Childhood Vasculitis Improved on Tocilizumab but Arteries Remain Damaged

A 12-year-old girl with refractory childhood-onset Takayasu arteritis developed extensive narrowing of arteries supplying the head, arms and legs despite several forms of immunosuppression. Switching from adalimumab to the interleukin-6 receptor blocker tocilizumab was followed by resolution of inflammatory changes around the right femoral artery, normalization of inflammatory blood markers and relief of hip pain and limping. The established stenoses, however, remained, and the child retained an absent radial pulse, weak pulses and markedly unequal blood pressures between her limbs. The retrospective case report shows why normal laboratory results cannot, by themselves, demonstrate that arterial inflammation has resolved or that vascular damage has reversed.

The girl initially presented in January 2026 after 13 days of recurrent, severe retrosternal chest pain and diffuse colicky abdominal pain. Each episode lasted about 15 minutes, occurred two or three times daily and sometimes woke her from sleep. For the preceding six months, she had experienced progressive exertional fatigue and intermittent pain with “drumstick” swelling of her right fingertips. Unlike the constitutional pattern that often triggers investigation for vasculitis, she had no fever, weight loss, night sweats or joint pain. Her eventual diagnosis depended on findings that were visible only when clinicians examined all four limbs: the right arm blood pressure was below the fifth percentile for her age and sex, the left arm and lower-limb pressures were between the 50th and 90th percentiles, her right radial pulse was absent and other peripheral pulses were weak or diminished.

Laboratory testing revealed a pronounced systemic inflammatory response, with an erythrocyte sedimentation rate of 110 mm per hour and C-reactive protein concentrations of 82 to 93 milligrams per liter. Mild normocytic anemia, leukocytosis and thrombocytosis were also present. Computed tomography angiography showed mild concentric thickening of the aortic arch, severe stenosis of the right common and internal carotid arteries, near-occlusion of the right subclavian artery, moderate-to-severe brachiocephalic stenosis and moderate narrowing of the left common carotid and left subclavian arteries. In the legs, the right common and superficial femoral arteries had moderate stenosis with concentric wall thickening. In contrast, the descending thoracic and abdominal aorta, visceral branches, renal arteries and other imaged territories were spared, with no aneurysm or intraluminal thrombus detected.

The imaging pattern corresponded to Numano type IIa disease, involving the ascending aorta, aortic arch and its branches, while the femoral lesions were recorded separately because that classification system does not cover those peripheral arteries. The girl met the mandatory angiographic criterion and four of five additional criteria in the EULAR/PRINTO/PRES Ankara 2008 classification for childhood Takayasu arteritis. She also met the 2022 ACR/EULAR criteria, although those were developed and validated in adults. Investigators considered fibromuscular dysplasia, infective endocarditis, tuberculous aortitis, systemic lupus erythematosus, antiphospholipid syndrome, ANCA-associated vasculitis, sarcoidosis, inflammatory bowel disease and several genetic mimics. Negative serology reduced the likelihood of some alternatives but did not exclude them; the diagnosis rested principally on the arterial distribution, clinical findings and imaging.

Digital clubbing was an unusual feature. It affected both hands and had begun in the right fingertips before the acute presentation, in parallel with documented upper-limb arterial insufficiency. Clubbing in children more commonly has cardiac, pulmonary, hepatic, gastrointestinal or endocrine causes, and the relevant investigations were not performed systematically. Echocardiography found no intracardiac shunt or structural congenital heart disease, but pulmonary function tests, dedicated pulmonary artery imaging, a formal shunt study and a hepatic work-up were not documented. A proposed link between chronic arterial insufficiency, local hypoxia and growth-factor release remains hypothetical, so the authors appropriately classified the association as unexplained rather than claiming that Takayasu arteritis caused the clubbing.

The origins of the chest and abdominal pain were likewise not established with certainty. The abdominal aorta and the coeliac, superior mesenteric and inferior mesenteric arteries were patent, normal in calibre and free of wall thickening; the pain was not consistently related to meals, and there was no diarrhoea, gastrointestinal bleeding or weight loss. Small-vessel mesenteric disease below the resolution of CT could not be excluded. The aortic root and ascending aorta were normal, with no aortic regurgitation, but coronary arteries were not imaged and electrocardiography and cardiac biomarkers were not documented. On a later admission with severe nocturnal right-sided chest pain, mild hypertension, mild tricuspid regurgitation and mildly reduced left-ventricular systolic function were recorded. The treating clinicians considered the chest pain likely multifactorial, although coronary involvement remained possible.

