Oclusão Completa do Arco Aórtico em Paciente Portadora de Arterite de Takayasu e Espondilite Anquilosante / Total Aortic arch Occlusion in a Patient with Takayasu's Arteritis and Spondylitis Ankylosans

1970 ◽  
Vol 5 (1) ◽  
pp. 52-60
Author(s):  
Rodolfo Souza Cardoso ◽  
Melissa Andreia de Moraes Silva ◽  
Seleno Glauber de Jesus Silva ◽  
Isabela de Godoy ◽  
Isabella Seixas Cenci Marin
Keyword(s):  
Ankylosing Spondylitis ◽  
Digital Subtraction Angiography ◽  
Takayasu Arteritis ◽  
Functional Limitation ◽  
Axial Skeleton ◽  
Chronic Inflammatory Disease ◽  
Systemic Hypertension ◽  
Unknown Etiology ◽  
Simultaneous Occurrence ◽  
Digital Subtraction

Introdução: A Arterite de Takayasu (AT) é uma vasculite que acomete a aorta e seus principais ramos, principalmente as artérias carótidas e subclávias. A espondilite anquilosante (EA) por sua vez é uma doença inflamatória crônica, que acomete preferencialmente a coluna vertebral, evoluindo com rigidez e limitação funcional progressiva do esqueleto axial. A associação dessas duas doenças é rara e de difícil diagnóstico. Casuística: Relata-se o caso de uma paciente do sexo feminino, 49 anos, apresentando dor e rigidez da coluna vertebral, cefaleia, vertigem, ausência de pulsos em membros superiores e hipertensão arterial sistêmica. A Angio-TC de aorta revelou oclusão dos troncossupra-aórticos (TSA) e sinais de espondilopatia, caracterizados por sindesmófitos entre os corpos vertebrais e sinais de anquilose da articulação sacroilíaca. A angiografia por subtração digital confirmou a oclusão completa dos TSA em suas origens e extensa circulação colateral. Foi iniciado tratamento com imunosupressores e corticoide, além de tratamento da hipertensão arterial sistêmica, observando-se redução dos níveis pressóricos e melhora da cefaleia, porém com manutenção da dor lombar e do enrijecimento articular. Conclusão: A ocorrência concomitante de duas doenças aparentemente distintas como a AT e a EA deve ser interpretada como a manifestação de um estado inflamatório sistêmico de etiologia ainda não totalmente definida. Palavras-chave: Arterite de Takayasu, Espondilite Anquilosante, Aorta Torácica, Angiografia Digital, Aortografia, Tomografia Computadorizada por Raios X.ABSTRACTIntroduction: Takayasu Arteritis (TA) is a large-vessel vasculitis that affects the aorta and its main branches, especially the carotid and subclavian arteries. Ankylosing Spondylitis (AS) in turn is a chronic inflammatory disease, that mainly affects the spine, evolving with stiffness and progressive functional limitation of the axial skeleton. The association of these two diseases is rare and difficult to diagnose. Case Report: We report the case of a female patient, 49 years, with pain and stiffness of the spine, headache, dizziness, absence of pulses in the upper limbs and systemic hypertension. The CT angiography revealed occlusion of the aorta of the supra-aortic trunks (SAT) and signals of spondylopathy, characterized by syndesmophytes between vertebral bodies and signs of ankylosis of the sacroiliac joint. The digital subtraction angiography confirmed a complete occlusion of the TSA in its origins and extensive collateral circulation. Treatment was started with immunosuppressants and corticosteroids, and treatment of hypertension, with a reduction in blood pressure and improvement of headache, although a maintenance of low back pain and joint stiffness was observed. Conclusion: The simultaneous occurrence of two seemingly distinct diseases such as TA and AS should be interpreted as the manifestation of a systemic inflammatory condition of unknown etiology not yet fully defined.Keywords: Takayasu Arteritis, Ankylosing Spondylitis, Thoracic Aorta, Digital Subtraction Angiography, Aortography, X-Ray Computed Tomography.

