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espanolLas vasculitis de gran vaso afectan predominantemente a la arteria aorta y sus ramas y se clasifican en dos entidades principales: la arteritis de celulas gigantes (ACG) y la arteritis de Takayasu (ATK). La ACG suele presentarse con cefalea temporal de reciente comienzo, claudicacion mandibular e hipersensibilidad del cuero cabelludo. Algunos pacientes presentaran tambien sintomas visuales (amaurosis fugaz y neuritis optica anterior) y manifestaciones sistemicas como la polimialgia reumatica, la fiebre y la perdida de peso. La biopsia de arteria temporal es una ayuda importante en su diagnostico. Otras herramientas son la ecografia de arterias temporales, la tomografia por emision de positrones combinada con tomografia computarizada (PET-TC) y la angiorresonancia magnetica (RM). Los glucocorticoides (GC) son el pilar del tratamiento de la ACG, reservando la asociacion de tocilizumab o metotrexato para pacientes con recurrencias o con toxicidad importante relacionada con los corticoides. La aparicion de sintomas en la ATK tiende a ser subaguda, lo que a menudo conduce a un retraso diagnostico. El diagnostico clinico de la ATK puede ser realizado en base a hallazgos clinicos sugestivos (sintomas constitucionales, carotidinia, hipertension, pulsos disminuidos o ausentes y/o soplos arteriales en pacientes de menos de 40 anos) y una prueba de imagen que muestre el estrechamiento vascular de la aorta y/o sus ramas primarias. La angio-TC ayuda a detectar complicaciones de la vasculitis y la PET/TC al evaluar la respuesta al tratamiento. El pilar del tratamiento de la ATK son los GC sistemicos, recomendando la asociacion precoz de un farmaco ahorrador de esteroides debido a la alta tasa de recaida. EnglishLarge-vessel vasculitis predominantly affects the aorta and its branches and is classified into two main entities: giant cell arteritis (GCA) and Takayasu arteritis (TKA). GCA usually presents with new-onset temporal headache, jaw claudication and scalp hypersensitivity. Some patients also have visual symptoms (amaurosis fugax and anterior optic neuritis) and systemic manifestations such as polymyalgia rheumatica, fever and weight loss. Temporal artery biopsy is an important diagnostic aid. Other tools include temporal artery ultrasound, positron emission tomography combined with computed tomography (PET-CT) and magnetic resonance angiography (MRA). Glucocorticoids (GC) are the mainstay of treatment for GCA, reserving combining with tocilizumab or methotrexate for patients with recurrences or significant corticosteroid-related toxicity. The onset of symptoms in TKA tends to be subacute, often resulting in diagnostic delay. A clinical diagnosis of TKA can be based on suggestive clinical findings (constitutional symptoms, carotid artery disease, hypertension, decreased or absent pulses and/or arterial murmurs in patients under 40 years of age) and an imaging test showing vascular narrowing of the aorta and/or its primary branches. Angio-CT helps to detect complications of vasculitis and PET/CT to assess response to treatment. Systemic GCs are the mainstay of treatment for TKA, and the early combination of a steroid-sparing drug due to the high rate of relapse.

The RIETE Investigators., [Background] The optimal therapy of patients with acute subsegmental pulmonary embolism (PE) is controversial., [Methods] We used the RIETE (Registro Informatizado Enfermedad TromboEmbólica) database to compare the rate of symptomatic PE recurrences during anticoagulation in patients with subsegmental, segmental, or more central PEs. [Results] Among 15 963 patients with a first episode of symptomatic PE, 834 (5.2%) had subsegmental PE, 3797 (24%) segmental, and 11 332 (71%) more central PE. Most patients in all subgroups received initial therapy with low‐molecular‐weight heparin, and then most switched to vitamin K antagonists. Median duration of therapy was 179, 185, and 204 days, respectively. During anticoagulation, 183 patients developed PE recurrences, 131 developed deep vein thrombosis (DVT), 543 bled, and 1718 died (fatal PE, 135). The rate of PE recurrences was twofold higher in patients with subsegmental PE than in those with segmental (hazard ratio [HR], 2.13; 95% confidence interval [CI], 1.16‐3.85) or more central PE (HR, 1.89; 95% CI, 1.12‐3.13). On multivariable analysis, patients with subsegmental PE had a higher risk for PE recurrences than those with central PE (adjusted HR, 1.75; 95% CI, 1.02‐3.03). After stratifying patients with subsegmental PE according to ultrasound imaging in the lower limbs, the rate of PE recurrences was similar in patients with DVT, in patients without DVT, and in those with no ultrasound imaging. [Conclusions] Our study reveals that the risk for PE recurrences in patients with segmental PE is not lower than in those with more central PE, thus suggesting that the risk of PE recurrences is not influenced by the anatomic location of PE.

