|
| | | Year : 2010 | Volume : 53 | Issue : 4 | Page : 624-627 | | | Syphilitic aortitis: Rearing of the ugly head | | | Pradeep Vaideeswar
Department of Pathology (Cardiovascular & Thoracic Division), Seth G.S. Medical College & KEM Hospital, Mumbai, India
Click here for correspondence address and email | Date of Web Publication | 27-Oct-2010 | | |      | |  Abstract | | | Context: Syphilitic aortitis has been relegated to the category of rare cardiovascular disease or a "medical curiosity" in the west. The same situation may not exist in developing countries due to the stigmata that continue to remain attached to sexually-transmitted diseases in general. Aims: To study the prevalence of syphilitic aortitis among autopsied non-atherosclerotic aortic diseases encountered in a span of 15 years. Settings and Design: Retrospective, autopsy-based study. Materials and Methods: Among 187 cases of non-atherosclerotic diseases of the aorta, 44 had been diagnosed as syphilitic aortitis on the basis of the pathological features and serology. The demographic details and modes of clinical presentation were retrieved from the health records. Depending on the presence of complicating lesions, the cases were classified as uncomplicated or complicated aortitis. Results: The 44 cases of syphilitic aortitis formed 23.5 % of the non-atherosclerotic aortic diseases. They were predominantly seen in males in the fifth decade, who often presented with valvular regurgitation, aneurysmal disease or myocardial ischemia; 13.6 % of patients were asymptomatic. Blood VDRL results were available in 19 patients; 84.2 % were positive. Concomitant involvement of the ascending, transverse and descending thoracic was seen in 45.5 % of cases. None had uncomplicated aortitis. Complications in the form of aortic regurgitation (72.7 %), coronary ostial stenosis (59 %) and aneurysms (59 %) frequently coexisted. Thirty-five aneurysms were present in 59 %, chiefly involving the aorta. Conclusions: We found syphilitic aortitis to be a common cause of aortitis at autopsy. Diagnosis should be made with the help of characteristic pathological features correlated to the clinical context and appropriate serological tests. Keywords: Aortic aneurysm, aortic regurgitation, aortitis, cardiovascular syphilis How to cite this article:
Vaideeswar P. Syphilitic aortitis: Rearing of the ugly head. Indian J Pathol Microbiol 2010;53:624-7 |
| Introduction | |  |
Syphilis is a prototype of sexually transmitted diseases, caused by the spirochetal bacterium Treponema pallidum, subspecies pallidum, which had developed as a new world disease through evolution of other treponemal species. [1] The disease progresses through the primary, secondary and tertiary phases. Involvement of the cardiovascular system is the most important sequel of the tertiary phase of syphilitic infection. This manifests usually as syphilitic aortitis and infrequently as gummatous myocarditis. The advent of effective antibiotic therapy saw a pronounced decline in the incidence of late cardiovascular syphilis and hence in the west, syphilitic aortitis has been relegated to the category of rare cardiovascular disease or a "medical curiosity". [2] But, the same may not be the situation in developing countries, and it was not surprising that syphilitic aortitis contributed to the bulk of the various non-atherosclerotic aortic diseases at autopsy, an observation that forms the basis of the present study.
