Cecily A. Clark-Ganheart1, Julia Timofeev1 and Virginia D. Steen2
(1)
Department of Obstetrics & Gynecology, Division of Maternal-Fetal Medicine, Medstar Washington Hospital Center, Medstar Georgetown University Hospital, Washington, DC, 20010, USA
(2)
Department of Medicine/Rheumatology, Medstar Georgetown University Hospital, 3800 Reservoir Road, PHC 3004, Washington, DC, 20007, USA
Virginia D. Steen
Email: steenv@georgetown.edu
Background
Scleroderma (also known as systemic sclerosis) is an autoimmune disease that is encountered less frequently than other connective tissue disorders. The diagnosis is more common in women vs. men, at a rate of 4:1. The prevalence of scleroderma is approximately 0.025 % with average age of onset between 40 and 50 years of age [1]. Manifestations of the disease can include involvement of the skin, lungs, kidneys, heart, gastrointestinal tract, and musculoskeletal system.
Because many of the initial complaints of scleroderma mimic other illnesses, the diagnosis is often difficult. Skin thickening proximal to the metacarpophalangeal joints (i.e., legs, face, trunk, etc.) is a hallmark of the disease [2]. Limited cutaneous scleroderma, also known as the CREST (Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasias) syndrome represents a subset of individuals with less skin thickening. While numerous antibodies have been described in patients with systemic sclerosis, anti-centromere, anti-topoisomerase I, and anti-RNA polymerase III antibodies are three of the more commonly encountered. These antibodies are associated with significant clinical features. For example, renal crisis occurs more frequently in patients that express anti-RNA polymerase III, and anti-topoisomerase I is associated with more severe interstitial lung disease [3].
Scleroderma is commonly classified into limited cutaneous and diffuse cutaneous types, with greater morbidity and mortality associated with the latter. Patients with skin changes on the trunk or proximal to the elbows and/or knees are said to have diffuse scleroderma. Those with skin changes distal to the elbows and/or knees and without trunk involvement are considered to have limited cutaneous disease. Some patients do not have any skin changes, except on the face, and this is termed limited scleroderma, or scleroderma sine scleroderma [2]. Disease progression and the extent of internal organ involvement are typically related to the extent of skin disease as well as the scleroderma-specific autoantibody. Patients with limited scleroderma typically have less severe organ impairment compared to patients with diffuse disease, although they can have significant gastrointestinal disturbance and pulmonary arterial hypertension. The early identification of organ disease provides the clinician an opportunity to institute treatment prior to the establishment of permanent damage.
The exact etiology of scleroderma is unknown. Multiple studies have demonstrated adverse pregnancy outcomes in women that later developed scleroderma. Fetal cells may remain in the maternal circulation for several years after delivery, and one theory involves a type of chronic graft versus host disease developing due to presence of fetal cells [4]. Increased rates of scleroderma development have been linked to antecedent pregnancies complicated by hypertensive disease and intrauterine growth restriction (IUGR) [4]. This theory, however, does not fully explain why some women develop scleroderma and others do not, along with the appearance of the disease in women who have never been pregnant or in men.
Scleroderma and Fertility
Early literature suggested the possibility of decreased fertility among women with scleroderma [5]. Given the fact that disease onset occurs most often during the ages of 40–50, many women with scleroderma diagnosed during this time period would be expected to have decreased pregnancy rates as a function of age alone. However, in a retrospective study of scleroderma patients compared to rheumatoid arthritis and neighborhood controls, there was no significant difference in the fertility rates in scleroderma when adjusted for patients who were sexually active [6].
Impact of Scleroderma on Pregnancy Outcomes
Miscarriage
Miscarriages in other connective tissue diseases are often a major problem. Early studies suggested that there was also an increased frequency of miscarriage in scleroderma and even in women prior to onset of scleroderma, but more recent studies have shown that miscarriage rates are generally not significantly increased [5, 7]. A prospective study by Steen and colleagues looked at 59 women and 91 pregnancies. Patients were divided by subset of disease and by stage of their illness. Miscarriage occurred with similar frequency to the historical controls except in the subgroup of patients with late diffuse scleroderma. These patients had a surprisingly high frequency of miscarriages, 42 % of the 15 women with late diffuse disease compared to 13 % in all of the other groups. Renal insufficiency and severe gastrointestinal malabsorption were present in two of the seven women who had miscarriages and several others had some interstitial lung disease [8]. Women with late, diffuse scleroderma were three times more likely to experience miscarriage compared to their peers [8]. Recently, an observational Italian study compared ninety-nine women with systemic sclerosis (SSc) receiving high risk pregnancy management, to the general Italian obstetric population and did not find an increase in miscarriage in these patients [9]. The series included 109 pregnancies resulting in 101 newborns. Miscarriages (<10 weeks) occurred in 4 % of women, fetal deaths (>10 weeks) in 2 %, and voluntary and therapeutic abortions in 4 % which was not different than the general Italian population. In all, the rate of spontaneous losses in SSc women regularly followed as high risk pregnancy seems comparable to that expected in the general population.