We report the case of a 77-year-old male with a history of aortic stenosis and interstitial lung disease, who debuted 3 years ago with an outbreak of necrotic and very painful canker sores. The severity of the lesions and their refractory response to treatment led to several hospital admissions and multiple consultations to different specialists (ENT, rheumatology, dermatology, ophthalmology, cardiology, and internal medicine). During this time, the patient received central parenteral nutrition with an episode of catheter-related septicemia, and he came to require psychiatric assistance for autolytic ideation. Numerous diagnostic tests were performed with inconclusive results, including biopsy of the lesion (histological study, immunohistochemistry for CD68 + , CD4 + , CD8 + , CD20 + , MCT +, and cytomegalovirus, PAS, Grocott–Gomori and Zielh–Neelsen staining, and in situ hybridization for Epstein Barr virus). Numerous treatments were unsuccessfully tested until thalidomide was administered, thus completely remitting lesions but leaving retractable scarring sequelae. Since then, the patient has had two recurrences, coinciding with the reduction of thalidomide dosages, which were controlled by increasing the dose of the immunomodulator. Recurrent necrotizing major aphthous stomatitis (Sutton’s disease) is a clinical variant of recurrent aphthous stomatitis that may have a dramatic course. Unfortunately, the lack of etiopathogenetic uniformity precludes any specific treatment. In severe cases, immunomodulators, including thalidomide, may represent a valid therapeutic option.

Essentials Recent randomized trials suggested fewer bleeding events in fragile patients with VTE receiving DOACs.The frequency, clinical characteristics and outcome of these patients have not been reported in real life.Fragile patients with VTE had a higher risk for major bleeding or death and a lower risk for recurrences than non‐fragile. Background Subgroup analyses from randomized trials suggested favorable results for the direct oral anticoagulants in fragile patients with venous thromboembolism (VTE). The frequency and natural history of fragile patients with VTE have not been studied yet. Objectives To compare the clinical characteristics, treatment and outcomes during the first 3 months of anticoagulation in fragile vs non‐fragile patients with VTE. Methods Retrospective study using consecutive patients enrolled in the RIETE (Registro Informatizado Enfermedad TromboEmbolica) registry. Fragile patients were defined as those having age ≥75 years, creatinine clearance (CrCl) levels ≤50 mL/min, and/or body weight ≤50 kg. Results From January 2013 to October 2016, 15 079 patients were recruited. Of these, 6260 (42%) were fragile: 37% were aged ≥75 years, 20% had CrCl levels ≤50 mL/min, and 3.6% weighed ≤50 kg. During the first 3 months of anticoagulant therapy, fragile patients had a lower risk of VTE recurrences (0.78% vs 1.4%; adjusted odds ratio [OR]: 0.52; 95% confidence intervals [CI]: 0.37‐0.74) and a higher risk of major bleeding (2.6% vs 1.4%; adjusted OR: 1.41; 95% CI: 1.10‐1.80), gastrointestinal bleeding (0.86% vs 0.35%; adjusted OR: 1.84; 95% CI: 1.16‐2.92), haematoma (0.51% vs 0.07%; adjusted OR: 5.05; 95% CI: 2.05‐12.4), all‐cause death (9.2% vs 3.5%; adjusted OR: 2.02; 95% CI: 1.75‐2.33), or fatal PE (0.85% vs 0.35%; adjusted OR: 1.77; 95% CI: 1.10‐2.85) than the non‐fragile. Conclusions In real life, 42% of VTE patients were fragile. During anticoagulation, they had fewer VTE recurrences and more major bleeding events than the non‐fragile.

Background Evidence-based recommendations do not adequately address the treatment of right heart thrombi in patients who present with acute symptomatic pulmonary embolism. Methods This study included patients who had acute pulmonary embolism associated with right heart thrombi and participated in the Registro Informatizado de la Enfermedad TromboEmbolica registry. We assessed the effectiveness of anticoagulation versus reperfusion treatment for the outcomes of all-cause mortality, pulmonary embolism–related mortality, recurrent venous thromboembolism, and major bleeding rates through 30 days after initiation of pulmonary embolism treatment. We used propensity score matching to adjust for the likelihood of receiving reperfusion treatment. Results Of 325 patients with pulmonary embolism and right heart thrombi, 255 (78%; 95% confidence interval, 74-83) received anticoagulation and 70 (22%; 95% confidence interval, 17-26) also received reperfusion treatment. Propensity score–matched pairs analyses did not detect a statistically lower risk of all-cause death (6.2% vs 14%, P = .15) or pulmonary embolism–related mortality (4.7% vs 7.8%; P = .47) for reperfusion compared with anticoagulation. Of the patients who received reperfusion treatment, 6.2% had a recurrence during the study follow-up period, compared with 0% of those who received anticoagulation ( P = .049). The incidence of major bleeding events was not statistically different between the 2 treatment groups (3.1% vs 3.1%; P = 1.00). Conclusions In patients with pulmonary embolism and right heart thrombi, no significant difference was found between reperfusion therapy and anticoagulant therapy for mortality and bleeding. The risk of recurrences was significantly higher for reperfusion therapy compared with anticoagulation. Right heart thrombi may not warrant riskier interventions than standard anticoagulation.

