RESUMEN
BACKGROUND AND OBJECTIVES: Recently published population-based cohort studies have shown a high prevalence of cardiovascular disease in Systemic Sclerosis (SSc) patients. The aim of this study is to compare three different methods to measure cardiovascular risk in patients with scleroderma. METHODS: Forty-three SSc patients were included. A prospective study was performed for evaluation of cardiovascular risk and subclinical atheromatosis using 3 non-invasive methods: cardiovascular risk tables, carotid Doppler ultrasonography and quantification of coronary calcium by computerized tomography (CT). RESULTS: The cardiovascular risk charts for the Spanish population did not identify patients at high cardiovascular risk. Framingham-REGICOR identified 13 intermediate-risk patients. Twenty-two patients (51.2%) had plaques on carotid ultrasonography. We performed a ROC curve to identify the best cutoff point for the quantification of coronary artery calcium (CACscore), the value of CACscoreâ¯>â¯28â¯AU (Agatston Units) had the highest sensitivity (73%) and specificity (81%) for the diagnosis of subclinical atheromatosis. In the multiple regression study, age and decreased HDL cholesterol levels were identified as independent factors for subclinical atherosclerotic disease. No disease-related factors were associated with increased subclinical arteriosclerosis. CONCLUSION: Carotid ultrasound and CACscore are useful for identifying subclinical atheromatosis in patients with SSc and are superior compared to risk charts used for general population. HDL cholesterol and age were independent factors for the presence of subclinical atherosclerotic disease. A carotid ultrasound or CT should be performed for early detection of subclinical atheromatosis if these factors are present.
Asunto(s)
Calcio/metabolismo , Enfermedades Cardiovasculares/complicaciones , Vasos Coronarios/patología , Esclerodermia Sistémica/etiología , Ultrasonografía/métodos , Adulto , Anciano , Anciano de 80 o más Años , Calcio/análisis , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Factores de RiesgoRESUMEN
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
OBJECTIVES: To date, the diagnostic utility of anti-SSA/Ro52 autoantibodies in scleroderma and the association of them with certain clinical manifestations, particularly inflammatory myositis, are still controversial. This paper aims to assess the correlation between the presence of anti-SSA/Ro52 antibodies and the demographic, clinical and prognosis characteristics of patients with systemic sclerosis (SSc). METHODS: This is a retrospective, cross-sectional and observational study in patients with SSc. Baseline demographic and clinical characteristics were recorded. Presence of anti-SSA/Ro52, anti-SSA/Ro, anti-SSB/La, snRNP/Sm, anti-centromere, anti-Scl-70 and anti-PM-Scl were analysed by immunoblot, and antinuclear antibodies (ANA) by indirect immunofluorescence. Statistical analysis was performed with PASW Statics 18 software. RESULTS: A total of 132 consecutive patients with analysis of anti-SSA/Ro52 antibodies were selected from a Spanish cohort of 408 patients with SSc, 87.1% of them being women. About half of patients had the limited form (51.5%), followed by diffused form (18.9%), sclerosis sine scleroderma (22.7%), and pre-scleroderma (6.8%). Prevalence of anti-SSA/Ro52 was 35.6%. No association between anti-SSA/Ro52 and clinical manifestations was found, while detection of anti-SSA/Ro52 was significantly associated with the presence of anti-Ro. CONCLUSIONS: The results of our study show that anti-SSA/Ro52 antibodies are often found in SSc patients. No clinical manifestations, including inflammatory myopathy, were related with anti-SSA/Ro antibodies.
