scholarly journals Serologic Profile and Mortality Rates of Scleroderma Renal Crisis in Italy

2009 ◽  
Vol 36 (7) ◽  
pp. 1464-1469 ◽  
Author(s):  
VERONICA CODULLO ◽  
ILARIA CAVAZZANA ◽  
CLAUDIA BONINO ◽  
CLAUDIA ALPINI ◽  
LORENZO CAVAGNA ◽  
...  

Objective.To analyze clinical and serological characteristics of subjects with scleroderma renal crisis (SRC) in Italian patients with systemic sclerosis (SSc).Methods.A retrospective analysis of medical records from 9 Italian rheumatologic referral centers was carried out. All patients with SRC and an available serum sample at the time of crisis were included. Antinuclear antibodies (ANA) by indirect immunofluorescence, anti-topoisomerase (topo) I by enzyme-linked assay (ELISA), anti-RNA polymerases (RNAP) by ELISA for the subunit III, and immunoprecipitation (IP) were performed.Results.Forty-six cases (38 female; 40 diffuse cutaneous SSc) were identified. Mean age at SSc and SRC onset was 52.8 years ± 13.2 and 55.4 years ± 11.8, respectively. ANA were present in 44 patients (96%). Anti-topo I antibodies were detected in 30 (65%), anti-RNAP I–III in 7 (15%). No differences emerged between these 2 groups for their main clinical characteristics. The proportion of patients in the anti-RNAP I–III group developing SRC early (< 18 mo) in the course of SSc was significantly higher (p = 0.03). Cumulative survival rates were 64%, 53%, and 35% at 1, 2, and 10 years of followup, respectively. Survival rates of SSc patients significantly differed according to their autoantibody profile, being lower in the anti-topo I than in the anti-RNAP I–III group (p = 0.034).Conclusion.SRC is a rare manifestation of SSc in Italy but it is still associated with severe prognosis. Anti-topo I reactivity was more frequent than anti-RNAP I–III in our patients with SRC and was associated with delayed onset and high mortality rates.

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Mieko Yamagata ◽  
Koichi Hirose ◽  
Kei Ikeda ◽  
Hiroshi Nakajima

AlthoughNocardiosishas considerable recurrence and mortality rates, characteristics and risk factors ofNocardiainfection have not been assessed in patients with rheumatic diseases. Here, we examined the characteristics and risk factors ofNocardiainfection in rheumatic disease patients in our hospital. Ten rheumatic disease patients who developedNocardiainfection were identified by retrospectively reviewing the medical records. Possible predisposing factors forNocardiainfection were high-dose glucocorticoid treatment, concomitant use of immunosuppressants, preexisting pulmonary diseases, and diabetes mellitus. All patients had pulmonaryNocardiosis, and six of them had disseminatedNocardiosiswhen their pulmonary lesions were identified.


2018 ◽  
Vol 46 (1) ◽  
pp. 85-92 ◽  
Author(s):  
Sarah M. Gordon ◽  
Rodger S. Stitt ◽  
Robert Nee ◽  
Wayne T. Bailey ◽  
Dustin J. Little ◽  
...  

