Capi-score: a quantitative algorithm for identifying disease patterns in nailfold videocapillaroscopy.

OBJECTIVES: The European Alliance of Associations for Rheumatology (EULAR) supports the use of nailfold videocapillaroscopy (NVC) to identify disease patterns (DPs) associated with systemic sclerosis (SSc) and Raynaud's phenomenon (RP). Recently, EULAR proposed an easy-to-manage procedure, a so-called Fast Track algorithm, to differentiate SSc from non-SSc patterns in NVC specimens. However, subjectivity among capillaroscopists remains a limitation. Our aim was to perform a software-based analysis of NVC peculiarities in a cohort of samples from SSc and RP patients and, subsequently, build a Fast Track-inspired algorithm to identify DPs without the constraint of interobserver variability. METHODS: NVCs were examined by 9 capillaroscopists. Those NVCs whose DPs were consensually agreed (≥2 out of 3 interobservers) were subsequently analysed with an in-house developed software. Each variable's results were grouped according to the consensually agreed DPs in order to identify useful hallmarks to categorise them. RESULTS: Eight-hundred and fifty-one NVCs (21 957 images) whose DPs had been consensually agreed were software-analysed. Appropriate cut-offs set in capillary density and percentage of abnormal and giant capillaries, tortuosities and hemorrhages allowed DP categorization and the development of the CAPI-Score algorithm. This consisted of 4 rules: Rule 1, SSc vs non-SSc, accuracy 0.88; Rules 2 and 3, SSc-early vs SSc-active vs SSc-late, accuracy 0.82; Rule 4, non-SSc normal vs non-SSc non-specific, accuracy 0.73. Accuracy improved when the analysis was limited to NVCs whose DPs had achieved full consensus among interobservers. CONCLUSIONS: The CAPI-Score algorithm may become a useful tool to assign DPs by overcoming the limitations of subjectivity.

Lupus nephritis with preserved kidney function associated with poorer cardiovascular risk control: A call for more awareness.

BACKGROUND: Despite the improvement in the prognosis of lupus nephritis (LN), the cardiovascular morbimortality remains high. The early recognition and remission of flares, while trying to avoid the metabolic adverse effects of medication, must be mandatory. AIM: The aim of our study was to assess the cardiovascular (CV) risk profile in a cohort of lupus patients with preserved kidney function after a nephritis episode, compared to patients without a nephritis flare. METHODS: 130 patients diagnosed of SLE (32 with previous nephritis flare and 98 without) were studied in order to evaluate the CV risk profile, despite the preserved kidney function. RESULTS: The most prevalent risk factors were sedentary lifestyle (57.6%), overweight/obesity (38.3%) and dyslipidemia (36%), followed by smoking (32%) and hypertension (16%). Though more than a half (53.1%) was taking CV medication, a high percentage did not reach a therapeutic target value, especially regarding obesity (11.5%) and cholesterol levels (LDL-C of 16%). The prevalence of dyslipidemia (53.1% vs 30.6%), smoking (46.6% vs 27.5%), left ventricular hypertrophy (LVH) (21.4% vs 6.4%) and lower HDL-C (48.6mg/dL vs 55.4mg/dL) were significantly different in the group with previous nephritis flare. Moreover, young patients with lupus nephritis, received more pulses of corticosteroids and cyclophosphamide, had higher prevalence of hypertension, LVH, higher proteinuria, hospital admissions and waist circumference, constituting the subgroup of patients with greater aggregation of CV risk factors. CONCLUSIONS: Patients with previous nephritis flare showed a poor control of CV risk factors despite the preserved renal function, these patients would require a closer therapeutic management.

