Takayasu Arteritis a Review S L Johnston R J Lock and M M Gompels

  • Journal Listing
  • J Clin Pathol
  • v.55(seven); 2002 Jul
  • PMC1769710

J Clin Pathol. 2002 Jul; 55(7): 481–486.

Takayasu arteritis: a review

S L Johnston

Department of Immunology and Immunogenetics, Southmead Hospital, Westbury on Trym, Bristol BS10 5NB, United kingdom

R J Lock

Section of Immunology and Immunogenetics, Southmead Infirmary, Westbury on Trym, Bristol BS10 5NB, UK

M M Gompels

Department of Immunology and Immunogenetics, Southmead Infirmary, Westbury on Trym, Bristol BS10 5NB, UK

Abstract

Takayasu arteritis is a well known nevertheless rare form of large vessel vasculitis. This review details the history, clinical features, differential diagnoses, classification, and immunology of the disorder. Suppression of inflammation and preservation of vascular competence are the aims of treatment. As with any rare illness, randomised controlled handling trials are either lacking or based on pocket-sized patient numbers, making management decisions hard. Electric current evidence based treatments are presented and discussed.

Keywords: arteritis, pathogensis, Takayasu, treatment

Takayasu arteritis, also known as pulseless disease, occlusive thromboaortopathy, and Martorell syndrome,1 is a chronic inflammatory arteritis affecting large vessels, predominantly the aorta and its main branches. Vessel inflammation leads to wall thickening, fibrosis, stenosis, and thrombus formation. Symptoms reflect finish organ ischaemia. More astute inflammation can destroy the arterial media and lead to aneurysm germination.2 Early reports suggested that the disease was confined to females from Eastern Asia, simply it has now been recognised worldwide in both sexes, although disease manifestations vary betwixt populations. The female person to male person ratio appears to pass up from Eastern Asia towards the Westward.

HISTORY

Published descriptions of this arteritis appointment back every bit far as 1830.ii Yamamoto described the case of a 45 year quondam human being with persistent fever who developed impalpable upper limb and carotid pulses associated with weight loss and dyspnoea.3 In 1905 Takayasu, professor of ophthalmology at Kanazawa Academy Japan, presented the instance of a 21 year former adult female with characteristic fundal arteriovenous anastamoses.4 In the aforementioned twelvemonth, Onishi and Kagosha each described similar cases associated with absent radial pulses.3 In 1920, the first postmortem case of a 25 year erstwhile woman demonstrated panarteritis and suggested that the fundal appearances resulted from retinal ischaemia.2 In 1951, Shimizu and Sano summarised the clinical features of this "pulseless illness".five

INCIDENCE

Takayasu arteritis is rare, but most commonly seen in Nippon, South East Asia, India, and United mexican states. In 1990, it was included in the list of intractable diseases maintained by the Japanese authorities,ii and to date 5000 patients have been registered. A study of North American patients past Hall et al constitute the incidence to be 2.6/million/yr.6 The U.k. incidence is unknown.

CLINICAL FEATURES

The clinical features take been well documented by accomplice studies of over 570 patients from different countries.1, 6– xiii Manifestations range from asymptomatic illness plant as a consequence of impalpable pulses or bruits, to catastrophic neurological impairment. A two stage procedure has been suggested with a "pre-pulseless" phase characterised by non-specific inflammatory features, followed by a chronic phase with the development of vascular insufficiency, in some cases accompanied by intermittent flares, although not all patients adapt to this blueprint.half dozen

"As the inflammation progresses and stenoses develop, the more characteristic features become apparent, influenced by the development of collateral circulation"

The disease unremarkably presents in the 2nd or 3rd decade of life, ofttimes with a delay in diagnosis from the onset of commencement symptoms of months to years. In i of the largest cohorts (due north = 107) lxxx% of patients were between eleven and 30 years, 77% had disease onset between the ages of 10 and 20 years, with time from onset of symptoms to diagnosis of ii to 11 years in 78%.1 A written report of 88 patients from India9 gave a hateful (SD) age at symptom onset of 24.0 (8.8) years and mean (SD) age at diagnosis of 28.three (ix.9) years. The National Institute of Wellness study by Kerr et al suggested that the delay in diagnosis was longer in juveniles, beingness up to four times that of adult patients.ten Notwithstanding, data from India12 looking at patients aged nether eighteen years demonstrated a delay of only 2.5 to 5.5 months. This discrepancy presumably relates to the difference in disease incidence between the two populations, which results in differences in sensation. The clinical features and progress of young patients with Takayasu arteritis appear to be very similar to those of adults.12

