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Year : 2003  |  Volume : 13  |  Issue : 1  |  Page : 61-66
Aortoarteritis : A study of 33 central Indian patients


Dept. of Radiodiagnosis, Govt. Medical College, Nagpur-440003, India

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   Abstract 

OBJECTIVE : To evaluate the clinical profile, complications, site of involvement and response to therapy and interventional management of aortoarteritis. MATERIAL AND METHODS : 33 patients with Aortoarteritis were studied over a period of two years between Jan 2001 to Nov 2002. Data on clinical features, laboratory and angiographic findings, disease course, response to therapy and interventional management were all recorded and stored in a computer based retrieval system. RESULTS : In our series of 33 patients (21 female, 12 male) with angiographically diagnosed Aortoarteritis, females (63.64 percent) were most frequently affected. The median age of onset was 29 years. The clinical presentation ranged from asymptomatic to catastrophic with stroke. Patients had both non-vascular symptoms (fever 21.21 percent, arthralgia 9 percent) and symptom of vascular stenosis such as limb claudication (66.67 percent), headache (66.67 percent), and hypertension due to renal artery Stenosis (42 percent). All patients had either multiple vascular bruits (76 percent), absent pulses (18 percent), or asymmetric pulses (66.67 percent) and blood pressure differences greater than 10 mm hg between arm (66.67 percent). Laboratory findings included elevated erythrocyte sedimentation rate (87.88 percent), C-reactive protein (30.30 percent), one patient had Mantax test positive. However, E.S.R. was not a consistently reliable surrogate marker of the disease activity. Almost all patients had multiple sites of arterial involvement documented by angiogram with various combinations of stenosis, Luminal irregularity and aneurysm formation. Type IV (63.64 percent) (i.e. involvement of abdominal aorta and or renal arteries) was more commonly found in our study. Response to corticosteroid treatment was usually very good, with dramatic improvement in non-vascular symptoms. SIX patients underwent percutaneous transluminal angioplasty involving the abdominal aorta (2 cases), subclavian artery (3cases) and renal artery (lease). CONCLUSIONS : In central India, Aortoarteritis is a rare disease. However, Type IV is more common. It is heterogeneous in presentation, progression, and response to therapy. Current laboratory markers of disease activity are insufficiently reliable to guide management. Most patients require repeated and at times, prolonged course of therapy. After percutaneous transluminal, balloon angioplasty (P.T.A.) clinical parameters were normal. Although mortality was low, substantial morbidity occurred in most patients.

Keywords: Takayasu arteritis, Aortitis syndrome, pulseless disease, occlusive thromboarteritis, occlusive thromboaortopathy

How to cite this article:
Salkar R G, Parate R, Taori K B, Parate T R, Salkar H R, Mahajan S. Aortoarteritis : A study of 33 central Indian patients. Indian J Radiol Imaging 2003;13:61-6

How to cite this URL:
Salkar R G, Parate R, Taori K B, Parate T R, Salkar H R, Mahajan S. Aortoarteritis : A study of 33 central Indian patients. Indian J Radiol Imaging [serial online] 2003 [cited 2014 Aug 1];13:61-6. Available from: http://www.ijri.org/text.asp?2003/13/1/61/28629

   Introduction Top


Aortoarteritis is a chronic inflammatory disorder of large elastic arteries usually affecting the aorta and its larger branches and pulmonary arteries.[1]

Non-specific aortoarteritis (NSAA) was introduced to medical literature in 1827 by R.Adams, who was the first physician to note the absence of pulses in all the four extremities. [2]. Mikito Takayasu, described a peculiar wreath -like appearance of the retinal blood vessels with the absence of the radial pulse in 1908, at the Annual Meeting of Japan Ophthalmic Society.[3]

The clinical features usually reflect limb or organ ischemia resulting from gradual stenosis of the involved arteries. Females are more commonly affected than males. NSAA has protean manifestations and symptoms and signs differ with geographical location. The diagnosis is made by a set of clinical and investigation parameters. Aortoarteritis results in occlusion and aneurysm formation of arteries in its chronic phase. Cases have been reported from Japan, Mexico, North America and India. The exact etiopathogenesis of the disease is not yet established.

