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- Springer Journals
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- Copyright © 2009 by Patarroyo et al; licensee BioMed Central Ltd.
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- Biomedicine; Immunology; Internal Medicine
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- 1740-2557
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- 10.1186/1740-2557-6-1
- pmid
- 19250526
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Abstract
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. Page 1 of 10 (page number not for citation purposes) Journal of Autoimmune Diseases 2009, 6:1 http://www.jautoimdis.com/content/6/1/1 Conclusion: Current vasculitis classification schemes are designed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. in the Universidad Nacional de Colombia's Pathology Introduction Systemic vasculitis represents a heterogeneous group of Department (Bogotá, Colombia) were compiled. The data clinical entities having non-specific etiology with the from the 1953–1990 cases were obtained from medical common characteristic of acute or chronic inflammatory record review of all patients with a positive biopsy speci- compromise of the small and large vessels walls, associ- men. After 1990, cases were compiled using a standard ated with fibrinoid necrosis. Histopathological examina- case report form (CRF) as patients attended consultation tion is the main basis for diagnosing these diseases. in the clinical setting. All reports from each examination Vasculitis may be primary, or associated with another dis- were reviewed and all those with vascular inflammatory eases process. Infections, hypersensibility to drugs, atrial compromise collected; 304 confirmed cases of vasculitis myxoma, solid cancers, and lymphoproliferative or other were found (187 between 1953 and 1990, and 117 connective tissue diseases are among the entities causing between 1991 and 1997). These included 187 specimens secondary vasculitis [1-17]. from the 1953–1990 period and 117 from 1991–1997. These specimens were reviewed by rheumatologists and Criteria have been defined for classifying the different by members of the pathology department of a third level types of vasculitis by the American College of Rheumatol- university hospital from Bogotá, Colombia (Hospital San ogy (ACR) Vasculitis Study Committee, which however Juan de Dios). limited itself to defining "primary" vasculitis characteris- tics. The ACR introduced criteria for some vasculitides in Results were expressed in frequencies since the total 1990, and has proposed different parameters for classify- number of patients that consulted during these years was ing each one of these entities [18-26]. Only seven types of not available, so that an approximate calculation of inci- vasculitis are considered in the ACR scheme. These and dence could not be performed. Of 165,556 biopsies proc- the classification system proposed by J. T. Lie [27] and by essed by the histopathology laboratory of our hospital, Chapel Hill consensus conference [28] in 1994 are in use 304 specimens from 292 patients had evidence of vascular today. These criteria are not designed for diagnosis of vas- inflammatory involvement. Once a diagnosis was made culitis, and are inadequate for clinical application, and on histopathological grounds, the cases were classified omit important clinical forms of vasculitis. according to the Chapel Hill [28] and J. T. Lie classifica- tion proposal [27]. The criteria defined by the ACR [18- With few exceptions, only sporadic reports of vasculitis 26] for classifying primary vasculitis were also applied, are found in Latin-American literature [29-56]. Some of however these encompass only 7 types of vasculitis, and it the larger series include classification studies of Alarcón- was necessary to expand the spectrum of vasculitis entities Segovia [57-59] and one cohort of patients with primary according to clinical, laboratory, and histological consid- vasculitis of the Cisterna's group in Chile [60]. The epide- erations. All histological studies were re-reviewed and miology of the primary vasculitides is best studied in diagnosed according to clinical, laboratory and his- Europe and North America [61-72]. There have been no topathological information. such studies in South America. Results With this study, we describe the different types of vasculi- Of the 165,556 tissue specimens, 0.18 percent could be tis most frequently found in the San Juan de Dios' Hospi- assigned a diagnosis of vasculitis, perivasculitis or vascu- tal from Bogotá, Colombia using two current lopathy (Tables 1 and 2). Three-hundred-four histopatho- classification systems as well as our own clinical-patho- logical biopsies from 292 patients with documented logical correlation. vasculitis were found during that period. More than one sample was obtained from twelve patients, those with two Methods histological specimens were: six with Buerger's disease (in This is a descriptive study performed in two phases. All one of these the diagnosis on first biopsy was not conclu- 165,556 histological specimens (129,192 between 1953 sive); two patients with polyarteritis nodosa (in both the and 1990 and 36,364 between 1991 and 1997) processed initial diagnosis was inconclusive); two patients with Page 2 of 10 (page number not for citation purposes) Journal of Autoimmune Diseases 2009, 6:1 http://www.jautoimdis.com/content/6/1/1 Table 1: Classification of histological specimens with vasculitis from the San Juan de Dios Hospital, Bogotá, Colombia, 1953 – 1997, according to the Chapel Hill system [28] Vasculitis diagnosis Number Frequencies Percentages N = 304 per 100,000 histological specimens groups of vasculitis subgroups of vasculitis I. Primary vasculitis 104 62.8 34.2 A. Large vessel vasculitis 1 0.6 1 1. Giant cell (Temporal) arteritis 1 0.6 B. Medium sized vessel vasculitis 24 14.5 23.1 1. Polyarteritis nodosa 24 14.5 C. Small vessel vasculitis 79 47.7 75.9 1. Wegener's granulomatosis 3 1.8 2. Churg-Strauss syndrome 2 1.2 3. Microscopic polyangiitis 1 0.6 4. Henoch-Schönlein purpura 9 5.4 5. Cutaneous leukocytoclastic vasculitis 64 38.7 II. Not classified vasculitis 200 120.8 65.8 Total 304 183.6 100 inconclusive results of histology, including one diagnosed Vasculitis associated with connective tissue diseases (33 with cutaneous leukocytoclastic vasculitis and the other specimens) was the most frequently encountered classifi- with connective tissue disease vasculitis. cation among those specimens classified as secondary vas- culitis according to the scheme proposed by J.T. Lie, while The average age of the patients from whom these speci- vasculitis associated with infection was the next most mens were obtained was 36 years, ranging from 10 to 99. common type (20 specimens; Tables 2 and 3). Male/female ratio was 1:2. Most specimens corresponding to vasculitis came from skin (64.5%), amputated extrem- In our format of clinical-pathological correlation we ities (11.5%) and muscle (8.2%). found that leukocytoclastic vasculitis was the most fre- quent with 107 specimens, followed by lymphomono- Primary vasculitis was the diagnosis in the minority of our cytic vasculitis with 53 specimens. Only 27 specimens biopsies; thirty-four percent (104 specimens) of the his- (8.9%) could not be classified with our or any scheme. topathological studies corresponded to primary vasculitis Within the cases that were unable to be classified using the according to the Chapel Hill Consensus Conference while system of JT Lie or the Chapel Hill proposal are the lym- forty-six percent (142 specimens) revealed a primary vas- phomonocytic vasculitis (53 specimens), several cases of culitis according to the system of J. T. Lie (Tables 1 and 2). granulomatous vasculitis, nodular vasculitis (19 speci- mens) and illnesses that resemble vasculitis, including The most frequently encountered types of primary vascu- arterial embolism (4 specimens) (Table 3). litis were cutaneous leukocytoclastic vasculitis (64 speci- mens), followed by thromboangiitis obliterans (38 Discussion specimens) (all of this obtained from tissue of amputated Vasculitis classification are diverse and employ several extremities), polyarteritis nodosa (24 specimens), and organizing principles including the size of the compro- Henoch-Schönlein purpura (Tables 1, 2, 3). mised vessel, whether an underlying pathoetiology is Page 3 of 10 (page number not for citation purposes) Journal of Autoimmune