RESUMEN
OBJECTIVE: To analyse the prevalence, the clinical characteristics, the overall survival and the event-free survival (EFS) of SSc patients who express anti-U11/U12 RNP (RNPC-3) antibodies. METHODS: A total of 447 SSc patients from Barcelona (n = 286) and Milan (n = 161) were selected. All samples were tested using a particle-based multi-analyte technology. We compared anti-RNPC-3 positive and negative patients. Epidemiological, clinical features and survival were analysed. End-stage lung disease (ESLD) was defined if the patient developed forced vital capacity <50% of predicted, needed oxygen therapy or lung transplantation. EFS was defined as the period of time free of either ESLD or death. RESULTS: Nineteen of 447 (4.3%) patients had anti-RNPC-3 antibodies and interstitial lung disease (ILD) was more frequent (11, 57.9% vs 144, 33.6%, P =0.030) in individuals with anti-RNPC-3 antibodies. More patients reached ESLD in the positive group (7, 36.8% vs 74, 17.3%, P = 0.006), and a higher use of non-glucocorticoid immunosuppressive drugs was observed (11, 57.9% vs 130, 30.4%, P = 0.012). Anti-RNPC-3 positive patients had lower EFS, both in the total cohort (log-rank P =0.001), as well as in patients with ILD (log-rank P = 0.002). In multivariate Cox regression analysis, diffuse cutaneous subtype, age at onset, the presence of ILD or pulmonary arterial hypertension and the expression of anti-RNPC-3 positivity or anti-topo I were independently associated with worse EFS. CONCLUSION: The presence of anti-RNPC-3 was associated with higher frequency of ILD and either ESLD or death. These data suggest anti-RNPC-3 is an independent poor prognosis antibody in SSc, especially if ILD is also present.
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Autoanticuerpos/inmunología , Enfermedades Pulmonares Intersticiales/inmunología , Proteínas Nucleares/inmunología , Proteínas de Unión al ARN/inmunología , Esclerodermia Sistémica/inmunología , Adulto , Supervivencia sin Enfermedad , Femenino , Humanos , Enfermedades Pulmonares Intersticiales/mortalidad , Masculino , Persona de Mediana Edad , Pronóstico , Ribonucleoproteínas Nucleares Pequeñas , Factores de Riesgo , Esclerodermia Sistémica/mortalidad , Tasa de SupervivenciaRESUMEN
OBJECTIVES: To evaluate the clinical features and survival of patients with positive anti-RNA polymerase III (anti-RNAP III) in a Spanish single centre. METHODS: We analysed 221 patients with SSc according to LeRoy and Medsger criteria. Twenty-six patients with positivity for anti-RNAP III antibodies were compared with 195 negative patients. Epidemiological, clinical, immunological features and survival were analysed. RESULTS: In patients with anti-RNAP III positivity diffuse cutaneous SSc (dcSSc) subset was the most prevalent (20, 76.9% vs. 35, 17.9%, p < 0.001), with shorter diagnosis delay (4.11 ± 7.34 years vs. 6.77 ± 9.22 years, p = 0.005). Patients with anti-RNAP III antibodies had higher frequency of arterial hypertension (13, 50% vs. 55, 28.2%, p = 0.024), scleroderma renal crisis (SRC) (3, 11.5% vs. 3, 1.5%, p = 0.023), arthritis (9, 34.6% vs. 35, 17.9%, p = 0.046), tendon friction rubs (4, 15.4% vs. 1, 0.5%, p = 0.001) and contractures (5, 19.2% vs. 10, 5.1%, p = 0.02). There were no differences found in the presence of cancer or in global survival. In the multivariate survival analysis, severe interstitial lung disease (ILD) (HR: 8.61, 95%CI 3.40 - 21.81), pulmonary arterial hypertension (PAH) (HR: 4.05, 95%CI 1.42 - 11.61) and SRC (HR: 17.27, 95%CI 3.36 - 88.97) were the only factors associated with poor prognosis. CONCLUSIONS: In this cohort anti-RNAP III antibodies are related with dcSSc subset, shorter diagnostic delay and higher prevalence of musculoskeletal involvement, arterial hypertension and SRC. ILD, PAH and SRC were independent prognostic factors.