Prevalence of Vertebral Fractures by Semiautomated Morphometry in Patients with Ankylosing Spondylitis

2011 ◽  
Vol 38 (5) ◽  
pp. 893-897 ◽  
Author(s):  
NURIA MONTALA ◽  
XAVIER JUANOLA ◽  
EDUARDO COLLANTES ◽  
ELISA MUÑOZ-GOMARIZ ◽  
CARLOS GONZALEZ ◽  
...  
Keyword(s):  
New York ◽  
Ankylosing Spondylitis ◽  
Vertebral Fractures ◽  
Axial Skeleton ◽  
Chronic Inflammatory Disease ◽  
National Registry ◽  
Prevalent Vertebral Fracture ◽  
High Prevalence ◽  
Prevalent Fracture ◽  
Semiquantitative Method

Objective.Ankylosing spondylitis (AS) is a chronic inflammatory disease mainly affecting the axial skeleton and characterized by ossification of the spinal disc, joints, and ligaments leading to progressive ankylosis. Vertebral osteoporosis is a recognized feature of AS. Studies have confirmed a moderate to high prevalence of vertebral fractures with extremely varying ranges in patients with AS. Our objective was to estimate the prevalence of vertebral fractures in a representative Spanish population of patients with AS using a validated semiquantitative method, MorphoXpress®.Methods.Patients were randomly selected from the 10 initial participating centers of the Spanish National Registry of Spondyloarthropathies (REGISPONSER) by consecutive sampling. All patients fulfilled the New York modified criteria for AS and had a baseline thoracolumbar radiograph. A prevalent vertebral fracture was defined according to the Genant classification criteria.Results.The estimated prevalence of vertebral fractures was 32.4% (95% CI 25.5%–39.3%). The majority of fractures were localized in the thoracic segment (n = 100; 82.%) and were mild (n = 79; 64.8%). In logistic regression analysis, age (odds ratio per year 1.05, 95% CI 1.03–1.08, p < 0.001), disease duration (OR per year 1.03, 95% CI 1.01–1.06, p = 0.011), Bath Ankylosing Spondylitis Functional Index score (OR per score 1.16, 95% CI 1.03–1.30, p = 0.015), Bath Ankylosing Spondylitis Radiographic Index-TS (OR per score 1.25, 95% CI 1.12–1.39, p < 0.001), and wall-occiput distance (OR per cm 1.15, 95% CI 1.08–1.23, p < 0.001) were all associated with prevalent fracture.Conclusion.Semiquantitative methods are needed to improve the diagnosis of vertebral fractures in AS in order to start early treatment and to avoid complications arising from osteoporosis.

scholarly journals The pathogenesis of ankylosing spondylitis

2008 ◽  
Vol 24 (1) ◽  
pp. E3 ◽  
Author(s):  
Mohammed F. Shamji ◽  
Mohammed Bafaquh ◽  
Eve Tsai
Keyword(s):  
Ankylosing Spondylitis ◽  
Molecular Mimicry ◽  
Human Leukocyte ◽  
Axial Skeleton ◽  
Chronic Inflammatory Disease ◽  
The Body ◽  
Hla B27 ◽  
Leukocyte Antigen ◽  
Molecular Events ◽  
Microbial Tolerance

✓ Ankylosing spondylitis (AS) is a chronic inflammatory disease that can cause significant functional complications by affecting the sacroiliac joints and axial skeleton. Despite a longstanding knowledge about the familial associations of this disease, particularly among patients positive for human leukocyte antigen (HLA)–B27, the fundamental pathogenetic mechanism by which this disease arises in genetically susceptible individuals remains ill defined. Furthermore, the molecular predilection for characteristic articular site involvement remains under ongoing investigation. Current theories about the HLA-B27 association range from the presentation of novel arthritogenic peptides, to abnormal autoimmune stimulation, to anomalous microbial tolerance. The immune effectors of this damage include CD4+, CD8+, and natural killer cells, with marked heterogeneity at different sites. Biomechanical stresses may trigger this disease by exposing the body to previously immune-sequestered autoantigens or by providing a route for bacterial seeding. Environmental triggers such as infection have not been definitively established but may represent a primary pathogenic step in a molecular-mimicry process. In this article, the authors review the current literature on the origin and pathophysiology of AS, focusing on genetic and molecular associations, consequent pathomechanisms, and associated triggers. An improved understanding of the sequence of molecular events that predispose and initiate the onset of this disease will allow for more specific and targeted therapy and better avoidance of the significant side effects of systemic immunomodulation.

