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Are classification criteria for vasculitis useful in clinical practice? Observations and lessons from Colombia
Paúl Alejandro Méndez Patarroyo1, José Félix Restrepo, Samanda Adriana Rojas
1Department of Internal Medicine, Rheumatology Unit, Universidad Nacional de Colombia, Bogota, Columbia.
Insights
Current vasculitis classification systems are inadequate for diagnosis. A clinical-histopathological correlation approach is suggested for better diagnosis and therapy of inflammatory vascular diseases.
Area of Science:
- Rheumatology
- Pathology
- Immunology
Background:
- Idiopathic systemic vasculitis involves inflammation of blood vessel walls with non-specific causes.
- Fibrinoid necrosis is a common characteristic in affected small and large vessels.
Purpose of the Study:
- To evaluate common inflammatory vascular diseases in a historical patient cohort from Bogota, Colombia.
- To compare the efficacy of two classification systems and a clinical-histopathological correlation format.
- To identify limitations in current vasculitis classification schemes.
Main Methods:
- Reviewed 304 histopathological biopsies from 292 patients diagnosed with vasculitis between 1953 and 1997.
- Classified cases using the Chapel Hill Consensus criteria and the J.T. Lie system.
- Collected data systematically for new cases from 1991 to 1997.
Main Results:
- Cutaneous leukocytoclastic vasculitis was the most frequent primary vasculitis.
- Secondary vasculitis associated with connective tissue diseases and infections were common.
- A significant percentage of cases (65.8% and 35.2%) were unclassifiable by existing systems, but only 8.9% remained unclassified with the proposed correlation method.
Conclusions:
- Existing vasculitis classification schemes are insufficient for clinical diagnosis, particularly for infectious and unusual etiologies.
- A classification approach emphasizing clinical-histopathological correlation is proposed for improved diagnosis and therapy.
- The suggested method may enhance clinical practice and standardization for research studies.
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.
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