Asunto(s)
Anticuerpos Antinucleares/inmunología , Esclerodermia Sistémica/inmunología , Adulto , Anciano , Autoanticuerpos/inmunología , Estudios Transversales , ADN-Topoisomerasas de Tipo I , Exorribonucleasas/inmunología , Complejo Multienzimático de Ribonucleasas del Exosoma/inmunología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Proteínas Nucleares/inmunología , Estudios Retrospectivos , Ribonucleoproteínas/inmunología , Ribonucleoproteínas Nucleares Pequeñas/inmunología , EspañaRESUMEN
OBJECTIVES: To compare a cohort of patients with systemic sclerosis sine scleroderma (ssSSc) vs. patients with limited cutaneous systemic sclerosis (lcSSc). METHODS: Forty-five patients with ssSSc and 186 patients with lcSSc were investigated. Demographic, clinical and immunologic features and survival were compared. RESULTS: There were no significant differences between ssSSc and lcSSc in gender, age at onset and interval between onset and diagnosis. ssSSc patients fulfilled the ACR criteria for SSc less than lcSSc patients (13%/77%, p<0.0001). There were no significant differences in articular involvement, myopathy, tendon friction rubs and gastrointestinal, pulmonary, cardiac and renal involvements. There was a trend to higher prevalence of pulmonary arterial hypertension (PAH) in ssSSc patients (29%/19%) but not reach significant difference. The prevalence of antinuclear and anticentromere antibodies and slow capilaroscopic pattern was similar. Sicca syndrome (13%/30%; p=0.024), digital ulcers (16%/50%; p<0.0001), calcinosis (11%/26%; p=0.047) and acroosteolysis (0% /10%; p=0.028) were more frequently in lcSSc. Survival at 5, 10, and 15 yr was not different in ssSSc and lcSSc patients (100%/98%, 100%/98%, and 92%/89%, respectively). CONCLUSIONS: ssSSc and lcSSc patients share demographic, clinical and immunologic features. Survival is also similar in both groups. Differences are mainly due to peripheral vascular manifestations. However, despite great similarities, we believe that ssSSc patients should be considered as a different subset in order to avoid misdiagnosis. ssSSc patients should be truly differentiated from early SSc using sensitive and specific studies looking for any asymptomatic organ involvement.
Asunto(s)
Calcinosis/etiología , Dermatosis de la Mano/etiología , Hipertensión Pulmonar/etiología , Esclerodermia Sistémica/complicaciones , Síndrome de Sjögren/etiología , Úlcera Cutánea/etiología , Acroosteólisis/etiología , Adulto , Anciano , Trastornos de la Motilidad Esofágica/etiología , Femenino , Humanos , Enfermedades Pulmonares Intersticiales/etiología , Masculino , Persona de Mediana Edad , Esclerodermia Difusa/clasificación , Esclerodermia Difusa/complicaciones , Esclerodermia Difusa/fisiopatología , Esclerodermia Limitada/clasificación , Esclerodermia Limitada/complicaciones , Esclerodermia Limitada/fisiopatología , Esclerodermia Sistémica/clasificación , Esclerodermia Sistémica/fisiopatologíaRESUMEN
Sweet's syndrome or acute febrile neutrophilic dermatosis (SS) is characterized by the sudden onset of painful erythematous lesions (papules, nodules, and plaques) together with fever and neutrophilia. The lesions are typically located on hands, arms, upper trunk, neck and face, showing an asymmetric distribution. Acute phase reactants are usually elevated and dermal infiltration of neutrophils without vasculitis is seen on skin biopsies. It is considered as a marker of systemic disease in over half of the cases, and is associated with infections, inflammatory bowel disease, autoimmune connective tissue disorders and various neoplasias. Its association with Crohn's disease (CD) is unusual and it appears mainly in association with colonic involvement. Fewer than 50 cases have been published in the medical literature since its first description in 1964, some concurrent with the first episode of CD. We present two patients with Crohn's disease and Sweet's syndrome diagnosed in our department at the time of CD diagnosis, as well as their response to treatment, subsequent course of the disease, and a review of the scientific literature.