Digital Subtraction Angiography in Takayasu Arteritis

1987 ◽  
Vol 28 (3) ◽  
pp. 247-252 ◽  
Author(s):  
N. Matsunaga ◽  
K. Hayashi ◽  
H. Aikawa ◽  
M. Uetani ◽  
M. Iwao ◽  
...  
Keyword(s):  
Digital Subtraction Angiography ◽  
Takayasu Arteritis ◽  
Vascular Involvement ◽  
Digital Subtraction ◽  
Initial Examination ◽  
Conventional Angiography ◽  
Intravenous Dsa ◽  
Pulmonary Parenchyma ◽  
Pulmonary Arterial ◽  
Collateral Vessels

The usefulness and limitation of digital subtraction angiography (DSA) in Takayasu arteritis were investigated in 32 patients. Intravenous DSA was particularly useful in the follow-up of patients with an established diagnosis of Takayasu arteritis. Pulmonary arterial involvement could also be demonstrated with intravenous DSA as obstructive arterial changes and lack of accumulation of contrast medium in the pulmonary parenchyma. Detailed information concerning the site and extent of vascular involvement and development of collateral vessels were obtained with intraarterial DSA. Thickening of the thoracic aortic wall, however, could not be recognized with either intravenous or intraarterial DSA. Conventional angiography of the descending thoracic aorta is still required as an initial examination, particularly when there is no aortic arch involvement. However, there is no doubt that DSA has the potential to become the diagnostic procedure of choice in Takayasu arteritis.

scholarly journals OP0255 THE ROLE OF PELVIC MORPHOLOGY IN AXIAL SPONDYLOARTHRITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 156.1-156
Author(s):  
E. Zaccagnino ◽  
R. Patel ◽  
L. S. Gensler
Keyword(s):  
New York ◽  
Ankylosing Spondylitis ◽  
Axial Spondyloarthritis ◽  
Axial Skeleton ◽  
Chronic Inflammatory Disease ◽  
Cross Sectional ◽  
Radiographic Damage ◽  
Pelvic Morphology ◽  
The Relationship

Background:Axial Spondyloarthritis (axSpA) is a chronic inflammatory disease affecting the axial skeleton. It includes non-radiographic axSpA and radiographic axSpA [Ankylosing Spondylitis (AS)]. Male axSpA patients often have greater damage, while women report a higher disease burden. The role of pelvic morphology in the axSpA phenotype has not been explored. There is anatomic sexual dimorphism between the male and female pelvis. Given the phenotypic gender differences in axSpA, the role of pelvic morphometry is of interest.Objectives:The purpose of this study is to determine whether an association exists between pelvic dimensions and radiographic damage in patients with axSpA, as well as to compare these measurements in axSpA patients and healthy controls.Methods:This was a cross-sectional analysis comparing axSpA cases from a prospective cohort and non-axSpA controls from the UCSF radiology databank. Informed consent was obtained from axSpA cohort patients and this study was approved by the institutional IRB. To be included in the analysis, we limited inclusion to age ≤ 50 with an Anterior Posterior (AP) pelvis radiograph in the system. We excluded non-nulliparity, pelvic fracture history, BMI ≥ 30kg/m2, any prosthetic history and avascular necrosis. We measured the pelvic inlet, pelvic outlet, and subpubic angle (based on validated scoring methods) (Figure 1) and assessed its relation to sacroiliac joint (SIJ) damage (average SIJ score, New York criteria) and modified Stoke Ankylosing Spondylitis Spine Score (mSASSS) in cases. AxSpA patients were also compared to age/gender matched controls. Pelvic measurements were performed by 2 blinded independent-trained readers in randomized, blinded image order. Inter-rater reliability was assessed. When examining the relationship between pelvic measurements and damage, linear regression was used to stratify by gender and adjust for potential confounders.Results:The axSpA cohort included 481 patients, of which 210 men and 89 women were included in this analysis and gender/age matched controls. Rater inter-class correlation was above 0.70 for pelvic outlet and above 0.80 for other measures. Cases and controls were similar (Table 1). The regression analysis showed a significant relationship between the sub-pubic angle and damage in the spine (coeff=-0.342, p=0.003) in men with axSpA. A sensitivity analysis, excluding mSASSS outliers (mSASSS ≥ 16) upheld the relationship (coeff=-1.40, p=0.002).Conclusion:In men with axSpA, there appears to be a relationship between sub-pubic angle and spinal radiographic damage. This is consistent with our finding that women have larger sub-pubic angles and lower spinal radiographic damage than men. A greater sub-pubic angle may protect against spinal involvement or associate with other protective factors. Further work should be performed to understand the contribution of pelvic anatomy to damage in axSpA.Disclosure of Interests:Ethan Zaccagnino: None declared, Rina Patel: None declared, Lianne S. Gensler Consultant of: AbbVie, Eli Lilly, Gilead, Novartis, Pfizer and UCB., Grant/research support from: Pfizer and UCB.

