Systemic sclerosis in pregnancy

Clark, Kristina E N; Etomi, Oseme; Ong, Voon H

doi:10.1177/1753495x19878042

Cited by 13 publications

(7 citation statements)

References 38 publications

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“…As discussed, early stage SSc is the highest risk period for development of SRC and patients are advised to avoid pregnancy during this time [ 68 ]. Pregnancy itself does not increase the risk of SRC [ 69 ].…”

Section: Managementmentioning

confidence: 99%

“…Other serious complications of pregnancy such as pre-eclampsia and HELLP (haemolysis, elevated liver enzymes, low platelet) syndrome can mimic SRC and renal biopsy may be required to differentiate the processes. Renal biopsy carries close to the same risk as the general population early in pregnancy but should be avoided in the third trimester unless it will determine management as the risk of complications increases with gestational age [ 68 ]. An ACEi should be started immediately if SRC suspected [ 68 , 70 ].…”

Section: Managementmentioning

confidence: 99%

“…Renal biopsy carries close to the same risk as the general population early in pregnancy but should be avoided in the third trimester unless it will determine management as the risk of complications increases with gestational age [ 68 ]. An ACEi should be started immediately if SRC suspected [ 68 , 70 ]. Captopril is the preferred choice due to lower risk of foetal renal complications.…”

Section: Managementmentioning

confidence: 99%

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Renal Disease and Systemic Sclerosis: an Update on Scleroderma Renal Crisis

Cole

Ong

Denton

2022

Clinic Rev Allerg Immunol

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Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc) with a mortality of 20% at 6 months. Once the leading cause of mortality in scleroderma (SSc), it remains a serious complication, often necessitating level three care for patients affected. Whilst renal outcomes have significantly improved following the advent of angiotensin-converting enzyme inhibitor (ACEi) therapy, SRC remains a precarious challenge for clinicians, due to lack of preventative measures and the fact that patients can rapidly decline despite best medical management. Large cohort studies spanning decades have allowed clear identification of phenotypes particularly at risk of developing SRC thus allowing enhanced monitoring and early identification in those individuals. Novel urinary biomarkers for renal disease in SSc may offer a new window for early identification of SRC patients and response to treatment. Multiple studies have demonstrated increased activity of complement pathways in SRC with some anecdotal cases exhibiting serological response to treatment with eculizumab where ACEi and therapeutic plasma exchange (TPE) were not successful. Endothelin-1 blockade, a therapeutic strategy in other SSc vasculopathies, has shown potential as a target but clinical trials are yet to show a clear treatment benefit. Clear guidelines for the management of SRC are in place to standardise care and facilitate early collaboration between rheumatology and renal physicians. Outcomes following renal transplant have improved but the mortality of SRC remains high, indicating the need for continued exploration of the mechanisms precipitating and exacerbating SRC in order to develop novel therapies.

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Section: Managementmentioning

confidence: 99%

Section: Managementmentioning

confidence: 99%

Section: Managementmentioning

confidence: 99%

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Renal Disease and Systemic Sclerosis: an Update on Scleroderma Renal Crisis

Cole

Ong

Denton

2022

Clinic Rev Allerg Immunol

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“…The primary concern in pregnancy is presence of PAH, which is the leading cause of maternal mortality. 58 Those considering pregnancy should be evaluated for PAH as part of monitoring and counselled when considering pregnancy. In cases of established PAH, patients should be strongly advised against pregnancy.…”

Section: Systemic Sclerosismentioning

confidence: 99%

Management of Pregnancy in Rheumatic Disease

Maguire

O’Shea

2021

EMJ Rheumatol

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Managing patients with rheumatic diseases (RMD) during pregnancy and the postpartum period can be a challenge for both rheumatologists and obstetricians. While disease activity during the course of pregnancy varies with regard to the presence of underlying conditions, maintenance of remission from conception through to delivery increases the chances of an uncomplicated pregnancy. A period of remission of at least 6 months prior to conception increases the chance of a successful conception while decreasing the risk of flares during pregnancy. For this reason, discussion of pregnancy in females with RMDs should begin prior to conception with risk stratification and pregnancy planning. This allows for the transfer to pregnancy-compatible medications, disease stabilisation, determination of autoantibody status, and evaluation of end-organ damage. During pregnancy, where possible, disease activity should be monitored with scores modified to allow use in pregnancy. Prompt recognition and treatment of active disease is essential to minimise the risk to the pregnancy. Systemic lupus erythematosus and axial spondyloarthropathy can present diagnostic dilemmas due to overlap of symptoms of disease activity and normal pregnancy. Patients with end-organ involvement, such as systemic lupus erythematosus or systemic sclerosis, face a higher risk of adverse pregnancy outcomes and disease progression. Close monitoring of patients with RMD should be done by both obstetrics and rheumatology, with regular communication between specialties. Medications should be reviewed at each stage of pregnancy to ensure compliance with the current American College of Rheumatology (ACR) guidelines and the adequate treatment of RMDs.

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“…Of note, EULAR recommendations currently advise to avoid pregnancy in SSc patients with PAH as this complication is associated with a significant pregnancy risk and a maternal mortality up to one third of cases. 60 Regardless of the concomitant treatment with teratogenic medications, contraceptives should be prescribed in SSc-PAH patients, if not contraindicated.…”

Section: Introductionmentioning

confidence: 99%

The Role of the Multidisciplinary Health Care Team in the Management of Patients with Systemic Sclerosis

Farina¹,

Benanti²,

Luca³

et al. 2022

JMDH

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Systemic sclerosis (SSc) is a rare connective tissue disease characterised by immune dysfunction, vascular damage and fibrosis affecting the skin and multiple internal organs. The clinical spectrum of SSc is wide and its manifestations may lead to severe morbidity and mortality, in addition to a great impact on patients’ quality of life. Due to the multifaceted clinical manifestations of SSc, its management requires a combined expertise of different medical specialists to guarantee an adequate disease control and prevent organ complications. Multi-disciplinary teams (MDT), which are composed by physicians and other specialized health professionals, represent therefore a key element for the comprehensive management of SSc patients. Moreover, MTD can improve communication and patients’ empowerment while the presence of dedicated nurses can help patients to ask questions about their condition. The scope of this narrative review is to analyse the available evidences regarding the role of MDT in the management of SSc patients, and how this holistic approach may improve different disease domains and the overall prognosis. MDT regarding the cardiovascular and lung complication are the more represented in literature, given the great impact in prognosis. Nonetheless, MDT have been shown to be fundamental also in other disease domains as they can intercept early manifestations, thus stratifying patients based on the individual risks in order to personalize patients’ follow-up. MDTs may also minimize the treatment delay, enabling fast-track specialist referral. On the other hand, there are few trials specifically studying MDT in SSc and several authors have highlight the lack of standardization.

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Systemic sclerosis in pregnancy

Cited by 13 publications

References 38 publications

Renal Disease and Systemic Sclerosis: an Update on Scleroderma Renal Crisis

Renal Disease and Systemic Sclerosis: an Update on Scleroderma Renal Crisis

Management of Pregnancy in Rheumatic Disease

The Role of the Multidisciplinary Health Care Team in the Management of Patients with Systemic Sclerosis

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