Diseases 2009, 6:1 http://www.jautoimdis.com/content/6/1/1 Table 2: Classification of histological specimens with vasculitis from the San Juan de Dios Hospital, Bogotá, Colombia, 1953 – 1997, according to the scheme of Lie [27] Vasculitis diagnosis Number Frequencies Percentages N = 304 per 100,000 histological specimens groups of vasculitis subgroups of vasculitis I. Primary vasculitis 142 74 46.7 A. Affecting large, medium and small sized blood 1 0.6 0.7 vessels 1. Giant cell (Temporal) arteritis 1 0.6 B. Affecting predominantly medium and small- 29 17.5 20.4 sized blood vessels 1. Polyarteritis nodosa 24 14.5 2. Churg-Strauss syndrome 2 1.2 3. Wegener's granulomatosis 3 1.8 C. Affecting predominantly small-sized blood 74 44.7 52.1 vessels 1. Microscopic polyangiitis 1 0.6 2. Henoch-Schönlein purpura 9 5.4 3. Cutaneous leukocytoclastic angiitis 64 38.7 D. Miscellaneous conditions 38 23 26.8 1. Buerger's disease 38 23 II. Secondary vasculitides 55 33.2 18.1 1. Infection-related vasculitis 20 12.1 2. Connective tissue disease vasculitis 33 20 3. Hypocomplementemic urticarial vasculitis 1 0.6 4. Vasculitis associated with neoplasia 1 0.6 III. Unclassified vasculitis 107 64.6 35.2 Total 304 183.6 100 found (primary versus secondary vasculitis); the main and others were taken into account during development type of vessel compromise (artery, vein, capillary, etc.), of the Chapel Hill consensus conference criteria [28,91]. the type of immune damage and others. Zeek's concept of classifying vasculitis according to the Attempts to classify vasculitis began with the work of Pearl size of the comprised vessel: small, medium-or large-sized Zeek in 1952 [61] and many others have proposed their continues to be widely used. This proposal, along with own classification schemes [27,28,57-59,62-91]. Consid- other proposals regarding vessel size, has been adapted by erations of the ACR [78], Lie [27,79-81], Churg [83,84], many authors who have since built upon it. Page 4 of 10 (page number not for citation purposes) Journal of Autoimmune Diseases 2009, 6:1 http://www.jautoimdis.com/content/6/1/1 Table 3: Histological specimens with vasculitis from the San Juan de Dios Hospital, Bogotá, Colombia, 1953 – 1997, according to a clinical-pathological correlation of vasculitis [92] Vasculitis diagnosis Number Frequencies Percentages N = 304 per 100,000 histological specimens groups of vasculitis subgroups of vasculitis 1. Leukocytoclastic vasculitis 107 64.6 35.2 1.1. Erythema elevatum diutinum 1 0.6 0.9 1.2. Secondary to diseases 42 25.4 39.3 1.2.1. SLE 14 8.5 1.2.2. PM/DM 14 8.5 1.2.3. Diffuse/limited scleroderma 5 6.7 1.2.4. Henoch-Schönlein purpura 9 5.4 1.3 Livedoid leukocytoclastic vasculitis 5 3 4.7 1.4. Idiopathic 59 35.6 55.1 2. Lymphomonocitic's vasculitis 53 32 17.4 2.1. Schamberg's purpura 1 0,6 1.9 2.2. Chronic urticaria, associated 1 0.6 1.9 2.3. Idiopathic 51 30.8 96.2 3. Nodular vasculitis 19 11.5 3.6 3.1. Erythema nodosum 7 4.2 36.8 3.2. Granulomatous panniculitis 2 1.2 10.5 3.3. Vasculitis in panniculitis 4 2.4 21.1 3.4. Idiopathic 6 3.6 31.6 4. Granulomatous vasculitis 11 6.6 3.6 4.1. Wegener's granulomatosis 3 1.8 27.3 4.2. Churg Strauss 2 1.2 18.2 4.3. Lymphomatoid granulomatosis (lung) 1 0.6 9.1 4.4. Granuloma annulare 1 0.6 9.1 4.5. Giant cell (Temporal) arteritis 1 0.6 9.1 4.6. Idiopathic 3 1.8 27.3 5. Polyarteritis nodosa 24 14.5 7.9 Page 5 of 10 (page number not for citation purposes) Journal of Autoimmune Diseases 2009, 6:1 http://www.jautoimdis.com/content/6/1/1 Table 3: Histological specimens with vasculitis from the San Juan de Dios Hospital, Bogotá, Colombia, 1953 – 1997, according to a clinical-pathological correlation of vasculitis [92] (Continued) 5.1. Generalized 16 4.8 66.7 5.2. Cutaneous 8 9.7 33.3 6. Vasculitis like diseases 4 2.4 1.3 6.1. Arterio-embolism 4 2.4 100 7. Infection-related vasculitis 20 12.1 6.6 7.1. Mycobacterium 12 7.2 60 7.2. Bacterial infections 8 4.8 40 8. Microscopic polyangiitis 1 0.6 0.3 9. Thromboangiitis obliterans 38 23 12.5 10. Unclassified vasculitis 27 16.3 8.9 TOTAL 304 100 Existing classification systems are incomplete and difficult classification schemes and compared them with our find- to use in the clinic, as they are not designed to be diagnos- ings based on