Takayasu's arteritis associated with Crohn's disease

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Guía de información al paciente sobre la enfermedad tromboembólica venosa

Contiene: qué es la enfermedad tromboembólica venosa, si es un problema frecuente y es grave, cuáles son sus causas, si puede ser un problema hereditario, cómo se manifiesta, si es necesario realizar alguna prueba diagnóstica, si es necesario ingresar en el hospital, si puede ser tratado en casa, qué debe saber para ser tratado en su domicilio, cómo se trata, qué precauciones hay que tener con el tratamiento, cómo puede prevenirse, qué secuelas pueden quedarle y cómo puede prevenirlas.

[Upper limb deep venous thrombosis. Risk factors, outcome, and posthrombotic syndrome]. / Trombosis venosa profunda de miembros superiores. Factores de riesgo, evolución y síndrome postrombótico.

OBJECTIVE: Our objective was that of describing a series of episodes of upper limb deep venous thrombosis, with special emphasis on the incidence of posthrombotic syndrome and its impact in the quality of life of the patients. METHODS: 33 episodes (29 patients) of upper limb deep venous thrombosis were studied, between January 1995 and January 2002; episodes until September 1999 (18 episodes) were considered retrospectively while those which took place starting in this date (15 episodes) were considered prospectively. In all, 24 patients were cared in hospital clinic. It was possible to obtain complete data in 16 cases, in which evolution toward posthrombotic syndrome, venous revascularization, and impact of the pathological process in the global quality of life of patients were studied. RESULTS: Average age of patients was 58.9 years. In 25 episodes (75.8%) some risk factor for venous thrombosis existed, the presence of a venous catheter being the most frequent. Subclavian axillary affectation was predominant (18 episodes, 55%) and in the left side (25 episodes, 76%). Twenty-eight episodes were diagnosed with echography (84.8%). Mean D-dimers concentration (N = 16) was 1,046 ng/ml (standard deviation [SD]: 826.9). One of the episodes (3%) was associated to lung thromboembolism. Treatment consisted on the administration of low molecular weight heparin during the acute phase in 70% of the cases. In 13 episodes (39%) additional treatment of thrombosis (endovascular in most) was carried out. In 29 episodes (88%) chronic treatment was administered with acenocoumarol, while in 4 episodes (12%) the administration of low molecular weight heparin was kept. No patient died during hospitalization. In 15 patients (52%) control of revascularizacion was performed. We detected complete revascularization in 6 patients (40%), revascularization with collateral circulation in 4 patients (27%), partial revascularization in other 4 patients (27%) and presence of permeability but with expansion and tortuosity of deep venous system in 1 case (6%). Posthrombotic syndrome was considered mild or nonexistent in 10 patients (62.5%) and moderate in 6 patients (37.5%). Quality of life of patients with moderate posthrombotic syndrome was significantly worse than that of patients with mild posthrombotic syndrome (73 vs 90, p < 0.035). CONCLUSIONS: Most of upper limb deep venous thrombosis are associated to the use of intravenous catheters. Echography is the primary diagnostic technique. At the time of the diagnosis, patients tend to show elevation in D-dimers levels. Evolution toward posthrombotic syndrome is frequent; this syndrome does not use to be serious but indeed influences the quality of life of patients.

Quiste hidatídico pélvico [Imagen de la semana] / Pelvic hydatid cyst [Weekly image]

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Trombosis venosa profunda de miembros superiores. Factores de riesgo, evolución y síndrome postrombótico / Upper limb deep venous thrombosis