Not-specific features include fever, nighttime sweats, malaise, weight loss, arthralgia, myalgia, and mild anaemia.6 As the inflammation progresses and stenoses develop, the more characteristic features go apparent, influenced past the development of collateral apportionment. Stenotic lesions predominatenine, 10 and tend to be bilateral. Nearly all patients with aneurysms also accept stenoses and about have extensive vascular lesions.

Feature FEATURES

  • Diminished or absent pulses in 84–96% of patientsi, nine associated with limb claudication and blood force per unit area discrepancies.

  • Vascular bruits in lxxx–94% of patients,1, half dozen, 10 oftentimes multiple, and specially affecting the carotids, subclavian, and abdominal vessels.

  • Hypertension in 33–83% of patients,i, 6, 7, ten, 12 more often than not reflecting renal artery stenosis, which is seen in 28–75% of patients.one, 10, 12

  • Takayasu retinopathy in upwardly to 37% of patients.six, 7

  • Aortic regurgitation resulting from dilatation of the ascending aorta, separation of the valve leaflets, and valve thickening in 20–24%.9, 10

  • Congestive cardiac failure associated with hypertension, aortic regurgitation, and dilated cardiomyopathy.ix

  • Neurological features secondary to hypertension and/or ischaemia, including postural dizziness, seizures, and amaurosis.

  • Pulmonary artery interest in 14–100% of patients, depending on the method used to assess pulmonary vasculature. Oligaemic lung fields on plain chest 10 ray correlate with pulmonary vasculopathy in approximately a third of cases.14 Pulmonary artery disease shows little correlation with the systemic pattern of arterial involvement,7, 14 merely can be useful in the differential diagnosis by helping to confirm Takayasu arteritis.

  • Other symptoms include dyspnoea, headaches, carotodynia, myocardial ischaemia, chest wall hurting, and erythema nodosum.

Variable disease presentation betwixt unlike populations is well illustrated past Moriwaki et al in their report of Indian and Japanese patients.xi The Japanese patients (n = 80) were predominantly female (96%), presenting with dizziness, vertigo, pulselessness, more prolonged and astringent inflammation, and more aortic regurgitation, reflecting involvement of the aortic arch and its main branches. This contrasted with the Indian patients (n = 102), 37% of whom were male. They tended to nowadays with headache, hypertension, and left ventricular hypertrophy as a result of vasculitis affecting the abdominal aorta and renal vessels. However, almost patients in both countries had diffuse disease.

DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS

From the more than typical features of Takayasu'south arteritis, the American College of Rheumatology (ACR) defined specific diagnostic criteria for this disorder in 1990 (table i ).15 Angiography remains the gold standard for diagnosis (figs 1, 2 ). Assessment of pulmonary vasculature by angiography is non universally recommended, being reserved for patients with symptoms of pulmonary hypertension.x Doppler ultrasound is a useful non-invasive procedure for the assessment of vessel wall inflammation. In view of the vessels involved, histological diagnosis is usually impractical and histological assessment is limited to those cases undergoing revascularisation procedures.

An external file that holds a picture, illustration, etc.  Object name is 01223.f1.jpg

Arch aortogram demonstrating (A) a severely narrowed right common carotid artery, (B) occlusion of the left common carotid artery and, (C) proximal stenosis of the left subclavian artery. (D) The right vertebral avenue provides the dominant cognitive supply.

An external file that holds a picture, illustration, etc.  Object name is 01223.f2.jpg

Curvation aortogram demonstrating severe involvement of all extracranial vessels; the descending thoracic aorta appears to be normal.

