Pathology of Takayasu arteritis: A brief review

Childhood-onset Takayasu arteritis (c-TA) is a distinct subset affecting a wide age group, ranging from young infants to adolescents and it differs from adult TA in many aspects. There is scarcity of data on c-TA worldwide. The disease is classified using the European League Against Rheumatism/Pediatric Rheumatology International Trials Organization/Pediatric Rheumatology European Society criteria. The non-specific nature of presenting complaints and lack of appropriate biomarkers delay the early diagnosis of this illness and many children present with complications, which become irreversible once they set in. One of the largest cohorts of 40 children with c-TA from our center reports hypertension as the commonest presenting feature. Systemic symptoms like headache, fever and weight loss are also described. Assessment of disease in c-TA is done by correlating clinical features with raised inflammatory markers. Advanced imaging plays an important role in diagnosis. In c-TA, the role of magnetic resonance angiography is advocated, taking into consideration the enormous amount of radiation exposure with other modalities. Complications of c-TA include cardiovascular, pulmonary, neurological and those arising secondary to long-term steroid and immunosuppression therapy.

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“…55 To date there are reports of a total of 120 TA patients treated with TNF agents. Most of these reported series comprise of adult TA patients.…”

Section: Biological Therapy In Tamentioning

confidence: 99%

Childhood‐onset Takayasu arteritis: an update

et al. 2015

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“…This variability in PTA effectiveness based on underlying conditions has been considered to be the result of different pathologies. In TA, for example, inflammatory processes fragment the elastic fibers of vessels, while FMD exhibits medial fibroplasia but preserves the intima and MMD results in fibrous thickening of the intima [35][36][37]. Beyond the different pathological factors that affect the efficacy of PTA, restenosis varied according to underlying conditions.…”

Section: Discussionmentioning

confidence: 99%

Long-Term Outcomes of Pediatric Renovascular Hypertension

Chung

Lee

Park

et al. 2017

Kidney Blood Press Res

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Background/Aims: Renovascular hypertension (RVHT) is an important cause of childhood hypertension. This study evaluated the clinical characteristics and outcomes of Korean children with RVHT. Methods: Children treated for RVHT between 2000 and 2015 at our center were retrospectively reviewed. Results: Forty-six children were followed for a median of 6.5 (0.66-27.23) years. Forty-five percutaneous transluminal angioplasties (PTAs) were performed in 32 children. At the last visit, clinical benefit was observed in 53.3% of children. Patients with comorbid cerebrovascular disease (CVD) showed less favorable long-term outcomes after PTA (clinical benefit in 41.7% vs. 61.1% in others) and higher restenosis rates (50% vs. 31.6% in others). Surgical procedures (bypass or nephrectomy) were performed in 8 patients. After surgery, blood pressure was normalized in 2 patients, improved in 3 patients, and unchanged in the remaining patients. Between PTA group (n=21) and medication group (n=14), percentage of atrophic kidneys became higher after follow-up period in medication group than in PTA group (60.0% vs. 26.1%, P=0.037). Conclusion: Aggressive treatment of pediatric RVHT yielded fair outcomes in our cohort. CVD comorbidity was associated with relatively poor PTA outcomes. To confirm our findings, larger cohort studies with a longer follow-up period are warranted.

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“…Note the linear wrinkles that have been described as having a "tree bark" appearance. These result from scar replacement of elastic tissue in the aortic wall although the apparent aggressiveness of this disease suggests a more active T-cellmediated process with acute infl ammation, followed by necrosis, neovascularization, intimal proliferation with smooth muscle integration, and fi nally giant cell formation in the remodeled vessel walls [ 7 ].…”

Section: Pathology/pathophysiologymentioning

confidence: 99%

“…It is also distinguished from GCA in that it almost exclusively affects younger women, often of Asian ancestry [ 7 ].…”

Section: Takayasu Arteritismentioning

confidence: 99%