For clinicians

Systemic sclerosis (scleroderma)

systemic sclerosis, scleroderma, SSc

Looking for the plain-language version? See the patient version of this page.

Bottom lineBottom line: pregnancy is feasible in many with SSc but requires pre-conception cardiopulmonary and renal assessment. Pulmonary arterial hypertension is a relative contraindication with high maternal mortality; scleroderma renal crisis is a major concern, complicated by the teratogenicity of ACE inhibitors that treat it. Higher rates of preterm birth and IUGR. Manage in a specialist multidisciplinary setting.

By domain

The pre-pregnancy check ●●●○moderate evidencestrong

Pre-conception evaluation: echocardiography for pulmonary hypertension, pulmonary function, and renal assessment; test anti-Ro/SSA and anti-La/SSB once (ACR 2020, verified). Taraborelli 2012 (verified): disease was usually stable through pregnancy, with progression in only 4/99 women within a year of delivery, all anti-Scl-70 positive and mostly recent-onset; the authors advise high-risk multidisciplinary care, folic acid from pre-conception, counselling against pregnancy with severe organ damage, and postponing pregnancy in recent-onset disease, particularly anti-Scl-70-positive.[1, 3, 5]

Lung artery pressure (pulmonary hypertension) ●●○○low evidencestrong

Significant pulmonary arterial hypertension is a recognised high-risk situation in pregnancy and falls under the severe organ involvement for which pregnancy is generally advised against; screen with echocardiography pre-conception. Note the Taraborelli cohort excluded severe organ disease and had no baseline PAH, so it does not quantify PAH-specific maternal risk; a dedicated PAH-in-pregnancy source is needed for specific mortality figures.[5]

Kidneys (scleroderma renal crisis) ●●○○low evidencestrong

Scleroderma renal crisis is a key risk. ACR 2020 STRONGLY recommends treating scleroderma renal crisis in pregnancy with an ACE inhibitor or ARB (verified, primary text), because the risk of maternal or fetal death from untreated crisis exceeds the medication risk. ACE inhibitors/ARBs are otherwise avoided electively in the 2nd/3rd trimester (oligohydramnios, fetal renal damage), so this exception is specific to active renal crisis. Monitor blood pressure and renal function; co-manage with nephrology and obstetric medicine. BSR 2023 (part 2) corroborates this, noting ACE inhibitors reduced scleroderma renal crisis mortality from up to 50% (1970s) to up to 20% at 6 months, and may be indicated in pregnancy in exceptional circumstances.[1, 4]

Pregnancy outcomes ●●●○moderate evidenceconditional

Taraborelli 2012 (IMPRESS, 99 women/109 pregnancies, verified): live births 90%. Versus the general obstetric population, SSc pregnancies had more preterm delivery <37 weeks (25% vs 12%; OR 2.53, 95% CI 1.55-4.10), severe preterm <34 weeks (10% vs 5%), IUGR (6% vs 1%; OR 4.36, 1.65-10.85), very-low birth weight (5% vs 1%; OR 4.88, 1.66-13.24), and caesarean (52% vs 31%). Corticosteroid use was independently associated with preterm delivery (OR 3.63); folic acid was protective (OR 0.30). Raynaud's improved in the second trimester in 32%, lost after delivery.[5]

Medicines ●●●○moderate evidenceconditional

Review medications pre-conception: avoid mycophenolate, methotrexate, and cyclophosphamide. ACE inhibitors are avoided electively in pregnancy but ACR 2020 strongly recommends their use (or an ARB) if scleroderma renal crisis develops. Individualise disease-modifying therapy with compatible agents.[1, 2, 3]

Key points

PointEvidence
Pre-conception cardiopulmonary and renal assessment is mandatory to stratify risk.[]●●●○moderate evidencestrong
Significant PAH falls under the severe organ involvement for which pregnancy is generally advised against (Taraborelli 2012 and guideline consensus).[5]●●●○moderate evidencestrong

Key numbers

References

  1. Sammaritano LR, et al. 2020 ACR Guideline for the Management of Reproductive Health in RMD. Arthritis Rheumatol. 2020;72(3):529-556. https://acrjournals.onlinelibrary.wiley.com/doi/10.1002/art.41191
  2. Ruegg L, et al. EULAR recommendations for antirheumatic drugs in reproduction, pregnancy, and lactation: 2024 update. Ann Rheum Dis. 2025;84(6):910-926. https://ard.eular.org/article/S0003-4967(25)00818-0/fulltext
  3. Report of the 2020 ACR reproductive health guideline recommendations, including use of ACE inhibitors or ARBs for scleroderma renal crisis in pregnancy and anti-Ro/La testing. https://www.medscape.com/viewarticle/926291
  4. Schreiber K, Frishman M, Russell MD, et al. British Society for Rheumatology guideline on prescribing drugs in pregnancy and breastfeeding: comorbidity medications used in rheumatology practice. Rheumatology (Oxford). 2023;62(4):e89-e104. https://doi.org/10.1093/rheumatology/keac552
  5. Taraborelli M, Ramoni V, Brucato A, et al. Brief report: successful pregnancies but a higher risk of preterm births in patients with systemic sclerosis: an Italian multicenter study. Arthritis Rheum. 2012;64(6):1970-1977. https://doi.org/10.1002/art.34350