Mortality in systemic sclerosis: An international meta-analysis of individual patient data

Ioannidis, John P. A.; Vlachoyiannopoulos, Panayiotis G.; Haidich, Anna Bettina; Medsger, Thomas A.; Lucas, Mary; Michet, Clement J.; Kuwana, Masataka; Yasuoka, Hidekata; Hoogen, Frank van den; Boome, Liane te; Laar, Jacob M van; Verbeet, Nicolette L.; Matucci‐Cerinic, Marco; Georgountzos, A.; Moutsopoulos, Haralampos Μ.

doi:10.1016/j.amjmed.2004.04.031

Cited by 390 publications

(269 citation statements)

References 31 publications

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“…The reported unadjusted SMRs for RA and SLE ranged from 1.15 to 2.64 (17-28) and from 2.17 to 3.81 (9,13-17), respectively. SSc had the highest SMR, with results ranging from 1.46 to 4.29 (33)(34)(35). The SMR of antineutrophil cytoplasmic antibody-associated vasculitis ranged from 1.60 to 4.80 (36)(37)(38).…”

Section: Life Expectancy Smrs and Causes Of Death In Rheumatic Disementioning

confidence: 98%

Life expectancy, standardized mortality ratios, and causes of death in six rheumatic diseases in Hong Kong, China

Mok¹,

Kwok²,

Ho³

et al. 2011

Arthritis & Rheumatism

193

161

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Objective. To examine the life expectancy, standardized mortality ratios (SMRs), and causes of death in 6 groups of patients from Hong Kong with different rheumatic diseases.Methods 7 years). In male patients, the loss in life expectancy was highest for SV (28.3 years), SLE (27 years), and SSc (16 years). There were 2,486 deaths during the study period (1999)(2000)(2001)(2002)(2003)(2004)(2005)(2006)(2007)(2008), and the principal causes were infections (28%), cardiovascular complications (18%), cancer (16%), and disease activity (7%). Infection was the leading cause of death in SLE, RA, AS, and PsA, whereas deaths from disease-related activity and cardiovascular complications were most frequent in SSc. Cancer was the most common cause of death in SV. Conclusion.Our findings indicate that patients with SLE, RA, AS, PsA, SV, and SSc have increased mortality rates and reduced life expectancy. SLE has the highest adjusted SMR, and female SSc patients have the greatest loss in life expectancy. Infection is the leading cause of death, followed by cardiovascular complications and malignancies.

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Section: Life Expectancy Smrs and Causes Of Death In Rheumatic Disementioning

confidence: 98%

Life expectancy, standardized mortality ratios, and causes of death in six rheumatic diseases in Hong Kong, China

Mok¹,

Kwok²,

Ho³

et al. 2011

Arthritis & Rheumatism

193

161

View full text Add to dashboard Cite

show abstract

“…ILD, which occurs at a frequency of 34%-60% in patients with SSc (Meyer et al 2007;Hunzelmann et al 2008;Hashimoto et al 2012), is currently the primary cause of SSc-related death (Ioannidis et al 2005;Steen and Medsger 2007). However, no agents have been proven to effectively control ILD .…”

Section: Introductionmentioning

confidence: 99%

“…The causes of death in patients with SSc have dramatically changed over the past 30 years (Ioannidis et al 2005;Steen and Medsger 2007). ILD, which occurs at a frequency of 34%-60% in patients with SSc (Meyer et al 2007;Hunzelmann et al 2008;Hashimoto et al 2012), is currently the primary cause of SSc-related death (Ioannidis et al 2005;Steen and Medsger 2007).…”

Section: Introductionmentioning

confidence: 99%

High Prevalence of Acute Exacerbation of Interstitial Lung Disease in Japanese Patients with Systemic Sclerosis

Tomiyama

Watanabe

Ishii

et al. 2016

Tohoku J. Exp. Med.