Background The benefits of a diagnostic workup for occult cancer in patients with VTE are controversial. Our aim was to provide and validate a risk score for occult cancer in patients with VTE. Methods We designed a nested case-control study in a cohort of patients with VTE included in the RIETE (Registro Informatizado Enfermedad TromboEmbolica) registry from 2001 to 2014. Cases included cancer detected beyond the first 30 days and up to 24 months after VTE. Control subjects were defined as patients with VTE with no cancer in the same period. Results Of 5,863 eligible patients, 444 (7.6%; 95% CI, 6.8%-8.2%) were diagnosed with occult cancer. On multivariable analysis, variables selected were male sex, age > 70 years, chronic lung disease, anemia, elevated platelet count, prior VTE, and recent surgery. We built a risk score assigning points to each variable. Internal validity was confirmed using bootstrap analysis. The proportion of patients with cancer who scored ≤ 2 points was 5.8% (241 of 4,150) and that proportion in those who scored ≥ 3 points was 12% (203 of 1,713). We also identified scores divided by sex and age subgroups. Conclusions This is the first risk score that has identified patients with VTE who are at increased risk for occult cancer. Our score needs to be externally validated.

Patient registries are useful tools for assessing rare diseases. Our objective is to present the Spanish registry of patients with systemic lupus erythematosus (Registro español de pacientes con lupus eritematoso sistémico, RELES).RELES was started in 2008 as an observational, prospective, multicentre cohort registry that included patients from the time they were diagnosed. The registry's objective is to analyse the incidence and noninflammatory complications of systemic lupus erythematosus (SLE). The departments of internal medicine of 38 Spanish hospitals participate in this registry.A total of 298 patients with a mean age of 40.8±15.7 years were included, 88.9% of whom were women and 85.6% of whom were white. In the first visit, there was a predominance of joint manifestations (74.5%). One hundred and seventy-seven patients (59.4%) were positive for anti-native DNA. In these patients, there was a higher rate of lupus nephritis (26.7% vs. 14%, p=.009; relative risk [RR], 1.33), haemolytic anaemia (13.6% vs. 4.1%, p=.07; RR, 1.46) and lymphopenia (55.4% vs. 43.8%, p=.05; RR, 1.21). The median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI 2K) score was 9.64 points (interquartile range, 4-13). The patients treated with antimalarial drugs before the diagnosis of SLE had a median SLEDAI score in the first visit of 5, compared with 8 for those who were not treated with these drugs (p=.02).RELES constitutes the first Spanish patient cohort with SLE recorded from the time of the diagnosis. The presence of anti-DNA has been related to severe manifestations such as nephritis and haemolytic anaemia. Treatment with antimalarial drugs before the diagnosis was associated with less active disease at the initial presentation.

Objetivo Evaluar las caracteristicas epidemiologicas, clinicas, microbiologicas y pronosticas de la bacteriemia por Enterococcus faecalis. Metodos Estudio retrospectivo de historias clinicas de pacientes con bacteriemia por E. faecalis en un periodo de 7 anos (enero 1995-diciembre 2001). Resultados Se documentaron 95 episodios de bacteriemia: el 83,2% de adquisicion intrahospitalaria, el 85,3% en relacion con procedimientos invasivos previos y el 9,5% en neonatos. El 57,9% de los sujetos tenia una enfermedad subyacente y el 41,1% habia recibido previamente antibioticos de amplio espectro sin actividad frente al enterococo. El 32,6% de las bacteriemias se considero primaria, y en el resto los focos de infeccion mas frecuentemente asociados fueron cardiovasculares, intraabdominales, urogenitales y pulmonares. El estudio de resistencias demostro un solo caso de resistencia a ampicilina y ninguno a glucopeptidos. La mortalidad global fue del 23,9%, aunque solo en el 9,9% se considero directamente atribuible a la bacteriemia. En el analisis multivariante el tratamiento inadecuado y la ventilacion mecanica fueron factores de mal pronostico. Conclusiones En nuestro medio la bacteriemia por E. faecalis es fundamentalmente una infeccion nosocomial que afecta a pacientes con graves enfermedades subyacentes sometidos a procedimientos invasivos y tratamientos antibioticos previos de amplio espectro. La ampicilina sigue siendo el tratamiento de eleccion. La inadecuacion del tratamiento antibiotico empirico inicial y la ventilacion mecanica son factores de mal pronostico.

