Lung and gastrointestinal complications are leading causes of death in SCORE, a multi-ethnic Singapore systemic sclerosis cohort

Santosa, Amelia; Tan, Chuen Seng; Teng, Gim Gee; Fong, Warren; Lim, Andrew Kean Seng; Law, Weng Giap; Chan, Gabriel; Sc, Ng; Low, Albert

doi:10.3109/03009742.2016.1153141

Cited by 25 publications

(36 citation statements)

References 34 publications

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“…In CSRG, upper gastrointestinal involvement was associated with mortality but not PAH and ILD, possibly due to less sensitive means of identifying the latter complications. Although mortality was highest in SCORE, there were no independent risk predictors of mortality in this analysis, although overlap syndrome and systolic pulmonary arterial pressure (sPAP) greater than 40 mmHg have been shown previously to predict mortality in this cohort (13). Also in contrast to the other cohorts (38), survival in lcSSc was relatively short in SCORE, especially in incident patients.…”

Section: Discussioncontrasting

confidence: 59%

“…Additional risk factors for mortality included older age at disease onset, high mRSS, low DLCOc, ILD and PAH. Cardiopulmonary complications such as PAH and ILD, were responsible for the majority of deaths (13, 18). Malignancy was more common in the older cohorts.…”

Section: Discussionmentioning

confidence: 99%

“…Systemic Sclerosis Cohort in Singapore (SCORE): SCORE was established in 2008 with the aim of determining disease characteristics and outcomes (13) in a multi-ethnic Asian population comprising Chinese (74%), Malays (13%) and Indians (9%) (14). Patients diagnosed with SSc fulfilling the ACR/EULAR 2013 criteria (8) are recruited from three tertiary rheumatology centres in Singapore.…”

Section: Methodsmentioning

confidence: 99%

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What have multicentre registries across the world taught us about the disease features of systemic sclerosis?

Proudman

Huq

Stevens

et al. 2017

Journal of Scleroderma and Related Disorders

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The aim of this study is to compare the clinical features, mortality and causes of death of systemic sclerosis (SSc) patients in four large multicentre registries. Methods: Patients seen at least once in the Australian Scleroderma Cohort Study (ASCS) (n = 1714), the Canadian Scleroderma Research Group (CSRG) (n = 1628), the European League Against Rheumatism Scleroderma Trials and Research (EUSTAR) Network (n = 13,996) and the Systemic Sclerosis Cohort in Singapore (SCORE) (n = 500) before August 2016 were included. Clinical manifestations and survival in cohorts and disease subtypes were compared. Results: Among 17,838 SSc patients, most were female (86.1%), Caucasian (84.6%) and had the limited cutaneous subtype (lcSSc) (65.0%). The anti-centromere autoantibody was the most prevalent (37.6%). More patients in SCORE had the diffuse subtype (dcSSc) (49.3%) and Scl-70 autoantibody (38.8%) (p<0.001). Patients with dcSSc were more likely to be younger and male (p<0.001) and have shorter disease duration, more calcinosis, tendon friction rubs and synovitis (all p<0.001). Interstitial lung disease (ILD) occurred more frequently in dcSSc but prevalence of pulmonary arterial hypertension (PAH) was similar in both subtypes. More deaths occurred among SCORE patients who had the shortest median survival (p<0.001). The survival of patients with early disease, males and those with dcSSc was shorter than that of patients with prevalent disease, female gender and lcSSc, respectively. SSc-related complications accounted for more than 50% of deaths, with PAH and ILD being the most common. Conclusions: This meta-cohort of SSc patients, the largest reported to date, provides insights into the impact of race and sex on disease manifestations and survival and confirms the early mortality in this disease.

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Section: Discussioncontrasting

confidence: 59%

Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

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What have multicentre registries across the world taught us about the disease features of systemic sclerosis?

