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INTRODUCTION: Idiopathic systemic vasculitis represents a group of clinical entities having non-specific etiology with the common characteristic of acute or chronic inflammatory compromise of the small and large vessels walls, associated with fibrinoid necrosis. OBJECTIVES: To describe the most common inflammatory vascular diseases in a long historical cohort of patients from San Juan de Dios Hospital located in Bogota, Colombia using two different systems and a clinical histopathological correlation format, and to make a comparison between them. METHODS: We reviewed all previously ascertained cases of vasculitis confirmed by biopsy processed between 1953 and 1990, and systematically collected data on all new cases of vasculitis from 1991 to 1997 at the Hospital San Juan de Dios (Bogota-Colombia). The cases were classified in accordance with the Chapel Hill Consensus criteria, and the system proposed by J.T. Lie. RESULTS: Of 165,556 biopsy tissue specimens obtained during this period from our hospital, 0.18% had vasculitis, perivasculitis or vasculopathy. These included 304 histopathological biopsies from 292 patients. Cutaneous leukocytoclastic vasculitis (64 histological specimens) was the most frequently encountered type of "primary" vasculitis followed by thromboangiitis obliterans (38 specimens), and polyarteritis nodosa (24 specimens). Vasculitis associated with connective tissue diseases (33 specimens) and infection (20 specimens) were the main forms of secondary vasculitis, a category that was omitted from the Chapel Hill consensus report. We found that 65.8% of our histopathological diagnoses could not be classified according to the Chapel Hill classification, and 35.2% could not be classified according to the classification of Lie. Only 8.9% of cases remained unclassified by our system after clinical and histological correlation. CONCLUSION: Current vasculitis classification schemes are designed for classification, rather that diagnosis of disease and do not adequately address some common forms of inflammatory vascular diseases, including those of infectious etiology and unusual etiology seen in clinical practice. Based on our clinical experience, we suggest a classification outline which practitioners can use which emphasizes correlation of the clinical picture to the histopathology findings for diagnosis and therapy, which may promote better clinical practice and standardization for clinical trials.
RESUMO
BACKGROUND: Paget disease of bone has an unknown etiology, having complex pathogenesis leading to increased bone resorption in the first phase and an excess of bone formation with more advanced disease. The disease has been associated to white ancestry in Europe and other countries, being less common in people without European origin. OBJECTIVES: The objectives of this study were to describe the Colombian cases of Paget disease and search the published literature for more Latin American cases and their characteristics. METHODS: Electronic databases were searched up to August 2004: MEDLINE, PUBMED, BIREME LILAC, and MEDCARIB, evaluating the entire bibliography regarding reports of Paget disease in Latin America during the last 30 years. Additionally, we searched the medical databases of local reference centers to describe new cases from Colombia. RESULTS: We found 14 cases of Paget disease from Colombia; 12 of them were previously reported elsewhere and 2 additional cases were found in the medical database of a local reference center. We describe the main clinic characteristics, including age, symptoms, type and stage of involved bone (monostotic or polyostotic), and treatment, which generally are similar to cases from Europe or the United States. The literature search showed that a total of 1149 cases of Paget disease have been previously published from Latin America in the last 30 years, more than half of them coming from Argentina and Brazil with predominant white ancestry. CONCLUSIONS: We emphasize the presence of white European origin or ancestry in the great majority of reported cases of Paget disease in Latin America and Colombia. Studies of factors that influence the etiology in cases of non-European ancestry would be of interest.