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Autoanticuerpos/inmunología , Enfermedades Pulmonares Intersticiales , ARN Polimerasa III/inmunología , Esclerodermia Sistémica , Adulto , Autoanticuerpos/sangre , Diagnóstico Tardío , Femenino , Humanos , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/inmunología , Enfermedades Pulmonares Intersticiales/metabolismo , Masculino , Persona de Mediana Edad , Esclerodermia Sistémica/diagnóstico , Esclerodermia Sistémica/inmunología , Esclerodermia Sistémica/metabolismo , EspañaRESUMEN
BACKGROUND: To determine whether lung involvement is related to microvascular perturbations, nailfold videocapillaroscopy (NVC) was performed in patients with systemic sclerosis (SSc). METHODS: A cross-sectional study was consecutively accomplished in 152 SSc patients. NVC, a pulmonary function test and echocardiography were undergone within a 3-month period. Finally, 134 patients with at least eight NVC (200× magnification) images were selected for quantitative and qualitative examinations. RESULTS: Patients with interstitial lung disease presented lower median capillary density (4.86/mm vs 5.88/mm, p = 0.005) and higher median of neoangiogenesis (0.56/mm vs 0.31/mm, p = 0.005). A higher quantity of neoangiogenesis capillaries was found in patients with pulmonary arterial hypertension (0.70/mm vs 0.33/mm, p = 0.008). Multivariate linear regression analysis established a correlation between neoangiogenesis and decreased forced vital capacity (FVC) (p < 0.001): for each capillary with neoangiogenesis visualized on average per 1 mm, FVC was 7.3% reduced. In qualitative NVC, a late pattern as defined by Cutolo was also associated with lower FVC (p = 0.018). The number of giant capillaries was associated with reduced diffusion capacity of the lung for carbon monoxide (DLCO) (p = 0.016); for each giant capillary per 1 mm, DLCO was 11.8% diminished. CONCLUSIONS: A good correlation was observed between distinctive quantitative and qualitative NVC features with lung functional parameters such as FVC and DLCO. It is suggested that vasculopathy could play a role in SSc lung involvement.
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Lesión Pulmonar/fisiopatología , Angioscopía Microscópica/métodos , Sistema Respiratorio/fisiopatología , Esclerodermia Sistémica/fisiopatología , Enfermedades Vasculares/fisiopatología , Adulto , Anciano , Capilares/fisiopatología , Estudios de Cohortes , Estudios Transversales , Ecocardiografía , Femenino , Humanos , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/fisiopatología , Pulmón/irrigación sanguínea , Pulmón/fisiopatología , Enfermedades Pulmonares Intersticiales/diagnóstico , Enfermedades Pulmonares Intersticiales/fisiopatología , Lesión Pulmonar/complicaciones , Lesión Pulmonar/diagnóstico , Masculino , Persona de Mediana Edad , Pruebas de Función Respiratoria/métodos , Esclerodermia Sistémica/complicaciones , Enfermedades Vasculares/complicaciones , Enfermedades Vasculares/diagnósticoRESUMEN
BACKGROUND: Pulmonary arterial hypertension (PAH) is one of the most relevant causes of death in systemic sclerosis. The aims of this study were to analyse the recently published DETECT algorithm comparing it with European Society of Cardiology/European Respiratory Society (ESC/ERS) 2009 guidelines: as screening of PAH; (2) identifying median pulmonary arterial pressure (mPAP) ≥21 mmHg; and (3) determining any group of pulmonary hypertension (PH). METHODS: Eighty-three patients fulfilling LeRoy's systemic sclerosis diagnostic criteria with at least right heart catheterization were studied retrospectively. Clinical data, serological biomarkers, echocardiographic and hemodynamic features were collected. SPSS 20.0 was used for statistical analysis. RESULTS: According to right heart catheterization findings, 35 patients with PAH and 28 with no PH met the standards for DETECT algorithm analysis: 27.0% of patients presented with functional class III/IV. Applying DETECT, the sensitivity was 100%, specificity 42.9%, the positive predictive value 68.6% and the negative predictive value 100%, whereas employing the ESC/ERS guidelines these were 91.4%, 85.7%, 88.9% and 89.3%, respectively. There were no missed diagnoses of PAH using DETECT compared with three patients missed (8.5%) using ESC/ERS guidelines. The DETECT algorithm also showed greater sensitivity and negative predictive value to identify patients with mPAP ≥21 mmHg or with any type of PH. CONCLUSIONS: The DETECT algorithm is confirmed as an excellent screening method due to its high sensitivity and negative predictive value, minimizing missed diagnosis of PAH. DETECT would be accurate either for early diagnosis of borderline mPAP or any group of PH.