Graft intolerance syndrome (GIS) is a common complication developed in failed kidney allografts left in situ when the patients returned to hemodialysis. GIS usually develops within the first 6 months after immunosuppression has been withdrawn. When medical treatment has failed, transplantectomy is the conventional therapy. Nevertheless, in recent years, transvascular ethanol embolization has been reported as an effective, safe, and less invasive technique than transplantectomy for the management of patients with GIS. Although infrequent, the most severe complication is infection of the graft or surrounding tissues, which usually appears in the first weeks after the procedure. We present the first case of late infection of an embolized renal graft, more than 2 years after embolization.

Although cocaine abuse is an increasingly important medical problem, many manifestations of its toxicity are not well understood. The aim of this study is to review the most serious clinical manifestations related to cocaine abuse.We reviewed the medical records of all patients over 16 years of age admitted to the hospital from January 1994 to December 2005 where cocaine abuse was recorded in their clinical history.A total of 170 patients, with 188 episodes and 268 pathologic manifestations related to cocaine abuse were included. Thirty two out of the 170 patients (18.8%) were females. Mean age was 33 +/- 11 years, with no significant difference between males and females. A total of 88.8% were smokers, 70% had alcohol abuse and 67.3% had other illegal drug abuses. The more frequent reason for their hospitalization was: pulmonary infection (29.6%), bronchial hyperreactivity (14%), acute psychotic attack related to drugs (12%), ischemic heart disease (10%), infectious endocarditis (7.8%), cerebrovascular disease (8.6%), seizures (6.2%) and severe abdominal complications (2.3%). Eight patients died (6.25%). The association with cocaine was only suspected in 46% of the toxic manifestations.Several clinical manifestations are associated to cocaine abuse and its consumption causes potentially fatal complications. The integral treatment of these patients could be improved if these complications are kept in mind.

Removal of graft with intravenous antibiotics is traditionally considered the most effective treatment of vascular graft-associated infections. However, an increasing number of reports suggests that this complication can be effectively treated without graft removal.A prospective study to evaluate the outcome of conservative management of vascular graft-associated infection seen in our center was performed. The diagnosis of graft-associated infection was based on microbiological tests, and imaging studies. In our study, patients were hemodynamically stable with functioning vascular graft as per clinical and imaging evaluations. Conservative management included antibiotic treatment and local debridement without removal of vascular graft.Fourteen patients with vascular graft-associated infection were enrolled in our study. Eleven out of 14 patients (78.6%) responded successfully to conservative management. Treatment failure was reported in 3 patients, 2 of whom required graft removal. After a two-year follow-up, these 14 patients were clinically stable without recurrence of infection.Our study has revealed that conservative management of vascular graft-associated infection seems to be effective and should be considered in hemodynamically stable patients with functioning vascular graft.

We performed a retrospective study about patients diagnosed of spinal epidural abscess without concurrent spondylodiscitis, collected in the last five years in our hospital. We describe four patients, all of them males, with ages ranged from 59 to 67 years old. All patients presented fever, elevated phase reactants and spinal pain, with cord compression in three of them. Two patients had risk factors: diabetes mellitus and non-Hodgkin lymphoma. S. aureus was isolated in three patients, and S. agalactiae in the other one. The posterior epidural space was the most frequently affected, with variable location along the spinal cord. Three patients required surgical operation, and the other one was treated only with antibiotics. All patients demonstrated a complete clinical, analytical and radiological resolution.

Fundamento: Conocer la evolucion de las neumonias desde 1995 a 2001. Metodo: Los datos se obtuvieron del Conjunto Minimo Basico de Datos, seleccionando tres causas de hospitalizacion por neumonias en la Comunidad Valenciana, bronconeumonia, neumonia por organismo no especificado y neumonia neumococica. El analisis estadistico se realizo mediante el programa SPSS, hallando las incidencias de las neumonias y su evolucion en el tiempo y observando interacciones entre las variables genero, edad, estancia y procedencia. Resultados: La incidencia fue de 209 por 100.000 habitantes, aumentando significativamente a lo largo del periodo hasta estabilizarse a partir de 1999. La neumonia afecta mas a hombres que a mujeres, sobre todo en mayores de 65 anos. Por edades, son mas frecuentes en menores de 5 anos y en mayores de 65, concentrandose el mayor numero de casos en 76-77 anos. Se han observado diferencias estadisticamente significativas en las incidencias por provincias. El numero de estancias es mayor en hombres que en mujeres y aumentan por grupos de edad, siendo mas elevadas en los mayores de 65 anos. La estancia media, varia segun la edad, sin existir diferencias significativas entre genero o grupo de edad. Conclusiones: La neumonia, patologia originada por diferentes causas, afecta mas a hombres que a mujeres, a menores de 5 anos y a mayores de 65. Las incidencias se estabilizan en los ultimos anos de estudio. Se deberian seguir estas investigaciones para dilucidar si las diferentes neumonias en este periodo, evolucionan igualmente y si se dan interacciones entre las variables estudiadas

