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Takayasu arteritis in major rheumatology centers in Malaysia

Khor CG, Tan BE, Kan SL, Tsang EE, Lim AL, Chong EY, et al.

J Clin Rheumatol, 2016 Jun;22(4):194-7.
PMID: 27219306 DOI: 10.1097/RHU.0000000000000362

OBJECTIVE: There is paucity of data for Takayasu arteritis (TAK) among South Asians. We aimed to evaluate the clinical features, angiographic findings, as well as treatment and outcome of TAK among Malaysian multiethnic groups.

METHODS: This is a retrospective review of 40 patients with TAK seen in major rheumatology centres in Malaysia between April 2006 and September 2013.

RESULTS: Majority were female patients (92.5%), with a female-to-male ratio of 12:1. Median duration of disease from diagnosis was 66 months (interquartile range, 33-177 months). Fifteen (37.5%) were Malays, 9 (22.5%) each were Indians and indigenous from East Malaysia and 7 (17.5%) were Chinese. Indian and indigenous from East Malaysia were overrepresented in this disease. The mean (SD) age of symptom onset and diagnosis were 25.5 (8.1) and 27.4 (8.4), respectively. The 3 most common clinical presentations at diagnosis were diminished or absent pulse, which occurred in 80% of the patients, followed by blood pressure discrepancy (60%) and arterial bruit (52.5%). There was no difference in clinical presentation among ethnic groups. The subclavian artery was the commonest vessel involved (72.5%), followed by the carotid artery (65%) and renal artery (47.5%). Eight patients had coronary artery involvement, and 2 patients had pulmonary artery involvement. Type I arterial involvement was the commonest (80.0%), followed by type IV (35%), present in isolation or mixed type. Glucocorticoid was the main medical treatment (90.0%). Nineteen patients (47.5%) underwent revascularization procedures. Five patients died during the follow-up period.

CONCLUSIONS: The Malaysian TAK cohort had similarities with and differences from other published TAK cohort. A nationwide TAK registry is needed to determine the prevalence of the disease among different ethnic groups.

Matched MeSH terms: Takayasu Arteritis/ethnology*; Takayasu Arteritis/therapy
Fulltext Positron emission tomography (PET) and single photon emission computed tomography (SPECT) imaging of macrophages in large vessel vasculitis: Current status and future prospects

Jiemy WF, Heeringa P, Kamps JAAM, van der Laken CJ, Slart RHJA, Brouwer E

Autoimmun Rev, 2018 Jul;17(7):715-726.
PMID: 29729443 DOI: 10.1016/j.autrev.2018.02.006

Macrophages are key players in the pathogenesis of large-vessel vasculitis (LVV) and may serve as a target for diagnostic imaging of LVV. The radiotracer, 18F-FDG has proven to be useful in the diagnosis of giant cell arteritis (GCA), a form of LVV. Although uptake of 18F-FDG is high in activated macrophages, it is not a specific radiotracer as its uptake is high in any proliferating cell and other activated immune cells resulting in high non-specific background radioactivity especially in aging and atherosclerotic vessels which dramatically lowers the diagnostic accuracy. Evidence also exists that the sensitivity of 18F-FDG PET drops in patients upon glucocorticoid treatment. Therefore, there is a clinical need for more specific radiotracers in imaging GCA to improve diagnostic accuracy. Numerous clinically established and newly developed macrophage targeted radiotracers for oncological and inflammatory diseases can potentially be utilized for LVV imaging. These tracers are more target specific and therefore may provide lower background radioactivity, higher diagnostic accuracy and the ability to assess treatment effectiveness. However, current knowledge regarding macrophage subsets in LVV lesions is limited. Further understanding regarding macrophage subsets in vasculitis lesion is needed for better selection of tracers and new targets for tracer development. This review summarizes the development of macrophage targeted tracers in the last decade and the potential application of macrophage targeted tracers currently used in other inflammatory diseases in imaging LVV.