Tabular array i

1990 ACR criteria for the nomenclature of Takayasu arteritisxv

Criterion Definition
Age at disease onset ≤40 years Development of symptoms or findings related to Takayasu arteritis at age ≤40 years
Claudication of extremities Evolution and worsening of fatigue and discomfort in muscles of 1 or more than extremity while in use, especially the upper extremities
Decreased brachial artery pulse Decreased pulsation of 1 or both brachial arteries
Blood pressure divergence >10 mm Hg Divergence of >10 mm Hg in systolic blood pressure between arms
Bruit over subclavian arteries or aorta Bruit aural on auscultation over one or both subclavian arteries or abdominal aorta
Arteriogram aberration Arteriographic narrowing or occlusion of the entire aorta, its master branches, or large arteries in the proximal upper or lower extremities, not caused by arteriosclerosis, fibromuscular dysplasia, or similar causes; changes commonly focal or segmental

A diagnosis of Takayasu arteritis requires that at least 3 of the 6 criteria are met.

The differential diagnoses include other causes of large vessel vasculitis: inflammatory aortitis (syphilis, tuberculosis, lupus, rheumatoid arthritis, spondyloarthropathies, Behçet'due south illness, Kawasaki disease, and giant cell arteritis); developmental abnormalities (coarctation of the aorta and Marfan syndrome), and other aortic pathologies, such as ergotism and neurofibromatosis. Most of these take specific features that enable diagnosis, simply tuberculosis has remained an important differential and possible aetiological factor. However, tuberculous aortitis tends to cause erosion of the vessel wall with the germination of true or false aneurysms, especially affecting the descending thoracic and abdominal aorta. Dissection and rupture are important complications rather than the stenoses typical of Takayasu arteritis. The incidence of rupture and haemorrhage complications of aneurysmal Takayasu arteritis is depression. Syphilis tends to affect an older age group, with calcification, sparing the descending thoracic aorta, and stenoses are non a characteristic.9 Hypertension as a result of fibromuscular dysplasia is an of import differential diagnosis.

Although similar in many respects, including aortic involvement in 10–fifteen% of patients with behemothic cell arteritis, Michel et al suggest that giant jail cell arteritis and Takayasu arteritis tin can be differentiated on clinical grounds. In a study of 280 patients, 217 with giant jail cell arteritis and 63 with Takayasu arteritis identified through the ACR vasculitis criteria databank, they found that age of forty years at disease onset was the single most discriminatory factor. Excluding age from the analysis, ethnic background and clinical signs of upper limb vascular insufficiency, shoulder stiffness, and scalp tenderness were variables that led to correct diagnoses in 95% of patients.16

Classification

An attempt has been made to classify the disease on the basis of angiographic findings. The early arrangement, revised by Lupi-Herrera et al in 1977,1 has been superseded by the new classification of Takayasu arteritis (table ii ).eleven These systems are useful in that they allow a comparison of patient characteristics according to the vessels involved and are helpful in planning surgery, simply they offer little by way of prognosis.

Table 2

New angiographic classification of Takayasu arteritis, Takayasu briefing 199411

Type Vessel involvement
Type I Branches from the aortic arch
Type IIa Ascending aorta, aortic arch and its branches
Blazon IIb Ascending aorta, aortic arch and its branches, thoracic descending aorta
Type III Thoracic descending aorta, intestinal aorta, and/or renal arteries
Type 4 Abdominal aorta and/or renal arteries
Type 5 Combined features of types IIb and IV

According to this classification organisation, involvement of the coronary or pulmonary arteries should be designated as C (+) or P (+), respectively.

Almost patients in the large series studied take diffuse illness.

The natural history of any disorder tin can merely be elucidated by following patients in the absence of specific treatment. Ishikawa defined clinical groups based on the natural history and complications of the disease.7 The 4 most important complications were defined every bit Takayasu retinopathy, secondary hypertension, aortic regurgitation, and aneurysm germination, each being graded as mild/moderate or astringent at the time of diagnosis. Iv grades of disease are described (tabular array 3 ).

Tabular array 3

Ishikawa clinical classification of Takayasu arteritisseven

Grouping Clinical features
Group I Uncomplicated disease, with or without pulmonary avenue involvement
Group IIA Balmy/moderate single complication together with unproblematic disease
Group IIB Severe unmarried complication together with unproblematic illness
Group Three Two or more complications together with uncomplicated disease

"Tuberculosis has remained an important differential and possible aetiological factor"

Ishikawa retrospectively studied 54 Japanese patients over half-dozen months to xviii years of follow upward between 1957 and 1975. The overall five yr survival rate after diagnosis was 83.1%. Vii patients died within five years of diagnosis, all were in groups IIB and III, and deaths were mostly from cerebrovascular disease and congestive cardiac failure. All patients with aortic regurgitation were in grouping III. The five year survival rate in combined groups IIB and 3 was lxx%, compared with 100% in group I. Five acute events occurred in the survivors during follow up, three of five occurring in patients from groups IIB and Three. No acute event occurred in patients from group I. Xix of the 54 patients were treated with steroids.

