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  Citation statistics : Table of Contents
   2015| December  | Volume 10 | Issue 5  
    Online since August 3, 2016

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Assessment of disease activity in Takayasu's arteritis
Durga Prasanna Misra, Ramnath Misra
December 2015, 10(5):43-47
Takayasu's arteritis (TA) is a large vessel vasculitis of unknown etiology, more common among Asians. Since it is a smoldering, chronic disease, assessment of disease activity is a challenge. Acute phase reactants, erythrocyte sedimentation rate and CRP, imperfectly correlate with disease activity on histopathology. The earliest clinical criteria to assess disease activity were the NIH criteria, taking a composite of clinical features, inflammatory markers, and imaging to assess disease activity in TA. Of late, clinical scoring systems like DEI.Tak (Disease Extent Index in TA), derived from the BVAS scoring for small vessel vasculitis, and the ITAS2010 and ITAS-A, derived from DEI.Tak, have been validated. Serum biomarkers like matrix metalloproteinases 2, 3, and 9, pentraxin-3 and soluble receptor for advanced glycation end products hold promise in assessing disease activity. Recently, endothelial microparticles have been shown to correlate with active TA. Evidence suggests wall edema, and contrast uptake in the vessel wall on angiography may suggest active TA. Scoring systems assessing angiographic extent of TA are a work in progress, validation of which shall help quantify extent of vascular involvement in TA, and serial follow-up might prove valuable for assessment of disease activity. PET-CT is useful to diagnose prepulseless TA; however, its utility in patients on immunosuppression is debatable. Analysis of serum metabolites using nuclear magnetic resonance spectroscopy is a promising exploratory approach towards identifying new biomarkers in TA. There remains an unmet need for a composite index, taking into account clinical features, serial radiography, and circulating biomarkers, to assess disease activity in TA.
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  11 3,339 434
Takayasu's arteritis: Review of epidemiology and etiopathogenesis
Sanjay Jain, Sathish Kumar Pondaiah
December 2015, 10(5):22-29
Takayasu's arteritis (TA) is a systemic large vessel chronic inflammatory disease affecting the aorta and its branches. Epidemiological data on the prevalence of TA are limited to the hospital-based studies. The prevalence, clinical features and outcomes of TA vary across the globe. Although TA is reported from all parts of the world, the prevalence of the disease appears to be high in oriental countries especially in Japan, India, Korea, and Thailand. The exact pathogenesis of the disease is unknown but an ongoing research is being carried out in the immunological and genetic aspects of the disease. HLA Bw52, cytotoxic T cells, and antiendothelial cell antibodies are significant highlights in the pathogenesis of TA. The exact antigenic targets of the immune system are still unknown. We attempt a review of the available literature on epidemiology and etiopathogenesis of TA.
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  9 4,020 1,102
Kawasaki disease - A common childhood vasculitis
Dhrubajyoti Sharma, Surjit Singh
December 2015, 10(5):78-83
Kawasaki disease (KD) is an acute self-limiting vasculitis of children predominantly affect- ing the medium sized arteries. The disease was first described by Dr. Tomisaku Kawasaki in 1967 from Japan. KD has now been reported from more than 60 countries and is the commonest cause of acquired heart disease in children in the developed countries. Japan reports the highest incidence of KD at 265/100,000 children below 5 years, followed by Korea and Taiwan. In North America and Europe, the incidence of KD is much lower (9-25/100,000 children below 5) and appears to have plateaued down over the last few decades. The reasons for these differences in epidemiology are not clearly understood. KD has been increasingly reported from India over the last 20 years. At Chandigarh, an incidence of 4.54/100,000 children below 15 years was reported in 2011. However, this was likely to be an underestimate. The etiology of KD remains unknown. Although a genetic basis of KD seems plausible, an intercurrent infectious process seems to act as a trigger for the inflammatory cascade. Like many other vasculitides, the diagnosis of KD is essentially clinical and is based on a set of criteria first elaborated by Dr. Kawasaki himself. However, several children (especially infants) with KD can have incomplete and atypical presentations. This can result in diagnostic and therapeutic delays. Approximately 15-25% children with KD can develop coronary artery abnormalities (CAAs) if left untreated. Two dimensional echocardiography remains the gold standard in detecting CAAs in patients with KD. Dual source CT coronary angiography is a recent advance in accurate detection of CAAs with minimal radiation risk. Intravenous immunoglobulin (2 g/kg) remains the drug of choice and is administered as an infusion. Other therapeutic agents that have been used include infliximab, cyclosporine, glucocorticoids, and statins. KD has been associated with several long-term sequelae.