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Algoritmos , Diagnóstico Precoz , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/etiología , Esclerodermia Sistémica/complicaciones , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Sensibilidad y EspecificidadRESUMEN
INTRODUCTION: Interstitial lung disease (ILD) is one of the major causes of death in systemic sclerosis (SSc). This study investigated exhaled breath (EB) and exhaled breath condensate (EBC) biomarkers in patients with SSc and analyzed their role as a prognostic tool in SSc-related ILD. METHODS: Fraction exhaled nitric oxide (FeNO) and exhaled carbon monoxide (eCO) measured in EB, together with pH, nitrite, nitrate and interleukin-6 levels measured in EBC were prospectively analyzed in 35 patients with SSc. Twelve patients had established ILD by chest high-resolution computed tomography (HRCT), and 23 patients showed no evidence of ILD. EB and EBC biomarkers were determined at inclusion, and pulmonary function tests were annually performed during 4 years of follow-up. RESULTS: No differences at baseline biomarkers levels were found between groups. In all patients studied, low EBC pH levels were associated with a decreased diffusing capacity for carbon monoxide (DLCO) during follow-up. Low FeNO levels were correlated with lower forced vital capacity (FVC) at baseline, 4years of follow-up and with a decrease in FVC and DLCO during monitoring. Among ILD patients, high eCO levels were correlated with lower baseline FVC. In the global cohort, a worse progression-free survival was identified in patients with EBC pH values lower than 7.88 and FeNO levels lower than 10.75ppb (Log Rank P=.03 and P<.01, respectively). CONCLUSIONS: EB and EBC could help to detect patients likely to present a deterioration on lung function during follow up.
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Enfermedades Pulmonares Intersticiales/metabolismo , Óxido Nítrico/metabolismo , Esclerodermia Sistémica/metabolismo , Adulto , Anciano , Biomarcadores/análisis , Pruebas Respiratorias , Espiración , Femenino , Humanos , Concentración de Iones de Hidrógeno , Enfermedades Pulmonares Intersticiales/etiología , Enfermedades Pulmonares Intersticiales/mortalidad , Masculino , Persona de Mediana Edad , Óxido Nítrico/análisis , Pronóstico , Estudios Prospectivos , Esclerodermia Sistémica/complicaciones , Esclerodermia Sistémica/mortalidad , Tasa de SupervivenciaRESUMEN
This study aims to evaluate the utility of magnetic resonance imaging (MRI) to assess interstitial lung disease (ILD) extent in patients with systemic sclerosis (SSc). Patients with SSc and varying degrees of ILD with a high-resolution computed tomography (HRCT), pulmonary function tests (PFTs), and a chest MRI containing an ultrafast SE sequence performed less than 1 year apart were included in the study. Wells global disease extent and Goh's staging algorithm were used to measure and categorize ILD both for MRI and HRCT. Correlation and diagnostic performance of MRI compared with HRCT and PFTs were calculated. Eighteen SSc patients were studied. MRI showed a good performance to detect ILD (AUC = 0.96) and was correlated with forced vital capacity (r = -0.60, p = 0.01), diffusing capacity of the lung for carbon monoxide (r = -0.79, p = 0.04), and also with HRCT (r = 0.85, p < 0.001), but MRI extent values were consistently lower than HRCT and, thus, not directly comparable. Goh's algorithm using HRCT and transformed to be used with MRI showed a good agreement (kappa = 0.73, p < 0.001) and MRI-measured ILD extent presented good intra-observer (ICC = 0.86) and inter-observer (ICC = 0.90) reliability. In SSc patients, MRI proved to be a good technique to detect and categorize ILD extent compared with HRCT, suggesting that it may be a valuable x-ray sparing technique for selected cases.
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Enfermedades Pulmonares Intersticiales/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Esclerodermia Sistémica/diagnóstico por imagen , Tórax/diagnóstico por imagen , Adulto , Anciano , Algoritmos , Femenino , Humanos , Enfermedades Pulmonares Intersticiales/etiología , Masculino , Persona de Mediana Edad , Reproducibilidad de los Resultados , Esclerodermia Sistémica/complicaciones , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND AND OBJECTIVES: Primary biliary cirrhosis (PBC) is associated to any systemic autoimmune disease (SAD), in particular systemic sclerosis (SSc). To investigate the prevalence of SAD in a cohort of patients with PBC, specifically the prevalence of SSc and its clinical subtypes, and determining the clinical and biological profile of patients with associated PBC and SSc. METHODS: Observational study of 62 patients with PBC following a protocol that included an anamnesis and physical examination to detect the presence of SAD as well as a nailfold capillaroscopy and an immunological study with specific SSc autoantibodies. A comparative analysis was conducted between patients with isolated PBC and patients with PBC and an associated SAD. RESULTS: SAD was associated to PBC in 22 patients (35,4%), and SSc was the most frequent illness, identified in 13 cases (21%). Five patients (8%) without previous diagnosis of SAD fulfilled pre-scleroderma criteria, according to LeRoy and Medsger criteria. The presence of anticentromere antibodies (54,5% vs. 5%, P<.001) was the unique immunological determination identified more frequently in patients with PBC-SAD. The SSc suggestive capillary pattern was visualized in 11 patients (20,4%), mainly the slow pattern. No factors associated with greater morbi-mortality were identified in the PBC-SAD group. CONCLUSIONS: It does exist a subgroup of patients with PBC and clinical-biological features suggestive of an SAD, which advise a protocolized study to detect early the association to an SAD.
