title: Pediatric-onset Takayasu's arteritis: clinical features and short-term outcome.
abstract: The aim of this was to assess clinical features and outcome in pediatric-onset Takayasu's arteritis (TA). Retrospective data analysis of patients diagnosed with TA over last 13 years with onset before 18 years of age was done. Their presenting features, activity (by NIH criteria, ITAS2010, ITAS-A), disease extent (by DEI.Tak) and angiographic findings were retrieved from clinic files. Treatment received and follow-up data on disease activity and damage by TA damage score (TADS) were also analyzed. Wherever repeated angiography data were available, the same was analyzed. Values are expressed as median with interquartile range in brackets. There were 29 patients (19 females) with median age at diagnosis of 14 (13-16) years and delay to diagnosis of 1 (0.4-2) year. Common presenting symptoms were pulse loss (23/29) and hypertension (22/29). Patients had extensive disease at presentation with median DEI.Tak of 12 (9.5-15); 23/29 had elevated acute-phase reactants, and 28/29 were active at presentation [median ITAS2010 13 (8-15.5), ITAS-A 14 (10-17)]. Numano's type V was the commonest angiographic type (22/29). At a median follow-up of 2.4 (1.5-5.1) years, 2/20 were active whereas all had sustained damage despite a majority (17/20) being on immunosuppression. The median TADS was 8 (6.3-9.8) with pulse loss, claudication and hypertension being the commonest damage item. Two needed renal artery stenting to control hypertension. Angiographic assessment at least 2 years apart demonstrated disease progression in 5 of 6 patients despite immunosuppression. Significant damage accrued on follow-up despite immunosuppression and control of disease activity. Hypertension remains the major long-term morbidity.
authors.affiliation: Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India. durgapmisra@gmail.com.
authors.forename: Durga Prasanna
authors.initials: DP
authors.lastname: Misra
authors.affiliation: Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India. aa.amita@gmail.com.
authors.forename: Amita
authors.initials: A
authors.lastname: Aggarwal
authors.affiliation: Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India. abledoc@gmail.com.
authors.forename: Able
authors.initials: A
authors.lastname: Lawrence
authors.affiliation: Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India. vikasagr@yahoo.com.
authors.forename: Vikas
authors.initials: V
authors.lastname: Agarwal
authors.affiliation: Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India. rnmisra2000@gmail.com.
authors.forename: Ramnath
authors.initials: R
authors.lastname: Misra
mesh_terms: D000293:Adolescent; D018450:Disease Progression; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D011379:Prognosis; D012720:Severity of Illness Index; D063189:Symptom Assessment; D013625:Takayasu Arteritis / Q000175:diagnosis* / Q000188:drug therapy; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents
keywords: Activity; Aortoarteritis; Childhood vasculitis; Damage; India; Large vessel vasculitis