Analysis of Enterococcus faecalis bacteremia epidemiological, clinical, microbiological and prognostic characteristics.Retrospective analysis of clinical records of patients with E. faecalis bacteremia throughout 7 years (January 1995-December 2001).95 episodes of bacteremia were documented, 83.2% with nosocomial origin, 85.3% associated to previous invasive procedures and 9.5% in neonates. 57.9% patients suffered an underlying disease and 41.1% had received previously broad-spectrum antibiotics without activity against enterococcus. 32.6% bacteremia episodes was considered primary and, in the rest, the most frequent associated sources of infection were cardiovascular, intra-abdominal, urogenital, and lung. The resistance study showed a single case of resistance to ampicillin and none to glucopeptides. Global mortality was 23.9%, although only in 9.9% was directly attributable to bacteremia. Inadecuate treatment and mechanical ventilation were factors of poor prognosis in the multivariate analysis.E. faecalis bacteremia is in our environment essentially a hospital-acquired infection in patients with severe underlying diseases, subject to invasive procedures, and previously treated with wide spectrum antibiotherapy. Ampicillin continues to be the treatment of choice. Inappropriateness of the initial empirical antibiotic treatment and mechanical ventilation are factors of poor prognosis.

The etiology of multiple sclerosis (MS) is currently unknown. Different viruses have tentatively been involved as causative agents of MS that would trigger an autoimmune response leading to demyelination plaques. There is controversy regarding the role that the human herpesvirus 6 (HHV-6) might play in this condition, and high antibody titers have been detected to HHV-6. HHV-6 DNA has also been detected by PCR both in blood and cerebrospinal fluid by means of the Polymerase Chain Reaction (PCR). Immunohistochemistry studies were performed with histologic specimens from the white matters of patientes with MS. All this has led some authors to incriminate this virus as the triggering etiologic agent of this disease.CSF specimens from 23 patients with Relapsing-Remitting MS were studied. The CSF specimens from 23 patients undergoing rachianesthesia were used as controls, and none of them had neurologic disorders. A nested PCR was performed in the collected specimens to detect specific DNA sequences of HHV-6.No DNA sequences of HHV-6, EBV, VZV, CMV and HSV were detected in the tested specimens.No HHV-6 DNA sequences were detected from CSF specimens of patients with MS. Further investigations on the association between HHV-6 and MS should be performed to elucidate the role of HHV-6 in the pathogenesis of this disease.

Histiocytic necrotizing lymphadenitis, Kikuchi Fujimoto's disease (KFD) is characterised by fever and lymphadenopathy, usually large cervical, unilateral lymph nodes. Such clinical presentation demands a work-up to exclude serious medical conditions like malignancy and infections. Foci of necrosis with lymphocytic Histiocytic predominance in association with scarce polymorphonuclear cells on lymph node examination, confirm the diagnosis of KFD. Many different patterns of computed tomographic (CT) appearance of KFD have been reported. We describe the CT scan finding in two patients with this disease. All our cases showed, after two and three weeks of evolution respectively, enlarged lymph nodes with hypodense centres and peripheral ring enhancement. These radiological alterations correlated with the central lymph node necrosis found in the anatomopathological studies. In conclusion, KFD should be considered in patients with fever, cervical lymph node enlargement and CT scan showing hypointense centres and peripheral ring enhancement.