| Materials and Methods | | |
Review of the autopsy records at our hospital in a 15-year-period (1994-2008) yielded 187 cases of non-atherosclerotic diseases of the aorta. Forty-four of these had been diagnosed as syphilitic aortitis. In all cases, the demographic details and modes of clinical presentation of the patients were noted. All the heart specimens had been perfusion-fixed with 10 % buffered formalin; 39 specimens were still available for review. All hearts were cut following the flow of blood and weighed after removing all the adherent post-mortem blood clots. Particular attention was paid to degree of cardiomegaly and left ventricular dilatation/hypertrophy, presence of myocardial scarring, status of the aortic annulus, aortic valve and coronary ostia, extent of involvement of the aortitic process, and presence (and degree) of atherosclerosis. Diffuse thickening of the wall of the ascending aorta (classic location) with gelatinous intimal plaques separated by furrows, creases and radiating scars, the 'tree bark' appearance [Figure 1] were considered to be characteristic gross morphological features of the disease. Since this appearance is mimicked by a variety of inflammatory aortic diseases, we also relied on blood serology and the presence of chronic mesaortitis (medial scarring, perivascular lympho-plasmacytic infiltrate, and endarteritis obliterans - [Figure 1] for the diagnosis. Sections were taken from the various aortic segments and stained by hematoxylin-eosin and elastic van Geison stains. Depending on the presence of complicating lesions such as aortic regurgitation (AR), coronary ostial stenosis (COS) and aneurysm formation, the cases were classified as uncomplicated or complicated aortitis.  | Figure 1 :(a) Classic tree-barking, multiple gelatinous plaques, separated by stellate scars and vertical furrows, seen in the ascending aorta and arch. Note that some plaques are yellow due to superimposed atherosclerosis; (b) Chronic mesoaortitis, where part of the media is replaced by fibrous scars; (c) The adventitia shows endarteritis of the vasa vasorum (Elastic van Gieson, ×250). The arrow points at the perivascular inflammatory infiltrate rich in plasma cells. (Elastic van Gieson, ×250)
Click here to view |
| Results | | |
The 44 cases of syphilitic aortitis formed 23.5 % of the 187 cases of non-atherosclerotic aortic diseases [Table 1]. The age range was 28 to 61 years, with predominance in the fifth decade of life. Only three of the 44 were females, and only one male was less than 30 years of age. Clinical features of aortic regurgitation or aneurysmal disease were present in 29 patients (65.9 %). Syphilitic infection was implicated in six and seven patients among the 13 and 16 clinically diagnosed cases of aortic insufficiency and aortic aneurysms, respectively. The other patients had diagnoses of ischemic heart disease (IHD)/hypertensive heart disease (five cases), head injury (three cases) and one case each with cardiomyopathy, with 90 % suicidal burns, sudden death, peripheral vascular disease (PVD), IHD with PVD, cerebrovascular accident, pancytopenia and malaria. Results of blood VDRL were available in 19 patients cases (43.2 %); among these, positivity was found in 16 (84.2 %). Three cases in whom VDRL had not been done, had given a history of exposure to venereal disease.
The typical "tree bark" appearance of the intima was the constant feature in the ascending aorta in all cases. However, it was confined to this segment in four hearts (9 %, [Figure 2]), while in the others; there was variable extension into the other aortic portions. The involvement of the ascending, transverse and descending thoracic (2.1 to 5.8 cm) segments was the commonest, seen in 20 patients (45.5 %, [Figure 2]). In the remaining 10 cases each (27.5%), the ascending aortic involvement was associated with aortic arch disease, and affection of the supra-renal portion of abdominal aorta (in continuity with thoracic aorta), respectively. Concomitant atherosclerosis was present in 14 aortae [Figure 3].  | Figure 2 :(a) Aortitis restricted to the ascending aorta. There is annular dilatation and commissural separation, especially the one between the non-coronary and right coronary cusps (black arrow). There was clinical valvular regurgitation. White arrows indicate ostial stenosis, right more than the left (MV mitral valve, LV left ventricle); (b) Commonest pattern of involvement, tree-barking seen in the ascending, arch and descending thoracic segments
Click here to view |  | Figure 3 :Syphilitic aortitis affecting the three segments of the aorta. Note marked atherosclerosis with ulceration in the thoracic portion. The patient, 45 years male, presented with peripheral vascular disease
Click here to view |