The experience from India supports this nomenclature for prognostic assessment.9 Cumulative survival at five years after disease onset was 91%, later on ten years the figure was 84%, whereas issue free survival figures were 74.9% and 64%, respectively. Patients with a single mild complexity or no complication at diagnosis had a five year upshot free survival of 97%, compared with 59.7% in patients with a single severe or multiple complications. No deaths occurred in patients in groups I and IIA, whereas 19.vi% of patients in groups IIB and III died during follow upward, by and large from cerebrovascular disease and cardiac failure. Twenty two major not-fatal events occurred during follow up, with 20 of 22 occurring in groups IIB and Iii. In this study, 63 of 88 patients received no specific disease modifying treatment. Other studies, which have included patients treated more than aggressively, give five year survival rates of xc–94%.6, thirteen Therefore, nomenclature co-ordinate to this system appears to requite useful prognostic information at diagnosis and may help to guide treatment.

HISTOLOGY, IMMUNOLOGY, AND PATHOGENESIS

Macroscopically, in the chronic phase, the aorta is thickened secondary to fibrosis of all three vessel layers. The lumen is narrowed in a patchy distribution, often affecting multiple areas. If affliction progression is rapid, fibrosis can be inadequate with subsequent aneurysm germination. The intima may exist ridged, with a "tree bark" appearance, a characteristic common to many aortitides.17

Microscopically, the vasculitis may be divided into an astute florid inflammatory phase and a healed fibrotic phase. In the acute phase a vasa vasoritis is seen in the adventitia. The media is infiltrated by lymphocytes and occasional behemothic cells with neovascularisation. Mucopolysaccharides, smooth muscle cells, and fibroblasts thicken the intima. In the chronic stage there is fibrosis with destruction of elastic tissue. Similar histopathological findings are also seen in giant cell arteritis; therefore, biopsy results may not differentiate between these two vasculitides. Clinical features usually allow right diagnosis,16 but difficulties can exist envisaged in older patients with Takayasu arteritis when the timing of disease onset is uncertain.

Recent investigation of the cellular limerick of the aortic wall18 has shown neovessels in the deep intima associated with the adventitial vasa vasorum. T cells and dendritic cells, with few B cells, granulocytes, and macrophages, surrounded the vessels. The media contained acellular fibrous tissue, with bundles of neovascularisation and sparse shine muscle cells. Inflammation was most prominent in the adventitia, with infiltration of B and T cells. In half of the cases these formed nodules, with central B cells and peripheral T cells in close proximity to antigen presenting dendritic cells. Granulocytes were located outside of the nodules and granulocyte destruction was observed. No giant cells were seen.

Infection has been considered to play a function in the pathogenesis of Takayasu arteritis. Tuberculosis has been specially implicated in view of the high prevalence of infection, past or present, in affected patients,i, ix largely from endemic areas. More than recently, viral infection is being investigated equally a trigger of vasculitis.nineteen

Seko et al take reported that γδT cells, αβT cells (CD4 and CD8), and natural killer cells play an important role in the vascular injury.20 The 65 kDa heat shock protein to which γδT cells respond is strongly expressed in the aortic tissue of patients with Takayasu arteritis. They have previously plant restricted VαVβ gene usage of the αβT cell receptor, suggesting that a specific antigen was beingness targeted. More recently, they take reported restricted usage of the VγVδ genes in the infiltrating γδT cells, supporting their hypothesis, along with the expression of diverse costimulatory molecules necessary for T cell activation.