Objective.Systemic sclerosis (SSc) is a disease of autoimmunity, fibrosis, and vasculopathy. Scleroderma renal crisis (SRC) is one of the most severe complications. Corticosteroid exposure, presence of anti-RNA polymerase III antibodies (ARA), skin thickness, and significant tendon friction rubs are among the known risk factors at SSc diagnosis for developing future SRC. Identification of additional clinical characteristics and laboratory findings could expand and improve the risk profile for future SRC at SSc diagnosis.Methods.In this retrospective cohort study of the entire military electronic medical record between 2005 and 2016, we compared the demographics, clinical characteristics, and laboratory results at SSc diagnosis for 31 cases who developed SRC after SSc diagnosis to 322 SSc without SRC disease controls.Results.After adjustment for potential confounding variables, at SSc diagnosis these conditions were all associated with future SRC: proteinuria (p < 0.001; OR 183, 95% CI 19.1–1750), anemia (p = 0.001; OR 9.9, 95% CI 2.7–36.2), hypertension (p < 0.001; OR 13.1, 95% CI 4.7–36.6), chronic kidney disease (p = 0.008; OR 20.7, 95% CI 2.2–190.7), elevated erythrocyte sedimentation rate (p < 0.001; OR 14.3, 95% CI 4.8–43.0), thrombocytopenia (p = 0.03; OR 7.0, 95% CI 1.2–42.7), hypothyroidism (p = 0.01; OR 2.8, 95% CI 1.2–6.7), Anti-Ro antibody seropositivity (p = 0.003; OR 3.9, 95% CI 1.6–9.8), and ARA (p = 0.02; OR 4.1, 95% CI 1.2–13.8). Three or more of these risk factors present at SSc diagnosis was sensitive (77%) and highly specific (97%) for future SRC. No SSc without SRC disease controls had ≥ 4 risk factors.Conclusion.In this SSc cohort, we present a panel of risk factors for future SRC. These patients may benefit from close observation of blood pressure, proteinuria, and estimated glomerular filtration rate, for earlier SRC identification and intervention. Future prospective therapeutic studies could focus specifically on this high-risk population.


2016 ◽  
Vol 25 (3) ◽  
pp. 155-159
Author(s):  
Iulia Andronache ◽  
◽  
Cristina Suta ◽  
Sabina Ciocodei ◽  
Liliana Tuta ◽  
...  

Scleroderma renal crisis is an important cause of morbidity and mortality in systemic sclerosis patients, occurring in about 5-10% of the cases. It is seen mostly early in the progression of the disease, about 75% of the scleroderma renal crises affecting the patients in their first 4 years since the onset of the disease. We present the case of a 62 year old woman who was admitted in our clinic for oliguria, edema, an important elevation of the kidney function tests, arterial hypertension and severe anemia. Immunological tests showed intensely positive antinuclear antibodies with positive anti RNA polymerase III antibodies. She was diagnosed with systemic sclerosis according to ACR/EULAR 2013 criteria and scleroderma renal crisis. Specific therapy was started along with hemodialysis imposed by the progression of the kidney failure.


Neonatology ◽  
2021 ◽  
pp. 1-9
Author(s):  
Matthias Fröhlich ◽  
Tatjana Tissen-Diabaté ◽  
Christoph Bührer ◽  
Stephanie Roll

<b><i>Introduction:</i></b> In very low birth weight (&#x3c;1,500 g, VLBW) infants, morbidity and mortality have decreased substantially during the past decades, and both are known to be lower in girls than in boys. In this study, we assessed sex-specific changes over time in length of hospital stay (LOHS) and postmenstrual age at discharge (PAD), in addition to survival in VLBW infants. <b><i>Methods:</i></b> This is a single-center retrospective cohort analysis based on quality assurance data of VLBW infants born from 1978 to 2018. Estimation of sex-specific LOHS over time was based on infants discharged home from neonatal care or deceased. Estimation of sex-specific PAD over time was based on infants discharged home exclusively. Analysis of in-hospital survival was performed for all VLBW infants. <b><i>Results:</i></b> In 4,336 of 4,499 VLBW infants admitted from 1978 to 2018 with complete data (96.4%), survival rates improved between 1978–1982 and 1993–1997 (70.8 vs. 88.3%; hazard ratio (HR) 0.20, 95% confidence interval 0.14, 0.30) and remained stable thereafter. Boys had consistently higher mortality rates than girls (15 vs. 12%, HR 1.23 [1.05, 1.45]). Nonsurviving boys died later compared to nonsurviving girls (adjusted mean survival time 23.0 [18.0, 27.9] vs. 20.7 [15.0, 26.3] days). LOHS and PAD assessed in 3,166 survivors displayed a continuous decrease over time (1978–1982 vs. 2013–2018: LOHS days 82.9 [79.3, 86.5] vs. 60.3 [58.4, 62.1] days); PAD 40.4 (39.9, 40.9) vs. 37.4 [37.1, 37.6] weeks). Girls had shorter LOHS than boys (69.4 [68.0, 70.8] vs. 73.0 [71.6, 74.4] days) and were discharged with lower PAD (38.6 [38.4, 38.8] vs. 39.2 [39.0, 39.4] weeks). <b><i>Discussion/Conclusions:</i></b> LOHS and PAD decreased over the last 40 years, while survival rates improved. Male sex was associated with longer LOHS, higher PAD, and higher mortality rates.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Wen Wang ◽  
Lei Chen ◽  
Qiao He ◽  
Mingqi Wang ◽  
Mei Liu ◽  
...  