The modern state of the art investigation like axial CT, COLOR DOPPLER, MRA and ANGIOGRAPHY have suggested that this disease involves continuous aortic segments, producing wall and luminal changes in some areas. Early diagnosis is also made possible by modern investigations. But the major breakthrough came in the field of management. Since 1990, the introduction of long term steroid with or without chemotherapeutic agents, angioplasty, stenting and reconstructional surgeries have revolutionized the treatment of aortoarteritis. This study has established the efficacy and safety of these treatment options in appropriate situations.


   Materials and Methods Top


Thirty-three patients with aortoarteritis were studied over a period of approx two years from Jan2001 To November 2002.Patients were recruited as per protocols and without bias to economic, social, or racial status. The diagnosis was based on the presence of symptoms and signs of ischemic, inflammatory large-vessel disease, as well as supportive arteriographic findings. All patients had arteriographic studies and had abnormalities that included multifocal areas of stenosis or aneurysm formation (or both) of the aorta or its primary branches (or both).

Once enrolled, patients were prospectively followed and assigned to treatment protocols if their disease was active. The frequency of follow-up depended on the disease activity and treatment varied from monthly to yearly visits. Each visit included a complete history and physical examination, routine laboratory tests, blood pressure measurement in all four limbs, electrocardiogram, and chest radiograph. A complete aortogram was done if active, new disease was suspected or if required by specific treatment protocols. Angiography was done by intra-arterial injection of approximately 100ml of non-ionic contrast media i.e. Iohexol (300 mg%) using femoral puncture under DSA guidance.


   Results Top


Thirty three patients of Aortoarteritis were followed during a median period of approx two years. The cohert included 21 females and 12 males. The median age of presentation was 29 years (range, 12 to 40 years).

Our study revealed the symptoms as: claudication of extremities (22 =66.67%)and headache (22 =66.67%), syncope (12=33.36%), fever (7=21.21%), weakness of the extremities (5=15.15%), blurring of vision (4=12.12%), abdominal pain (4=12.12%), arthralgia (3=9.09%), diplopia and convulsions (2=6.06%), chest pain, palpitation and myalgia (1=3.03%). Clinically the patients had asymmetric pulses in 22 cases (66.67%), blood pressure difference (>10 mm of Hg.) in 22 cases (66.67%) and hypertension in 14 cases (42.42%). Bruit was heard in the abdominal aorta (8=24.24%), renal (7=21.21%), subclavian (6=18.18%) and common carotid artery (4=12.12%).

Laboratory investigations showed a raised ESR in 29 cases (87.88%), raised C-reactive protein in 10 (30.30%) and Mantoux test positive in 1 (3.03%). Hypertensive ischemic retinopathy stage-I was found in 4 cases (12.12%). Complications were hypertension (14=42.42%), stroke (5=15.15%), aneurysm/dilatation (4=12.12%).

In our study the vascular procedure was performed in 6 cases, percutaneous balloon angioplasty and stenting in subclavian 3 (9.09%), abdominal aorta 2 (6.06%) and left renal artery 1 (3.03%). Follow up of these six patients after percutaneous transluminal balloon angioplasty with stenting was found to normalize the clinical symptoms, blood pressure within 24-hours with improvement of exercise tolerance and restoration of peripheral pulses.


   Discussion Top


We have prospectively studied 33 cases with Aortoarteritis to evaluate their clinical profile and outcome of therapeutic interventions. This study is unique: 1) Angiographic evidence of disease was required in all patients. 2) Sequential angiography was used to follow disease progression. 3) Defined criteria for disease activity were used and 4) Established guideline for the treatment of active disease was applied. The median period of follow up for the patient was two years.