Takayasu arteritis has been associated with different human leucocyte antigen (HLA) alleles in different populations.21– 23 For example, in Japan and Korea at that place is a clear association with the extended haplotype: HLA B*52, DRB1*1502, DRB5*0102, DQA1*0103, DQB1*0601, DPA1*02-DPB1*0901.21 Sequence assay has shown that some of the alleles share specific epitopes and it may be that the epitopes are more of import equally a disease susceptibility gene than the allele in which they are found. The HLA association is thought past some to strengthen the argument in favour of an autoimmune pathogenesis. Even so, no specific autoantigens take all the same been identified and for any adaptive immune response to occur, whether against exogenous or endogenous antigen, presentation of antigen to T cells in the context of the major histocompatibility complex is central.

"A written report reported in 1998 concluded that no known serological test was able to supersede vascular histopathology in determining affliction activity"

Several studies have examined the acute stage response in Takayasu arteritis. Ishikawa found that the erythrocyte sedimentation rate (ESR) was raised in 29 of 54 patients studied,vii with an equal distribution in the four disease categories. Higher values were seen in the younger patients, declining with age, perhaps representing the natural history of the disease. Hall et al found that the ESR was raised in three quarters of 32 cases, and that it showed an fantabulous correlation with handling effect.6 Nonetheless, Kerr et al concluded that the ESR was not a consistently reliable mark of illness course, being raised in 72% with active affliction but as well in almost half of patients in clinical remission.10 In their study, 44% of arterial biopsy specimens obtained from patients with clinically inactive affliction demonstrated vasculitis, suggesting that affliction activity may be underestimated, a view as well supported by P Salary (personal communication, 2001).

This inconsistency has led to a search for improve serological markers. A study reported in 199824 ended that no known serological test was able to supplant vascular histopathology in determining affliction activity. This study compared 29 patients (22 with clinically inactive disease and seven with clinically active vasculitis) with 26 healthy control volunteers; no serological examination reliably distinguished healthy volunteers from patients with active disease. The markers assessed included ESR, C reactive poly peptide (CRP), tissue cistron, von Willebrand gene, thrombomodulin, and tissue plasminogen activator, in addition to diverse adhesion molecules. The numbers with clinically active disease were pocket-sized and over again may have been underestimated in the absenteeism of histological assessment. ESR and CRP values were not directly compared. Although affliction activeness may non be discriminated by these markers at a unmarried point in time, for private patients the use of a given parameter longitudinally may still be of value.

Serum concentrations of the pro-inflammatory and chemotactic cytokines interleukin 1β (IL-1β), IL-6, and RANTES have been assessed by enzyme linked immunoabsorbent assay.25 All of xviii patients studied had increased concentrations of IL-half-dozen and RANTES during active disease compared with healthy controls, and concentrations parallelled disease activity. These cytokines correlated with the ESR but not with CRP values. This lack of CRP correlation (CRP being driven by IL-1 and IL-6) was not adequately explained. The positive correlation with disease activeness suggested that these cytokines may contribute to the vasculitis and raised the possibility of their use in monitoring disease and handling. Nevertheless, serum cytokine assays are not necessarily a reflection of tissue cytokine concentrations and may not accurately detect biologically active cytokine. Their utilize over and above the ESR remains to be established.

TREATMENT OPTIONS

Medical treatment

Steroids have formed the mainstay of handling for Takayasu arteritis and reports of efficacy vary. This may relate to the stage of illness at which handling is introduced in addition to disease extent. Early on data suggested little benefit,1 with six of eight patients treated showing no improvement. Data from the USA in 19856 from 29 steroid treated patients demonstrated a reduction in ESR, a reduction of inflammatory symptoms, and eight of 16 patients with absent pulses were shown to accept a render of a pulse later on a delay of several months. In a later study, of 48 treated patients, remission was achieved at least once with steroids alone in 60%.10

It is at present accepted that approximately one-half of patients treated with steroids will respond.8 This lack of universal success and the side furnishings associated with steroid use take led to a search for a more effective treatment.

Comparisons accept been made with the handling of other systemic vasculitides, such as Wegener'southward granulomatosis.26 Therefore, immunosuppressive agents including cyclophosphamide, azathioprine, and methotrexate take all been tried. However, the difficulty of comparing Takayasu arteritis with Wegener'due south granulomatosis relates not only to the size of vessel afflicted by the affliction process, only also to the very different morbidity and mortality associated with these disorders. Untreated systemic Wegener'southward granulomatosis has a mean survival from affliction onset of five months and a one year mortality of 82%,27 which is in sharp contrast to that of Takayasu arteritis.