clinical-pathological correlation. tic criteria, but rather are more useful in distinguishing one type of vasculitis from another in populations of In our experience, "primary" vasculitis seems to be patients for whom a diagnosis of vasculitis is established uncommon in Colombia [41], although it does occur, [92]. For this reason, in 1993 we proposed a format for including Takayasu's disease [42,92]. Since our review ordering vasculitides based on their pathogenesis, histo- concentrates on cases with biopsy evidence of disease, the logic features, and causal agents if known, with the intent true frequency of primary vasculitis diagnosed on clinical, of improving understanding of them without proposing a but not histological grounds, is certainly higher. Further- new classification scheme (Table 3). We consequently more, we observed that a significant number of our cases included numerous entities with defined histology which could not be classified by Lie's and the Chapel Hill Con- are not captured in existing classification schemes [93]. sensus criteria (Table 3, 4). We also encountered difficul- This provides a clinical-pathologic correlation which goes ties in assigning a diagnosis of vasculitis to some of our beyond the incomplete current classification schemes histological studies according to whether they were pri- [93]. mary or secondary. Many difficulties emerged while eval- uating these cases. It was particularly difficult to make a It is our opinion that the most representative, recognized diagnosis based on the ACR diagnostic criteria of classic and utilized classifications in worldwide literature are types, since these were created for classifying patients hav- those of J.T. Lie, and Jennette and Falk et al., described at ing defined vasculitis and not for making a specific or the Chapel Hill Consensus Conference. We therefore ana- even general diagnosis in patients where other vasculitis lyzed this Colombian cohort in accordance with these two or vasculopathies are suspected. Table 4: Comparison of Two Vasculitis Classifications Schemes With a Clinical-Pathological Correlation from the San Juan de Dios hospital, Bogotá, Colombia, 1953 – 1997 * Percentages Chapel Hill Lie Clinical-pathological Correlation Classifiable 34.2 64.8 92.1 Unclassifiable 65.8 35.2 8.9 * from 304 histological specimens with inflammatory vascular compromise Page 6 of 10 (page number not for citation purposes) Journal of Autoimmune Diseases 2009, 6:1 http://www.jautoimdis.com/content/6/1/1 The difficulty in applying these criteria clinically was illus- Thromboangiitis obliterans was more frequent before trated in a study using chart audits to classify 198 consec- 1990 (26 specimens), probably because the national utive patients by ACR criteria, with re-audit of patient health promotion effort from the 1980s onward against charts after 2–8 months to ascertain the patient's ultimate tobacco-use could account for its diminishing frequency diagnosis, Rao et al, found that vasculitis was diagnosed since then. The difference found in primary vasculitis in 51 (26%) patients. Thirty-eight (75%) of 51 patients between the two proposed classifications is explained with vasculitis and 31 (21%) of 147 patients without vas- largely by the absence of Buerger's disease in the Chapel culitis met ACR criteria for one or more types of vasculitis. Hill scheme. Thromboangiitis obliterans (Buerger's dis- The positive predictive values for the four vasculitides ease) is included as a vasculitis, understanding that it is according to ACR criteria were 17% to 29% for the entire histopathologically distinguished from other forms of cohort and 29% to 75% for only the patients with a final vasculitis, having an inflammatory thrombus with rela- diagnosis of vasculitis [94]. It was also difficult to obtain tively sparse evidence of inflammation in the vessel wall. samples in cases where visceral compromise or large ves- sel involvement was present, although clearly the his- Our study found JT Lie's classification was better adapted topathologic examination continues to be the gold- to those pathologies associated with vasculitis in our insti- standard for diagnosis. tution compared to that of the Chapel Hill Consensus Conference (Table 4). This may