Intrauterine Growth Restriction
IUGR is classically defined as a fetus who fails to reach their maximum growth potential in utero, with estimated fetal weight less than the tenth percentile. Etiologies of IUGR can result from either maternal or fetal factors. Several retrospective studies suggest that systemic sclerosis confers an increased risk of delivering a growth-restricted fetus. Based on available data, women with a diagnosis of scleroderma have a 3.7-fold increase of IUGR compared to women without the disease [10].
Though small in number, various studies have examined placental pathology in women with systemic sclerosis [11]. Findings of fibrosis and abnormal vascular remodeling may predispose women with systemic sclerosis to having small for gestational age infants.
In a recent prospective study that examined pregnancy outcomes in 59 women with scleroderma, none of the pregnancies were complicated by IUGR. Thirty-three patients had diffuse disease, while 26 patients had limited scleroderma [8]. These findings raise the question on the degree of impact that systemic sclerosis has on fetal growth. However, in an Italian study which reviewed data on 109 pregnancies in women with scleroderma, the rate of IUGR was 6 vs. 1 % in controls [9]. Given this, it is reasonable to perform serial growth ultrasounds in women with scleroderma.
Preterm Delivery
Preterm delivery occurs at a greater frequency in women affected by systemic sclerosis. Both retrospective and prospective studies have demonstrated an increase in the number of women delivering prematurely compared with controls. Patients with early diffuse scleroderma are a subset that has a particularly high rate of preterm birth. In a series of 91 pregnancies in 59 women, 65 % of pregnancies in women with early diffuse disease delivered prior to 37 weeks [12]. The overall rate of preterm delivery for women with systemic sclerosis in this series was 29 %, which is a 5.8-fold risk compared to controls. The average gestational age at birth was 34.9 weeks, with the majority of neonates having positive outcomes.
A retrospective study by Taraborelli et al. also reported an increased incidence of preterm delivery compared with controls (25 vs. 12 %) [9]. Interestingly, in this study 72 % of the preterm deliveries were iatrogenic which suggests that the obstetrician made the decision to deliver the baby early and therefore the reasons of prematurity might be related more to other pregnancy complications than to scleroderma itself. However, given that the indications for preterm delivery are not always clear, regardless of etiology, the patients with scleroderma should be aware of the increased risk of preterm birth.
Fetal Death
Infant deaths were quite common in the individual case reports and many cases were associated with the acute exacerbation of scleroderma complications in the mother, particularly renal crisis. Neonatal deaths are occasionally noted in the series and case–control studies, but none found a statistically or clinically excessive number compared with controls [6].
Pregnancy Complications
Renal Crisis
Ten to twenty percent of women with diffuse systemic sclerosis will experience a renal crisis at some point in their disease course [12]. Although early literature described many cases of renal crisis during pregnancy, cohort studies were unable to confirm this [6]. It is unlikely that pregnancy increases the risk of developing renal crisis; however, literature on this specific subject is lacking. The risk is greatest in those with recent disease onset or diffuse systemic sclerosis; therefore, it is important that women in these categories have stabilization of their condition prior to conception [13]. This life-threatening condition is characterized by malignant hypertension, acute renal failure, proteinuria, and microangiopathic hemolytic anemia, and “onion skin” appearance of the renal arteries on histology [14]. Angiotensin-converting-enzyme (ACE)-inhibitors are the cornerstone of treatment in renal crisis during the nonpregnant state. Unfortunately, treatment with ACE-inhibitors is generally avoided in pregnancy due to potential adverse fetal effects. Early data suggested a possible link between fetal malformations and first trimester exposure to ACE-inhibitors; however, recent studies seem to dispel these findings [14].
