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Objective:To examine the association between baseline serum albumin level and short-term, long-term outcomes in patients with systemic sclerosis associated interstitial lung disease (SSc-ILD).Methods:A total of 259 patients with SSc-ILD who were hospitalized in West China Hospital of Sichuan University from January 2008 to December 2018 were enrolled. The data of serum albumin, demographic characteristics, peripheral blood hemoglobin at admission, and treatment plan were obtained from the hospital information management system, and the survival of the patients were followed up to June 1, 2019. The mean baseline serum albumin of 259 patients was 37.67 g/L, there were 118 patients with serum albumin ≤37.67 g/L (low protein group) and 141 patients with serum albumin>37.67 g/L (high protein group).Results:There were 64 males and 195 females with a mean age of 50.0(41.0, 61.0) years. The follow-up time was 627(61, 1 426) days. Compared to high protein group, the low protein group had higher proportion of male patients [30.5%(36/118) and 19.9%(28/141),χ 2=3.92, P=0.048], and higher levels of the erythrocyte sedimentation rate [45.0(27.0,69.0) vs. 29.0 (19.0,46.0)mm/1 h,χ 2=4.07, P<0.001], neutrophil percentage [71.50(63.35,77.13) vs. 65.60(59.50,72.50)%,χ 2=3.65, P<0.001], platelet [196(140,273) vs. 172(126,240)×10 9/L,χ 2=1.99, P=0.046], nutrophil/lymphocyte ratio [33.85(2.53,5.28) vs. 2.61(1.97,3.83),χ 2=4.57, P<0.001], platelet/lymphocyte ratio [149.0(112.0,216.8) vs. 113.5(72.76,158.8),χ 2=4.98, P<0.001], aspartate aminotransferase [27.0(21.0,39.0) vs. 23.0 (19.5,30.0) IU/L,χ 2=2.93, P=0.003], globulin [31.20(26.90,36.83) vs. 29.50(25.65,32.80) g/L,χ 2=2.28, P=0.023], serum cystatin C[1.14(0.98,1.33) vs. 1.02(0.88,1.16) mg/L,χ 2=3.80, P<0.001], IgA[2 710 (1 965,3 505) vs. 2 460 (1 862,3 105) mg/L,χ 2=2.13, P=0.033], IgG[15.05(12.83,21.08) vs. 13.60(11.53,17.23)g/L,χ 2=3.24, P=0.001], IgE[60.44(24.92,197.99) vs. 34.82(14.72,85.04) kIU/L,χ 2=3.33, P=0.001] and circulating immune complex [0.13(0.08,0.19) vs. 0.10(0.08,0.13)O.D,χ 2=2.60, P=0.009]; and lower levels of hemoglobin [121.5(101.8,132.0) vs. 129.0(119.0,142.0) g/L,χ 2=5.05, P<0.001], albumin [(33.28±3.49) vs.(41.34±2.95) g/L,χ 2=20.17, P<0.001] and IgM[1 320 (932,1 745) vs.1 560(1 170,2 030) mg/L, χ 2=2.63, P=0.009]. The utilization rate of antibiotics was higher in the low protein group (60 vs. 43 cases, χ 2=11.10, P=0.001). The number of patients followed up to 1, 5, and 10 years were 248, 245, and 244, respectively. The 1-year, 5-year, and 10-year cumulative survival rates of patients in low protein group and high protein group were(91.0% vs. 98.4%, χ 2=6.23, P=0.013;87.0% vs. 97.1%, χ 2=6.15, P=0.013; 81.6% vs. 97.1%, χ 2=7.00, P=0.008) respectively. Conclusions:Patients with scleroderma-associated interstitial lung disease have an increased risk of poorer prognosis when serum albumin ≤37.67 g/L.