Objetivo. Nos propusimos describir una serie de episodios de trombosis venosa profunda de miembro superior, con especial énfasis en la incidencia posterior de síndrome postflebítico y surepercusión en la calidad de vida de los pacientes. Métodos. Se estudiaron 33 episodios (29 pacientes) de trombosis venosa profunda del miembro superior entre enero de 1995 y enero de 2002, incluidos retrospectivamente hasta septiembre de 1999 (18 episodios) y prospectivamente a partir de esta fecha (15 episodios). Se analizaron los factores de riesgo, la clínica, los procedimientos diagnósticos y el tratamiento administrado. Fueron controlados en la policlínica24 pacientes. Se dispuso de datos completos evaluables en16 casos, estudiándose la evolución a síndrome postflebítico, la repermeabilización venosa y la repercusión del proceso en la calidad de vida global de los pacientes. Resultados. La edad media fue de 58,9 años. En 25 episodios(75,8%) se apreció algún factor de riesgo para trombosisvenosa, siendo el más frecuente la presencia de un catéter venoso. Predominó la afectación axilosubclavia (18 episodios,55%) y del lado izquierdo (25 episodios, 76%). Veintiocho episodios (84,8%) se diagnosticaron con ecografía. La media de los niveles de D-dímeros (n=16) fue de 1.046 ng/ml.(DE: 862,9). En un episodio (3%) se asoció tromboembolismo pulmonar. El 70% de los casos se trató con heparina de bajo peso molecular en la fase aguda. En 13 episodios (39%) se practicó tratamiento adicional de la trombosis (endovascularla mayoría). En 29 episodios (88%) se administró tratamiento crónico con acenocumarol y en 4 (12%) se continuó con heparina de bajo peso molecular. No falleció ningún paciente durante el ingreso hospitalario. Se practicó control de repermeabilización en15 pacientes (52%). Detectamos repermeabilización completa en6 pacientes (40%), repermeabilización con circulación colateral en 4 (27%), repermeabilización parcial en otros 4 pacientes(27%) y eje permeable pero con dilatación y tortuosidad del sistema venoso profundo en un caso (6%). El síndrome postflebítico se clasificó como leve o ausente en 10 pacientes(62,5%) y moderado en 6 (37,5%). La calidad de vida de los pacientes con síndrome posflebítico moderado fue significativamente peor que la de los pacientes con síndrome postflebítico leve (73 frente a 90, p<0,035).Conclusiones. Las trombosis venosas profundas de miembros superiores más frecuentes se asocian al uso de catéteres intravenosos. La ecografía es la técnica diagnóstica predominante. Los D-dímeros se elevan al diagnóstico. La evolución a síndrome postflebítico es frecuente, pero éste no suele ser grave. aunque sí repercute en la calidad de vida de los pacientes

Objective. Our objective was that of describing a series of episodes of upper limb deep venous thrombosis, with special emphasis on the incidence of posthrombotic syndrome and its impact in the quality of life of the patients. Methods. 33 episodes (29 patients) of upper limb deep venous thrombosis were studied, between January 1995 and January2002; episodes until September 1999 (18 episodes) were considered retrospectively while those which took place starting in this date (15 episodes) were considered prospectively. In all,24 patients were cared in hospital clinic. It was possible to obtain complete data in 16 cases, in which evolution toward posthrombotic syndrome, venous revascularization, and impact of the pathological process in the global quality of life of patients were studied. Results. Average age of patients was 58.9 years. In25 episodes (75.8%) some risk factor for venous thrombosis existed, the presence of a venous catheter being the most frequent. Subclavian axillary affectation was predominant(18 episodes, 55%) and in the left side (25 episodes, 76%).Twenty-eight episodes were diagnosed with echography (84.8%).Mean D-dimers concentration (N = 16) was 1,046 ng/ml(standard deviation [SD]: 826.9). One of the episodes (3%) was associated to lung thromboembolism. Treatment consisted on the administration of low molecular weight heparin during the acute phase in 70% of the cases. In 13 episodes (39%)additional treatment of thrombosis (endovascular in most)was carried out. In 29 episodes (88%) chronic treatment was administered with acenocoumarol, while in 4 episodes (12%)the administration of low molecular weight heparin was kept. No patient died during hospitalization. In 15 patients (52%)control of revascularización was performed. We detected complete revascularization in 6 patients (40%),revascularization with collateral circulation in 4 patients (27%),partial revascularization in other 4 patients (27%) and presence of permeability but with expansion and tortuosity of deep venous system in 1 case (6%). Posthrombotic syndrome was considered mild or nonexistent in 10 patients (62.5%) and moderate in 6 patients (37.5%). Quality of life of patients with moderate posthrombotic syndrome was significantly worse than that of patients with mild posthrombotic syndrome (73 vs 90,p<0,035).Conclusions. Most of upper limb deep venous thrombosis are associated to the use of intravenous catheters. Echography is the primary diagnostic technique. At the time of the diagnosis, patients tend to show elevation in D-dimers levels. Evolution toward posthrombotic syndrome is frequent; this syndrome does not use to be serious but indeed influences the quality of life of patients