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  5 2,204 600
Giant Cell Arteritis and Takayasu Arteritis: Are they a different spectrum of the same disease?
Lorraine O'Neill, Cristina Ponte, Jan Sznajd, Anna P Rodrigues, Benjamin Seeliger, Raashid A Luqmani
December 2015, 10(5):11-21
Giant Cell Arteritis (GCA) and Takayasu Arteritis (TAK) are the two major forms of large vessel vasculitis (LVV). Traditionally GCA and TAK have been considered as two separate diseases based on a number of differences including age of onset, ethnicity, clinical features and vascular distribution. However, the realisation that large vessel involvement in GCA is more common than previously thought, has prompted the discussion that GCA and TAK may in fact represent a spectrum of the same disease. Greater understanding of the pathogenesis of GCA and TAK has highlighted striking similarities. Cell mediated immunity plays a critical role in the pathogenesis of large vessel vasculitis with T helper subsets 1 and 17 (Th1 & Th17) predominating.1,2 Interleukin 6 (IL 6) is upregulated in both GCA and TAK and promotes differentiation of T cells towards the Interleukin 17 producing (IL17), Th17 lineage, which directs the systemic inflammatory response. Interleukin 12 (IL 12) polarises T cells to differentiate into Th 1 cells, of which the signature cytokine is interferon gamma (IFN-g). IFN-g is responsible for driving vascular remodelling, intimal hyperplasia and ultimately luminal occlusion and increased expres- sion is found in patients with TAK vs. GCA,2 perhaps accounting for increased vessel stenosis in TAK. In GCA, the Th17 pathway appears to be important in early disease and is rapidly suppressed by glucocorticoid therapy, while the Th1 axis is responsible for ongoing vascular inflammation and is relatively steroid resistant.1,3 The opposite appears to be true for TAK, with Th17 cytokines persisting despite treatment, and the Th1 axis adequately suppressed by glucocorticoids. Therefore, while glucocorticoids are effective they do not fully eliminate the aberrant immune response and insights into the subtle differences in pathogenesis will help guide future targeted therapies. GCA itself could be considered to comprise of a number of clinical subtypes; PMR with systemic inflammation, myalgias, subclinical vasculitis with an IL 6 but not and IFN-g cytokine signature; uncomplicated cranial GCA with classical cranial symptoms, a systemic inflammatory response but without neuro-ophthalmic ischaemia; cranial GCA complicated by an ischaemic event such as blindness or stroke, often without a significant acute phase
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  4 2,311 78
Antineutrophil cytoplasmic antibodies (ANCA): Role in disease pathogenesis, diagnosis, and monitoring ANCA associated vasculitis
Ashish Aggarwal, Aman Sharma, Manish Rathi, Kusum Sharma, Ranjana Walker Minz
December 2015, 10(5):48-53
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a small-vessel vasculitis. The exact mechanism of ANCA involvement in the pathogenesis of AAV is not fully understood. It is an autoimmune disease with multifactorial pathophysiology. The most common ANCA target antigens are myeloperoxidase and proteinase 3. In addition to autoantibodies, dysregulated T and B cells have a pivotal pathophysiological role in this disease. Though the mechanism of action of ANCA is still being explored, ANCA serology is being increasingly used for classification of AAV, though the histological examination still remains the gold standard of diagnosis. The international consensus statement on reporting of ANCA recommended use of both IIF and ELISA as 5% of IIF negative results are ELISA positive. Though ANCA positivity increases the risk of recurrence four or more times, only a weak association is observed between ANCA and disease activity. Controversy exists regarding the utility of serial measurements of ANCA in patients with AAV to predict disease relapse. However, based upon current evidence the treatment decisions should not be based solely on the levels of ANCA titers in patients with AAV.
