely been reported. To our knowledge this is the first report of severe WG, from conception, treated with cyclophosphamide from the second trimester, which went to term, where both mother and child had a good outcome. Fields (2) has previously reported cyclophosphamide use in WG during the second trimester with both mother and child surviving. However, the infant was born prematurely at 33 weeks and the mother required ongoing haemodialysis. </p><p>Cyclophosphamide does cross the placenta but when used in the second trimester appears safe for the fetus. Whilst rarely used in WG, high dose intravenous cyclophosphamide has often been used in pregnant women with leukaemia and non-Hcdgkin lymphoma, once as early as 12 weeks (lo), without damage to the fetus. Caution should still be cxprcsscd however, as none of thesc studies have follow-up longer than 12 months: hence. the long-term risks o f malignancy are unknown. Its usc in the first trimester is more contentious and certainly in the past. termination of pregnancy has been rccommcndcd before beginning cyclophosphamide for WG (3). The evidence for fetal abnormality associatcd with cyclophosphamide comes from 3 case reports (7-9). In these reports intravenous cyclophosphamidc was used between weeks 6 and 8 which is the peritd that peripheral limb budding oxcurs. The first infant was exposed to 2.4 g at 6 weeks and was born with absent toes and a single coronary artery. The second was exposed to 1.8 g at week 7 and was born with 4 toes on each foot. bilateral finger hypoplasia, cleft palate and bilateral hernias. The third was exposed t o ZOO mg at weeks 2 and 7 and was born with bilaterally absent thumbs, cleft palate, dysmorphic features. and multiple eye abnormalities. It is likely that cyclophosphamide is causally related to these anomalies because of the recurring pattern of malformation. </p></li><li><p>ALAN P. PARNHAM AND GEOFR~EY N. THATCHER 363 </p><p>Because of the maternal risk from severe WG, adequate remission-inducing therapy is mandatory in anyone with this disease. From week 12 it seems that there is little, at least short-term, fetal risk in using cyclophosphamide and prednisolone. Azathioprine which has not been described to cause fetal abnor- mality has been used as a remission-inducing drug but is not as effective as cyclophosphamide ( 1 ) . It however could be used in the first trimester if the mother was unwilling to accept the risks of cyclo- phosphamide and wanted to continue the pregnancy. Oral cyclophosphamide is as good at inducing remission as intravenous treatment. Peak tissue levels do not go as high with oral treatment but it is unknown if it carries the same risk of first trimester malformation. </p><p>Use of immunosuppressive agents in pregnancy is rare and the optimum patient management in pregnant WC patients is uncertain within the first 12 weeks. However, it seems that cyclophosphamide and pred- nisolone can safely be given after this time. </p><p>References 1. Fauci AS, Haynes BF, Katz P, Wolff MS. Wegeners Granulo- </p><p>matosis: Prospective Clinical and therapeutic Experience with 85 patients for 21 years. Ann Intern M d 1983; 98: 76-85. </p><p>2. Fields CL, Ossorio MA, Roy TM, Bunke CM. Wegeners gran- ulomatosis complicated by pregnancy, a case report. J Reprod Med 1991; 36: 463-466. </p><p>3. Palit J, Clague RB. Wegeners granulomatosis presenting during first trimester of pregnancy. Br J Rheurnatol 1990,29: 389-390. </p><p>4. Milford CA, Bellini M. Wegeners Granulomatosis arising in pregnancy. J Laryngol Otol 1986; 100: 475476. </p><p>5. Mrad S, Moalla M, Ben-Mild K et al. Wegeners Granulo- matosis and pregnancy. Review. Med. Interne 1989; 10: 69-72. </p><p>6. DeRernee RA. The Treatment of Wegeners Granulomatosis with Trirnethoprim/Sulfamethoxazole: Illusion or Vision? Arthrit Rheum 1988; 31: 1068-1072. </p><p>7.Toledo TM, Harper RC, Mosher RH. Fetal effects during cyclophosphamide and irradiation therapy. Ann Intern Med 1971; 74: 87-91. </p><p>8.Kirshon B, Wasseatrom N. Willis R, Herman GE, McCabe ERB. Teratogenic effects of first trimester cyclophosphamide therapy. Obstet Gynecol 1988; 7 2 462-464. </p><p>9. Greenberg LH, Tanaka KR. Congenital anomalies probably induced by cyclophosphamide. JAMA 1964, 188: 423-4215. </p><p>10. Ba-Thike K, 00 N. Non-Hodgkins lymphoma in pregnancy. Asia-Oceania J Obstet Gynaecol 1990: 229-232. </p><p>Aust. NZ J Obstet Gynuecd 1996; 36: 3: 363 </p><p>A Case of Acute Colonic Pseudoobstruction in Pregnancy </p><p>Nicholas A. Rieger, FRACS, William J. Lyon, MBBS, Robert L. Bryce, MSc, PhD, FRCOG, FRACOG and Stephen N. Birrell, BMedSc, FRCS, FRACS </p><p>Departments of Surgery and Obstetrics and Gynaecology, Flinders Medical Centre, Bedford Park, South Australia </p><p>EDITORIAL COMMENT: We accepted this case for publication because it shows that acute colonic pseudoobstruction can occur during pregnancy and may be successfully managed by colonoscopy as in this patient. For readers interest we direct them to 2 previous case reports on Ogilvie syndrome which occurred post- Caesarean section and which were complicated by spontaneous perforation of the colon: Atypical Ogilvie syndrome. Paraskevaides EC. Aust NZ J Obstet Gynaecol 1988; 28: 148- 149; Ogilvie syndrome with caecal perforation in the post-Caesarean patient. Tang PTM, Collopy BT, Somerville M. Aust NZ J Obstet GynaecolI995; 35: 104-106. These papers indicate that perforation is likely to occur if the caecum is dilaied to a diameter between 9-12 cm; the dilatation of the transverse colon to 18 cm in the present case is remarkable. </p><p>I . Senior Registrar in General Surgery. 2. Registrar in General Surgery. 3. Senior Consultant Obstetrician and Gynaecologist. 4. Senior Consultant Surgeon. Address for correspondence: Dr Nick Rieger, Hinders Medical Centre, Bedford Park, South Australia, 5042. </p></li></ul>