[Cranial hyperostosis as a metastasic adenocarcinoma presentation form]. / Hiperostosis craneal como forma de presentación de un adenocarcinoma metastásico.

Hyperostosis is a volume-unit osseous increase of very diverse etiology. We present the case of a 68-year woman with a cranial hyperostosis debuting with frontal protrusion, headache and neurologic symptoms. Image proves demonstrated a hyperostosis in the calotte and meningeal enhancement, without intracerebral lesions nor malignant cells in the cerebrospinal fluid. Analytic data were unspecific. Cranial biopsy showed huge neoplastic infiltration in bone and meninges. Primary site remained unknown after a CAT and a mammography.

Hiperostosis craneal como forma de presentación de un adenocarcinoma metastásico / Cranial hyperostosis as a metastasic adenocarcinoma presentation form

La hiperostosis es un aumento de masa ósea por unidad de volumen de etiología muy diversa. Presentamos el caso de una mujer de 68 años con hiperostosis craneal que debutó con clínica de protusión frontal derecha, cefalea y sintomatología neurológica. Las pruebas de imagen demostraron la existencia de hiperostosis de calota con afectación meníngea, sin lesiones cerebrales ni células malignas en líquido cefalorraquídeo. Los datos analíticos eran inespecíficos. La biopsia craneal mostró amplia infiltración neoplásica por adenocarcinoma metastásico tanto en hueso como en meninges. No se localizó el tumor primario tras realizarse TAC y mamografía (AU)

La enfermedad de Rendu-Osler-Weber. Estudio descriptivo de 17 casos / Rendu-Osler-Weber disease. Descriptive study of 17 cases

Objetivo. Estudiar la prevalencia y formas de presentación de la telangiectasia hemorrágica hereditaria (THH) en la población de nuestro ámbito hospitalario.Material y métodos. Se realizó un estudio descriptivo retrospectivo de los pacientes diagnosticados de THH entre los años 1972 y 1999 en el Hospital La Fe de Valencia. Se valoró la presencia de antecedentes familiares, epistaxis de repetición, telangiectasias cutaneomucosas y lesiones viscerales, así como los tratamientos administrados y la evolución de la enfermedad. Resultados. Se diagnosticaron 17 pacientes en dicho período con edades comprendidas entre los 24 y los 80 años. Ocho pacientes presentaban antecedentes familiares. Los síntomas más frecuentes fueron la epistaxis y la anemia ferropénica, seguidas en frecuencia por las telangiectasias cutaneomucosas. Seis pacientes presentaron manifestaciones neurológicas, cinco pulmonares, cinco gastrointestinales y tres pacientes hepáticas, generalmente secundarias a malformaciones vasculares a esos niveles.Conclusiones. La prevalencia de la enfermedad puede ser más elevada de lo que había sido descrito anteriormente. Ante pacientes con epistaxis de repetición y/o anemias ferropénicas hay que tener en cuenta esta enfermedad y buscar la presencia de telangiectasias que puedan orientar al diagnóstico. El diagnóstico precoz de la THH puede ayudar al control temprano de malformaciones viscerales asociadas. El tratamiento de la THH de ser individualizado según las manifestaciones clínicas del paciente (AU)

Trombopenia asociada a enfermedad mixta del tejido conectivo y esplenectomía laparoscópica / Laparoscopic esplenectomy and thrombocytopenia associated to mixed connective tissue disease

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[Rendu-Osler-Weber disease. Descriptive study of 17 cases]. / La enfermedad de Rendu-Osler-Weber. Estudio descriptivo de 17 casos.