Asunto(s)
Enfermedad de Crohn/complicaciones , Síndrome de Sweet/complicaciones , Adulto , Antiinflamatorios/uso terapéutico , Colonoscopía , Enfermedad de Crohn/diagnóstico , Enfermedad de Crohn/terapia , Eritema/etiología , Eritema/patología , Femenino , Fluidoterapia , Humanos , Masculino , Persona de Mediana Edad , Prednisona/uso terapéutico , Piel/patología , Síndrome de Sweet/diagnóstico , Síndrome de Sweet/terapia , Tomografía Computarizada por Rayos XRESUMEN
El síndrome de Sweet o dermatosis neutrofílica febril aguda(SS) se caracteriza por la aparición brusca de lesiones eritematosas,(pápulas, nódulos y placas) dolorosas, junto con fiebre y neutrofilia,siendo de presentación poco frecuente. Las lesiones se localizanpreferentemente en manos, brazos, parte superior deltronco, cuello y cara, con distribución asimétrica. Suele haber elevaciónde reactantes de fase aguda y en las biopsias cutáneas seidentifica una infiltración dérmica de neutrófilos sin vasculitis. Seconsidera un marcador de enfermedad sistémica en más de la mitadde los casos, asociándose a infecciones, enfermedad inflamatoriaintestinal, conectivopatías autoinmunes y diversas neoplasias.Su asociación con la enfermedad de Crohn (EC) es poco habitual,asociado sobre todo a afectación colónica. Se han publicadomenos de 50 casos en la literatura médica desde su primera descripciónen 1964, algunos de ellos simultáneos con el primer brotede la EC. Presentamos dos pacientes con enfermedad deCrohn y síndrome de Sweet diagnosticados en nuestro servicio enel momento del diagnóstico de la EC, así como su respuesta al tratamiento,evolución posterior y revisión de la literatura científica(AU)
The uncommon Sweets syndrome or acute febrile neutrophilicdermatosis (SS) is characterized by the sudden onset of painfulerythematous lesions (papules, nodules, and plaques) togetherwith fever and neutrophilia. The lesions are typically located onhands, arms, upper trunk, neck and face, showing an asymmetricdistribution. Acute phase reactants are usually elevated and dermalinfiltration of neutrophils without vasculitis is seen on skinbiopsies. It is considered as a marker of systemic disease in overhalf of the cases, and is associated with infections, inflammatorybowel disease, autoimmune connective tissue disorders and variousneoplasias.Its association with Crohns disease (CD) is unusual and it appearsmainly in association with colonic involvement. Fewer than50 cases have been published in the medical literature since itsfirst description in 1964, some concurrent with the first episodeof CD. We present two patients with Crohns disease and Sweetssyndrome diagnosed in our department at the time of CD diagnosis,as well as their response to treatment, subsequent course ofthe disease, and a review of the scientific literature(AU)
Asunto(s)
Humanos , Masculino , Femenino , Adulto , Persona de Mediana Edad , Enfermedad de Crohn/complicaciones , Enfermedad de Crohn/terapia , Síndrome de Sweet/complicaciones , Síndrome de Sweet/diagnóstico , Fluidoterapia/métodos , Metilprednisolona/uso terapéutico , Ciprofloxacina/uso terapéutico , Metronidazol/uso terapéutico , Dermatosis de la Mano/complicaciones , Dermatosis de la Mano/terapia , Enfermedades de la Piel/complicaciones , Síndrome de Sweet/fisiopatología , Astenia/complicaciones , Fluidoterapia/tendencias , Nutrición Enteral , Mesalamina/uso terapéutico , Beclometasona/uso terapéuticoRESUMEN
Raynaud's phenomenon (RP) is a clinical picture characterized by the presence of recurrent episodes of vasospasm that is precipitated by cold or other stimuli and especially affects the fingers and/or toes. It may be primary or idiopathic or secondary and be due to many causes, among which connective diseases, and specifically scleroderma, stand out. Primary RP does not generally require drug treatment, only requiring general measures. Calcium channel antagonists continue to be the drug of first choice. However, when RP is severe and is accompanied by digital ulcers/tissue necrosis, the therapeutic regime must be individualized and combinations should be established with difference drugs such as prostanoids, bosentan, sildenafil, antiaggregants/anticoagulants, antibiotics and analgesics.
