The most common fibrotic pulmonary lesion in young women is pulmonary fibrosis secondary to radiotherapy. Other causes include idiopathic pulmonary fibrosis, hypersensitivity pneumonitis (formerly known as extrinsic allergic alveolitis), autoimmune/connec- tive tissue disease-related fibrosis, occupational lung disease and drug-induced fibrosis. The progressive alveolar and pulmonary fibrotic conditions tend to occur in later life; the general principles are the same, although these conditions are potentially life-threatening. There are few case reports of pregnancy in women with idiopathic pulmonary fibrosis, as two-thirds of presentations are diagnosed after the age of 60, with a median survival from diagnosis of 3 years; however, successful vaginal and caesarean deliveries have been described.
Problems and special considerations
Many women who have had a successfully treated malignancy expect to have children. Many of them are unaware that their pulmonary function is not normal, as they have no symptoms during ordinary activities, and they may not have been told that there has been any pulmonary damage as a result of treatment of their malignancy. Pulmonary function tests show a reduction in the vital capacity and forced expiratory volume, with no evidence of restrictive lung disease unless there is other pathology. Most of these women tolerate their pregnancy with little or no problem, although they need a plan for their delivery.
Patients who have had a treated malignancy have usually had chemotherapy and may have had drugs that cause myocardial damage, such as bleomycin. It is therefore important that these women have echocardiography to assess their cardiac function.
Women who are symptomatic before pregnancy may find the additional physiological challenges of pregnancy difficult; restrictive lung disease does not allow for the usual increases in minute volume seen in pregnancy.
Management options
It is important to assess these women antenatally so that they may understand their restrictions and how these might affect the management of labour and delivery. The delivery plan will depend on the degree of pulmonary damage. Some women may be treated as entirely normal, whereas others require a clear plan comprising avoidance of general anaesthesia and sedative drugs, epidural analgesia during labour to avoid further increases in minute ventilation and oxygen consumption, and regional anaesthesia for operative delivery.
Key points
• Antenatal assessment of the woman with pulmonary fibrosis is essential, including pulmonary function tests and echocardiography when appropriate.
• Intrapartum management is as for respiratory disease in general.
Further reading
Budev MM, Arroliga AC, Emery S. Exacerbation of underlying pulmonary disease in pregnancy. Crit Care Med 2005; 33 (10 Suppl): S313-18.
Wexler ID, Johannesson M, Edenborough FP, Sufian BS, Kerem E. Pregnancy and chronic progressive pulmonary disease. Am J Respir Crit Care Med 2007; 175: 300-5.