Perspectiva histórica de la clasificación de las vasculitis / Historical perspective on the classification of vasculitis

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[Crohn s disease: is still the reason of fever of unknown origin?]. / Enfermedad de Crohn: ¿todavía hoy causa de fiebre de origen desconocido clásica?

We present two patients with Crohn's disease who presented with fever unknown origin, and mild intestinal symptoms. In case 1, the debut was with intermittent fever and symmetrical polyarthritis of the wrists, elbows, ankles and knees; in the case 2, prolonged fever associated to unspecific colicky abdominal pain. The initial approach was fever unknown origin yielded no etiology in both of them. The barium studies of the intestinal tract of paramount importance to reach a positive diagnosis in both cases. We strongly recommend the use of barium studies as a first line diagnostic tool in the approach of fever unknown origin.

[Mixed hereditary polyposis with atypical extracolonic manifestations]. / Poliposis hereditaria mixta con manifestaciones extracolónicas atípicas.

We present the case of a 31 year-old-man with mixed hereditary polyposis and atypical extracolonic manifestations, as patent ductus arteriosus and mental retardation, with cranial hyperostosis. This is an extremely uncommon polyposis syndrome and has a moderate risk to progress to colon cancer.

[Fever of unknown origin: differences in two different clinical series in a universitary hospital]. / Fiebre de origen desconocido clásica: cambios en dos series asíncronas en un hospital universitario.

We compare two clinical series of patients with fever of unknown origin (FUO) during two different periods of time (1982-1989 and 1997-2004) in the department of Internal Medicine at La Paz University Hospital, Madrid. We have analysed the changes in aetiology and diagnostic methods in these separated periods. We describe a decrease in the incidence of this clinical entity which we think to be due to the improvement of the diagnostic methods. This provides an earlier diagnosis and so patients do not reach criteria for classical FUO. Due to these improvements we have also assessed a change in the final diagnoses and the means used to reach them which, in general, have been less interventionist.

ANCA-associated vasculitis as paraneoplastic syndrome with colon cancer: a case report.

Coexistence of a vasculitis and a neoplastic disease is rare and the pathogenesis is unknown. Most of these associations refer to leukocytoclastic or poliarteritis nodosa (PAN)-type vasculitis and hematological malignancies. There are few reports of vasculitis in patients with solid tumours and there are also few reports of paraneoplastic ANCA-associated vasculitis. We report a case of p-ANCA-positive vasculitis with peripheral nerve involvement associated with a colon cancer. Vasculitis resolved after corticoid treatment and surgical removal of the tumour.

[Nonalcoholic steatohepatitis, the enigma of bad progression]. / Esteatohepatitis no alcohólica, el enigma de una mala evolucion.

Still, very little is known about the precise pathogenetic mechanisms, the triggering events and in particular, the evolution and treatment of nonalcoholic steatohepatitis (NASH). It is part of the broad spectrum of nonalcoholic fatty liver diseases (NAFLDs). Mainly, it has been reported as a benign disease, associated with metabolic disorders commonly occurrence en the general population. Nevertheless, the syndrome can lead to cirrhosis, liver failure or hepatocellular carcinoma, requiring liver transplantation. We present one patient with diagnosis of NASH, who was treated initially for overweight, HTA and hyperlipaemia with incompleted response and who showed a quickly progress to cirrhosis but no cause of liver decompensated disease could be identified. Currently she is at end-stage waiting a liver transplantation. Controlled and multicentric studies with the same definition of NASH and the study end-points are needed, and will provide information about diagnosis features and novel therapies to early management of the disease.

Computed tomography findings of peritoneal tuberculosis: systematic review of seven patients diagnosed in 6 years (1996-2001).

OBJECTIVE: The aim of this study was to describe the computed tomography (CT) findings in patients with demonstrated peritoneal tuberculosis (TB) and their concordance with the three types from the traditional classification (wet, fibrotic, and dry plastic). METHODS: We reviewed the CT images of all patients with microbiologically proven peritoneal tuberculosis over a 6-year period (1996-2001). RESULTS: Seven patients were included. Ascites was present in 5 patients (free ascites in 3 patients and loculated in 2). Involvement of the mesentery was found in 5 patients, the omentum in 4, and the parietal peritoneum in 3. Tuberculous lymphadenitis was the most common associated finding (6 patients). Two patients had hepatic lesions. The fibrotic type was found in all the patients, and 5 patients had an association of the fibrotic and wet types. None of the patients had lesions consistent with the dry plastic type. CONCLUSION: Peritoneal tuberculosis is best described as a combination of ascites, peritoneal lesions, and lymphadenopathy, rather than the three types from the traditional classification.

Fabry's disease: long-term study of a family.

Fabry's disease is the second most prevalent lysosomal storage disorder after Gaucher's disease. It occurs as the result of a deficit in the alpha-galactosidase A enzyme. The gene coding for it is located on the long arm of the X chromosome (Xq22.1). This deficit causes the gradual accumulation of a glycosphingolipid. The main substance accumulated is globotriaosylceramide (Gb3). This accumulation leads to pain and angiokeratomas, and to cardiac, cerebral, and vascular involvement as the disease progresses. The treatment of Fabry's disease has so far only been symptomatic; however, new advances have now made it possible to prescribe alpha-galactosidase replacement therapy, which not only improves symptoms, but also enhances these patients' quality of life and lowers mortality. In this paper we review the status of Fabry's disease and we report the follow-up of a family with Fabry's disease, with some members receiving replacement therapy with alpha-galactosidase A and demonstrating good progress.

[Treatment of malignant pheochromocytoma with 131I MIBG: a long survival]. / Feocromocitoma maligno tratado con MIBG I131:una larga supervivencia.