However, during the second and third trimesters, this class of medications has been associated with decreased fetal blood volume, oligohydramnios, renal failure, IUGR, and hypocalvaria [14, 15]. More importantly, some of the in utero effects are not completely reversed after drug discontinuation. A systematic review of the literature by Bullo et al. provided postnatal follow-up of children with prenatal exposure to ACE-inhibitors and angiotensin receptor blockers (ARBs). Twenty-two children were exposed during the second and third trimesters or for the duration of the entire pregnancy. Of these children, ten had no long-term sequelae, ten had mild impairment (i.e., mild renal insufficiency, arterial hypertension, proteinuria, or developmental delay), while two children had end-stage renal disease, requiring dialysis or transplantation [15]. Due to these findings, ACE-inhibitors and ARBs are generally not used during the second and third trimesters of pregnancy.
Although typically contraindicated during pregnancy, treatment of renal crisis with ACE-inhibitors may prove lifesaving to the mother despite the risks to the fetus. Prior to the initiation of ACE-inhibitor therapy for the treatment of renal crisis, the overwhelming majority of patients with disease died within the first year [12]. Therefore, ACE-inhibitors are indicated for the management of renal crisis given the substantial mortality risk if treatment is withheld.
Another complexity of renal crisis in pregnancy is differentiating it from preeclampsia which has a similar presentation, thus possibly delaying the diagnosis and treatment. While delivery is curative for preeclampsia, it will not address the pathology underlying renal crisis in scleroderma. Preeclampsia and renal crisis share several features, which makes differentiating between the two pathologies difficult. Renal crisis is most likely to develop in women with early, rapidly progressive, diffuse scleroderma [13]. Again, women with this disease subtype are strongly advised to delay pregnancy until stabilization of their disease.
Baseline antibody profiles in women with systemic sclerosis may assist clinicians in making the diagnosis of renal crisis. In a study by Nikpour et al., patients presenting with renal crisis had a significantly increased prevalence of anti-RNA polymerase III compared to patients with other antibody profiles. In patients with anti-RNA polymerase III antibodies, 24.6 % developed renal crisis compared to only 1.8 % in individuals in which anti-RNA antibodies were absent [16]. Diffuse scleroderma patients with anti-topoisomerase (anti-Scl 70) antibody are also at significant increased risk for renal crisis but patients with anti-centromere antibody rarely if ever get this complication. Antibody titers do not correlate with the likelihood of disease development, nor the severity of renal crisis, therefore the presence of anti-RNA antibodies even in low titers should alert the clinician to the possibility of renal crisis [16].
Both complications are associated with severe hypertension and early onset of renal insufficiency. Even just a 50 % change in serum creatinine from baseline should raise major concern for renal crisis. The serum creatinine may increase rapidly if blood pressure is not controlled promptly. Finally, measurement of liver transaminase can also assist in making a diagnosis. Elevation of aspartate aminotransferase (AST) and alanine aminotransferase (ALT) is more characteristic of preeclampsia, though it is important to note that normal liver function tests do not rule out the disease. A 24 h urine protein in the beginning of pregnancy will help to establish baseline renal function and may aid the clinician in differentiating between new onset and preexisting proteinuria later in pregnancy, although increased proteinuria prior to renal crisis is not common. Serum renin levels are usually markedly elevated during renal crisis and are typically within the normal or low range in preeclampsia although these results often take several weeks and clinically are not very useful [13].
Although distinguishing renal crisis from preeclampsia is difficult, ACE-inhibitor therapy should be instituted immediately as this could prove to be lifesaving. A reasonable approach in women with scleroderma presenting with new onset malignant hypertension and proteinuria would be an immediate trial of ACE-inhibitors, while administering steroids for fetal lung maturity at gestational ages less than 34 weeks, and magnesium sulfate for seizure prophylaxis. Failure to control blood pressure within 36–48 h of initiation of ACE-inhibitors should alert the clinician to the possibility of severe preeclampsia and delivery. ACE-inhibitor therapy should continue postpartum, until it is determined that the etiology of hypertension was not due to renal crisis [13, 17].