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Enfermedades Autoinmunes/diagnóstico , Cirrosis Hepática Biliar/etiología , Angioscopía Microscópica , Esclerodermia Sistémica/diagnóstico , Adulto , Anciano , Anciano de 80 o más Años , Enfermedades Autoinmunes/complicaciones , Enfermedades Autoinmunes/epidemiología , Femenino , Humanos , Cirrosis Hepática Biliar/diagnóstico , Cirrosis Hepática Biliar/fisiopatología , Masculino , Persona de Mediana Edad , Prevalencia , Pronóstico , Estudios Retrospectivos , Esclerodermia Sistémica/complicaciones , Esclerodermia Sistémica/epidemiologíaRESUMEN
OBJECTIVE: To analyse the differences in SSc clinical features and survival in patients aged > or = 65 years compared with young SSc patients. METHODS: Of a total of 319 SSc patients, we identified 67 (21%) patients aged >65 years. Demographical data such as SSc subsets, the cutaneous complaint, internal organ involvement and the causes of morbidity and mortality were collected. Results of the elderly and young patients were compared. RESULTS: There were 61 (91%) women and 6 (9%) men aged > or = 65 years. The limited SSc (lSSc) subset was more prevalent in elderly than in young patients (74.6 vs 54%, P = 0.002). Pulmonary disease (86.6% in elderly vs 73.8% in young patients, P = 0.034) and cardiac involvement (70.1% in elderly vs 49.6% in young patients, P = 0.004) were significantly more prevalent in elderly patients. In contrast, signs of oesophageal involvement (43.3% in elderly vs 57.5% in young patients, P = 0.040) were less frequent in aged patients. In addition, pulmonary and heart disease appeared significantly earlier after the diagnosis in patients aged > or = 65 years. Mortality was significantly higher in elderly than in young patients (35.8 vs 19%, P = 0.005), but when standardized mortality ratios (SMRs) were analysed, there was no significant mortality increase in the elderly. CONCLUSION: In elderly patients, the lSSc subset is more prevalent than the diffuse. Pulmonary and cardiac involvement are more prevalent in aged patients and appears sooner after the disease diagnosis. SSc is clearly related to increased mortality, although it is not significant in the elderly group.
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Cardiopatías/fisiopatología , Enfermedades Pulmonares/fisiopatología , Esclerodermia Sistémica/fisiopatología , Adulto , Factores de Edad , Anciano , Anciano de 80 o más Años , Causas de Muerte , Progresión de la Enfermedad , Femenino , Cardiopatías/complicaciones , Cardiopatías/mortalidad , Humanos , Enfermedades Pulmonares/complicaciones , Enfermedades Pulmonares/mortalidad , Masculino , Persona de Mediana Edad , Esclerodermia Sistémica/complicaciones , Esclerodermia Sistémica/mortalidad , Índice de Severidad de la Enfermedad , Tasa de SupervivenciaRESUMEN
OBJECTIVES: To study the presence and characteristics of nailfold capillary changes in a cohort of adult patients with inflammatory myopathies, determine correlations with disease activity and severity, and investigate any relationship between capillary findings and the immunological or clinical characteristics of the groups. METHODS: Fifty-three consecutive adult patients followed in our outpatient clinic were evaluated using a Wild M3 stereomicroscope with an Intralux 5000 Volpi cold light lamp. A semiquantitative rating scale was used to score capillaroscopy changes. Disease activity and severity were assessed with the Myositis Disease Activity Assessment Tool and Myositis Damage Index, respectively. Associations between capillaroscopy findings and other factors were calculated with the chi(2) and Mann-Whitney U tests. Serum autoantibody profile was determined in all patients. RESULTS: Twenty-three patients (43%) showed relevant capillaroscopy changes. No significant association was observed between the number of capillaroscopy alterations and the clinical or immunological groups, or disease duration. Disease activity and severity were both significantly associated with a larger number of capillaroscopy findings (P < 0.05). The combination of microhemorrhages and capillary enlargement was significantly more frequent in patients with dermatomyositis (OR, 8.9; 95% CI, 1.8-45.2), and a characteristic capillaroscopy pattern was associated with paraneoplastic myositis (OR, 14.7; 95% CI, 2.0-106.4). Interstitial lung disease significantly correlated with higher capillary score (OR, 3.7; 95% CI, 1.1-13.0). CONCLUSIONS: Nailfold microcirculation as determined by semiquantitative simple capillaroscopy appears to provide useful information in patients with idiopathic inflammatory myopathy, contributing to an early diagnosis and identifying patients with a poor prognosis.