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Objective:To explore the effect and mechanism of different concentrations of metformin on bleomycin (BLM)-induced systemic sclerosis (SSc) mice model.Methods:C57BL/6 mice were divided into the normal group, the model group, the high, the medium and the low metformin (MET) treatment groups randomly. All mice were sacrificed after BLM and metformin treatment for 4 weeks. Local skin was exminedby histopathological staining method to measure the thickness of dermis and collagen, and immunohistochemistry and real-time fluorescence quantitative polymerase chain reaction (RT-qPCR) were used to detect the protein and mRNA levels of Interleukin (IL)-17, forkhead box P3 (Foxp3) and α-smooth muscle actin (α-SMA). Flow cytometry was used to detect the percentage of effector T cell (Teff) and regulatory cells (Treg) in splenic mononuclear cells. The data such as dermal collagen thickness, α-SMA, IL-17, Foxp3, Teff and Treg levels were statistically analyzed by one-way analysis of variance. The data such as dermal collagen thickness, α-SMA, IL-17, Foxp3, Teff and Treg levels were analyzed by one-way analysis of variance, and least significant difference (LSD)- t or Kruskal-Wallis test was used for comparison between groups. Results:Compared with the normal group, remarkable fibrotic lesions appeared in the skin of mice in the model group, and the levels of T-helper cells (Th)1, Th2, Th17, and T follicular helper cells (Tfh) cells were increased, accompanied by a significant decrease in the level of Treg cells. After high-dose metformin treatment, the dermal thickness [(131±25) μm], collagen thickness [(119±18) μm], and α-SMA [(3.0±0.5)/HPH] were significantly reduced( F=14.390, P<0.01; F=40.245, P<0.01; F=44.626, P<0.01). Th1[(27.00±6.68)%], Th17[(0.56±0.20)%], Tfh[(6.4±1.6)%] cells ware significantlyreduced ( F=32.390, P<0.01; F=16.083, P<0.01; F=16.546, P<0.01), and Treg[(11.23±1.52)%] cells were significantly increased ( F=10.171, P<0.01). Conclusion:Metformin can effectively reverse the local skin changesin BLM-induced SSc mouse model, and show immune regulation and anti-fibrosis effects by restoring the Teff/Treg balance.
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INTRODUCTION: Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by progressive fibrosis of the skin and internal organs that promotes high morbidity and mortality. OBJECTIVE: To evaluate the functionality, disability and quality of life of patients with systemic sclerosis and to compare the clinical forms of the disease. METHODS: Cross-sectional, descriptive and analytical study performed at the Rheumatology Clinic of the Hospital das Clínicas of the Federal University of Pernambuco (HC-UFPE) from August 2018 to April 2019. The non-probabilistic, convenience sample consisted of 60 patients diagnosed with systemic sclerosis (SSc), followed at the Rheumatology outpatient clinic of the Hospital das Clínicas, Federal University of Pernambuco. To evaluate the outcomes, the following instruments were used: Cochin Hand Functional Scale (CHFS) for hand function; 12-Item Short-Form Health Survey (SF-12) for quality of life; and Scleroderma Health Assessment Questionnaire (SHAQ) for functionality and disability. RESULTS: The mean results for CHFS, SHAQ, SF-12 Physical Component Summary and SF-12 Mental Component Summary were 14.5 (6.0-29.75), 1.01±0.56, 35.04±8.09, 40.94±10.56, respectively. There were no significant differences in CHFS outcomes between patients with diffuse and limited forms of SSc, SHAQ and the mental component of SF-12. However, in the physical component of SF-12, a better score was found in patients with the diffuse form of the disease (p=0.04). CONCLUSION: Patients with SSc present an important impairment of hand function, quality of life and functional capacity, and those with limited cutaneous form present worse scores of the physical component in the evaluation of quality of life.
INTRODUÇÃO: A esclerose sistêmica (ES) é uma doença autoimune crônica que se caracteriza por fibrose progressiva da pele e órgãos internos, promovendo grande morbimortalidade. OBJETIVO: Avaliar a funcionalidade, incapacidade e qualidade de vida em pacientes com esclerose sistêmica e comparar as formas clínicas da doença. MÉTODOS: Estudo transversal, descritivo e analítico, realizado na Clínica de Reumatologia do Hospital das Clínicas da Universidade Federal de Pernambuco (HC-UFPE), de agosto de 2018 a abril de 2019. A amostra não probabilística do tipo conveniência foi composta por 60 pacientes com diagnóstico de ES, acompanhados no ambulatório de Reumatologia do Hospital das Clínicas da Universidade Federal de Pernambuco. Para avaliar os resultados, foram utilizados os seguintes instrumentos: Escala Funcional da Mão de Cochin (CHFS) para função da mão; 12-Item Short-Form Health Survey (SF-12) para qualidade de vida; Questionário de Avaliação de Saúde da Esclerodermia (SHAQ) para funcionalidade e incapacidade. RESULTADOS: Os resultados médios para CHFS, SHAQ, SF-12 componente físico e SF-12 componente mental foram 14,5 (6,0-29,75), 1,01±0,56; 35,04±8,09; 40,94±10,56, respectivamente. Não houve diferenças significativas nos resultados do CHFS entre pacientes com formas difusas e limitadas de ES, SHAQ e o componente mental do SF-12. No entanto, no componente físico do SF-12, foi encontrado melhor escore dos pacientes com a forma difusa da doença (p=0,04). CONCLUSÃO: Pacientes com ES apresentam comprometimento importante da função da mão, qualidade de vida e capacidade funcional, e aqueles com forma cutânea limitada apresentam piores escores do componente físico na avaliação da qualidade de vida.