Impact of Pregnancy on Scleroderma
Concern of the impact of pregnancy on the disease is a common question among clinicians and patients. Ten-year survival appears equivalent in women with a previous pregnancy compared to those who are nulliparous [13]. In a retrospective study of 99 pregnant women with scleroderma, the authors found no evidence of significant disease progression during pregnancy. A prospective study of pregnant women with scleroderma demonstrated that the majority of women reported no change in symptoms throughout the gestation. In the 16 pregnancies that did indicate worsening of symptoms, the majority of complaints were related to esophageal reflux [8]. However, gastroesophageal reflux disease (GERD) is common among gravidas in the general population, and it is likely that this finding has more to do with the physiologic changes of pregnancy than from the worsening of scleroderma itself. Women with scleroderma do not seem to have greater adverse outcomes from cardiopulmonary problems than one would expect from other pregnant patient with similar cardiopulmonary disease. All are at high risk. Other than increased shortness of breath (typical in most pregnant women due to increased basal oxygen consumption and alveolar ventilation), there is no evidence that pregnancy increases the severity of scleroderma pulmonary fibrosis. Scleroderma patients may have undetected myocardial damage and this could in theory cause cardiac dysfunction and compromise during the cardiovascular stress of pregnancy. Pregnancy in patients with pulmonary arterial hypertension (PAH) is strongly discouraged. Any woman with PAH who becomes pregnant is at extremely high risk for severe hemodynamic complications during pregnancy and especially in the peripartum state. Reports estimate a 36–50 % maternal death rate in women with PAH, with the most vulnerable period occurring with the delivery and the first 2 weeks postpartum [18].
If improvements in scleroderma symptoms are noted during pregnancy, it is most likely with respect to Raynaud’s phenomenon and digital ulcers. It is thought that the natural decrease in peripheral vascular resistance is responsible for this improvement. Unfortunately, this improvement is lost postpartum, as Raynaud’s phenomenon is the most common symptom noted in postpartum women experiencing aggravation of their condition [8]. Overall, current published literature indicates that pregnancy has little impact on systemic sclerosis or disease progression.
Management
Preconception and Antepartum Management
With advances in the care of women with rheumatologic disease, providers are more likely to encounter pregnant women with autoimmune conditions. Though preconception counseling is underutilized, understanding of the unique considerations brought to pregnancy by scleroderma is the key to successful management (Table 8.1).
Table 8.1
Recommended evaluations for women with scleroderma prior to pregnancy
|
Manifestation |
Study |
|
Overall |
Disease duration, disease subtype, autoantibodies, evaluate medications |
|
Cardiovascular diseaseb |
CBC, LFTs, echocardiogram, electrocardiography, blood pressure |
|
Pulmonary diseaseb |
Pulmonary function tests, chest X-ray or high resolution CT scan to evaluate for fibrosisa |
|
Renal diseaseb |
Blood pressure, urinalysis, BUN/creatinine, electrolytes, 24 h UTP |
|
Miscellaneous |
Evaluation of esophageal disease, assessment of airway, range of neck motion, mucosal telangiectasia, examination of extremities including pulses, sites for IV access, and gangrene of digits |
LFT liver function tests, UTP urinary total protein, BUN blood urea nitrogen, 24 h UTP 24 h urine protein
aPerform prior to pregnancy unless patient is already pregnant, then perform as clinically indicated
bAppropriate for preconception evaluation and/or at first prenatal visit to establish baseline risk
Successful pregnancy outcomes are possible for women with systemic sclerosis, however, prior to becoming pregnant consultations with Maternal-Fetal Medicine and Rheumatology specialists are highly recommended. Women with diffuse systemic sclerosis or disease duration of less than 4 years are more likely to experience adverse neonatal outcomes. This is likely due to the fact that women with these characteristics are more likely to experience active disease. In this group of women, pregnancy can be attempted after stabilization of disease. Patients should be aware of the increased risk of preterm delivery and IUGR, and possible long-term neonatal risks if treatment with an ACE-inhibitor is indicated given the potential of renal crisis occurring during this time period.
An antibody profile of women desiring pregnancy should be obtained. This includes an investigation for the presence of anti-topoisomerase (scl 70), anti-RNA polymerase III, anti-centromere, anti-Ro/SSA and anti-La/SSB antibody titers [13]. Women with anti-topoisomerase are more likely to have severe pulmonary fibrosis and those with anti-RNA polymerase III are more likely to get renal crisis. Both groups experience a more aggressive and active disease course. Additionally, the presence of anti-Ro and anti-La, although more common in systemic lupus erythematosus, is associated with the development of congenital heart block and therefore the presence of these antibodies should alert the clinician to this complication (detailed in Chap. 4).
