Survival in scleroderma.

Bennett, Robert M.; Bluestone, Rodney; Holt, P J; Bywaters, E. G. L.

doi:10.1136/ard.30.6.581

Cited by 108 publications

(42 citation statements)

References 8 publications

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“…Perhaps not surprisingly, individuals who already present with evidence of internal organ involvement, particularly elevated blood urea levels (6)(7)(8), reduction in lung capacity (4,6,7,(10)(11)(12), and evidence of cardiac involvement (4,7,9,10,14), have increased risks of mortality. In addition, more generalized aspects of the disease, such as an elevated erythrocyte sedimentation rate (ESR) (11)(12)(13) and widespread skin disease (6,7,12,14,15), are also linked to poorer prognosis.…”

mentioning

confidence: 99%

Prediction of five-year survival following presentation with scleroderma: Development of a simple model using three disease factors at first visit

Bryan

Knight

Black

et al. 1999

Arthritis & Rheumatism

172

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Objective. To identify combinations of easily acquired clinical variables, at first presentation with scleroderma, that would predict subsequent mortality. Methods. In this prospective study of all new patients at one major scleroderma center, 280 patients with definite scleroderma (according to the American College of Rheumatology criteria) whose disease onset occurred from 1982 to 1991 and who were followed up to the end of 1996 were identified. Standardized data collection was performed at entry to obtain data on major clinical and laboratory variables. Vital status was determined by linkage to the National Death Index. Results. At 5 years, 55 (26%) of 215 women and 21 (32%) of 65 men had died. Univariate analysis showed that older age, diffuse skin disease, higher skin score, low carbon monoxide diffusing capacity, abnormal elec-trocardiogram findings, proteinuria, hematuria, low he-moglobin level, elevated erythrocyte sedimentation rate (ESR), and presence of antitopoisomerase antibody were all associated with increased mortality. A logistic regression model, validated by Monte Carlo simulation, identified 3 factors, proteinuria, elevated ESR, and low carbon monoxide diffusing capacity, that in combination , had an accuracy of >80% in predicting mortality. The absence of these 3 factors was associated with 93% survival. Conclusion. A simple model has been developed that appears to accurately predict mortality over 5 years in a cohort of patients newly presenting with sclero-derma.

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mentioning

confidence: 99%

Prediction of five-year survival following presentation with scleroderma: Development of a simple model using three disease factors at first visit

Bryan

Knight

Black

et al. 1999

Arthritis & Rheumatism

172

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“…Multiple studies have focused on the survival after the Wrst admission to hospital, Wrst appearance of clinical manifestations, or Wrst skin manifestation of the disease [5][6][7][8][15][16][17][18]. In the present study, we considered any initial systemic manifestation of disease, any manifestation besides Raynaud's phenomenon, any initial pulmonary manifestation and the detection time of honeycombing on HRCT for analysis of survival.…”

Section: Discussionmentioning

confidence: 99%

Survival in progressive systemic sclerosis with pulmonary involvement: a single-center experience in Istanbul, Turkey

zuhur

Zuhur

et al. 2011

Rheumatol Int

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The objective of the current study was to determine survival and factors that affect survival in progressive systemic sclerosis (SSc) with pulmonary involvement. A total of 102 SSc patients with pulmonary involvement, diagnosed between 1994 and 2008, enrolled into the study. Pulmonary involvement was defined based on the presence of interstitial changes on high-resolution CT (HRCT). Demographical, clinical, radiological, and laboratory data of the patients were found from patient records and were used for the evaluation of survival. The mean age of the patients at diagnosis was 50 ± 12 years. In follow-up, 22 (21.5%) patients were deceased. The mean age of the patients at death was 55 ± 12 years. Seventy-eight percent of known causes of mortality were related to pulmonary complications. After any initial systemic manifestation of disease, the mean survival was 447 ± 27 months and 5, 10, 15, and 20 year survival rates were 99, 92, 83, and 72%, respectively. The mean survival after initial pulmonary manifestation was 269 ± 23 months and 5, 10 and 15 year survival rates were 91, 73 and 57%, respectively. Mean survival was 113 ± 5 months, and 5- and 10-year survival rates after the lung involvement detected with HRCT were 85 and 66%, respectively. Pulmonary artery hypertension, disease onset after the age of 40, and honeycombing on HRCT were associated with poor survival. Our data suggest that pulmonary involvement is the most important determinant factor for poor prognosis in patients with SSc. Therefore, echocardiography, pulmonary function tests, and HRCT should be performed in early stages of the disease for early diagnosis of pulmonary artery hypertension and lung involvement before irreversible vascular and interstitial changes exist.

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“…Myocardial involvement in PSS is very fre quent in postmortem studies with an overall incidence of about 80% [1,3,4], The organ system most obviously affected is the skin, but involvement of visceral organs including the esophagus, kidney, lungs and heart occurs frequently [2], The cardiac involvement nega tively affects survival [5,6]. Diastolic left ven tricular disturbances often precede systolic disturbances [12][13][14], Diastolic dysfunction is often clinically silent.…”

Section: Discussionmentioning

confidence: 99%

“…Cardiovascular involvement may have varied manifestations; previous pathologic re- ports [2] show that diffuse myocardial fibrosis and pericardial lesion are frequent. Cardiac involvement in PSS is especially important since it is often closely associated with early death [5,6]. Previous Doppler échocardio graphie studies [4,7,8] have shown that PSS patients have left ventricular hypertrophy probably due to myocardial fibrosis which leads to perturbed diastolic function.…”

Section: Introductionmentioning

confidence: 99%

Doppler Echocardiographic Assessment of Left Ventricular Diastolic Function in Patients with Progressive Systemic Sclerosis

Fujimoto

Kimura²,

Nakajima³

et al. 1993

Cardiology

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To investigate the diastolic function of the left ventricle in patients with progressive systemic sclerosis (PSS), 13 patients with PSS (3 males and 10 females; group PSS) were examined by Doppler echocardiography. As a control 8 age-matched healthy volunteers (3 males and 5 females; group H) were also examined. Pulse-wave transmitral Doppler flow velocity curves were digitized and curves of the first derivatives were obtained. In group PSS, total left ventricular wall thickness and left atrial dimension were increased, the ratio between the left ventricular end-diastolic dimension and the left ventricular wall thickness was decreased, late peak diastolic filling was increased, isovolumic relaxation time was prolonged, compared with group H. The patients with left ventricular wall thickening out of proportion to left ventricular end-diastolic dimension had decreased early peak diastolic filling. We conclude that patients with PSS have deterioration of left ventricular diastolic function, and the diastolic function is severely disturbed in patients with left ventricular wall thickening out of proportion to left ventricular end-diastolic dimensions.

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Survival in scleroderma.

Cited by 108 publications

References 8 publications

Prediction of five-year survival following presentation with scleroderma: Development of a simple model using three disease factors at first visit

Prediction of five-year survival following presentation with scleroderma: Development of a simple model using three disease factors at first visit

Survival in progressive systemic sclerosis with pulmonary involvement: a single-center experience in Istanbul, Turkey

Doppler Echocardiographic Assessment of Left Ventricular Diastolic Function in Patients with Progressive Systemic Sclerosis

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