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  3 2,226 451
Imaging in Takayasu arteritis
Santosh Kumar Mandal, Nikhil Gupta, Ruchika Goel, Aswin Nair, Suvrat Arya, Arvind Ganapati, Ashish Jacob Mathew, George Joseph, Shyamkumar Nindugala Keshava, Debashish Danda
December 2015, 10(5):30-42
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  2 2,269 575
Granulomatosis with polyangiitis: Indian experience
Ashok Kumar, Anil Abrol
December 2015, 10(5):59-63
Granulomatosis with polyangiitis (GPA) is now a well-recognised clinical entity in India. The earliest description of GPA in India (then called Wegener's granulomatosis) appeared as a single case in a series of 108 cases of systemic vasculitis published in 1985. Later, several short case-series (mostly from northern India) and a number of case reports were published. The number of published cases of GPA from India till date is about 130 (actual burden of disease is certainly much larger as only a few cases get published). The overall clinical profile, response to treatment and long-term outcome of GPA do not differ significantly from what is described in western literature. A female preponderance has been noted in the Indian series, whereas no gender predilection was seen in western series. Although many cases used to masquerade as pulmonary tuberculosis in the past, the situation has improved considerably, with better awareness about the disease and availability of ANCA as a diagnostic test. High relapse rate and significant organ damage continue to pose a challenge. Cyclophosphamide combined with prednisolone is the standard induction regimen. Ritux- imab and its two biosimilars are being increasingly used in the management of refractory cases as well as for induction of remission in certain situations.
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  2 2,737 543
Epidemiology of vasculitis - Lessons learnt from the differences in different geographical areas
Richard A Watts
December 2015, 10(5):6-10
The epidemiology of the vasculitides has been increasingly investigated over the past 30 years and we have now gathered significant knowledge about the occurrence of vasculitis in many populations. There is however still a lack of reliable data from the Indian subconti- nent. For most types of vasculitis it is not known if differences in occurrence represent variation in genes, environment, or ascertainment. Giant cell arteritis is most common in populations of Northern Europe or Scandinavian origin and is rarer in the far East, whilst Takayasu arteritis is apparently commoner in Asia. The ANCA vasculitides have an overall occurrence that is similar in most populations, but in China and Japan microscopic poly- angiitis is more common than granulomatosis with polyangiitis, whilst in Northern Europe the opposite occurs. Kawasaki disease is markedly more common in South East Asian populations than Caucasians. Behcet's disease is most common along the ancient silk route between the Mediterranean and China. Increasing genetic knowledge is beginning to explain these differences. Behcet's disease is strongly associated with HLA-B*51, Takayasu arteritis with HLA-B*52 and granulomatosis wit polyangiitis with the HLA-DPB1*0401 allele. Varia- tions in allelic frequency might partially explain the global variation in occurrence.
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  1 2,507 870
Polyarteritis nodosa - Challenges and options in management
Christian Pagnoux, Nader A Khalidi
December 2015, 10(5):64-71
Polyarteritis nodosa (PAN) was one of the first systemic necrotizing vasculitides to be described, in the late 19th century. Hepatitis B virus (HBV) was the major cause of the observed PAN cases between 1970 and the early 2000s. However, in the revised 2012 Chapel Hill Consensus Conference nomenclature, HBV-related PAN is now included with vasculiti- des associated with probable etiologies (and named HBV-associated vasculitis). PAN should thus now refer exclusively to primary cases, without an identified or probable cause. Besides systemic forms of the primary and ''classical'' PAN, which have become more rare over the past decade, isolated and/or single organs can be affected, including the skin. In this article, we review the main clinical, biological, and radiological characteristics of ''classical'' primary PAN and its different forms, their treatment options, and outcomes. We also discuss other possible etiologies (other than HBV) of PAN-like medium-sized vessel vasculi- tis and newly described PAN-like vasculopathies, such as recessive loss-of-function muta- tions in adenosine deaminase 2, as well as the treatment options for isolated or refractory cases and the current place of biologic agents for the treatment of PAN.