Asunto(s)
Enfermedad de Raynaud/terapia , Algoritmos , HumanosRESUMEN
El fenómeno de Raynaud (FR) es un cuadro clínico caracterizado por la presencia de episodios recurrentes de vasospasmo, que es desencadenado por el frío u otros estímulos y afecta especialmente a los dedos de las manos y/o pies. Puede ser primario o idiopático, o secundario y obedecer a muchas causas, entre las que destacan las conectivopatías y, en particular, la esclerodermia. El FR primario no requiere, en general, tratamiento farmacológico, sólo medidas generales. Los antagonistas de los canales del calcio continúan siendo los fármacos de primera elección. Sin embargo, cuando el FR es grave y se acompaña de úlceras digitales/necrosis hística, la pauta terapéutica ha de individualizarse y deben establecerse combinaciones con distintos fármacos como: prostanoides, bosentan, sildenafilo, antiagregantes/anticoagulantes, antibióticos y analgésicos (AU)
Raynaud's phenomenon (RP) is a clinical picture characterized by the presence of recurrent episodes of vasospasm that is precipitated by cold or other stimuli and especially affects the fingers and/or toes. It may be primary or idiopathic or secondary and be due to many causes, among which connective diseases, and specifically scleroderma, stand out. Primary RP does not generally require drug treatment, only requiring general measures. Calcium channel antagonists continue to be the drug of first choice. However, when RP is severe and is accompanied by digital ulcers/tissue necrosis, the therapeutic regime must be individualized and combinations should be established with difference drugs such as prostanoids, bosentan, sildenafil, antiaggregants/anticoagulants, antibiotics and analgesics (AU)
Asunto(s)
Humanos , Enfermedad de Raynaud/tratamiento farmacológico , Vasodilatadores/uso terapéutico , Esclerodermia Localizada/complicaciones , Anticoagulantes/uso terapéutico , Antibacterianos/uso terapéutico , Analgésicos/uso terapéuticoRESUMEN
OBJECTIVE: To evaluate the presence of anti-PDGFR-alpha antibodies by immunological methods in patients with systemic sclerosis (SSc). METHODS: Fifty-eight women diagnosed with SSc and 36 healthy women controls were included. IgG anti-PDGFR-alpha were measured by ELISA and immunoblot. Associations with clinical and immunological findings were also studied. RESULTS: Non-significant differences were detected between patients with SSc and controls: median value 0.287 (range 0-2.06) versus median value 0.226 (range 0-2.94), respectively (p = 0.583). No correlation between the presence of anti-PDGFR-alpha antibodies and clinical and serological features was found. Serum samples from patients with SSc and healthy people who had high titres of anti-PDGFR-alpha antibodies by ELISA recognised the same band corresponding to PDGFR-alpha by immunoblot. CONCLUSION: Although anti-PDGFR-alpha antibodies seem to be disease-specific when determined by bioactivity assays, these antibodies are also detected in normal subjects when immunological methods are used. Thus, anti-PDGFR-alpha antibodies may arise from natural autoantibodies. Possibly, SSc autoantibodies recognise a different epitope on the PDGFR-alpha molecule which triggers its stimulatory effect when analysed by functional assays. Alternatively, naturally occurring autoantibodies may even become pathogenic after affinity maturation and class switching in genetically susceptible subjects.
Asunto(s)
Autoanticuerpos/sangre , Receptor alfa de Factor de Crecimiento Derivado de Plaquetas/inmunología , Esclerodermia Sistémica/inmunología , Adulto , Anciano , Anciano de 80 o más Años , Biomarcadores/sangre , Electroforesis en Gel de Poliacrilamida/métodos , Ensayo de Inmunoadsorción Enzimática/métodos , Femenino , Humanos , Persona de Mediana Edad , Sensibilidad y EspecificidadRESUMEN
OBJECTIVE: Interstitial lung disease (ILD) frequently complicates systemic sclerosis (SSc). Cyclophosphamide (CYC) is a promising immunosuppressive therapy for SSc-related ILD. Our objective was to investigate the effectiveness of an intravenous CYC (iv CYC) pulse regime in SSc-related ILD during treatment and thereafter. METHODS: In a prospective observational study ten consecutive patients with SSc-related ILD were treated with iv CYC in a pulse regime lasting from 6 to 24 months. Clinical status, pulmonary functional testing (PFT) and high resolution computed tomography (HRCT) of the chest were evaluated at enrolment and 6, 12 and 24 months thereafter. After treatment withdrawal, patients were followed up every 6 months with PFT and chest HRCT to monitor lung disease. RESULTS: Clinical improvement was apparent in 8 out of 10 patients. The median values of forced vital capacity (FVC), forced expiratory volume in the first second (FEV1) and diffusion lung capacity for carbon monoxide (DLCO) as well as ground-glass pattern on HRCT did not change significantly after 6, 12 and 24 months of therapy. The follow-up continued in 8 out of 10 patients after treatment withdrawal for a median of 26.5 months (range: 12-48 months). The final median FVC was 54.5% of predicted value (interquartile range, IQR= 31.6%-94%). Only one patient suffered a FVC deterioration greater than 10%, even though less than 160 ml. The final median DLCO was 68% of predicted value (IQR=38.3-83.6%). Only 2 patients who developed pulmonary arterial hypertension deteriorated their DLCO values of more than 15%. CONCLUSIONS: An iv CYC pulse regimen over 24 months may stabilize pulmonary activity in patients with SSc-related ILD during the course of treatment and for a median of 26.5 months thereafter.
