Systemic phenotype of sarcoidosis associated with radiological stages. Analysis of 1230 patients.

BACKGROUND: To analyze the association between Scadding radiological stages of sarcoidosis at diagnosis and the disease phenotype (epidemiology, clinical presentation and extrathoracic involvement) in one of the largest cohorts of patients with sarcoidosis reported from southern Europe. METHODS: The SARCOGEAS-Study Group includes a multicenter database of consecutive patients diagnosed with sarcoidosis according to the WASOG 1999 criteria. Extrathoracic disease at diagnosis was defined according to the 2014 instrument and the clusters proposed by Schupp et al. RESULTS: We analyzed 1230 patients (712 female, mean age 47 yrs.) who showed the following Scadding radiologic stages at diagnosis: stage 0 (n = 98), stage I (n = 395), stage II (n = 500), stage III (n = 195) and stage IV (n = 42). Women were overrepresented in patients presenting with extrathoracic/extrapulmonary disease, while the diagnosis was made at younger ages in patients presenting with BHL, and at older ages in those presenting with pulmonary fibrosis (q values <0.05). Multivariable adjusted analysis showed that patients presenting with pulmonary involvement (especially those with stages II and III) had a lower frequency of concomitant systemic involvement in some specific extrathoracic clusters (cutaneous-adenopathic/musculoskeletal, ENT and neuro-ocular/OCCC) but a higher frequency for others (hepatosplenic), in comparison with patients with extrapulmonary involvement (stages 0 and I). The presence of either BHL or fibrotic lesions did not influence the systemic phenotype of patients with pulmonary involvement. CONCLUSIONS: The key determinant associated with a differentiated systemic phenotype of sarcoidosis at diagnosis was interstitial pulmonary involvement rather than the individual Scadding radiological stage.

First clinical symptom as a prognostic factor in systemic sclerosis: results of a retrospective nationwide cohort study.

The objective of the study is to determine the importance of the mode of onset as prognostic factor in systemic sclerosis (SSc). Data were collected from the Spanish Scleroderma Registry (RESCLE), a nationwide retrospective multicenter database created in 2006. As first symptom, we included Raynaud's phenomenon (RP), cutaneous sclerosis, arthralgia/arthritis, puffy hands, interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), and digestive hypomotility. A total of 1625 patients were recruited. One thousand three hundred forty-two patients (83%) presented with RP as first symptom and 283 patients (17%) did not. Survival from first symptom in those patients with RP mode of onset was higher at any time than those with onset as non-Raynaud's phenomenon: 97 vs. 90% at 5 years, 93 vs. 82% at 10 years, 83 vs. 62% at 20 years, and 71 vs. 50% at 30 years (p < 0.001). In multivariate analysis, factors related to mortality were older age at onset, male gender, dcSSc subset, ILD, PAH, scleroderma renal crisis (SRC), heart involvement, and the mode of onset with non-Raynaud's phenomenon, especially in the form of puffy hands or pulmonary involvement. The mode of onset should be considered an independent prognostic factor in systemic sclerosis and, in particular, patients who initially present with non-Raynaud's phenomenon may be considered of poor prognosis.

First month prednisone dose predicts prednisone burden during the following 11†months: an observational study from the RELES cohort.

AIM: To study the influence of prednisone dose during the first month after systemic lupus erythematosus (SLE) diagnosis (prednisone-1) on glucocorticoid burden during the subsequent 11 months (prednisone-2-12). METHODS: 223 patients from the Registro Español de Lupus Eritematoso Sistémico inception cohort were studied. The cumulative dose of prednisone-1 and prednisone-2-12 were calculated and recoded into a four-level categorical variable: no prednisone, low dose (up to 7.5 mg/day), medium dose (up to 30 mg/day) and high dose (over 30 mg/day). The association between the cumulative prednisone-1 and prednisone-2-12 doses was tested. We analysed whether the four-level prednisone-1 categorical variable was an independent predictor of an average dose >7.5 mg/day of prednisone-2-12. Adjusting variables included age, immunosuppressives, antimalarials, methyl-prednisolone pulses, lupus nephritis and baseline SLE Disease Activity Index (SLEDAI). RESULTS: Within the first month, 113 patients (51%) did not receive any prednisone, 24 patients (11%) received average low doses, 46 patients (21%) received medium doses and 40 patients (18%) received high doses. There was a strong association between prednisone-1 and prednisone-2-12 dose categories (p<0.001). The cumulative prednisone-1 dose was directly associated with the cumulative prednisone-2-12 dose (p<0.001). Compared with patients on no prednisone, patients taking medium (adjusted OR 5.27, 95% CI 2.18 to 12.73) or high-dose prednisone-1 (adjusted OR 10.5, 95% CI 3.8 to 29.17) were more likely to receive prednisone-2-12 doses of >7.5 mg/day, while patients receiving low-dose prednisone-1 were not (adjusted OR 1.4, 95% CI 0. 0.38 to 5.2). If the analysis was restricted to the 158 patients with a baseline SLEDAI of ≥6, the model did not change. CONCLUSION: The dose of prednisone during the first month after the diagnosis of SLE is an independent predictor of prednisone burden during the following 11 months.

[Differences in variables representative of cardiovascular disease or repercussion between subjects with high cardiovascular risk with and without any family history of early cardiovascular events]. / Diferencias en variables representativas de enfermedad o repercusión cardiovascular entre sujetos de alto riesgo cardiovascular con y sin antecedentes familiares de eventos cardiovasculares precoces.