Uncomplicated aortitis was not seen in any case. Aortic valvular regurgitation [Figure 2], clinical or on the basis of pathology occurred in 32 patients (72.7 %, [Table 2]), three of whom required valve replacements. Jet lesion of valvular incompetence was present in 12 hearts. One case had a bicuspid valve. Narrowing of the coronary ostia was observed in 26 (59 %). Eleven had narrowing of both ostia [Figure 2] and one patient had arteritis of 2.3 cm of the proximal right coronary artery [Figure 4]. Ostial stenosis often coupled with left ventricular hypertrophy to produce myocardial scarring, ischemia, micro infarctions or even grossly visible infarction (two hearts). Thirty-five aneurysms were present in 26 patients (59 %, [Table 3]). One case each had a right sinus-of-Valsalva aneurysm and sub-aortic aneurysm. The latter was associated with degenerated prosthetic aortic valve. Of the remaining 33 aneurysms, 30 involved the aorta [Figure 5] and the other three were seen in the aortic branches. Two patients had five aneurysms, one of which was the subject of an earlier publication [3] ; another had shown two aneurysms. Ascending aorta was the commonest site involved (isolated involvement in nine cases); 18 aneurysms were saccular. Eight aneurysms had ruptured; the sites depended on the location of the aneurysms. Seven patients underwent graft interpositions or aneurysmorraphy.  | Table 3 :Syphilitic Aortitis (n=44) Sites of 33 aorto-arterial aneurysms
Click here to view |  | Figure 4 :(a) Thickening of the wall of the proximal right coronary artery (H and E, ×100); (b) Destruction of the media by inflammation and fibrosis (H and E, ×250); (c) Fibrotic adventitia showing obliterative change (H and E, x250); (d) Inflammation in the media rich in plasma cells (H and E, ×400)
Click here to view |  | Figure 5 :The heart has been cut longitudinally to show a saccular protrusion in the proximal ascending aorta (arrows) with a larger fusiform aneurysm involving the remaining portion
Click here to view |
| Discussion | | |
Cardiovascular syphilis, a relatively common disease at the turn of the 20 th century, [2] showed a steady decline in the incidence in the developed countries from 1-15 % to 0.7-4 %. [4] However, syphilitic aortitis continues to be the basis of many case reports in the west, thereby indicating that 'it is dying but not dead'. [5] This evolution into the tertiary stage of syphilis can be explained on the basis of poorly defined optimal curative dosage of penicillin. [6] We found a prevalence of 23.5% among the non-atherosclerotic aortic disease. The prevalence, pattern of age and gender predilection and involvement of various segments of the aorta are almost similar when compared to the two large autopsy series. [4],[7] Surprisingly, the abdominal aorta (supra-renal portion) was variably affected in 22.7% of patients, compared to an incidence of 3 to 9% in the other studies. [4],[7] We did not have a single case of uncomplicated syphilitic aortitis, though silent disease was found in six patients (13.6 %); asymptomatic disease is said to be the most prevalent form. [2] Complications in the form of aortic regurgitation (72.7%), coronary ostial stenosis (59%) and aneurysms (59%) were present in almost equal proportions and frequently coexisted, an incidence higher than those quoted. [2],[4],[7] Though aortic regurgitation and aneurysms occurred as sole complications, coronary ostial stenosis was not seen as an isolated complication. One of our patients also showed arteritis of the right coronary artery, a rare manifestation [8] and the patient had presented with features of IHD. Combination of aortic regurgitation and saccular aneurysm is considered as an uncharacteristic finding. [2] This was identified in eight patients. Besides, five of the 33 aneurysms (15%) also involved the abdominal aorta, which is relatively a higher incidence as compared to those in literature. [9] Superimposed atherosclerosis can also have its effects, since PVD was the primary manifestation in one patient; infrequently the atherosclerosis in syphilitic aortitis can present as penetrating atherosclerotic ulcer. [10]
The diagnosis of syphilis in general and aortitis in particular faces hurdles in the developing countries. The control of the disease is often plagued by stigma and secrecy associated with it, improper or lack of safety measures and incomplete therapy, explaining a high seroprevalence. [11] Also, the principle "It is a sound rule rarely to diagnose conditions that occur rarely" is often deeply ingrained so that the condition is sometimes overlooked. [5]
Among the 29 patients with a clinical diagnosis of aortic regurgitation and/or aneurysm, the cause was attributed to syphilitic aortitis in 44.9 % of the patients. Therefore, it would be important to employ serological tests to aid the diagnoses in aortic incompetence, especially when there is absence of involvement of the mitral valve clinically. [12] In addition, a cut-off value of 1:8 VDRL positivity and use of improvised serological reactions for the diagnosis would be required. [13] Along with serological tests in all thoracic aneurysms, it would also be important to sample of the diseased aorta during aortic valve replacements and/or aneurysm surgeries; this would certainly help in clinching the etiologic diagnosis.