RESUMEN
OBJECTIVE: It has recently been reported that some autoimmune diseases seem to be associated with a functional polymorphism in PTPN22, a gene which encodes a phosphatase known to be important in T-cell signaling. The aim of our study was to check for the prevalence of the PTPN22 R620W polymorphism in patients with systemic sclerosis. METHODS: DNA samples from 54 systemic sclerosis patients and 55 healthy controls were obtained from peripheral blood and genotyping was performed by means of a restriction fragment length polymorphism analysis of PCR products (RFLP-PCR). RESULTS: Allele frequency for the T allele was slightly higher in the patients group (0.074 versus 0.055). Eight out of the 54 systemic sclerosis patients (14.8 %) were heterozygous for this single nucleotide polymorphism whereas the CT genotype was found in 6 out of the 55 controls (10.9%). Nevertheless, the difference did not reach statistical significance (p = 0.542). Neither certain antibodies linked to systemic sclerosis (anti-centromere and anti-topoisomerase I antibodies) nor any particular clinical involvement were associated with the polymorphism. CONCLUSION: This particular single nucleotide polymorphism of PTPN22 does not seem to be associated with systemic sclerosis.
Asunto(s)
Predisposición Genética a la Enfermedad , Polimorfismo de Longitud del Fragmento de Restricción , Polimorfismo de Nucleótido Simple , Proteínas Tirosina Fosfatasas/genética , Esclerodermia Sistémica/genética , Anticuerpos Antinucleares/sangre , Centrómero/inmunología , ADN-Topoisomerasas de Tipo I/inmunología , Femenino , Frecuencia de los Genes , Humanos , Reacción en Cadena de la Polimerasa , Proteína Tirosina Fosfatasa no Receptora Tipo 22 , Proteínas Tirosina Fosfatasas/metabolismo , Esclerodermia Sistémica/inmunología , Esclerodermia Sistémica/patologíaRESUMEN
Our objective was to determine the prevalence of antinuclear antibodies (ANAs) in patients with malignancies and to investigate if their presence might be related with development of musculoskeletal symptoms or paraneoplastic rheumatic syndromes. Antinuclear antibodies were determined by indirect immunofluorescence on Hep-2 cells in 274 neoplastic patients and in a control group of 140 age-adjusted healthy subjects. Antinuclear antibody specificities (anti-DNA and anti-ENA) were investigated in patients with rheumatological symptoms and positive ANA. Antinuclear antibodies were detected in 76 of 274 (27.7%) patients with malignancies and in nine of 140 (6.4%) healthy subjects. Twenty patients reported paraneoplastic rheumatic symptoms or syndromes. Two of them developed clinical symptoms mimicking rheumatoid arthritis (rheumatoid-like arthropathy), one systemic lupus erythematosus (lupus-like syndrome), one dermatomyositis and four cutaneous vasculitides. Musculoskeletal symptoms and paraneoplastic rheumatic symptoms and syndromes were both more frequently observed in patients with positive ANA. Antinuclear antibody specificities were found in only two cases. We can conclude that there is an increased incidence of antinuclear antibodies in malignant conditions. Musculoskeletal symptoms and rheumatic paraneoplastic symptoms and syndromes seem to be more frequent in patients with cancer-related positive ANAs. The failure to find ANA specificities (anti-ENA, anti-DNA) in patients with malignancies and positive ANAs in our study may simply reflect molecular differences between the autoantigens involved in cancer and those characteristically involved in the systemic autoimmune diseases.