Background 85-90% of patients with systemic sclerosis (SSc) are women, so there are few series that have evaluated distinguishing clinical features in men (1). Objectives To describe clinical features of the male subgroup of a cohort of patients diagnosed with scleroderma in our hospital and evaluate the differences within the female subgroup. Methods Encoded patients with diagnosis of SSc in our hospital from 1985 until December 2013 were collected. They were classified into four groups using a modification of LeRoy and Medsger proposed criteria (2): diffuse systemic sclerosis (dcSSc), limited systemic sclerosis (lcSSc), systemic sclerosis sine scleroderma (ssSSc) and pre-scleroderma (pre-SSc). Medical records were reviewed, recording clinico-epidemiological data and results of immunological tests. Results Of the 114 patients diagnosed with SSc in this period, 17 (15%) were male, the group described below: 10 (59%) were classified as dcSSc and 7 (41%) as lcSSc. Mean age at diagnosis was 49 years. 9 (53%) had a history of exposure to SSc related toxic - 8 silica, 1 vinyl chloride-. All patients had positive ANA, 3 ACA, 8 Scl70, 3 dsDNA, 2 antiRo, 1 antiLa, 1 antiRNP and 1 antiSm. Capillaroscopy was performed in 3 patients (18%), finding a scleroderma pattern throughout, with a presence in all three of giant capillaries and capillary loss and ramified capillaries in two. Along the follow-up (mean 11 years), 15 patients (88%) had Raynaud9s phenomenon, 10 (59%), dyspnea, 9 (53%) ulcers, 7 (41%) telangiectasia, and 4 (23%) digestive disorders. Interstitial lung disease was documented in 10 patients (55%) and PAH in 3 (16%). 12 patients (71%) died during the monitoring, an average of 10 years after initial diagnosis. The cause of death was: respiratory failure in 4 (33%), heart failure in 2 (17%), carcinoma in 3 (25%), cirrhosis in 1 (8%) and unknown in 2 (17%). Multivariate analysis comparing men with women subgroup, showed in male less age at diagnosis (p 0.017) and death (p 0.001), a predominance therein of exposure to toxic (p 0.05), dcSSc (p 0.003), Scl70 positivity (p 0.004), interstitial lung disease (p 0.002), and presence of giant capillaries (p 0.05), capillary loss (p 0.02) and ramified capillaries (p 0.003) in nailfold capillaroscopy. No statistically significant difference was found in mortality or survival time from diagnosis. Conclusions 1. In more than half of our male patients with SSc, a related toxic was found, mainly silica. 2. Our male subgroup was differentiated clinically in an younger occurrence of the disease, a predominance of dcSSc, positivity for Scl70, presence of interstitial lung disease, and advanced endothelial damage capillaroscopic patterns. References J.-B. Gaultier et al. Systemic sclerosis in men. La Revue de medecine interne 2008: 181–186. LeRoy EC, Medsger TA, Jr. Criteria for the classification of early systemic sclerosis. J Rheumatol. 2001;28(7):1573-6. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.2014

Hereditary hemochromatosis is the most common inherited disorder in white population (2-8 cases per 1000 habitants). Hemochromatosis is characterized by increased intestinal absorption of iron leading to its deposition into multiple organs. An early diagnosis and proper management with frequent phlebotomies are known to improve life expectancy and quality of life. Diagnosis is suggested by an elevated Transferrin saturation (TS) (more than 60%).Prospective study of the level of TS among 1131 healthy workers, who came to the Security and Hygiene Official Centre for their annual revision had been undertaken.Twenty-wo workers had high TS; in 10 of them the increase of TS was confirmed on repeated determinations. Liver biopsy was performed in six (and refused by the other four), eventually a diagnosis of hemochromatosis was confirmed in three (in-group prevalence of 2.6 per 1000 people).In our experience, TS is the most appropriate initial screening test for detecting hereditary hemochromatosis in a normal population.

Objectives To evaluate the clinical significance of Streptomyces isolates in different clinical samples. Material and methods Review of the records of all cases of Streptomyces isolated from any clinical sample at a tertiary Hospital, during a seven-year period. Results Streptomyces was isolated from 13 patients. All of them had underlying diseases. Only in one patient Streptomyces was considered to have a pathogenic role in the clinical picture. We report the third case of catheter-related infection caused by this microorganism. Conclusions Streptomyces is usually isolated from patients with underlying diseases. Before considering them significative, Streptomyces isolates must be interpreted in the clinical context.

We had reviewed all the patients with Histiocytic Necrotizing Lymphadenitis (Kikuchi's disease) seen in a General Hospital during two years. Three of the six cases were young females who showed the same clinical symptoms: Fever and unilateral painful, lymphadenopathy, usually at the latero-cervical region. All patients have mild neutropenia and high levels of serum transaminases. The lymph node biopsy yielded the proper diagnostic in all cases. The course of disease was good, and all patients healed without treatment one to two month after the symptom started. To date, we have not recorded any relapse. Our finding are quite similar to the other cases published in the medical literature. The benign course of this disease, and the need for biopsy to get a correct diagnosis, can explain why this disease may be unrecognized in clinical practice.