Kerr et al studied 25 steroid unresponsive patients10 receiving cytotoxic medications including cyclophosphamide, azathioprine, or methotrexate, although not concurrently. The overall remission rate was 33%. Twenty iii per cent of all treated patients in their report never achieved remission.

Because no single cytotoxic drug appears to be better than any other in terms of efficacy, side effect profiles have been an of import driving force in determining treatment. An early on report of methotrexate28 suggested that it was a clinically useful, well tolerated drug. A follow up study of 16 steroid unresponsive patients treated with methotrexate and steroid demonstrated remission in 81%.29 Yet, 7 of 16 relapsed as they were weaned off of steroids. Overall, 8 patients sustained remissions of iv to 34 months and four of these were able to discontinue treatment altogether. Three of 16 progressed despite treatment. A Brazilian study included 12 patients treated with methotrexate and prednisolonexiii; 58% had a adept response. Three had to discontinue treatment because of leucopenia or abnormal liver part.

"Because no single cytotoxic drug appears to be better than any other in terms of efficacy, side outcome profiles have been an important driving forcefulness in determining handling"

More recently, three patients have been reported after treatment with mycophenolate mofetil.30 All three showed clinical benefit, steroids were tapered or discontinued, and no toxicity was observed. Larger studies volition exist necessary to ostend these findings and institute the place of this drug in the treatment of Takayasu arteritis.

Currently, the best show based treatments include steroids, to which l% respond, and methotrexate to which a further 50% answer. The use of methotrexate as a steroid sparing drug is logical and safe. 20 five percent of patients with active disease will not answer to current treatments and care should be taken non to expose these patients to the hazards of prolonged immunosuppression in the absence of clinical do good.

The other important medical issues chronicle to the management of hypertension and the prevention and handling of thrombosis. Hypertension can exist particularly difficult, and worsened by the utilize of steroids with their fluid retaining side effects. The use of angiotensin converting enzyme inhibitors requires careful monitoring in view of the frequency of renal artery stenosis.31

Surgical treatment

Indications for surgery include hypertension with critical renal artery stenosis, extremity claudication limiting activities of daily living, cerebrovascular ischaemia or disquisitional stenoses of three or more cerebral vessels, moderate aortic regurgitation, and cardiac ischaemia with confirmed coronary avenue involvement.x In full general, surgery is recommended at a fourth dimension of quiescent disease to avoid complications, which include restenosis, anastamotic failure, thrombosis, bleeding, and infection.6, 10

Surgery may be unnecessary for aortic curvation and splanchnic affliction equally a consequence of all-encompassing collateral development.31 Yet, contempo surgical experience of critical thoracic aortic arch stenoses and stroke risk from the National Institutes of Health, USA32, 33 concluded that critical stenoses should be corrected to prevent stroke, with grafts originating from the ascending aorta. Renal avenue involvement is best treated by percutaneous transluminal angioplasty.33 Stent placement following angioplasty for ostial lesions, long segment lesions, incomplete relief of stenoses, and autopsy is condom and effective.34 Radical surgical treatment of thoracic aneurysms is recommended if technically possible considering more palliative procedures neglect to prevent recurrent aneurysm germination or to minimise risk of after surgery.35

PREGNANCY

Considering Takayasu arteritis predominantly affects women of reproductive age, the issue of pregnancy is important. Kerr et al reported v pregnancies in their series of lx patients, all of whom had normal deliveries of a normal live infant.10 Only one patient had illness exacerbation during pregnancy.

A written report from Hong Kong in 198336 reported on 13 women who had experienced a full of 30 pregnancies. Autonomously from hypertension, there were no major obstetric problems and no maternal deaths straight related to pregnancy. Fetal outcome could exist predicted on the basis of maternal vessel interest (abdominal aorta and renal), severity of maternal hypertension, superimposed pre-eclampsia, and timing of acceptable blood force per unit area control.

Maternal complications reported in 12 patients from India37 included superimposed pre-eclampsia, congestive cardiac failure, progressive renal impairment, and one example of postpartum sepsis. Intestinal aortic involvement and a filibuster in seeking medical attention predicted a poor perinatal outcome.