131I-metaiodobenzylguanidine (131I-MIBG) is a structural analogue of adrenal hormone norepinephrine employed to localise tumours derived of neural crest and in the treatment of malignant pheochromocytomas. Although the number of patients is low, in the revised literature there are objective remissions and reduction of hormonal activity, with symptomatic answers. However, the reduction of size of tumour only has been described in some times and hardly ever in the presence of osseous metastasis. We present one patient diagnoses of malignant pheochromocytoma with osseous metastasis, who was treated with 131I-MIBG. It was administrated a total dose of 1,800 mCu, with an excellent tolerance and short adverse symptoms. There were a partial tumour response and a complete hormonal response, with a survival of 30 years after the diagnoses.

[Fabry's disease associated with rheumatoid arthritis. Multisystemic crossroads]. / Enfermedad de Fabry asociada a artritis reumatoide. Una encrucijada multisistémica.

Fabry's disease is a rare congenic disorder of glycosphingolipid catabolism resulting from deficient activity of the alpha galactosidasa. Is an X-linked disorder and in hemizygous males the activity of this enzyme is very low, resulting in severe manifestations. Fabry disease is confirmed by the lack alfa-galactosidase in serum. In the literature have been reported a few cases of coexistent Fabry's disease and connective disorders, but there is not cases of rheumatoid arthritis coexistent. This report describes a case of a female with Fabry's disease who vas subsequently diagnosed with rheumatoid arthritis. The suspect diagnosis was very important because the two disorders are multisystem and new symptoms could be attributed to Fabry's disease. The accumulation of lipids may results in numerous pathogenic autoantibodies, which could make immunocomplex. This is the potential pathogenic mechanisms explaining the association between Fabry's disease and autoimmune diseases.

[Systemic necrotizing vasculitis in autopsy]. / Vasculitis necrosante sistémica en la autopsia.

OBJECTIVE: To analyze the characteristics of systemic necrotizing vasculitis observed in necropsy emphasizing the non-diagnosed cases until necropsy and to identify the reasons of clinical confusion. MATERIALS AND METHODS: Necropsies of adult patients performed in Hospital General La Paz, Madrid, from 1966 to 1977 were reviewed. Clinical manifestations, analytical changes and involvement of different organs were analyzed. The correspondence degree between clinical and necropsic diagnoses was studied. RESULTS: A total of 18 cases of necrotizing vasculitis were observed out of 3,980 necropsies. The most common clinical manifestations were fever and general syndrome (60%). The organs most commonly involved were kidney (94%) and gastrointestinal tract (56%). Diagnosis was revealed by necropsy in 39% of cases; in these cases, the incidence of heart failure and peripheral neuropathy was significantly lower, whereas digestive hemorrhage and liver, pancreas, adrenal gland, and bladder involvement was significantly higher. CONCLUSIONS: Systemic necrotizing vasculitis in underdiagnosed, which can be partly explained by the lack of specificity of the most common symptoms and the paucity of characteristic clinical and organ-specific manifestations which leads to confusion with more prevalent conditions.

[Homocysteine. The cardiovascular risk factor of the next millennium?]. / La homocisteína. El factor de riesgo cardiovascular del próximo milenio?

Several epidemiologic studies have demonstrated that hyperhomocysteinemia is a risk factor for arteriosclerosis in coronary, cerebral, peripheral and aortic arteries. This risk is independent of other cardiovascular risk factors, and it is dose related. However, prospective studies show contradictory findings. Hyperhomocysteinemia is also associated with a higher risk of venous thrombosis to which other coagulation disorders, such as factor V Leiden, could contribute. Hyperhomocysteinemia can be due to genetic defects in the enzymes that control homocysteine metabolism, and also to other factors, mainly nutritional (deficiencies in vitamin B6, vitamin B12, or folic acid). Dietary supplements of these vitamins reduce plasma homocysteine levels. Randomized clinical trials are still needed to demonstrate that reducing plasma homocysteine levels will reduce the risk for cardiovascular disease.

[Churg-Strauss syndrome. Our experience in 2 decades]. / Síndrome de Churg-Strauss. Nuestra experiencia en dos décadas.

BACKGROUND: Since 1951, the year when the Churg-Strauss syndrome was reported, several isolated cases have been reported in the literature but works containing a high number of cases are exceptional. The features of the syndrome as well as diagnostic criteria have not been contrasted in large series. We therefore tried to analyze these parameters in 16 patients diagnosed of Churg-Strauss syndrome at our hospital. METHODS: A retrospective analysis was made of 16 patients diagnosed of Churg-Strauss syndrome at Hospital Universitario La Paz from 1978 at 1998. Data regarding personal antecedents, clinical manifestations, analytical parameters, histology and clinical course were collected. Also, the main diagnostic criteria reported in the literature were applied to each case. RESULTS: Of the 16 cases, thirteen (81%) were women, six were diagnosed at an age between 21 and 40 years; other six cases from 51 to 70 years. All patients had antecedents of bronchial asthma. Among clinical manifestations, 60% had fever and 50% a general syndrome. The main involved organs by decreasing frequency were lung (100%), nervous system (63%), and heart (44%). The mean eosinophilic count was 7,640/mm3. The histological diagnosis was obtained in 14 out of the 16 patients (88%). The most efficient biopsies corresponded to muscle and nerve. After therapy, no relapse was noted of vasculitis although bronchial asthma persisted in most patients (12). Twelve cases (75%) fulfilled the Lanham diagnostic criteria. CONCLUSIONS: Churg-Strauss syndrome affects mainly women with severe eosinophilia. Electromyogram and muscle and peripheral nerve biopsy are useful tools for diagnosis. The Lanham criteria lead to the diagnosis in most patients.