It must be remembered that both gross and histological features of syphilitic mesoaortitis may be produced by other disease entities, even systemic lupus erythematosus. [14] To circumvent the discrepancies in the serological and pathological features in syphilis, use of polymerase chain reaction has been advocated. [15] This may not be feasible at all centers and hence it would be prudent to fall back on the clinical acumen and relate the clinical context to the interpretation of the serology and pathological features of the disease. Infection with HIV and syphilis occur in similar settings and hence it is possible that syphilis may have an increased incidence and may also pursue a more aggressive course.
| References | | |
| 1. | Harper KN, Ocampo PS, Steiner BM, George RW, Silverman MS, Bolotin S, et al. On the origin of the treponematoses: a phylogenetic approach. PLoS Negl Trop Dis 2008;2:e148. 
[PUBMED] [FULLTEXT] | | 2. | Jackman JD, Radolf JD. Cardiovascular syphilis. Am J Med 1989;87:425-33.
| | 3. | Vaideeswar P, Deshpande JR, Sivaraman A. Aortocaval fistula: a rare complication of abdominal aortic aneurysm. Indian Heart J 2000;52:597-8.
[PUBMED] | | 4. | Bhaskara Reddy D, Ranganayakamma I. Syphilitic aortitis (A clinico-pathologic and autopsy study of 32 cases during the 11-year period from 1955to 1966). Indian Heart J 1967;19:86-95.
| | 5. | Cheng TO. Syphilitic aortitis is dying but not dead. Catheter Cardiovasc Interv 2001;52:240-1.
[PUBMED] [FULLTEXT] | | 6. | Augenbraun M. Treatment of latent and tertiary syphilis. Hosp Pract 2000;35:89-95.
| | 7. | Heggtveit HA. Syphilitic aortitis. A clinicopathologic autopsy study of 100 cases, 1950 to 1960. Circulation 1964;29:346-55.
[PUBMED] [FULLTEXT] | | 8. | Lie JT. Coronary vasculitis. A review in the current scheme of classification of vasculitis. Arch Pathol Lab Med 1987;111:224-33.
[PUBMED] | | 9. | Williams J, Krishnan G, Devarajan H. Syphilitic abdominal aortic aneurysm. AIDS Patient Care STDS 2002;16:467-70.
[PUBMED] [FULLTEXT] | | 10. | Saleem MA, McNeeley M, Zias E, Pucillo A, Ro JH, Weiss MB. Penetrating ulcer of ascending thoracic aorta in syphilis. Catheter Cardiovasc Interv 2004;61:16-9.
[PUBMED] [FULLTEXT] | | 11. | Kaur V, Kaur P, Singh S. Sexual and treatment behaviour of STD patients. Indian J Sex Transm Dis 1992;13;83-6.
| | 12. | Reynolds DL, Evangelista F, Ward BM, Notenboom RH, Young ER, D'Cunha CO. Syphilis in an urban community. Can J Public Health 1998;89:248-52.