Abstract Background The outbreak of COVID-19 has resulted in serious concerns in China and abroad. To investigate clinical features of confirmed and suspected patients with COVID-19 in west China, and to examine differences between severe versus non-severe patients. Methods Patients admitted for COVID-19 between January 21 and February 11 from fifteen hospitals in Sichuan Province, China were included. Experienced clinicians trained with methods abstracted data from medical records using pre-defined, pilot-tested forms. Clinical characteristics between severe and non-severe patients were compared. Results Of the 169 patients included, 147 were laboratory-confirmed, 22 were suspected. For confirmed cases, the most common symptoms from onset to admission were cough (70·7%), fever (70·5%) and sputum (33·3%), and the most common chest CT patterns were patchy or stripes shadowing (78·0%); throughout the course of disease, 19·0% had no fever, and 12·4% had no radiologic abnormality; twelve (8·2%) received mechanical ventilation, four (2·7%) were transferred to ICU, and no death occurred. Compared to non-severe cases, severe ones were more likely to have underlying comorbidities (62·5% vs 26·2%, P = 0·001), to present with cough (92·0% vs 66·4%, P = 0·02), sputum (60·0% vs 27·9%, P = 0·004) and shortness of breath (40·0% vs 8·2%, P <  0·0001), and to have more frequent lymphopenia (79·2% vs 43·7%, P = 0·003) and eosinopenia (84·2% vs 57·0%, P = 0·046). Conclusions The symptoms of patients in west China were relatively mild, and an appreciable proportion of infected cases had no fever, warranting special attention.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1004.1-1004
Author(s):  
D. Xu ◽  
R. Mu

Background:Scleroderma renal crisis (SRC) is a life-threatening syndrome. The early identification of patients at risk is essential for timely treatment to improve the outcome[1].Objectives:We aimed to provide a personalized tool to predict risk of SRC in systemic sclerosis (SSc).Methods:We tried to set up a SRC prediction model based on the PKUPH-SSc cohort of 302 SSc patients. The least absolute shrinkage and selection operator (Lasso) regression was used to optimize disease features. Multivariable logistic regression analysis was applied to build a SRC prediction model incorporating the features of SSc selected in the Lasso regression. Then, a multi-predictor nomogram combining clinical characteristics was constructed and evaluated by discrimination and calibration.Results:A multi-predictor nomogram for evaluating the risk of SRC was successfully developed. In the nomogram, four easily available predictors were contained including disease duration <2 years, cardiac involvement, anemia and corticosteroid >15mg/d exposure. The nomogram displayed good discrimination with an area under the curve (AUC) of 0.843 (95% CI: 0.797-0.882) and good calibration.Conclusion:The multi-predictor nomogram for SRC could be reliably and conveniently used to predict the individual risk of SRC in SSc patients, and be a step towards more personalized medicine.References:[1]Woodworth TG, Suliman YA, Li W, Furst DE, Clements P (2016) Scleroderma renal crisis and renal involvement in systemic sclerosis. Nat Rev Nephrol 12 (11):678-91.Disclosure of Interests:None declared


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