The median age at disease onset of (29 year) is similar to that seen in Asian countries. [4],[6][7] This finding is in contrast to the later age of onset (41 years) reported in Europe [8-9] Females predominated, as previously reported in most other series of the patients. Males are not exempt, however, and some authors report almost equal occurrence in both sexes [10].

The spectrum of presentation, disease severity, and pace of disease progression can often lead to an inaccurate assessment and a delay in diagnosis. In this study the delay in diagnosis may reflect failure to recognize the disease and, therefore, the hesitancy of clinicians to request diagnostic angiographic studies. The limited reliability of clinical, laboratory and angiographic markers of disease activity was also evident. Systemic symptoms occurred in only a third of patients at the onset of disease and in an additional ten percent throughout their disease course. These findings fail to support the notion that most patients with Takayasu arteritis have a "triphasic " illness characterized by asymmetric pulses, prepulseless inflammation, followed by painful ischemic vessels, and ending in "burnt-out disease " [11][12].

Vascular ischemic symptoms were the hallmark of the disease and were apparent as a bruit, claudication, or diminished pulses. Most of the patients we studied had extensive disease of the aorta and it's branches, and stenotic lesions were more common than were aneurysms. Calcification was uncommon but has been reported in 10-25% of patients [13].

Hypertension was seen in 42.42% cases in our series of patients and correlated significantly with the presence of renal artery stenosis [11]. Abnormal vascular compliance and dysfunctional baroreceptors are alternative pathophysiologic mechanisms that may account for hypertension in the absence of renal artery stenosis [14].In our series of patients the complication was found as hypertensive ischemic retinopathy stage-I in 12.12%, stroke in 15.15% due to carotid artery involvement.

The erythrocyte sedimentation rate was elevated in 87.88% of patients and C-reactive proteins was elevated in 30.30% of patients with active disease. There the erythrocyte sedimentation rate was an unreliable marker of the disease course. Other reports support this evaluation, although some have found the erythrocyte sedimentation rate to be a reliable and usual marker to determine the therapy [11][15]. One patient was found Mantoux positive (3.03%). Angiography is considered the gold standard in delineating abnormal vessels in patients with Takayasu arteritis.

In this study, angiograms were done in all clinically suspected patients of aortoarteritis. Most of the patients showed affection of the abdominal aorta and/or renal artery i.e. type-IV (63.64%) of the proposed classification at the international conference on Takayasu arteritis (Masato Okada et al-2001). Similar angiographic features of Takayasu arteritis have been reported in India, Thailand and Mexico[6],[11].Our data established that although angiography was sensitive in detecting abnormal anatomy in patients with Takayasu arteritis, it could not determine the cause of vascular narrowing(e.g. active inflammation as apposed to intimal and adventitial fibrosis) of the vessel wall. Therefore, angiographic findings in patients with Takayasu arteritis, like ESR, have to be interpreted within the clinical context.

Glucocorticoids still remains an effective palliative agent for most patients with active Takayasu arteritis. Anti-hypertensive and decongestive therapy is given according to individual need. The drug response to renovascular hypertension was poor.

Because of multifocal involvement and dense transmural fibrosis, surgical reconstructive procedures are difficult and are associated with significant morbidity, mortality and postoperative complications. Percutaneous transfemoral balloon angioplasty (PTA) has emerged as an effective, less invasive and safer technique in this debilitating disease. Recently implantations of endovascular stent are used in aortoarteritis [16],[17]. In our study PTA with stenting was performed in six patients with 100% success rate. After PTA and stenting clinical parameters were normal.

So we can conclude that aortoarteritis is a rare disease of unknown etiology. It has a heterogeneous presentation, progression and response to therapy. Current laboratory markers of the disease activity are insufficiently reliable to guide management. Most patients require repeated and, at times, prolonged course of therapy, although mortality is low, substantial morbidity occurred in most patients. Angiography is a gold standard to detect not only the multiple sites but also the extent and severity of the disease. PTA with stenting is an effective, less invasive and safer technique in this debilitating disease.