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Capilares/patología , Microcirculación/fisiología , Microscopía/métodos , Miositis/diagnóstico , Uñas/irrigación sanguínea , Adulto , Anciano , Capilares/fisiopatología , Estudios de Cohortes , Estudios Transversales , Dermatomiositis/diagnóstico , Dermatomiositis/patología , Dermatomiositis/fisiopatología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Miositis/patología , Miositis/fisiopatología , Polimiositis/diagnóstico , Polimiositis/patología , Polimiositis/fisiopatología , Pronóstico , Índice de Severidad de la EnfermedadRESUMEN
INTRODUCTION: Presacral tumors are a rare and little known pathology. We present our experience in its surgical treatment. METHODS: Analysis of a series (1995-2008). RESULTS: 20 patients (14 women and 6 men), average age 46 (29-71) years. Patients with benign tumors were younger (43.5+/-10.2) years that those who had malignant tumors (62+/-10.7; p = 0.002). The median duration of symptoms was 1 year (2 month-50 years). The first symptom was suppuration, palpable mass or constipation in 10 cases or constipation, abdominal pain in 4, back pain in 4 and a casual finding in 3. The surgical approach was perineal in 9 (45%), abdominal in 8 (40%) and combined in 3 (15%) cases. Fifteen (75%) tumors were benign, with teratomas being the most frequent (5 cases). Another 5 were malignant: 2 chordomas, 1 malignant cystic teratoma, 1 malignant mucinous cystadenocarcinoma and 1 lymphoma. Mean postoperative stay was 6.6+/-5 days. There were post-surgical complications in 5 (20%) patients. After an average follow-up of 3.5+/-4 years, 2 patients died (malignant teratoma and cystadenocarcinoma, respectively) due to morbidity and to a non-resectable recurrence, and a benign schwannoma has recurred, the rest being free of disease. CONCLUSIONS: Surgical treatment by a group of surgeons experienced in perineal and pelvic surgery can obtain good results with few complications, with the exception of some malignant tumors, depending on the infiltration of adjacent structures and their histological type.
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Neoplasias/cirugía , Región Sacrococcígea , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana EdadRESUMEN
OBJECTIVE: To evaluate the results of the treatment of patients with thoracic esophageal perforation in order to determine the most appropriate management of this entity. PATIENTS AND METHOD: We performed a retrospective study of 21 patients (mean age 59 years; 24-82) who presented with thoracic esophageal perforation to our hospital between 1991 and 2004. RESULTS: In 13 patients (62%) treatment was performed within 24 hours. In the remaining 8 patients the mean delay was 7.2 (2-12) days. In 4 patients (26%) the perforation was confined to the mediastinum and conservative treatment was provided. Of these patients, 1 developed empyema and underwent esophageal resection. Extramediastinal involvement was confirmed in 17 patients (73%) and was treated by a variety of surgical procedures: esophagectomy (n=2), drainage alone (n=2), primary closure (n=2) and reinforced primary repair (n=11). Two patients with simple closure and 1 with reinforced primary closure developed leakage of the suture line resulting in death. The 3 patients who underwent esophagectomy survived. In patients with perforation confined to the mediastinum mortality was 0%, whereas in those with extramediastinal involvement mortality was 23%. CONCLUSIONS: Thoracic esophageal perforation leads to high mortality rates and requires early diagnosis and immediate treatment. Conservative management is appropriate in only a few selected patients. When surgical treatment is indicated, we advocate reinforced primary repair regardless of the interval between injury and operation, except when the esophagus is in such poor condition that esophagectomy is the only option.