Kinesiotherapy in patients with ankylosing spondylitis

2020 ◽  
Vol 96 (2) ◽  
pp. 18-23
Author(s):  
R.R. Ahunova ◽  
R.A. Bodrova
Keyword(s):  
Quality Of Life ◽  
Ankylosing Spondylitis ◽  
Axial Skeleton ◽  
Poor Quality ◽  
Chronic Inflammatory Disease ◽  
Functional Disorders ◽  
Regular Group ◽  
Permanent Disability ◽  
Positive Effect

Ankylosing spondylitis is a chronic inflammatory disease from the group of spondylarthritis, characterized by damage to the predominantly axial skeleton, gradual formation of functional disorders of the spine and joints, leading to temporary or permanent disability and poor quality of life for patients, mostly young. In recent years, much attention has been paid to the study of the effectiveness of kinesitherapy in patients with ankylosing spondylitis. Studies have been conducted that confirmed the positive effect on the function of the spine and joints of regular exercises performed at home, regular group exercise and their combination with physiotherapy methods. However, due to the imperfect methodology of conducting these studies and the lack of a standardized approach in evaluating the effectiveness of therapy in these patients, further detailed researches are needed to select the required amount of kinesitherapy for patients with ankylosing spondylitis and develop a standardized assessment of its effectiveness.

Ankylosing spondylitis

2013 ◽  
Author(s):  
Joachim Sieper
Keyword(s):  
Ankylosing Spondylitis ◽  
Axial Skeleton ◽  
Chronic Inflammatory Disease ◽  
Hla B27 ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Male Predominance ◽  
Inflammatory Drugs ◽  
Active Inflammation

Ankylosing spondylitis (AS) is a chronic inflammatory disease predominantly of the sacroiliac joint (SIJ) and the spine. It starts normally in the second decade of life and has a slight male predominance. The prevalence is between 0.2 and 0.8% and is strongly dependent on the prevalence of HLA B27 in a given population. For the diagnosis of AS, the presence of radiographic sacroiliitis is mandatory. However, radiographs do not detect active inflammation but only structural bony damage. Most recently new classification criteria for axial spondyloarthritis (SpA) have been developed by the Assessement of Spondylo-Arthritis international Society (ASAS) which cover AS but also the earlier form of non-radiographic axial SpA. MRI has become an important new tool for the detection of subchondral bone marrow inflammation in SIJ and spine and has become increasingly important for an early diagnosis. HLA B27 plays a central role in the pathogenesis but its exact interaction with the immune system has not yet been clarified. Besides pain and stiffness in the axial skeleton patients suffer also from periods of peripheral arthritis, enthesitis, and uveitis. New bone formation as a reaction to inflammation and subsequent ankylosis of the spine determine long-term outcome in a subgroup of patients. Currently only non-steroidal anti-inflammatory drugs (NSAIDs) and tumour necrosis factor (TNF) blockers have been proven to be effective in the medical treatment of axial SpA, and international ASAS recommendations for the structured management of axial SpA have been published based on these two types of drugs. Conventional disease-modifying anti-rheumatic drugs (DMARDs) such as methotrexate are not effective.

scholarly journals Ankylosing Spondylitis: From Cells to Genes

2013 ◽  
Vol 2013 ◽  
pp. 1-16 ◽  
Author(s):  
José Francisco Zambrano-Zaragoza ◽  
Juan Manuel Agraz-Cibrian ◽  
Christian González-Reyes ◽  
Ma. de Jesús Durán-Avelar ◽  
Norberto Vibanco-Pérez
Keyword(s):  
Ankylosing Spondylitis ◽  
Th17 Cells ◽  
Chronic Inflammatory Disease ◽  
Unknown Etiology ◽  
Hla B27 ◽  
Promoter Regions ◽  
Protein Coding ◽  
Coding Regions ◽  
Genetic Linkages