OBJECTIVE: To study the prevalence and presentation forms of hereditary hemorrhagic telangiectasia (HHT) among the population in our hospital area. MATERIALS AND METHODS: Descriptive, retrospective study of patients with the diagnosis of HHT from to 1999 at La Fe Hospital, Valencia. Family history, repeated epistaxis, mucocutaneous telangiectasis and visceral lesions, as well as administered treatments, and clinical course were evaluated. RESULTS: Seventeen patients were diagnosed during such time period, with ages ranging from 23 to 80 years. Eight patients had family histories. The most common symptoms included epistaxis and iron-deficiency anemia, followed by muco-cutaneous telangiectasis. Six patients had neurological, five pulmonary, five gastrointestinal, and three hepatic manifestations, usually secondary to vascular malformations at those levels. CONCLUSIONS: The prevalence of the disease maybe higher than previously reported. This disease should be considered in patients with repeated epistaxis and/or iron-deficiency anemia. The presence of telangiectasis that can suggest the diagnosis should be investigated. An early diagnosis of HHT can be useful for the early control of associated visceral malformations. Treatment of HHT should be tailored according to the clinical manifestations of the patient.

[Association of multiple myeloma and solid neoplasms: analysis of 13 cases]. / Asociación de mieloma múltiple y neoplasias sólidas: análisis de trece casos.

BACKGROUND: The diagnosis of second malignancies in patients with multiple myeloma is uncommon. It is debatable whether this tumor is in itself a risk factor for the incidence of second malignancies. Etiopathogenic factors which might account for this association were analyzed. MATERIALS AND METHODS: Retrospective analysis of a series of 210 patients with myeloma controlled by a Medical Oncology Department from 1984 to 1998. After searching for the diagnosis of a second malignancy, thirteen patients were identified with both diagnoses. A descriptive statistical study was elaborated as well as an analysis of survival. RESULTS: There were seven males and six females, with a mean age at diagnosis of 69 years (57-80). The Durie-Salmon stages at diagnosis were: I-A (3), I-B (1), II-A (4), III-A (4). Associated solid malignancies included: hepatocarcinoma (2), prostate adenocarcinoma (2), soft tissue sarcoma (2), lung adenocarcinoma (1), cholangiocarcinoma (1), breast carcinoma (1), endometrial carcinoma (2) and bladder carcinoma (1). At diagnosis, seven of the solid malignancies were metastatic. At the time of this analysis, eleven patients had died, eight because of progression of the solid cancer, two because of progression of myeloma and one because of therapy toxicity. Two patients are still alive with stability of the myeloma and solid malignancy controlled after surgical resection. CONCLUSIONS: The association was observed in 6.2% of patients with myeloma and occurs at an advanced age. IgG myelomas and in early stages predominated. Solid malignancies were diagnosed in advanced stages in most cases. A short term high mortality rate was observed due to progression of the solid malignancy. The frequency of the association did not seem to be higher than the overall incidence of second malignancies in patients with cancer.

[Bronchial granular cell myoblastoma associated with epidermoid carcinoma]. / Mioblastoma bronquial de células granulares asociado a carcinoma epidermoide.

A 43-year-old male simultaneously presented a bronchial granular cell myoblastoma affecting the bronchi of the lower right lobe, and a bronchogenic epidermoid carcinoma with mediastinal spread. In both cases diagnosis was established through bronchial and mediastinal biopsies, with posterior immunohistochemical evaluation to confirm the nature of the neoplastic lesions. The clinicopathological characteristics of this rare observation not previously reported in our country are commented.