Clinical course of 602 patients with Takayasu's arteritis: comparison between Childhood-onset versus adult onset disease

Abstract

Objectives To describe the clinical profile of Asian Indian patients with Takayasu’s arteritis (TAK) and to compare clinical features and outcome of childhood-onset Takayasu’s arteritis (cTAK) with adult-onset TAK (aTAK). Methods Data related to clinical features and response to treatment of patients with cTAK (age of onset <16 years) and aTAK from a large observational cohort in our tertiary care teaching hospital were noted and compared. Results Altogether, 602 patients (cTAK = 119; aTAK = 483) were studied. Patients with cTAK had a blunted female: male ratio; but fever, elevated acute phase reactants, involvement of abdominal aorta or its branches, hypertension, abdominal pain, elevated serum creatinine and cardiomyopathy were more common in cTAK as compared with aTAK. Patients with aTAK were more likely to have aortic-arch disease and claudication than cTAK. During follow-up, complete remission was more common in cTAK (87% vs 66%; P < 0.01), but subsequent relapses were equally common (30% vs 27%; P = 0.63). Independent associations of disease duration at presentation with disease extent [Disease Extent Index in TAK (DEI.Tak)] and damage [TAK Damage Score (TADS)] were observed (P ≤ 0.01). Moreover, 54% of patients with symptom duration of >5 years at presentation still continued to have elevated CRP suggesting continued and active inflammation warranting escalation or inititation of immunosuppression. Conclusion Patients with cTAK are more likely to have arterial disease below the diaphragm, systemic inflammation and achieve remission. Disease of the aortic arch is more common in patients with aTAK. Longer duration of symptoms prior to initiation of immunosuppression, thereby leading to extensive disease and damage, reflects ongoing disease activity as the rule rather than exception in untreated TAK.

Childhood-onset Takayasu arteritis

“…Although hypertension is the most common form of presentation in children and adults, the overall clinical spectrum at the presentation of children with Takayasu arteritis differs from that in adults [8,10,[13][14][15][16][17]. Specifically, headache, cardiomyopathy, renal failure, and systolic hypertension were significantly more common in initial presentation in children, as compared with adults [14][15][16]. Further, abdominal aorta and renal artery involvement appear to be more common in pediatrics as well [13,[15][16][17].…”

An Update on Childhood-Onset Takayasu Arteritis

“…In children, the aorta (arch, thoracic or abdominal) is most commonly affected, followed by the renal, subclavian, carotid, and splanchnic arteries ( 5 , 52 ; Figure 1 ). Stenotic lesions predominate, but occlusion, concentric vessel wall thickening and aneurysms may also be observed.…”

“…Table 2 summarizes childhood-onset TAK cohorts published during the last decade ( 6 , 7 , 44 – 46 , 52 , 54 – 62 ). Children may present with non-specific symptoms such as fever, dyspnea, headaches, weight loss or abdominal pain.…”

“…More recently, several groups have been directly comparing pediatric and adult TAK cohorts ( 5 , 52 , 54 , 60 – 62 ). Childhood-onset TAK has a lower female predominance ( 5 , 52 , 54 ) and a shorter diagnostic delay, possibly due to a more pro-inflammatory presentation ( 5 , 62 ). Children have more commonly arterial hypertension, less claudication of the upper extremities, and less carotidynia.…”

Screening Ultrasonography in Children with Prolonged Fever Can Detect Early Takayasu Arteritis

“…However, if such an attempt is made in children, especially in clinical scenario like FUO, it may upscale the pretest probability of diagnosing early c-TAK. The higher prevalence of abdominal vessel involvement in c-TAK than in adult-onset TAK makes it an even more relevant procedure in c-TAK (7) . Interestingly, in children, ultrasound may be superior to CECT for neck vessels also, according to this study, because the low fat in neck regions of children contributes to poor differentiation between the periarticular soft tissue and the arterial wall in CECT.…”