To determine the prevalence and the clinical and serological findings of neurosyphilis in HIV-infected patients,Treponema pallidum hemagglutination (TPHA) tests, CD4+ lymphocyte counts and determination of rapid plasma reagin (RPR) titers were performed in 972 HIV-infected patients over a period of 3.5 years. Patients were scored according to the Centers for Disease Control's classification for HIV infection. Reactive serum syphilis tests and positive cerebrospinal fluid (CSF)-Venereal Disease Research Laboratory (VDRL) tests, with or without clinical symptoms, were used as the criteria for diagnosis of neurosyphilis. The TPHA test was positive in 31 patients, representing 3.1 % of all HIV-infected patients included in the study. Of these, 13 were intravenous drug addicts, 14 were homosexuals and 4 were heterosexuals. Diagnosis of syphilis was concurrent with HIV infection in 19 patients, prior to HIV infection in 6 patients and after HIV infection in 6 patients. CSF examinations were performed in 28 of the 31 (90.3 %) patients with serologically evident syphilis. Four patients had positive CSF-VDRL tests with pleocytosis (23.5 % of untreated syphilis patients in whom CSF was examined), three of whom reported mild headache, which was considered a doubtful manifestation of neurosyphilis. Patients with syphilis diagnosed and treated prior to diagnosis of HIV infection did not have evidence of neurosyphilis. Seven patients had pleocytosis with a negative CSF-VDRL test, without any clinical manifestations of neurosyphilis. There was no significant difference in the mean CD4+ lymphocyte count between patients with and without neurosyphilis (p=0.5). RPR titers in neurosyphilis patients were greater than those in patients previously treated for syphilis and in those with pleocytosis only (p=0.046 and 0.036, respectively). All neurosyphilis patients had an RPR titer > 1∶8. After therapy, neurosyphilis patients had negative CSF-VDRL tests with a lower level of pleocytosis. The prevalence of neurosyphilis was 0.4% in HIV-infected patients and 23.5% in HIV-infected patients with untreated syphilis. This high prevalence of neurosyphilis warrants CSF examination in HIV-infected patients with syphilis, regardless of the stage of syhilis.

We describe the case of a 70-years-old woman with ischemic-hemorrhagic lesions in the cutaneous surface of both feet and analytical data of disseminated intravascular coagulation, in which the studies conducted were negative except for the presence of cryofibrinogen in plasma. We also review the clinical manifestations and the diseases associated to cryofibrinogenemia previously described in the literature.

Seventeen cases of cerebral abscesses undergoing surgery were reviewed, underlying the characteristics of predisposing factors, infectious sources, microbiological and radiological studies, as well as clinical and evolutive aspects. The average age of the patients was 34 years, with a higher incidence in the second (35%) and sixth (22%) decades. The young patients (40 years) showed a greater frequency of adjacent infectious sources (83%) and the older patients (40 years), distant infectious sources (75%). The average time gap between the onset of symptoms and the diagnosis was 7 +/- 13 days. CAT showed in all the patients typical hypodense images with a peripheral ring; three patients had multiple abscesses and the remainder, single abscesses. In 12 patients (70.5%), microbiological cultures were positive, 3 (25%) for aerobe germs, 7 (50.3%) for anaerobe germs, 1 (8.33%) for multiple germs and 1 (8.33%) for fungi. Eleven patients underwent surgical drainage, four of which required latter exeresis. Six other patients underwent exeresis as the only surgical treatment. One patient died and the remainder showed a positive evolution. The hospital length of stay was 42.3 +/- 52.3 days. The most frequent sequela was the epilepsia present in 23.5% of the patients. Our findings are similar to the results of recent works, although in our series, there is a higher frequency of anaerobe germs. No differences were observed between the surgical techniques used nor between the past and current antibiotherapy patterns.

To Study the main characteristics of intestinal tuberculosis (ITB), comparing patients with and without infection by HIV (HIV and non-HIV, respectively).The clinical records of patients diagnosed of this disease in the past five years in our center, were retrospectively reviewed. We used as inclusion criteria the histological and/or microbiological corroboration of the intestinal anatomical piece or the bacteriological and/or histological corroboration of tuberculosis at any other location with clinical and radiological signs compatible with intestinal affection. Two groups were established according to the serological results for HIV: HIV and non-HIV.We studied seven cases, three of them infected by the HIV. The mean age of HIV patients was 23 years, whereas the mean age of non-HIV patients was 49 years. Three non-HIV patients referred pulmonary tuberculosis when they were young. The interval between the onset of symptoms and the diagnosis in HIV patients was 45 days, ranging in the non-HIV patients between one month and four years. All the patients had abdominal pain. Six patients, including three HIV, had fever and constitutional syndrome. Thoracal radiography showed tuberculosis activity, bacteriologically demonstrated, in a HIV patient and in a non-HIV patient. Except one HIV-patient, the remainder were laparotomized. In all the HIV patients, intestinal tuberculosis was suspected upon admission, but this was not the case in the four non-HIV patients. CAT was the most useful of all the supplementary explorations conducted. Retrospectively, only four laparotomies were justified, although before this procedure, four patients were incorrectly oriented, one of them infected by the HIV. All the patients showed a good response to an specific treatment.Laparotomy is still a frequent diagnostic method. The characteristics of the HIV patients are similar to the ones of the non-HIV patients. The main differences are: younger ages and shorter time of evolution until diagnosis in the HIV group, and evidence of former or current pulmonary tuberculosis in the non-HIV group.

