Connective-tissue disorders, also referred to as collagen-vascular disorders, are a group of diseases that are not organ specific and thus cause generalized clinical findings. They are principally characterized by connective-tissue abnormalities that are immunopathologically mediated as the consequence of a variety of autoantibodies. Systemic lupus erythematosus (see Chapter 55) and the antiphospholipid syndrome (see Chapter 54) are examples of connective-tissue disorders that are discussed elsewhere in this manual. In this chapter, we will focus on several less common connective-tissue disorders that may be seen in pregnant women.
This is a chronic polyarthritis with symptoms of synovitis, fatigue, anorexia, weakness, weight loss, depression, and vague musculoskeletal symptoms. The hands, wrists, knees, and feet are commonly involved. Pain, aggravated by movement, is accompanied by swelling and tenderness. Extra-articular manifestations include rheumatoid nodules, vasculitis, and pleuropulmonary symptoms. The 1987 revised criteria of the American Rheumatism Association have approximately a 90-percent specificity and sensitivity for the diagnosis (Table 56-1).
TABLE 56-1. The 1987 Revised Criteria for the Classification of Rheumatoid Arthritis
There are no obvious adverse effects of rheumatoid arthritis on pregnancy outcome. In most instances, women with rheumatoid arthritis can be reassured that successful pregnancy is likely. Indeed, most women with rheumatoid arthritis improve during pregnancy. Conversely, postpartum exacerbation is common.
Treatment is directed at pain relief, reduction of inflammation, protection of articular structures, and preservation of function. Physical and occupational therapy and self-management instructions are essential. Aspirin or another one of the nonsteroidal anti-inflammatory drugs are the cornerstone of therapy. The relatively new cyclooxygenase-2 (COX-2) inhibitors are used widely because of decreased risk of gastrointestinal ulceration. Glucocorticoid therapy may be added, and 7.5 mg of prednisone daily for the first 2 years of active disease substantively reduces progressive joint erosions. Otherwise, corticosteroids are avoided if possible, but low-dose therapy is used by some clinicians, along with salicylates.
Immunosuppressive therapy with azathioprine, cyclophosphamide, or methotrexate is not routinely used during pregnancy. Of these, only azathioprine could be considered for use during pregnancy because the other agents are teratogenic.
If cervical spine involvement exists, particular attention is warranted during pregnancy. Subluxation is common with such involvement, and pregnancy, at least theoretically, predisposes to this because of joint laxity. Intense involvement of certain joints may interfere with delivery; for example, severe hip deformities may preclude vaginal delivery.
SYSTEMIC SCLEROSIS (SCLERODERMA)
The hallmark of this disease is overproduction of normal collagen. This results in fibrosis of skin, blood vessels, and visceral organs (i.e., gastrointestinal tract, heart, lungs, and kidneys). Pulmonary interstitial fibrosis along with vascular changes may cause pulmonary hypertension. There is no effective treatment. Therapy is symptomatic and directed at end-organ involvement. Corticosteroids are helpful only for inflammatory myositis, pericarditis, and hemolytic anemia.
Pregnancy outcome with scleroderma probably is related to the severity of underlying disease. Women with diffuse scleroderma or with hypertension, renal or cardiac involvement, or pulmonary fibrosis do poorly. Women with renal insufficiency and malignant hypertension have an increased incidence of superimposed preeclampsia. In the presence of rapidly worsening renal or cardiac disease, pregnancy termination should be considered.
Vaginal delivery may be anticipated, unless the soft-tissue changes wrought by scleroderma produce dystocia requiring abdominal delivery. Tracheal intubation for general anesthesia has special concerns because these women typically have limited ability to open their mouths. Because of esophageal dysfunction, aspiration is also more likely, and epidural analgesia is preferable. An overview of other uncommon connective tissue disorders is shown in Table 56-2.
TABLE 56-2. Uncommon Connective Tissue Disorders
For further reading in Williams Obstetrics, 23rd ed.,
see Chapter 54, “Connective Tissue Disorders.”