[PUBMED] | | 13. | Thakar YS, Chande C, Mahalley AD, Saoji AM, Seroprevalence of syphilis by TPHA test. Indian J Pathol Microbiol 1996;39:135-8.
| | 14. | MacCleod CS, Johnson D, Frable WJ. "Tree-barking" of the ascending aorta. Syphilitic or systemic lupus erythematosus. Am J Clin Pathol 1992;97:58-62.
| | 15. | O'Regan AW, Castro C, Lukehart SA, Kasznica JM, Rice PA, Joyce-Brady ME. Barking up the wrong tree? Use of polymerase chain reaction to diagnose syphilitic aortitis. Thorax 2002;57:917-8.
|
Correspondence Address:
Pradeep Vaideeswar
Department of Pathology, Seth G. S. Medical College and KEM Hospital, Parel, Mumbai 400 012
India
  | 8 |
DOI: 10.4103/0377-4929.72002 PMID: 21045381
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
[Table 1], [Table 2], [Table 3] | |
| This article has been cited by | | 1 | Early recognition of aortitis of the aorta ascendens with 18F-FDG PET/CT: syphilitic? | | | H. Balink,A. Spoorenberg,P. M. Houtman,A. Brandenburg,H. J. Verberne | | Clinical Rheumatology. 2013; 32(5): 705 | | [Pubmed] | | | 2 | Patología aórtica no urgente: diagnóstico clínico-radiológico de la aortitis | | | J. Cabero Moyano,M. Andreu Magarolas,E. Castañer González,X. Gallardo Cistaré,E. Belmonte Castan | | Radiología. 2013; 55(6): 469 | | [Pubmed] | | | 3 | Anévrismes infectieux de l’aorte thoracique : présentation de 7 cas et revue de la littérature | | | S. Roux,T. Ferry,C. Chidiac,A. Bouaziz,J. Ninet,L. Pérard,F. Farhat,C. Broussolle,P. Sève | | La Revue de Médecine Interne. 2013; | | [Pubmed] | | | 4 | Nonurgent aortic disease: Clinical-radiological diagnosis of aortitis | | | J. Cabero Moyano,M. Andreu Magarolas,E. Castañer González,X. Gallardo Cistaré,E. Belmonte Castan | | Radiología (English Edition). 2013; 55(6): 469 | | [Pubmed] | | | 5 | Infectious and Noninfectious Aortitis: Cross-Sectional Imaging Findings | | | Katabathina, V.S., Restrepo, C.S. | | Seminars in Ultrasound, CT and MRI. 2012; 33(3): 207-221 | | [Pubmed] | | | 6 | Primary Vascular Infection | | | Valentine, R.J., Chung, J. | | Current Problems in Surgery. 2012; 49(3): 128-182 | | [Pubmed] | | | 7 | Syphilitic aortic aneurysm with spastic paraparesis: A novel presentation and review of the literature | | | Seid Ali Gugssa,James C. Johnston | | Journal of the Neurological Sciences. 2012; 323(1-2): 241 | | [Pubmed] | | | 8 | Infectious and Noninfectious Aortitis: Cross-Sectional Imaging Findings | | | Venkata S. Katabathina,Carlos S. Restrepo | | Seminars in Ultrasound, CT and MRI. 2012; 33(3): 207 | | [Pubmed] | | | 9 | Primary Vascular Infection | | | R. James Valentine,Jayer Chung | | Current Problems in Surgery. 2012; 49(3): 128 | | [Pubmed] | | | 10 | Syphilitic Aortic Aneurysm in a Young HIV-Infected Man: Case Presentation | | | Juan Carlos Cataño,Isabel Cristina Ramirez | | Case Reports in Infectious Diseases. 2011; 2011: 1 | | [Pubmed] | |
| | | | | | | | | | | |
| Article Access Statistics | | | Viewed | 5887 | | | Printed | 112 | | | Emailed | 4 | | | PDF Downloaded | 111 | | | Comments | [Add] | | | Cited by others | 10 | | | | 
|