Fertility is not adversely affected, pregnancy per se does not appear to exacerbate the disease, but management of hypertension is essential. Hypertension in the 2d stage of labour is a hazard factor for cerebral haemorrhage; shortening this stage by use of low forceps delivery or vacuum extraction appears to be a reasonable solution.36, 37

LONG TERM FOLLOW Upward

Takayasu arteritis is a systemic vasculopathy that tin progress to cause vital organ ischaemia. Therefore, long term follow up is recommended. The limitations of monitoring the acute stage response have been discussed; improve tools are required then far these accept focused on vascular imaging techniques, with non-invasive methods obviously being near appropriate.

Doppler ultrasound is easily applied to extracranial vessels and can determine vessel wall thickness. Magnetic resonance angiography (MRA) is now being investigated in the evaluation of large vessel vasculitides.38 Information technology provides high resolution detail of vessel wall thickness and lumen configuration, and allows the measurement of wall enhancement every bit a reflection of oedema and agile inflammation. Compared with the gold standard of conventional angiography, approximately two% of stenosed arteries are overestimated every bit occluded on MRA. The reduction of enhancement on follow up is presumed to reflect reduced inflammatory activity. Therefore, MRA is probable to be used increasingly every bit an accurate follow upward tool.

The management of patients with Takayasu arteritis can exist problematic. There may be incertitude with regard to the onset and course of the disease, a poor correlation betwixt clinical assessment and illness activity, poor disease activeness markers in peripheral blood, and a lack of useful handling in upwardly to 25% of patients with progressive disease. The risk of increased morbidity and bloodshed means that most patients who present volition ultimately receive immunosuppression. The vasculitides, specially those affecting modest vessels, mostly require aggressive treatment. The same may non be truthful of all patients with Takayasu arteritis despite the angiographic appearances. Cohort studies suggest a good prognosis for those with uncomplicated or monocomplicated disease. Thus, the temptation to immunosuppress such patients aggressively should be questioned. In contrast, early treatment of those with progressive complicated disease may pb to a better prognosis for this group. Considering inflammation is a risk cistron for atherosclerosis,2 more than atherosclerotic complications are likely in the longer term.

Take abode letters

  • Takayasu arteritis is rare, affects mainly women, and is well-nigh unremarkably seen in Japan, South East asia, Bharat, and Mexico, where it usually presents in the 2nd or 3rd decade of life

  • Manifestations range from asymptomatic disease, found equally a result of impalpable pulses or bruits, to catastrophic neurological harm

  • Disease presentation varies between unlike populations

  • Angiography remains the gold standard for diagnosis

  • The iv nearly important complications for classification are Takayasu retinopathy, secondary hypertension, aortic regurgitation, and aneurysm formation, each being graded as mild/moderate or severe at the time of diagnosis

  • Iv grades of illness are described, which can be used for prognostic and handling assessment: cohort studies suggest a good prognosis for those with uncomplicated or monocomplicated disease

  • Approximately half of those patients treated with steroids will reply, and half of the remaining patients answer to methotrexate; mycophenolate mofetil may be useful

  • Treatment should aim to command disease activeness and preserve vascular competence, with minimal long term side effects; those with disease that carries a expert prognosis should non be put at risk past treatment that is more than harmful than the disease itself

  • Fertility is non adversely affected and pregnancy does not appear to exacerbate the illness, although management of hypertension is essential

"Takayasu arteritis is a systemic vasculopathy that can progress to cause vital organ ischaemia"

Every bit with whatever rare disorder, sufficient patient numbers for randomised controlled treatment trials are lacking. The aim of handling must be the control of disease action and the preservation of vascular competence, with minimal long term side furnishings. Patients with disease that carries a expert prognosis should non be put at risk by handling that is more harmful than the affliction itself. Current prove favours the use of steroids and methotrexate, but mycophenolate mofetil may prove to have a role.

Acknowledgments

The authors would like to thank Dr M Thornton, Consultant Radiologist, Southmead Hospital, Westbury on Trym, Bristol for help in provision of the radiographic material.

Abbreviations

  • ACR, American College of Rheumatology

  • CRP, C reactive poly peptide

  • ESR, erythrocyte sedimentation charge per unit

  • HLA, homo leucocyte antigen

  • IL, interleukin

  • MRA, magnetic resonance angiography

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Source: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1769710/

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