UNLABELLED: INTRODUCTION, BASES AND AIMS: Study the possible associations between any family history of early heart disease and variables expressive of heart disease, lesion or repercussion in subjects of high cardiovascular risk. MATERIAL AND METHODS: Nationwide cross-sectional study with 2264 consecutive patients in the Outpatient Clinic of Internal Medicine 18 years of age or older and with high or extremely high cardiovascular risk taken from the CIFARC (Integral Control of High Risk Cardiovascular Factors) study run by the SEMI (Spanish Internal Medicine Association) cardiovascular Risk Group. We have studied the relationship between the presence or lack of same in family histories of early heart disease (AFP-Family History of early heart disease, hereafter FH) and different variables in the factors concerning cardiovascular risk and target organ lesion. The statistical treatment (Chi-2, ANOVA and Pearson s linear regression) was performed using the STATISTIX programme. RESULTS AND DISCUSSION: We observed a significative increase (p<0.05) in the group with FH with a percentage of the following variables: Total cholesterol>250 mg/dl, HDL<40 mg/dl, LDL>130 mg/dl, LVH (left ventricular hypertrophy), creatinine 1.2-2mg/dl, retinopathy I-II, serious retinopathy, smoking, proteinuria>300 mg/dl, kidney insufficiency and peripheral vascular disease. This significative increase in the variables under study appeared in males of over 55 years of age and in females of over 65 years of age; nevertheless, this increase in the incidence rate is becoming more common as of age 50 in males, with a maximum in both sexes after the age of 70. No significative differences were noted with regard to sex, BMI (Body Mass Index), atheromatosis, ischemic cardiopathy and cerebrovascular disease. On the whole, subjects of both sexes of the FH group have come to the clinic 3 years earlier than those of the group without FH. CONCLUSIONS: Patients with FH show a greater incidence of certain factors concerning heart risk and target organ lesion.

Diferencias en variables representativas de enfermedad o repercusión cardiovascular entre sujetos de alto riesgo cardiovascular con y sin antecedentes familiares de eventos cardiovasculares precoces / Differences in variables representatives of cardiovascular disease or repercussion between subjects with high cardiovascular risk with and without any family history of early cardiovascular events

Introducción, fundamentos y objetivos: Estudiar las posibles asociaciones entre antecedentes familiares precoces (AFP) de enfermedad cardiovascular y variables expresivas de enfermedad, lesión o repercusión cardiovascular en los sujetos de alto riesgo cardiovascular. Material y métodos: Estudio transversal de ámbito nacional con 2.264 pacientes consecutivos de las Consultas Externas de Medicina Interna, mayores de 18 años y con riesgo cardiovascular alto o muy alto, procedentes del Estudio CIFARC del Grupo de Riesgo Vascular de la SEMI. Se estudia la relación entre la presencia o no de antecedentes familiares de enfermedad cardiovascular precoz (AFP) y distintas variables de factores de riesgo cardiovascular y lesión de órgano diana. El tratamiento estadístico (Chi cuadrado, ANOVA y regresión líneal de Pearson) se ha realizado mediante el programa STATISTIX. Resultados y discusión: Observamos un incremento significativo (p 250 mg/dl, HDL 130 mg/dl, HVI (hipertrofia ventricular izquierda), creatinina 1,2-2 mg/dl, retinopatía I-II, retinopatía grave, tabaquismo, proteinuria > 300 mg/dl, Insuficiencia renal y enfermedad vascular periférica. Este incremento significativo de las variables estudiadas se produce en varones de más de 55 años y en mujeres de más de 65 años; no obstante comienza a observarse este aumento del porcentaje de incidencia a partir de los 50 años en varones, con un máximo en ambos sexos tras los setenta. No se observan diferencias significativas respecto a sexo, IMC (índice de masa corporal) ateromatosis, cardiopatía isquémica y enfermedad cerebrovascular. Existe en general en ambos sexos un adelanto de tres años en el grupo de AFP en acudir a la consulta médica respecto al grupo que no posee AFP. Conclusiones: Los pacientes con AFP presentan mayor incidencia de ciertos factores de riesgo cardiovascular y de lesión de órgano diana

Introduction, bases and aims: Study the possible associations between any family history of early heart disease and variables expressive of heart disease, lesion or repercussion in subjects of high cardiovascular risk. Material and methods: Nationwide cross-sectional study with 2264 consecutive patients in the Outpatient Clinic of Internal Medicine 18 years of age or older and with high or extremely high cardiovascular risk taken from the CIFARC (Integral Control of High Risk Cardiovascular Factors) study run by the SEMI (Spanish Internal Medicine Association) cardiovascular Risk Group. We have studied the relationship between the presence or lack of same in family histories of early heart disease (AFP – Family History of early heart disease, hereinafter FH) and different variables in the factors concerning cardiovascular risk and target organ lesion. The statistical treatment (Chi-2, ANOVA and Pearson’s linear regression) was performed using the STATISTIX programme. Results and discussion: We observed a significative increase (p 250 mg/dl, HDL 130 mg/dl, LVH (left ventricular hypertrophy), creatinine 1.2-2mg/dl, retinopathy I-II, serious retinopathy, smoking, proteinuria > 300 mg/dl, kidney insufficiency and peripheral vascular disease. This significative increase in the variables under study appeared in males of over 55 years of age and in females of over 65 years of age; nevertheless, this increase in the incidence rate is becoming more common as of age 50 in males, with a maximum in both sexes after the age of 70. No significative differences were noted with regard to sex, BMI (Body Mass Index), atheromatosis, ischemic cardiopathy and cerebrovascular disease. On the whole, subjects of both sexes of the FH group have come to the clinic 3 years earlier than those of the group without FH. Conclusions: Patients with FH show a greater incidence of certain factors concerning heart risk and target organ lesion