Matched MeSH terms: Giant Cell Arteritis/diagnosis*; Giant Cell Arteritis/pathology*
Fulltext Giant cell arteritis with panocular involvement in an Indian male

Fathilah J, Jamaliah R

Med J Malaysia, 2003 Mar;58(1):111-4.
PMID: 14556335

A case of giant cell arteritis with systemic and panocular involvement is reported here. This elderly Indian male presented with symptoms of unilateral temporal headache and intermittent jaw claudication for a month followed by diplopia and blurring of vision and later loss of vision in the right eye. The right eye showed some limitation of ocular movements, presence of relative afferent pupil defect, anterior segment ischaemic changes and anterior ischaemic optic neuropathy. Visual evoked potential showed an absent P1 wave while the left eye with normal 6/6 vision sowed a prolonged P1 wave. Fundus fluoresceine angiography showed delay in choroidal perfusion. His erythrocyte sedimentation rate (ESR) was 120 mm/hr and he was started on oral prednisolone. Superficial temporal artery biopsy obtained one week after starting steroids was positive for giant cell arteritis. Steroids led to the resolution of optic disc swelling, disappearance of anterior segment signs, full recovery of right ocular movements and no further deterioration of the fellow eye. On steroids, he developed insomnia and progressive myopathy which resolved and is now symptom free at lower doses of steroids.

Matched MeSH terms: Giant Cell Arteritis/complications*; Giant Cell Arteritis/diagnosis*; Giant Cell Arteritis/therapy
A case of Takayasu Arteritis presenting with young stroke

Cheo SW, Mohd Zamin H, Low QJ, Tan YA, Chia YK

Med J Malaysia, 2020 11;75(6):745-747.
PMID: 33219190

Stroke is a debilitating disease as it carries significant morbidity especially when it affects the younger population. There are various etiologies of young stroke, namely arterial dissection, cardioembolism, thrombophilia, inherited genetic disorder and vasculitis. Young patient with stroke should undergo complete evaluation to identify the underlying etiology in order to prevent recurrence of stroke. Here, we would like to illustrate a case of Takayasu arteritis presenting as young stroke in a 17-years-old lady with no known medical illness.

Matched MeSH terms: Takayasu Arteritis
Fulltext Primary arteritis of aorta causing renal artery stenosis and hypertension

Danaraj TJ, Wong HO, Thomas MA

Br Heart J, 1963 Mar;25(2):153-65.
PMID: 14024854

The evidence presented by the nine cases described in this paper indicates that primary arteritis of the aorta is a single clinico-pathological entity of which Takayashu's syndrome is a part. Different segments of the aorta may be affected resulting in a variety of symptom complexes. In this series, the mode of presentation was hypertension consequent on renal artery stenosis. Early diagnosis is important and surgical treatment is indicated to relieve the hypertension which carries an immediately serious prognosis.

Matched MeSH terms: Arteritis*; Takayasu Arteritis*
Paediatric middle aortic syndrome with endovascular treatment in East Malaysia

Hong Ong G, Benjamin Leong DK

Med J Malaysia, 2018 02;73(1):57-59.
PMID: 29531207 MyJurnal

Middle-aortic syndrome is a surgically curable cause of childhood hypertension. Open surgery is traditionally offered but with the advance of medical technology, endovascular approached is available in many country. Failure to control BP in open surgery is as low as 4.1% compares to 13% in endovascular approaches. However, mortality is 4% in open surgery almost 2 times higher than 2.3% in endovascular approach. This article presents a case of 10 years old child treated successfully without complication with endovascular balloon dilatation, as a first case of such disease in East Malaysia.

Matched MeSH terms: Takayasu Arteritis
Giant cell arteritis presenting as chronic cough and prolonged fever

Lim KH, Liam CK, Vasudevan AE, Wong CM

Respirology, 1999 Sep;4(3):299-301.
PMID: 10489679 DOI: 10.1046/j.1440-1843.1999.00195.x

A 62-year-old man presented with a 3-month history of chronic non-productive cough and unexplained fever. Further questioning revealed that he had headaches and myalgia. Bilateral thickened temporal arteries were noted on physical examination. The erythrocyte sedimentation rate was 96 mm in 1 h. A biopsy specimen of the left temporal artery showed inflammatory changes consistent with the diagnosis of giant cell arteritis. Commencement of prednisolone resulted in rapid and dramatic resolution of his symptoms. Physicians should be aware of respiratory symptoms in patients with giant cell arteritis in order to avoid delay in diagnosis and therapy of this condition.

Matched MeSH terms: Giant Cell Arteritis/diagnosis*
Melioidosis and the vascular surgeon: Hospital Kuala Lumpur experience

Azizi ZA, Yahya M, Lee SK

Asian J Surg, 2005 Oct;28(4):309-11.
PMID: 16234087

Bacterial arteritis is relatively uncommon and management of this condition, which carries high morbidity and mortality, is difficult and time-consuming. Common organisms implicated include Salmonella and Staphylococcus. Arteritis as a result of infection by Burkholderia pseudomallei (formerly Pseudomonas pseudomallei) has been rarely reported in the English literature. This organism, which is endemic in our part of the world, is well known to cause a wide spectrum of septic conditions. A review of cases managed at Hospital Kuala Lumpur revealed that bacterial arteritis due to melioidosis is not such a rare entity. We share our experience in the management of this condition using three cases as examples.