Treatment began with intravenous methylprednisolone at 20 milligrams per kilogram daily, followed by oral prednisolone at 1 milligram per kilogram daily. Methotrexate was added weekly at 15 milligrams per square metre, together with low-dose aspirin for critical stenoses. When the right common femoral lesion progressed to 80% stenosis, causing severe hip pain and a limp, therapeutic low-molecular-weight heparin was also used empirically. No thrombus had been demonstrated, and no thrombophilia screen was performed; the team described this as a pragmatic decision under uncertainty rather than an evidence-based standard. Revascularization was discussed but deferred because collateral circulation was adequate, critical limb ischemia was absent and active inflammation was considered likely to increase the risk of restenosis.

Follow-up Doppler ultrasonography showed mild improvement in the calibre of several upper-limb arteries, but repeat CT angiography continued to show active arteritis, diffuse mural thickening and peri-arterial fat stranding. With progression despite glucocorticoids and methotrexate, the team introduced the tumour necrosis factor inhibitor adalimumab. Vascular disease continued to progress, prompting a switch to intravenous tocilizumab. The choice reflected the established role of interleukin-6 in Takayasu arteritis, pediatric series reporting benefit from switching between anti-TNF and anti-IL-6 treatments in refractory disease, comparative adult evidence and local availability. After the switch, the acute peri-arterial inflammatory change around the right common femoral artery resolved on imaging, the hip pain and limp subsided, and ESR and CRP returned to normal. Residual lower-limb wall thickening remained. The report does not provide exact doses, intervals or treatment durations for several agents, including adalimumab and tocilizumab, nor a full schedule for tapering corticosteroids.

At the latest documented admission in June 2026, the child was clinically stable and ambulatory, with no acute ischemic complication and stable hemoglobin. She had received another pulse of methylprednisolone and her scheduled tocilizumab dose, while methotrexate continued and enalapril was introduced for hypertension and mild left-ventricular dysfunction. No infusion reaction, cytopenia, transaminitis or serious infection was reported during the observed treatment period. Nevertheless, four-limb blood-pressure asymmetry and pulse deficits remained: the right arm pressure stayed below the fifth percentile, the right radial pulse remained absent, and the left radial and brachial pulses remained diminished. Renal Doppler imaging showed preserved perfusion.

This single-patient report cannot establish the effectiveness of tocilizumab or support broad causal conclusions. Follow-up ended at the June admission, approximately five months after presentation and only a short time after the switch to tocilizumab, so the record does not demonstrate sustained remission. Complete serial laboratory data, exact medication regimens, systematic imaging of every arterial territory, electrocardiography, cardiac biomarkers, pulmonary and hepatic evaluations of clubbing, ophthalmologic assessment, thrombophilia screening and genetic testing were unavailable or not performed. Magnetic resonance angiography and fluorine-18 fluorodeoxyglucose positron-emission tomography were also unavailable. No validated disease-activity score was applied prospectively, and coronary and pulmonary arteries were not specifically imaged, preventing assignment of the corresponding Numano modifiers. The central clinical lesson is narrow but important: in a child with unexplained systemic inflammation, measuring blood pressure in all four limbs and checking every pulse can expose major vascular asymmetry. In patients receiving IL-6 blockade, ESR and CRP require particular caution because treatment can suppress these markers independently of arterial-wall inflammation. Clinical symptoms and serial vascular imaging must therefore be interpreted together, and normalized blood tests must not be mistaken for either biological remission or recovery from established arterial damage.

Subject of Research: A retrospective case report of refractory childhood-onset Takayasu arteritis treated with escalating biologic immunosuppression.

Article Title: Case Report: Refractory childhood-onset takayasu arteritis with digital clubbing and extensive supra-aortic and lower-limb arterial disease

Article References: Abunejma, F. M., Jabari, L. M., Hamad, M. N., Tamimi, T. O., Bseiso, O., Dweik, H. A., Isayed, R. M. A., Qunibi, H., Alzaro, W., & Alhroub, W. (2026). Case Report: Refractory childhood-onset takayasu arteritis with digital clubbing and extensive supra-aortic and lower-limb arterial disease. Frontiers in Pediatrics, 14, Article 1961784. https://doi.org/10.3389/fped.2026.1961784

Image Credits: AI Generated

DOI: 10.3389/fped.2026.1961784

Keywords: Takayasu arteritis, childhood vasculitis, tocilizumab, adalimumab, arterial stenosis, digital clubbing, vascular imaging, pediatric rheumatology, IL-6 inhibition, case report, Case, Report