   Acknowledgements Top


We extend our sincere thanks to the department of Radiology, GMC, Nagpur with special reference to Dr.Varsha Rangankar, Dr.S.Kothekar, Dr.A Rewatkar and R. Mundhada .for extending their full support and co-operation.

 
   References Top

1.Di Giacomo V:A case of Takayasu disease occurred over two hundred years ago; Angiology ,1984;35(11):750-4.  Back to cited text no. 1    
2.Adam R: Cases of diseases of the heart accompanied with pathological observations; Medical classics3:690:1939(Reprinted from Dublin Hospital Reports and communications in Medicine and Surgery;1827;4:443.  Back to cited text no. 2    
3.Takayasu M: A case with unusual changes of the central vessel in the retina. Acta.soc.ophth Jap1908;12:554.  Back to cited text no. 3    
4.Ishikawa K:Diagnostic approach and proposed criteria for the clinical diagnosis of Takayasu arteriopathy; J Am Col Cardiol.1988;12:964-72.  Back to cited text no. 4    
5.Masato Okada, Jastin D Pearlman et al:Aortitis: emedicine Journal,Octomber18 ,2001;2:10.  Back to cited text no. 5    
6.Subramanyan R, Joy J, Balakrishnan KG: Natural history of Aortoarteritis(Takayasu disease); Circulation. 1989; 80:429-37  Back to cited text no. 6    
7.Zheng DY,Liu LS,Fan DJ:Clinical studies in 500 patients with aortoarteritis. Chin Med J (Engl).1990;103:536-40.  Back to cited text no. 7    
8.Waern AU,Andersson P,Hemmingsson A; Takayasu arteritis:A hospital region based study on occurance,treatment and prognosis; Angiology. 1983; 34: 311-20.  Back to cited text no. 8    
9.Di Giacomo V,Meloni F,Transi MG et all; Takayasu disease in middle -aged women :a clinicopathologic study; Angiology.1985;36:70-4.  Back to cited text no. 9    
10.Chugh KS,Sakhuja V.; Takayasu arteritis as a cause of renovascular hypertension in Asiancountries[editorial]; Am J Nephrol.1992;12:1-8.  Back to cited text no. 10  [PUBMED]  
11.Lupi-Herrera E,Sanchez-Torres G,Marchushamer J et al; Clinical study of 107 cases; Am Heart J.1977;93:94-103  Back to cited text no. 11    
12.Hall S,Barr W, Lie JT,Stanson AW,et al;Takayasu arteritis.A study of 32 North American patients; Medicine. 1985; 64: 89-99.  Back to cited text no. 12    
13.Yamato M,Lecky JW,Hiramatsu K et al; Takayasu arteritis:radiographic and angiographic findings in 59 patients; Radiology. 1986;161:329-34.  Back to cited text no. 13    
14.Ask-Upmark E.; On pathogenesis of the hypertension in Takayasu syndrome. Acta Medica Scandinavica. 1961; 169: 467-77.  Back to cited text no. 14    
15.Weaver FA, Yellin AE.; Surgical treatment of Takayasu arteritis. Heart Vessels Suppl.1992;7:154-8.  Back to cited text no. 15    
16.Tyagi S, Kaul UA,Arora R et al; Endovascular stenting for unsuccessful angioplasty of the aorta in aortoarteritis; Intervent.Radiol.1999;22:452-56.  Back to cited text no. 16    
17.Rao SA,Mandalam KR, Rao VR et al; Takayasu arteritis initial and long term follow up in 16 patients after percutaneous angioplasty of the descending thoracic and abdominal aorta; Radiology ,1993;189:173-79.   Back to cited text no. 17    

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Correspondence Address:
R G Salkar
Dept. of Radiodiagnosis, Govt. Medical College, Nagpur-440003
India
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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8]

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[Table - 1], [Table - 2]

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