Matched MeSH terms: Arteritis/drug therapy; Arteritis/etiology*; Arteritis/surgery
Is coronary arteritis a feature in secondary haemophagocytic lymphohistiocytosis?

See BT, Yip KX, Ang HL

Paediatr Int Child Health, 2018 02;38(1):76-79.
PMID: 28222654 DOI: 10.1080/20469047.2017.1289312

Haemophagocytic lymphohistiocytosis (HLH) is rare. Although Kawasaki disease (KD) has been reported as a precursor to HLH, coronary arteritis occurring at the onset of secondary HLH, not in association with KD, has not been reported. An 8-year-old girl presented with virus-induced secondary HLH associated with a giant aneurysm and ectasia of the coronary arteries which was detected incidentally at onset of the disease. She did not fulfill the criteria for diagnosis of KD. The coronary lesions improved after 6 months of treatment with dexamethasone and etoposide. Echocardiography early in the course of HLH is a useful tool to detect the unusual finding of coronary arteritis which may carry significant clinical sequelae.

Matched MeSH terms: Arteritis/diagnosis*; Arteritis/pathology*
Fulltext Two cases of retinal vasculitis in ocular tuberculosis involving different parts of the vascular system

Kok, Howe Sen, Tara, Mary Georg, Mae-Lynn, Catherine Bastion, Muhaya Hj Mohamad

Medicine & Health, 2006;1(1):91-93.
MyJurnal

Two case reports of retinal vasculitis in presumptive ocular tuberculosis are presented. Case 1 is a 26-year-old Malay woman who had phlebitis with choroidal lesions and case 2 is a 27 year old Malay woman who had occlusive arteritis. Both subjects had positive Mantoux tests with negative systemic screen. Both responded clinically to anti-tuberculous therapy within days of commencing therapy. There was however concurrent increase in vitritis which decreased following anti-inflammatory doses of oral corticosteroids. These two cases represent a rare mode of presentation of ocular tuberculosis for which a high index of suspicion is needed. Multidrug anti-tuberculous therapy should be combined with oral corticosteroids for effective treatment.

Matched MeSH terms: Arteritis
Fulltext Extracranial carotid aneurysm secondary to Takayasu Arteritis (TA): a rare complication

Irfan Mohamad, Ikhwan Sani Mohamad, Prahalad Ramanathan, Win Mar@Salmah

Malaysian Journal of Medicine and Health Sciences, 2015;11(2):89-92.
MyJurnal

Extracranial aneurysms are a rare presentation accounting for only less than 5% of all peripheral artery
aneurysms. The left common carotid artery aneurysm is considered even rarer. We present a case of a 46-year-old gentleman who presented to casualty unit with a neck mass, stridor and acute respiratory distress. Prior to this admission patient was being investigated for Takayasu arteritis (TA). Patient was subsequently intubated due to respiratory compromise. A CT angiography of the thorax was done
showed a proximal left common carotid artery aneurysm with contained leakage of aneurysm with
severe compression of the trachea. Patient was subsequently transferred to the vascular team in National Heart Institute for further management.

Matched MeSH terms: Takayasu Arteritis
Fulltext Problem based review: The patient with a pyrexia of unknown origin

Woolley AK, Hedger NA, Veettil RP

Acute Med, 2013;12(2):107-10.
PMID: 23732136

Pyrexia of unknown origin (PUO) is a frequent presentation to the Acute Medical Unit, and is a source of significant morbidity, both the psychological burden of an uncertain diagnosis and prognosis and untreated complications of the underlying pathology. We present a problem based review of the management of PUO, illustrated by a patient who recently presented to our unit with fever and systemic malaise after returning from abroad and in whom no cause could be found for more than two months. We describe a structured approach making use of complex modern techniques such as Positron Emission Tomography-Computed Tomography (PET-CT) which ultimately provided the diagnosis for our patient.