Proudman

Huq

Stevens

et al. 2017

Journal of Scleroderma and Related Disorders

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“…Further supporting the strong association between severe GI involvement and mortality across SSc subtypes and geographic areas, a study of a large SSc cohort (N = 349) from Singapore found that the use of parenteral nutrition was also a strong independent predictor of mortality. 18 GI involvement remains one of the leading causes of deaths related to SSc over decades. 7,16 Primary causes of death for SSc patients observed during a 30-year time were evaluated in 2007.…”

Section: Effects Of Gi Involvement On Mortality In Sscmentioning

confidence: 99%

Gastrointestinal involvement in systemic sclerosis: Effects on morbidity and mortality and new therapeutic approaches

Hoffmann‐Vold

Volkmann

2019

Journal of Scleroderma and Related Disorders

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The majority of research studies in systemic sclerosis focus largely on addressing skin and cardiopulmonary manifestations. Fewer studies assess the pathogenesis and treatment of gastrointestinal tract involvement in systemic sclerosis, despite the fact that the majority of patients with systemic sclerosis have gastrointestinal manifestations and these manifestations are a leading cause of death in systemic sclerosis. The present review provides a comprehensive update on morbidity and mortality outcomes related to gastrointestinal involvement in systemic sclerosis. This review also describes conventional and emerging approaches to managing gastrointestinal symptoms in systemic sclerosis. Recent developments in systemic sclerosis–gastrointestinal research efforts have revealed promising treatment targets, including specific auto-antibodies and microbiota alterations. This review will conclude with an overview of future research directions that may improve our understanding of systemic sclerosis–gastrointestinal involvement and ultimately help to alleviate suffering from this devastating dimension of systemic sclerosis.

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“…Gastrointestinal involvement in SSc is associated with significant morbidity, mortality, and impact on health-related quality of life [ 7 ]. In a well-characterised local multiethnic systemic sclerosis cohort Singapore (SCORE) where patients fulfilled the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) or Very Early Diagnosis of Systemic Sclerosis (VEDOSS) criteria, 57% of deaths were attributed to SSc, with pulmonary arterial hypertension (PAH), interstitial lung disease (ILD), and gastrointestinal (GI) complications as the leading causes [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

Superior Mesenteric Artery Syndrome: A Potentially Fatal but Reversible Gastrointestinal Manifestation of Systemic Sclerosis

Chua

Wansaicheong

Lim

et al. 2020

Case Reports in Rheumatology

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Superior mesenteric artery syndrome (SMAS) is a rare gastrointestinal disorder characterised by vascular compression of the third part of the duodenum, in the angle between the superior mesenteric artery (SMA) and the abdominal aorta. It presents as an uncommon cause of upper gastrointestinal obstruction. In patients with systemic sclerosis (SSc), gastrointestinal involvement may result in oesophageal dysmotility, gastroesophageal reflux disease (GERD), gastroparesis, small intestinal bacterial overgrowth (SIBO), chronic intestinal pseudoobstruction (CIPO), and fecal incontinence. Malnutrition may thus result in weight loss and reduced mesenteric and retroperitoneal adipose tissue, decreasing the angle between the SMA and aorta causing SMAS. Enteral or parenteral feeding can potentially reverse SMAS in SSc. We report a case of SMAS in an elderly female with SSc and concurrent gastrointestinal involvement, and discuss the important management considerations and potential adverse outcomes when untreated.

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Lung and gastrointestinal complications are leading causes of death in SCORE, a multi-ethnic Singapore systemic sclerosis cohort

Abstract: PAH, ILD, and GI complications were leading causes of death in this cohort. We identified a high-risk group of patients who would benefit from closer monitoring and early intervention.

Cited by 25 publications

References 34 publications

What have multicentre registries across the world taught us about the disease features of systemic sclerosis?

What have multicentre registries across the world taught us about the disease features of systemic sclerosis?

Gastrointestinal involvement in systemic sclerosis: Effects on morbidity and mortality and new therapeutic approaches

Superior Mesenteric Artery Syndrome: A Potentially Fatal but Reversible Gastrointestinal Manifestation of Systemic Sclerosis

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