Ankylosing spondylitis (AS) is a chronic inflammatory disease of unknown etiology, though it is considered an autoimmune disease. HLA-B27 is the risk factor most often associated with AS, and although the mechanism of involvement is unclear, the subtypes and other features of the relationship between HLA-B27 and AS have been studied for years. Additionally, the key role of IL-17 and Th17 cells in autoimmunity and inflammation suggests that the latter and the cytokines involved in their generation could play a role in the pathogenesis of this disease. Recent studies have described the sources of IL-17 and IL-23, as well as the characterization of Th17 cells in autoimmune diseases. Other cells, such as NK and regulatory T cells, have been implicated in autoimmunity and have been evaluated to ascertain their possible role in AS. Moreover, several polymorphisms, mutations and deletions in the regulatory proteins, protein-coding regions, and promoter regions of different genes involved in immune responses have been discovered and evaluated for possible genetic linkages to AS. In this review, we analyze the features of HLA-B27 and the suggested mechanisms of its involvement in AS while also focusing on the characterization of the immune response and the identification of genes associated with AS.

scholarly journals Takayasu Arteritis - A Case Study

2021 ◽  
Vol 11 (6) ◽  
pp. 362-366
Author(s):  
Minal Dhairya Bhavsar ◽  
Vinal Charpot
Keyword(s):  
Takayasu Arteritis ◽  
Cerebrovascular Accident ◽  
Inflammatory Process ◽  
Clinical Laboratory ◽  
Clinical Manifestations ◽  
Inflammatory Cells ◽  
Chronic Inflammatory Disease ◽  
Unknown Etiology ◽  
Cerebrovascular Accidents

Takayasu arteritis (TA) is a chronic inflammatory disease of unknown etiology. The mechanism of this disease is not exactly defined. The inflammatory process is generally (but not exclusively) initiated in the second or third decade of life through the actions of non-specific inflammatory cells. As the disease progresses, fibrotic stenosis occurs in aorta and its main branches. The consequence of this inflammatory process can be stenosis, thrombosis, dilatation or aneurysm formation in aorta and/ or its branches. In Asia, its incidence (2.69 in a million per year) has been reported to be 100 times higher than in Europe and North America. Because of the delay in diagnosing the disease, patients often experience claudication, absence of pulses, hypertension, myocardial infarction (MI), and cerebrovascular accidents (CVAs). Accurate and early diagnosis of TA can reduce the economic, social, and psychological burdens. We present clinical, laboratory and imaging findings of a 15 years old girl with TA, who initially presented with clinical manifestations of stroke in form of weakness in Lt Side upper and lower limb and aphasia. The rarity of the disease and especially such a presentation can cause considerable delay in the diagnosis and treatment. Key words: Takayasu arteritis, Cerebrovascular accident, Infarction, physical therapy.

scholarly journals Biologic therapies for the treatment of ankylosing spondylitis

2021 ◽  
Vol 64 (2) ◽  
pp. 116-123
Author(s):  
Soo Min Ahn ◽  
Yong-Gil Kim
Keyword(s):  
Ankylosing Spondylitis ◽  
Kinase Inhibitors ◽  
Axial Skeleton ◽  
Chronic Inflammatory Disease ◽  
Tnf Inhibitors ◽  
Janus Kinase ◽  
Interleukin 17 ◽  
Biological Products ◽  
Axial Symptoms ◽  
Anti Inflammatory

Ankylosing spondylitis (AS) is a chronic inflammatory disease predominantly affecting the axial skeleton. AS treatment aims to reduce pain and inflammation and maintain functional capacity. Nonsteroidal anti-inflammatory drugs are the first-line treatment for those with active AS. While for peripheral arthritis, sulfasalazine or local glucocorticoid injection can be used, these are not recommended for axial symptoms. Twenty years ago, biological products that target the tumor necrosis factor (TNF) were developed. These have been approved for use in rheumatoid arthritis and AS. Since the introduction of these TNF inhibitors, the control of disease activity in AS has improved markedly. TNF inhibitors, including both anti-TNF-alpha monoclonal antibodies and recombinant TNF soluble receptors, can be considered for patients with persistently active disease despite nonsteroidal anti-inflammatory drug treatment. Recently, interleukin-17 inhibitors have also been approved for use in AS patients with insufficient responses to TNF inhibitors. Ongoing clinical trials are exploring the effect of Janus kinase inhibitors against AS. This review summarizes the current pharmaceutical treatment for AS, focusing on the biological products. Recommendations for AS management are also discussed in this review.

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