Matched MeSH terms: Takayasu Arteritis/complications; Takayasu Arteritis/diagnosis*; Takayasu Arteritis/drug therapy
Dural arteriovenous fistula mimicking temporal arteritis

Naserrudin NS, Mohammad Raffiq MA

Clin Neurol Neurosurg, 2019 01;176:44-46.
PMID: 30504098 DOI: 10.1016/j.clineuro.2018.11.021

INTRODUCTION: Dural arteriovenous fistula (dAVF) is a very rare disease characterized by an abnormal vascular communication between arteries and veins in dural mater. It frequently presents with intracranial haemorrhage. Common presenting symptoms are headache and seizures.
CASE REPORT: Here we report a case of dAVF in which the patient's symptoms mimic a temporal arteritis in a 23-year-old woman. She presented with painful mass at forehead for 9 months with frontotemporal headache. Magnetic resonance imaging demonstrated dural arteriovenous fistula.
CONCLUSION: Since both diseases have different prognosis but similar presentation, it is important to ensure that there is no dural arteriovenous fistula in patient with suspected temporal arteritis.

Matched MeSH terms: Giant Cell Arteritis
Fulltext 18F-FDG positron emission tomography/computed tomography and the "underground map" appearance in imaging Horton's arteritis

Abdul Jalil N, Abdul Rahim N, Md Shalleh N, Rossetti C

Singapore Med J, 2008 Jul;49(7):e178-82.
PMID: 18695852

A majority of the clinical use of positron emission tomography (PET)-computed tomography (CT) is related to cancer management. Its application in evaluating inflammatory diseases and pyrexia of unknown origin is becoming popular. We reviewed the fluorine-18-fluorodeoxyglucose PET-CT findings of an 80-year-old woman with nonspecific clinical presentation consisting of generalised malaise, moderately high fever and weight loss. Prior CT and magnetic resonance imaging were not helpful in providing a clinical diagnosis. The diagnosis was Horton's arteritis, and the patient responded well to high-dose steroids.

Matched MeSH terms: Arteritis/diagnosis*
A Distinct Macrophage Subset Mediating Tissue Destruction and Neovascularization in Giant Cell Arteritis: Implication of the YKL-40/Interleukin-13 Receptor α2 Axis

van Sleen Y, Jiemy WF, Pringle S, van der Geest KSM, Abdulahad WH, Sandovici M, et al.

Arthritis Rheumatol, 2021 12;73(12):2327-2337.
PMID: 34105308 DOI: 10.1002/art.41887

OBJECTIVE: Macrophages mediate inflammation, angiogenesis, and tissue destruction in giant cell arteritis (GCA). Serum levels of the macrophage-associated protein YKL-40 (chitinase 3-like protein 1), previously linked to angiogenesis and tissue remodeling, remain elevated in GCA despite glucocorticoid treatment. This study was undertaken to investigate the contribution of YKL-40 to vasculopathy in GCA.
METHODS: Immunohistochemistry was performed on GCA temporal artery biopsy specimens (n = 12) and aortas (n = 10) for detection of YKL-40, its receptor interleukin-13 receptor α2 (IL-13Rα2), macrophage markers PU.1 and CD206, and the tissue-destructive protein matrix metalloproteinase 9 (MMP-9). Ten noninflamed temporal artery biopsy specimens served as controls. In vitro experiments with granulocyte-macrophage colony-stimulating factor (GM-CSF)- or macrophage colony-stimulating factor (M-CSF)-skewed monocyte-derived macrophages were conducted to study the dynamics of YKL-40 production. Next, small interfering RNA-mediated knockdown of YKL-40 in GM-CSF-skewed macrophages was performed to study its effect on MMP-9 production. Finally, the angiogenic potential of YKL-40 was investigated by tube formation experiments using human microvascular endothelial cells (HMVECs).
RESULTS: YKL-40 was abundantly expressed by a CD206+MMP-9+ macrophage subset in inflamed temporal arteries and aortas. GM-CSF-skewed macrophages from GCA patients, but not healthy controls, released significantly higher levels of YKL-40 compared to M-CSF-skewed macrophages (P = 0.039). In inflamed temporal arteries, IL-13Rα2 was expressed by macrophages and endothelial cells. Functionally, knockdown of YKL-40 led to a 10-50% reduction in MMP-9 production by macrophages, whereas exposure of HMVECS to YKL-40 led to significantly increased tube formation.
CONCLUSION: In GCA, a GM-CSF-skewed, CD206+MMP-9+ macrophage subset expresses high levels of YKL-40 which may stimulate tissue destruction and angiogenesis through IL-13Rα2 signaling. Targeting YKL-40 or GM-CSF may inhibit macrophages that are currently insufficiently suppressed by glucocorticoids.

Matched MeSH terms: Giant Cell Arteritis/metabolism; Giant Cell Arteritis/pathology*

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