A case of successful pregnancy in a woman with systemic sclerosis treated with cyclosporin

M. Basso, M. Ghio, G. Filaci, M. Setti and F. Indiveri

Department of Internal Medicine, Faculty of Medicine, Genoa, Italy.

Correspondence to: M. Ghio, Department of Internal Medicine, University of Genoa, Viale Benedetto XV/6, Genoa 16132, Italy. E-mail: mghio{at}unige.it

SIR, Systemic sclerosis (SSc) is a generalized connective tissue disease characterized by a strong female predilection. In our experience low-dose cyclosporin A (CyA) (1–3 mg/kg per day) provides a therapeutic option for patients with multivisceral involvement [1]. Its use during pregnancy in female transplant recipients has been proved to be safe, but there is little experience in autoimmune patients [2].

We present a case of successful pregnancy in a 25-yr-old woman with SSc who was treated with CyA. She presented to our department with severe Raynaud's phenomenon attacks. Physical examination showed scleroderma skin changes proximal to the metacarpophalangeal joints and to the face. Mild restrictive lung disease associated with reduction of diffusion capacity for carbon monoxide, microvascular abnormalities (evaluated by nailfold videocapillaroscopy), a slight increase in renal vascular resistance and abnormal oesophageal motility were revealed by instrumental evaluation. Haematological parameters and urinalysis showed no significant abnormalities. Anti-topoisomerase 1 antibodies were positive. Anti-Ro (SSA), anti-La (SSB) and anticardiolipin antibodies were not found. Total functional haemolytic complement (CH 50) and C3 levels were decreased, while C4 level was normal. On the basis of these data, SSc was diagnosed and CyA (3 mg/kg per day) was started, together with omeprazole (20 mg/day) and courses of intravenous (i.v.) vasodilators: iloprost (1 ng/kg per min in a 6 h i.v. infusion, for 5 consecutive days, 1 week per month). The patient showed partial remission after 24 months of therapy with CyA. The drug was then tapered until complete suspension, but evidence of active disease after 6 months led to the restoration of immunosuppressive treatment, with improvement in the involved organ and no new disease-specific visceral damage. The patient strongly desired a baby and her disease appeared to be stabilized. Hence, she underwent a detailed gynaecological evaluation; no major contraindication to pregnancy was found and she was allowed to get pregnant, which she did 4 months after a second round of cyclosporin therapy. Prophylactic supplementation with iron and folic acid was administered. During pregnancy no serious complication occurred and the biochemical profile showed no significant pathological alterations. She stayed on immunosuppressive treatment with no dose modification, and no major side-effects occurred. When the patient was 29 yr of age she delivered a girl (2.2 kg) at the 34th week, with Caesarean section. Pregnancy outcome was a preterm birth (according to ACOG definitions) [3]. The baby had no deformities. The mother did not breast-feed. Maternal clinical, biochemical and instrumental follow-up (24th month after pregnancy) showed no significant pathological modifications; the infant grew and developed normally.

The frequency of maternal complications in SSc patients, either in diffuse or in limited form, is not increased compared with healthy controls, except for renal crisis [4]. This risk is lower if pregnancy is planned within 3–5 yr from onset of symptoms [5]. Our patient became pregnant when the duration of disease, considered to have started at the time of the onset of Raynaud's phenomenon, was 8 yr and cutaneous and visceral manifestations were stable. The prevalence of delivery of premature or small infants has been reported to be increased: the underlying pathophysiological mechanisms remain unknown and standard obstetric problems, not strictly related to SSc, cannot be excluded [6].

Our data suggest an encouraging clinical effect for low-dose, long-term CyA treatment in SSc: tolerability and clinical improvement were observed in nine patients consecutively treated for at least 3 yr. Cyclosporin is currently administered to female organ transplant recipients, associated or not with azathioprine and steroids: their pregnancies are at high risk but most outcomes are favourable for mother and baby. Fewer than 100 cases of pregnancy in women with autoimmune diseases treated with CyA have been reported in the literature: most were rheumatoid arthritis patients [6]; there were few SLE patients [7].

To our knowledge this is the first report of successful pregnancy in an SSc patient treated with low-dose CyA.

The authors have declared no conflicts of interest.

References

  1. Filaci G, Cutolo M, Basso M et al. Longterm treatment of patients affected by systemic sclerosis with cyclosporin A. Rheumatology 2001;40:1431–2.[Free Full Text]
  2. Janssen NM, Genta MS. The effects of immunosuppressive and anti-inflammatory medications on fertility, pregnancy, and lactation. Arch Intern Med 2000;160:610–9.[Abstract/Free Full Text]
  3. Standard terminology for reporting of reproductive health statistics in the United States. Washington, DC: American College of Obstetricians and Gynecologists, 1987.
  4. Steen VD. Pregnancy in women with systemic sclerosis. Obstet Gynecol 1999;94:15–20.[Abstract/Free Full Text]
  5. Steen VD. Pregnancy in systemic sclerosis. Rheum Dis Clin North Am 1997;23:133–47.[ISI][Medline]
  6. Ghandour FZ, Knauss TC, Hricik DE. Immunosuppressive drug in pregnancy. Adv Ren Replace Ther 1998;5:31–7.[Medline]
  7. Maeshima E, Yamada Y, Kodama N, Mune M, Yukawa S. Successful pregnancy and delivery in a case of systemic lupus erythematosus treated with immunoadsorption therapy and cyclosporin A. Scand J Rheumatol 1999;28:54–7.[CrossRef][ISI][Medline]
Accepted 18 June 2004





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