The degree of organ involvement should also be assessed, ideally prior to conception. Depending on the degree of underlying organ dysfunction, pregnancy may be contraindicated. Patients with PAH are strongly discouraged from becoming pregnant because of the high mortality for these mothers. Also, it is a widely held consensus that women with cardiac ejection fraction less than 40 % should not become pregnant. In fact, pregnancy termination is recommended due to the one in five risk of maternal mortality during or immediately postpartum [19]. Likewise, the degree of renal impairment should be evaluated prior to conception. Women with serum creatinine above 1.4 gm/dL are at increased risk for pregnancy complications and renal deterioration irrespective of their scleroderma; for example, there is a 40 % risk of IUGR and preeclampsia, 5 % risk of perinatal death, and a 20 % risk of further deterioration of renal function persisting postpartum, with up to 2 % risk of end-stage renal disease within 1 year [20].
With respect to the restrictive lung disease, limited datum exists of the impact on pregnancy. A series of nine patients with restrictive lung disease resulted in favorable neonatal outcomes. Five mothers developed exercise-induced desaturation with four patients requiring supplemental oxygen. One patient was delivered at 31 weeks via general anesthesia and subsequently required mechanical ventilation for 3 days [21]. Consultation with pulmonology is recommended to assess the decrease of lung involvement prior to and during pregnancy. The clinician should keep in mind that underlying restrictive lung disease may present without alveolitis and therefore pulmonary function tests are recommended in the first trimester and should be repeated as clinically indicated thereafter [13].
Review of medications prior to pregnancy is extremely important since immunosuppressive medications such as methotrexate and mycophenolate are frequently used for treatment of skin, muscle, joint and lung disease in early scleroderma. These medications must be discontinued before conception. Azathioprine and plaquenil, which have been used throughout pregnancy in lupus patients, have not been particularly useful for management of skin or lung problems in scleroderma although they may be helpful in maintenance of prior improvement of skin, joint, muscle, or lung problems (medication safety in pregnancy is detailed in Chap. 14).
Women who have experienced a prior renal crisis require ACE-inhibitors as part of the management of the disease. A trial off ACE-inhibitors can be considered prior to pregnancy to see whether there is even the potential of managing the blood pressure without ACE-inhibitors. However, prompt resumption of therapy must occur if even the slightest blood pressure elevation occurs. Discontinuation of this class of medicine could prove fatal; therefore, the patient must have clear understanding of the potential fetal risks associated with ACE-inhibitor use and the implications on her health if the medication is discontinued. It appears that captopril may have the most favorable side effect profile regarding fetal complications of the available ACE-inhibitors. The use of enalapril was associated with a higher percentage of fetal complications than was noted with use of captopril. This is potentially related to the shorter half-life of captopril [15]. Therefore, in women with a prior renal crisis who require the use of ACE-inhibitors to maintain their health, consideration can be given to using captopril during the pregnancy.
Because ACE-inhibitor use in the second and third trimesters is associated with fetal complications, it would be reasonable to increase fetal surveillance during this time. Serial growth sonograms beginning in the second trimester are indicated given the association with growth restriction. Modified biophysical profile (non-stress test combined with measurement of the amniotic fluid index) could be instituted in the third trimester given the risk of oligohydramnios. Testing should be initiated earlier in pregnancy or at an increased frequency depending on the clinical scenario and at the provider’s discretion.
Intrapartum Management
Women with systemic sclerosis require special considerations during the intrapartum course (Table 8.2). Consultation with an anesthesiologist should be considered prior to the labor process. Scleroderma presents several unique considerations to both the anesthesiologist and obstetrician. Systemic sclerosis in general is not a contraindication for vaginal delivery. Contractures may provide limitations in range of motion, which the clinician should take into consideration. The presence of severe pulmonary or cardiac disease may necessitate an assisted second stage of labor (with forceps or vacuum delivery) or cesarean delivery depending on the degree of compromise.
Table 8.2
Intrapartum considerations for patients with scleroderma
|
Specialty |
Consideration |
|
Obstetrics |
Route of delivery |
|
– Lower extremity range of motion |
|
|
Degree of pulmonary disease |
|
|
– Assisted second stage for severe disease |
|
|
Degree of cardiac disease |
|
|
– Assisted second stage for severe disease |
|
|
Anesthesia |
Airway |
|
– Mandibular range of motion |
|
|
Ventilation |
|
|
– Pulmonary fibrosis and/or restrictive lung disease |
|
|
Regional anesthesia |
|
|
– Skin thickening |
|
|
– Vasoconstriction |
|
|
– Hypotension |
|
|
– Prolonged sensory blockade |
|
|
– Cardiac disease |
|
|
Nursing |
Positioning |
|
– Contractures |
|
|
Temperature |
|
|
– Raynaud’s phenomena/vasoconstriction in cold environment |
|
|
Skin thickening |
|
|
– IV access and blood draws |
Furthermore, features of the disease such as vasoconstriction, anatomic deformities of the airway, and skin changes may present additional challenges for the anesthesiologist. Prolonged sensory blockade has been reported with the use of regional anesthesia [22]. Spinal anesthesia can be associated with profound hypotension that is refractory to treatment with vasopressors. Even more challenging, vasodilatation below the level of the spinal block may not respond to vasopressors, thus predisposing to worsening of the patient’s upper extremity vasoconstriction. Aggressive fluid management may be required to address hypotension; therefore, epidural anesthesia may be a better choice in patients with scleroderma if feasible [22].