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  1 2,249 521
Richard A Watts
December 2015, 10(5):1-2
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  - 3,073 1,287
Which way is vasculitis research heading?
Paul Bacon
December 2015, 10(5):3-5
Clinical research is driving the improved prognosis for patients with systemic vasculitis. The major progress has been in ANCA-related vasculitis, where the need now is to incorporate the lessons from limited term controlled trials into evidence-based treat-to-target regimes related to individual responses over a prolonged period. This perspective examines the research aimed to establish the biomarkers needed for such an approach to succeed. It also looks at the more basic research needed to establish the aetio-pathogenesis of other major forms of vasculitis so that rational treatments can be devised for them too.
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  - 2,172 647
Polyarteritis nodosa and microscopic polyangiitis - The Indian experience
Rohini Handa
December 2015, 10(5):72-77
Polyarteritis nodosa (PAN) and microscopic polyangiitis (MPA) are systemic necrotising vasculitides, with a propensity to involve multiple organs. MPA is an ANCA (antineutrophil cytoplasmic antibody)-associated vasculitis while PAN is characterised by the absence of ANCA/other autoantibodies. The clinical profile in India is no different from that reported elsewhere. Lack of physician awareness contributes to diagnostic delays. In absence of disease registries, long-term outcome data from India are sparse.
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  - 2,255 438
ANCA-associated vasculitis - Should we change the standard of care?
Poonam Sharma, Max Yates, Chetan Mukhtyar
December 2015, 10(5):54-58
Collaborative clinical trials over the last 25 years have revolutionised the care of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. This has led to production of management recommendations and standards of care. This paper reviews the existing standards and the recent evidence that has fed further evolution of standards of care. Pattern recognition remains vital to early diagnosis and therefore initiation treatment. While cyclophosphamide remains the treatment of choice, the advent of rituximab has been shown to be beneficial to patients with relapsing disease. It may be safer in young females and those with a risk of urothelial cancers. Methotrexate and mycophenolate mofetil may not be as good as previously thought for inducing remission. Azathioprine and rituximab are the standards for remission maintenance. There have been recent changes to the nomen- clature of vasculitides. It is possible that these will continue to evolve over time to make them more meaningful and inform treatment and prognosis. In the absence of gold- standard biomarkers, we discuss the role of ANCA and histopathology, especially in the Indian setting. Follow-up and monitoring of these patients should include structured evaluation using validated clinical tools, assessing cardiovascular risk, vigilance for infec- tions and other co-morbidities due to exposure to glucocorticoids and immunosuppression.
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  - 2,026 514
Behçet's disease: Review of management
Jagdish R Nair, Robert J Moots
December 2015, 10(5):84-94
Introduction: Behçet's disease (BD) is a chronic relapsing and remitting vasculitis of unknown aetiology, which has the potential to affect almost all body systems due to its capacity to involve both arterial and venous sides of the vascular system and results in significant organ-specific morbidity and mortality. Whilst occurring worldwide, the prevalence is highest along the traditional 'silk road'. The pathological processes underlying BD remain poorly understood and the evidence base to inform optimal management is limited. Aim: To briefly discuss clinical features of BD and, through analysis of the current literature, provide a concise review of the currently available treatment modalities available. Opinion: There is a paucity of randomised controlled trials and robust clinical studies to guide treatment on various manifestations of BD. We have attempted to provide a system- wise step-up treatment guidance for BD. The current evidence base for BD management is based on limited high quality studies, clinical trials and various expert committee opinions.
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  - 2,395 473