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Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by extensive fibrosis and autoantibodies. Its clinical manifestations are diverse and include Raynaud's phenomenon, gastrointestinal dysmotility, interstitial lung disease (ILD), pulmonary hypertension, and renal crisis. Among these, ILD is the primary cause of SSc-related death. It has been considered that acute exacerbation of ILD (AE-ILD) is not common in patients with SSc; however, little is known about the prevalence of AE-ILD in Japanese patients with SSc. In this study, we aimed to clarify the prevalence, clinical characteristics, and prognosis of patients with SSc who developed AE-ILD and to identify predictive factors for AE-ILD in our Japanese cohorts. Clinical data of patients who visited our department from 1990 to 2014 and fulfilled the 2013 classification criteria for SSc were retrospectively reviewed. A total of 139 patients were enrolled. The mean age of onset was 49.1 years, and 113 (81.3%) patients were female; 116 (83.5%) had limited cutaneous involvement, and the overall 10-year survival rate was 92.0%. Among 66 (47.5%) patients with ILD, 13 (9.4%) developed AE-ILD. Patients with AE-ILD had a significantly higher incidence of overlap with polymyositis (PM) or dermatomyositis (DM) and lower prevalence of anticentromere antibodies with higher mortality rate compared with those without AE-ILD. Multivariate Cox regression analysis identified that an overlap with PM or DM was the most significant predictive factor for AE-ILD. Our study results suggest that Japanese patients with SSc, particularly patients overlapped with PM or DM, have a high risk of AE-ILD.

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“…Since adverse prognostic factors have been reported to include male gender (Ferri et al 2002), diffuse cutaneous SSc, visceral involvement such as heart (Ferri et al 2002;Ioannidis et al 2002;Scussel-Lonzetti et al 2002), and the presence of anti-Scl-70 antibodies (Ioannidis et al 2002), we considered that our patient would have a very poor prognosis, and was eligible for auto-PBSCT.…”

Section: Discussionmentioning

confidence: 99%

Successful Autologous Peripheral Blood Stem Cell Transplantation Using Thiotepa in a Patient with Systemic Sclerosis and Cardiac Involvement

Komatsuda

Kawabata

Horiuchi

et al. 2006

Tohoku J. Exp. Med.

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A 19-year-old man with systemic sclerosis (SSc) was hospitalized for autologous peripheral blood stem cell transplantation (auto-PBSCT) due to progressive scleroderma and cardiac involvement despite conventional treatment. During the administration of cyclophosphamide (60 mg/kg/day for 2 days) for mobilization and collection of CD34 + selected peripheral blood stem cells, he developed congestive heart failure. Echocardiogram showed hypokinetic asynergy from the septum to posterior wall, which might indicate underlying cardiac damage. We were also concerned about the risk of high-dose cyclophosphamide-induced cardiotoxicity. Since the dose-limiting toxicity of thiotepa, an alkylating agent, is myelosuppression, and cardiac toxicity due to thiotepa is less common, we used a conditioning regimen consisting of thiotepa (10 mg/kg/day, day -5) and lowdose cyclophosphamide (50 mg/kg/day, days -3 and -2), instead of the conventional highdose cyclophosphamide (50 mg/kg/day × 4 days/course). The post-transplant course was uneventful, and the modified Rodnan skin thickness score improved from 32 to 15. The present case report demonstrates that thiotepa can be employed as a conditioning regimen for auto-PBSCT in SSc patients with cardiac involvement in order to reduce cyclophosphamide-induced cardiotoxicity.autologous peripheral blood stem cell transplantation (PBSCT); cardiotoxicity; cyclophosphamide; systemic sclerosis; thiotepa

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Mortality in systemic sclerosis: An international meta-analysis of individual patient data

Cited by 390 publications

References 31 publications

Life expectancy, standardized mortality ratios, and causes of death in six rheumatic diseases in Hong Kong, China

Life expectancy, standardized mortality ratios, and causes of death in six rheumatic diseases in Hong Kong, China

High Prevalence of Acute Exacerbation of Interstitial Lung Disease in Japanese Patients with Systemic Sclerosis

Successful Autologous Peripheral Blood Stem Cell Transplantation Using Thiotepa in a Patient with Systemic Sclerosis and Cardiac Involvement

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