be due to the consensus We have pioneered the study of vasculitis in Colombia. excluding secondary vasculitis, such as those associated The San Juan de Dios Hospital was the Colombian refer- with connective tissue diseases, solid tumor, atrial ence center for this type of pathology during the time of myxoma, drug- or infection-related reactions, among oth- this study, and our reports may reflect the state of these ers. A majority, 65.8%, of the analyzed specimens were entities in Colombia and perhaps reveal Latin-American not classified on applying Chapel Hill consensus criteria, trends in frequency of the different types of vasculitis while fewer remained unclassified using Lie's proposed occurring. Small-sized vessel vasculitis is the predominant criteria (35.2%). When we used a correlation based on type of primary vasculitis. clinical diagnosis with pathological findings, only 8,9% of the specimens were unable to be classified. In the Latin American literature most reports of vasculitis are those of idiopathic types. Takayasu's arteritis in Brazil Histopathologic examination of biopsy specimens is the has been reported by Sato et al's group [43], and Behçet's gold standard for studying vasculitis in spite of existing disease by Heyman et al and Ferraz et al [44,46]. Kawa- limitations. Currently used classifications leave many vas- saki's disease has been reported by Saraiva et al [45], and cular entities, including some forms of vasculitis which Lucio's phenomenon has been reported by Souza et al are thus far ill-defined, without being classified. Knowl- [38]. Cisternas et al evaluated described their patient edge concerning these entities is limited in Latin America, cohort of "primary" vasculitis in Chile [60]. Other work so efforts made in studying these diseases will benefit our groups in Mexico have made significant contributions to patients. It is also necessary to make extra efforts towards the study of vasculitis, especially that of Alarcón-Segovia. creating more suitable classifications in which Latin- The immuno-pathological aspects of Takayasu's arteritis Americans and other countries study groups may partici- have been reported on by Vargas-Alarcon [48]. Other pate. studies include cases of Takayasu's arteritis by Castro, Castanon, and Robles [49,53,56]. Henoch-Schönlein pur- It is likely that the ethnicity, immunogenetics, and the pura has been reported by Reyes-Vasquez [50], Kawasaki's socioeconomic status of patients with vasculitis, among diseases in children by Vizcaino-Alarcon [52], and other factors, is different in our patients than in those Behçet's disease by Alarcon-Segovia's [55]. In Peru, described in North American and Western European Sanchez et al [95] described 29 patients with microscopic cohorts and accounts for many of the clinical differences polyangiitis and compared them with those of Guillevin's seen in these different groups of patients. Consequently, group [96]. The Peruvian patients had more multiple an attempt must be made to include the totality of condi- mononeuritis, less cutaneous involvement, more benign tions with clinical and histopathological findings of vas- renal disease, and a better prognosis. There are no studies culitis and even vasculopathies mimicking or closely of vasculitis epidemiology in Latin American. resembling established vasculitis conditions in such new classification schemes to facilitate clinical diagnosis and Cutaneous leukocytoclastic vasculitis predominated in research. our study of primary vasculitis correlating with other stud- ies reporting this as being the most frequent type. Conclusion Current classification criteria for vasculitis do not ade- quately address some common forms of inflammatory Page 7 of 10 (page number not for citation purposes) Journal of Autoimmune Diseases 2009, 6:1 http://www.jautoimdis.com/content/6/1/1 vascular diseases, including those of infectious etiology References 1. Mandell BF, Calabrese LH: Infections and systemic vasculitis. and unusual etiology seen in clinical practice. They are Curr Opin Rheumatol 1998, 10:51-7. based primarily on the vessel size, rather than on his- 2. 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Journal of Autoimmune Diseases – Springer Journals
Published: Feb 27, 2009