Asunto(s)
Anticuerpos Antinucleares/metabolismo , Biomarcadores de Tumor/análisis , Enfermedades del Tejido Conjuntivo/diagnóstico , Neoplasias/diagnóstico , Síndromes Paraneoplásicos/diagnóstico , Enfermedades Reumáticas/diagnóstico , Adulto , Anciano , Anciano de 80 o más Años , Anticuerpos Antinucleares/análisis , Estudios de Cohortes , Enfermedades del Tejido Conjuntivo/sangre , Enfermedades del Tejido Conjuntivo/epidemiología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Neoplasias/sangre , Neoplasias/epidemiología , Síndromes Paraneoplásicos/sangre , Síndromes Paraneoplásicos/epidemiología , Pronóstico , Enfermedades Reumáticas/sangre , Enfermedades Reumáticas/epidemiología , Sensibilidad y EspecificidadRESUMEN
Our objective was to study the presence of microchimerism in a series of 47 female Spanish patients with scleroderma (SSc) and to compare with a control group. Polymerase chain reaction was used to identify Y-chromosome sequences in DNA extracted from peripheral blood cells. Y-chromosome sequences were found in DNA from peripheral blood cells in four out of 47 (8.5%) patients with scleroderma (two limited and two diffuse) and in two out of 40 (5%) healthy women (no statistical differences were found). When we compared SSc patients and healthy controls who had had at least one male child, four out of 29 (13.7%) and two out of 26 (7.6%) had microchimerism respectively (no statistically significant differences were found). Patients with both scleroderma and persistent microchimerism had had a male offspring. Foetal microchimerism does not seem to play a major role in most cases of female Spanish patients with SSc.
Asunto(s)
Quimera , Intercambio Materno-Fetal , Complicaciones del Embarazo/epidemiología , Esclerodermia Sistémica/epidemiología , Esclerodermia Sistémica/genética , Adulto , Cromosomas Humanos Y , Femenino , Humanos , Incidencia , Leucocitos Mononucleares , Masculino , Persona de Mediana Edad , Embarazo , EspañaRESUMEN
Las metástasis gastrointestinales extrahepáticas del cáncer de mama son poco frecuentes. Presentamos, por su rareza, un caso de metástasis de carcinoma ductal infiltrante de mama simulando un cáncer de colon obstructivo primario. Debe realizarse un correcto diagnóstico diferencial en pacientes con cáncer de mama que tienen síntomas o hallazgos radiológicos de cáncer de colon para evitar el tratamiento quirúrgico innecesario de la lesión metastásica como si fuese un segundo primario (AU)
Asunto(s)
Anciano , Femenino , Humanos , Carcinoma/cirugía , Carcinoma/diagnóstico , Carcinoma/complicaciones , Colon/cirugía , Colon/patología , Colon , Inmunohistoquímica/métodos , Metástasis de la Neoplasia , Neoplasias del Colon/diagnóstico , Neoplasias del Colon/complicaciones , Neoplasias de la Mama/complicaciones , Neoplasias de la Mama/diagnóstico , Neoplasias Gastrointestinales/clasificación , Neoplasias Gastrointestinales/epidemiología , Neoplasias Gastrointestinales/fisiopatologíaAsunto(s)
Enfermedades de los Nervios Craneales/complicaciones , Esclerodermia Sistémica/complicaciones , Nervio Trigémino/patología , Enfermedades de los Nervios Craneales/patología , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Esclerodermia Sistémica/patologíaRESUMEN
OBJECTIVE: To assess cardiovascular abnormalities in patients with limited systemic sclerosis (SSc), using noninvasive cardiac techniques. METHODS: Sixty-three patients with limited SSc were prospectively evaluated with Doppler echocardiography and thallium-201 perfusion scintigraphy after a cold-stress test and radionuclide ventriculography. RESULTS: In the patients with limited SSc, there was a significantly high prevalence of abnormal left- and right-diastolic function parameters (P = 0.001 and P = 0.0002, respectively), thickening of papillary muscles (46%; P = 0.003), and mild mitral regurgitation (49%; P < 0.0001), compared with controls. Systolic pulmonary arterial hypertension was detected in 9 patients (14%), and pericardial effusion in 11 patients (18%). In 64% of patients with limited SSc, an ischemic response was detected on the thallium cold-stress scan; similarly, an ischemic response was detected in 57% of patients with primary Raynaud's phenomenon (P < 0.0001 versus controls). CONCLUSION: Although the frequency of cardiovascular symptoms was low in patients with limited SSc, a significant rate of cardiovascular abnormalities was found by noninvasive cardiac techniques.