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Humanos , Masculino , Femenino , Calidad de Vida , Esclerodermia Sistémica , Fuerza de la Mano , Destreza Motora , Perfil de Salud , Estudios Transversales , Hospitales UniversitariosRESUMEN
Objective To determine the prognostic factors in systemic sclerosis.Methods Clinical data of definite systemic sclerosis patients were collected,including disease onset age,sex,disease course,Raynaud's phenomenon,skin involvement,gastroesophageal reflux,interstitial pneumonia,cardiac lesions,kidney lesions and scleroderma renal crisis.serum antibodies to scl-70.RNP and anti-centromere antibody were detected.Pulmonary artery pressure was measured by ultrasound cardiography.Cox hazard ratio model was employed to assess the mortality risk of systemic sclerosis patients.Results No difference in Raynaud's phenomenon,gastroesophageal reflux,anti-nuclear antybody,anti-sol-70 antibody,anti-centromere antibody,interstitial pneumonia,diffusion capacity (DLco),coronary artery disease,and peripheral artery atherosclerotic disease could be found between the dead and alive systemic sclerosis patients(P>0.05).Dead systemic sclerosis patients had later disease,onset(older than 60 years old)(P=0.002).Male gender(P=0.023),more diffuse skin involvement(P=0.000),more positive anti-RNP antibody(P=0.014),more pulmonary artery hypertension(P=0.000).more cardiac lesions(P=0.000),more cerebral infarets (P=0.035),more kidney lesions(P=0.000),and more scleroderma renal crisis(P=0.000) could be found jn dead sclerosis patients.Cox regression analysis showed that,onset later than 60 years old(OR=5.441.95% CI 2.126～13.926,P=0.000),male sex (OR=5.531,95%CI 2.014～15.190,P=0.001),anti-RNP antibedy positivity (OR=2.664,95%CI 1.016～6.592,P=0.034),diffuse skin involvement(OR=3.432,95%CI 1.400～8.411,P=0.007),pulmonary artery hypertension (OR=25.718,95% CI 5.954～111.085,P=0.000),cardiac lesions (OR=4.141.95%CI 1.685～10.159,P=0.002),kidney lesions(OR=4.214,95%CI 1.654～10.737,P=0.003) and scleroderma renal crisis (OR=20.677,95% CI 4.161～102.764.P=0.000)were risk factors for mortality in systemic sclerosis.Severe pulmonary hypertension was the most strong predictive factor for mortality in systemic sclerosis (OR=55.809,95% CI 12.879～241.832.P=0.000).Conclusion Aggressive therapy should be given to those systemic sclerosis patients with onset later than 60 years old,male sex,diffuse skin involvement,anti-RNP antibody positivity,cardiac involvement,kidney lesions,scleroderma renal crisis and pulmonary artery hypertension,especially seevere pulmonary hypertension.
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Summary: This study investigated the contents and distribution of collagen Ⅴ (Col Ⅴ) in skin lesions of the patients with systemic sclerosis (SSc) and its roles in the pathogenesis. The contents and distribution for al chain of collagen type Ⅰ,Ⅲ and [α1 (Ⅰ), α1 (Ⅲ) and α1 (Ⅴ)] in skin lesions of 36 patients with SSc (9 cases of mild fibrosis, 14 moderate, and 13 severe) were detected by using immunohistochemical SP method. Six cases of normal skin tissues served as controls. The results showed that there was diffuse distribution for three kinds of collagens in dennis. The deep staining α1 (Ⅰ) and α1 (Ⅲ) masses or bands were seen in reticular layer, while α1 (Ⅴ) was distributed more homogeneously. From control to weak, moderate and severe fibrosis stages, α1(Ⅰ), α1 (Ⅲ) and α1 (Ⅴ) showed a gradually increased trend in skin lesions (P<0.05). α1(Ⅴ) was obviously elevated in skin lesions at early stage and persisted in whole fibrotic process and risen in greater contents, while α1 (1) and α1(Ⅲ) were to go higher late and were apparently elevated at moderate and late stages. Compared with α1(Ⅰ), α1(Ⅴ) took leading increase at early stage in skin lesions (P<0.01), and had more elevated contents than α1 (Ⅲ) at moderate and late stages. The fibrotic changes in dermal reticular layer occurred earlier than those in papillary layer, and the abnormalities of α1 (Ⅴ)/ α1(Ⅰ) ratio appeared before α1 (Ⅲ)/α1 (Ⅰ) ratio. It was concluded that a lot of α1(Ⅴ) began to deposit in greater contents prior to α1 (Ⅰ) and α1(Ⅲ) at early stage in SSc and persisted in whole fibrotic process. The changes of α1(Ⅴ) contents in reticular layer occurred earlier than those in papillary layer, and it suggested that the fibrosis in reticular layer appeared earlier.