RESUMEN
OBJECTIVES: To evaluate the clinical characteristics of patients with interstitial lung disease (ILD) in the setting of a large cohort of systemic sclerosis (SSc) patients, and to analyse the differences according to the SSc subtype (following the modification of classification criteria of the American College of Rheumatology for SSc proposed by LeRoy and Medsger), factors are associated with moderate-to-severe impairment of lung function, as well as mortality and causes of death. METHODS: A descriptive study was performed, using the available data from the Spanish Scleroderma Study Group. RESULTS: Twenty-one referral centers participated in the registry. By April 2014, 1374 patients with SSc had been enrolled, and 595 of whom (43%) had ILD: 316 (53%) with limited cutaneous SSc (lcSSc), 240 (40%) with diffuse cutaneous SSc (dcSSc), and 39 (7%) with SSc sine scleroderma (ssSSc). ILD in the lcSSc and the ssSSc subsets tended to develop later, and showed a less impaired forced vital capacity (FVC) and a ground glass pattern on high-resolution computed tomography (HRCT) less frequently, compared with the dcSSc subset. Factors related to an FVC < 70% of predicted in the multivariate analysis were: dcSSc, positivity to anti-topoisomerase I antibodies, a ground glass pattern on HCRT, an active nailfold capillaroscopy pattern, lower DLco, older age at symptoms onset, and longer time between symptoms onset and ILD diagnosis. Finally, SSc-associated mortality and ILD-related mortality were highest in dcSSc patients, whereas that related to pulmonary arterial hypertension was highest in those with lcSSc-associated ILD. CONCLUSIONS: Our study indicates that ILD constitutes a remarkable complication of SSc with significant morbidity and mortality, which should be borne in mind in all three subgroups (lcSSc, dcSSc, and ssSSc).
Asunto(s)
Enfermedades Pulmonares Intersticiales , Pulmón , Esclerodermia Difusa , Esclerodermia Limitada , Adulto , Anciano , Causas de Muerte , Distribución de Chi-Cuadrado , Femenino , Cardiopatías/mortalidad , Cardiopatías/fisiopatología , Humanos , Hipertensión Pulmonar/mortalidad , Hipertensión Pulmonar/fisiopatología , Modelos Logísticos , Pulmón/diagnóstico por imagen , Pulmón/patología , Pulmón/fisiopatología , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/mortalidad , Enfermedades Pulmonares Intersticiales/fisiopatología , Enfermedades Pulmonares Intersticiales/terapia , Masculino , Angioscopía Microscópica , Persona de Mediana Edad , Análisis Multivariante , Oportunidad Relativa , Prevalencia , Pronóstico , Sistema de Registros , Factores de Riesgo , Esclerodermia Difusa/diagnóstico , Esclerodermia Difusa/mortalidad , Esclerodermia Difusa/fisiopatología , Esclerodermia Difusa/terapia , Esclerodermia Limitada/diagnóstico , Esclerodermia Limitada/mortalidad , Esclerodermia Limitada/fisiopatología , Esclerodermia Limitada/terapia , Índice de Severidad de la Enfermedad , Piel/patología , España/epidemiología , Tomografía Computarizada por Rayos X , Capacidad VitalRESUMEN
Systemic sclerosis (SSc) is a rare, multisystem disease showing a large individual variability in disease progression and prognosis. In the present study, we assess survival, causes of death, and risk factors of mortality in a large series of Spanish SSc patients. Consecutive SSc patients fulfilling criteria of the classification by LeRoy were recruited in the survey. Kaplan-Meier and Cox proportional-hazards models were used to analyze survival and to identify predictors of mortality. Among 879 consecutive patients, 138 (15.7%) deaths were registered. Seventy-six out of 138 (55%) deceased patients were due to causes attributed to SSc, and pulmonary hypertension (PH) was the leading cause in 23 (16.6%) patients. Survival rates were 96%, 93%, 83%, and 73% at 5, 10, 20, and 30 years after the first symptom, respectively. Survival rates for diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc were 91%, 86%, 64%, and 39%; and 97%, 95%, 85%, and 81% at 5, 10, 20, and 30 years, respectively (log-rank: 67.63, Pâ<â0.0001). The dcSSc subset, male sex, age at disease onset older than 65 years, digital ulcers, interstitial lung disease (ILD), PH, heart involvement, scleroderma renal crisis (SRC), presence of antitopoisomerase I and absence of anticentromere antibodies, and active capillaroscopic pattern showed reduced survival rate. In a multivariate analysis, older age at disease onset, dcSSc, ILD, PH, and SRC were independent risk factors for mortality. In the present study involving a large cohort of SSc patients, a high prevalence of disease-related causes of death was demonstrated. Older age at disease onset, dcSSc, ILD, PH, and SRC were identified as independent prognostic factors.
Asunto(s)
Sistema de Registros , Esclerodermia Sistémica/mortalidad , Adulto , Anciano , Causas de Muerte , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Factores de Riesgo , España/epidemiologíaRESUMEN
Although adrenal hemorrhage is an unusual feature of antiphospholipid syndrome (APS), it can be its first clinical manifestation. The pathogenetic substratum is the thrombosis of the adrenal veins leading to hemorrhagic infarction. Recurrences are frequent in patients with APS-related thrombosis, thus long-term anticoagulation is advocated by most authors.
Asunto(s)
Enfermedades de las Glándulas Suprarrenales/etiología , Síndrome Antifosfolípido/complicaciones , Hemorragia/complicaciones , Enfermedad Aguda , Síndrome Antifosfolípido/diagnóstico , Femenino , Humanos , Persona de Mediana EdadRESUMEN
Valvular replacement was performed in a patient with primary antiphospholipid syndrome and severe mitral insufficiency. The valvular tissue presented thrombotic phenomena and fibrosis with minimum inflammatory component.