Describe the clinico-evolutionary characteristics of cases of primary antiphospholipid syndrome (PAPLS) diagnosed at our center.Retrospective study of all patients with circulating anticoagulant type lupus (AL) (62 patients) and/or anticardiolipin antibodies (ACLA) found in our center since 1981 and 1988, respectively. TTPA, Kaolin's test (KT), and Russel's viper venom time (RVVT) and ACLA type IgG and IgM by ELISA: Only those patients meeting the criteria for PAPLS are included.Twenty-four patients were diagnosed with PAPLS: Eleven patients were from the enlarged TTPA group, 11 of the total with positive ACLA, and two by KT and abnormal RVVT. As for gender, 18 were women. In the past three years, 19 patients were diagnosed. The diagnostic criteria were from the very beginning: venous thrombosis in nine patients, arterial thrombosis in four, aborted repetitions in three, and plateletopenia in eight. Six patients presented pulmonary thromboembolism, the cause of death in one patient. Thrombopenia was manifested in the evolutionary course of 14 patients, only one of which being severe. Two patients have died (one of them from neoplasia), and two others have had antiphospholipid antibodies become negative. Seven patients remain without treatment, the others undergoing anti-aggregating or anti-coagulant treatment.Plateletopenia, alone or accompanied by other symptoms, is the most common alteration of PAPLS in our area, followed by venous thrombosis which caused death by pulmonary thromboembolism in one patient. Arterial thrombotic events and aborted repetitions are by no means infrequent.

A retrospective study of patients infected by Salmonella typhi (ST) was conducted in the southern area of Pontevedra during a 17-month period in order to assess the clinical and evolutive characteristics and the pattern of antibiotic susceptibility of the infection. ST was isolated from samples of 30 patients: 25 hemocultures, 7 coprocultores, 1 urine and 1 aortic aneurysm. Nineteen cases had consumed non-sanitary controlled water, 95% from rural areas. The clinical characteristics were similar to the ones traditionally described. Seven patients (23%) developed complications and their presence was associated to a poorer prognosis. We did not observe any resistance to the commonly used antibiotics. In summary, the infection by ST is still a health problem in our environment, probably associated to insufficient measures in the enloration of water for human consumption.

The presence of high serum concentrations of immunoglobulins and hypocomplementemia is commonly observed in alcoholic patients; however, the mechanism behind their production is unknown. We studied 70 subjects (52 alcoholics and 18 healthy controls) prospectively. All the patients were active drinkers who consumed more than 100 gr of ethanol daily for at least 10 years. A biopsy of the liver was performed for all the subjects, along with immunoglobulins, C3, C4, CH100 and tests of liver function. Of the 52 alcoholics in the study, 20 showed minimal changes in hepatic steatosis (group II), 6 isolated severe alcoholic hepatitis (group III), 11 cirrhosis of the liver without alcoholic hepatitis (group IV), and 15 cirrhosis with alcoholic hepatitis (group V). There were no significant differences between the various groups according to age or quantity and duration of intake. The highest concentrations of immunoglobulins was observed in those with cirrhosis of the liver (p0.001) regardless of the degree of inflammation. The best correlation was found between IgA and liver histology (r = 0.64; p0.001). Hypocomplementemia was a factor only in those patients belonging to histological groups IV and V. Both the immunoglobulins and the complement proteins were narrowly correlated with the tests of liver function, and its alterations were more pronounced in patients with cirrhosis in Child's stage C. We conclude that the hypergammaglobulinemia and hypocomplementemia observed in the alcoholic patients are conditioned fundamentally by the degree of deterioration in liver function, indicated by cirrhosis of the liver upon presentation.

Testicular adrenal-like tissue (TALT) have been observed in patients with congenital adrenal hyperplasia, and is usually associated with 21-hydroxylase deficiency; in 3 cases with 11 beta-hydroxylase deficiency. We report a case of male pseudohermaphroditism with 17 alpha-hydroxylase deficiency (17OHD) who also had TALT. To our knowledge, this is the first report about the association of 17OHD and TALT. Also, the patient had high levels of serum aldosterone--an unusual finding in patients with 17OHD. A possible pathogenic mechanism is discussed.