Anatomic deformities of the upper airway including fibrosis of the temporomandibular joint, poor mouth opening, skin tightening of the face with reduced mobility, etc. can result in difficult intubation for patients with scleroderma. Likewise, esophageal dysmotility in addition to the intrinsic relaxation of the lower esophageal sphincter during pregnancy may increase the risk of aspiration. Ventilation may also prove difficult in the setting of underlying restrictive lung disease, which is not always apparent prior to conception or during the antepartum period. In general, if a cesarean section is indicated, it would be best to avoid general anesthesia.
Contraceptive Options
Given that up to 50 % of pregnancies are unplanned, an understanding of contraceptive options for women with scleroderma, particularly for women with very early diffuse scleroderma and those with concomitant use of teratogenic medications, is key. Literature specifically addressing contraception in patients with scleroderma is lacking, but there is nothing to suggest that hormones are a problem in scleroderma. Thus, the principles of contraception management in women with other autoimmune diseases may be applied to patients with systemic sclerosis. It is important to note that women with rheumatologic conditions have contraceptive options, and contraception should not be withheld solely based on the presence of autoimmune disease (contraception in rheumatic disease patients is reviewed in detail in Chap. 11).
Barrier methods, intrauterine devices, and progestin-only methods are options for women with scleroderma. As in women without rheumatologic disease, the etonogestrel single-rod implant and intrauterine devices have the lowest rate of unintended pregnancy. Rates of unintended pregnancy per 100 women during the first year of typical use for the implant, levonorgestrel intrauterine system, and the Copper T intrauterine devices are 0.05, 0.2, and 0.8, respectively [23]. Devices containing progestin do not appear to alter immunogenicity in patients with rheumatologic disease nor do they increase the risk of thrombosis [24]. Medroxyprogesterone acetate is another option for women seeking a reliable form of contraception with an unintended pregnancy rate of 3 % during the first year with typical use.
The use of barrier methods should be encouraged for the prevention of sexually transmitted diseases and as a contraceptive adjunct to the methods listed above. Patients should be aware that natural skin condoms do not prevent disease transmission. Rates of unintended pregnancy are greater with the use of male or female condoms compared to the other contraceptive options. Thus, in patients where avoidance of pregnancy is preferred, use of a more reliable form of contraception should be advocated (for example, intrauterine device). Additionally, if childbearing is complete, consideration of a permanent sterilization procedure is reasonable.
Summary
Despite the fact that early literature suggested an increased risk of infertility and poor pregnancy complications, positive pregnancy and neonatal outcomes in patients with scleroderma are generally the rule and not the exception. Most of the adverse pregnancy outcomes occur in women with early, diffuse systemic sclerosis. In this group, childbearing should be deferred until disease stabilization. The most commonly reported complications in pregnancies with systemic sclerosis are prematurity and IUGR. In the absence of other risk factors, the rates of intrauterine fetal demise are not increased [12]. Ideally, patients should undergo preconception counseling to determine if multi-organ involvement precludes pregnancy.
A multidisciplinary approach including consultation with Maternal-Fetal Medicine, Rheumatology, and Anesthesiology are important to classify the patient’s disease and provide recommendations for antepartum, intrapartum, and postpartum management. While successful pregnancies have been reported in women with a history of renal crisis, generally pregnancy is not advised in these patients. For women with renal crisis, a trial off ACE-inhibitors can be considered; however, prompt resumption of therapy must occur if even the slightest blood pressure elevation occurs. If renal crisis is suspected during pregnancy, immediate treatment with ACE-inhibitors is indicated. With appropriate preconception counseling and management with a multidisciplinary team, women with systemic sclerosis can achieve successful pregnancy outcomes.
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