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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. [ABSTRACT FROM AUTHOR]

An introduction is presented in which the editor discusses articles in the issue on topics including vasculitis, Kawasaki's disease.

The status of vascular lesion treatment using percutaneous intervention (PI) in Takayasu arteritis (TAK) remains unresolved. This study sought to develop PI strategies appropriate for TAK. A prospectively maintained single-center database of TAK PI procedures from 1996 to 2022 was analyzed retrospectively. Obstructive lesions were treated by elective stenting (using bare or covered stents), balloon angioplasty (BA), or cutting-balloon angioplasty (CBA), with adjunctive stenting for suboptimal BA or CBA results. PIs were repeated in restenotic lesions until sustained success was obtained. Aortic or peripheral aneurysms and spontaneous aortic dissections were treated with covered stents or endografts. Immunosuppressive therapy, started before PI, was continued long term. A total of 942 patients underwent PI to treat 2,450 arterial lesions (2,365 stenoses or occlusions, 85 aneurysms or dissections) in 630 subclavian or axillary, 586 renal, 463 aortic, 333 carotid, 188 mesenteric, 116 iliac, 71 coronary, and 63 other arteries; 3,805 PIs were performed (1.55 PIs per lesion; range 1-7 PIs per lesion). Early success was obtained in 2,262 (92.3%), and late success in 1,460 (84.5%) of 1,727 lesions with a median of 39 months (IQR: 15-85 months) of follow-up. Repeated PIs increased late success in obstructive lesions from 48.6% to 83.3%. A total of 1,687 elective stenting lesions achieved 88% late success with 1.49 PIs per lesion; covered stents (1.18 PIs per lesion) restenosed less than bare stents (1.51 PIs per lesion; P < 0.001). A total of 183 (36%) of 513 BA-treated lesions had good outcomes without adjunctive stenting; 122 CBA-treated lesions had 19% dissections and 8% ruptures or pseudoaneurysm formations. Aneurysms or dissections had 91.3% late success after PI. A total of 472 complications occurred in 415 (17%) lesions; 375 (79%) were resolved. Most vascular lesions in TAK can be effectively, safely, and durably treated using predominantly stent-based PI strategies. [Display omitted] [ABSTRACT FROM AUTHOR]

Introduction: Buerger’s disease is an unclassifiable vasculitis of the small and medium-sized distal arteries and superficial veins. BVAS (Birmingham Vasculitis Activity Score) is a validated disease activity index for vasculitis of different types. DEI.Tak (Disease Extent Index. Takayasu’s arteritis) scoring system is a validated disease extent Index used for Takayasu arteritis. Aim: The aim of this study was to assess outcome in patients with Buerger’s disease using various clinical features, laboratory parameters, BVAS and DEI.Tak scoring system. Materials and Methods: Case records of 136 patients diagnosed with Buerger’s disease in our hospital between August 1996 and July 2006 were studied retrospectively. Various clinical features (smoking history, claudication pain, loss of pulse), laboratory parameters, treatment modalities and outcome measures were recorded in a defined proforma. BVAS and DEI.Tak scoring was done using the data from medical records documented during the patients’ last visit to the hospital. Outcome of any patient requiring amputation was considered as bad outcome. Others, who could be managed by antiplatelet drugs, sympathectomy and revascularization procedures without any amputation were classified within the good outcome subset. Statistical analysis was done using Chi-square test and Non parametric Mann- Whitney test was performed to correlate outcome with all the recorded parameters including those embedded in BVAS and DEI.Tak scoring systems. Results: Sixty eight patients were in each group, namely the good outcome and bad outcome subsets. The mean BVAS score were 10.29±1.26 and 10.88±2.57 and mean DEI.Tak score were 5.29±1.75 and 7.93±2.43 and these scores were observed to be significantly different (p=0.038, p=0.014 respectively) among the good outcome subsets and bad outcome patients with respectively. Proportion of patients with claudication pain and absent upper limb pulse were observed significantly higher in the bad outcome group. Conclusion: Buerger’s disease with higher DEI.Tak score has significantly higher risk of bad outcome. DEI.Tak score can be used as an important predictor of outcome in Buerger’s disease. [ABSTRACT FROM AUTHOR]

Objective: To develop and validate new classification criteria for Takayasu arteritis (TAK). Methods: Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in 6 phases: 1) identification of candidate criteria items, 2) collection of candidate items present at diagnosis, 3) expert panel review of cases, 4) data‐driven reduction of candidate items, 5) derivation of a points‐based classification score in a development data set, and 6) validation in an independent data set. Results: The development data set consisted of 316 cases of TAK and 323 comparators. The validation data set consisted of an additional 146 cases of TAK and 127 comparators. Age ≤60 years at diagnosis and imaging evidence of large‐vessel vasculitis were absolute requirements to classify a patient as having TAK. The final criteria items and weights were as follows: female sex (+1), angina (+2), limb claudication (+2), arterial bruit (+2), reduced upper extremity pulse (+2), reduced pulse or tenderness of a carotid artery (+2), blood pressure difference between arms of ≥20 mm Hg (+1), number of affected arterial territories (+1 to +3), paired artery involvement (+1), and abdominal aorta plus renal or mesenteric involvement (+3). A patient could be classified as having TAK with a cumulative score of ≥5 points. When these criteria were tested in the validation data set, the model area under the curve was 0.97 (95% confidence interval [95% CI] 0.94–0.99) with a sensitivity of 93.8% (95% CI 88.6–97.1%) and specificity of 99.2% (95% CI 96.7–100.0%). Conclusion: The 2022 American College of Rheumatology/EULAR classification criteria for TAK are now validated for use in research. [ABSTRACT FROM AUTHOR]

Objective: To study obstetric and perinatal outcomes among pregnant women with Takayasu arteritis (TA), attending our hospital for pregnancy and childbirth between January 2011 to December 2016. Material and Methods: Retrospective study was carried out by abstracting clinical charts on all pregnant women with TA who underwent antenatal care and/or delivery in our hospital during this period. American College of Rheumatology criteria was used for diagnosis of TA. Sixteen women with TA were included in the study. Maternal demographic data, stage of disease, complications related to disease, details of treatment taken prior to pregnancy, pregnancy outcomes, and neonatal outcomes were studied. Results: Forty-four percentage (7/16) belonged to type 5 angiographic type, however the same proportion (7/16) had undergone surgical corrections prior to pregnancy and the majority (15/16) were on medical management. Only three women (19%) were diagnosed during pregnancy. Most did not have active disease measured by Kerr's criteria (n=12; 75%), and Indian Takayasu clinical activity scores A. Chronic hypertension was the commonest antenatal complication (56.2%), nearly one-third had growth restricted babies and 25% had preterm labour. There were no cardiovascular events, no maternal deaths, nor fetal or neonatal deaths. Two-thirds of our women were delivered by caesarean section. Conclusion: Preconceptional counselling is of paramount importance in women with TA. Good maternal and fetal outcomes are observed with close antenatal surveillance and multidisciplinary care. Pregnancy should be planned during disease remission, with good antenatal care, close monitoring of clinical symptoms, early diagnosis and treatment of complications. [ABSTRACT FROM AUTHOR]

The aim of the study was to explore utility of serial serum myeloid-related protein 8/14 (MRP8/14) as a biomarker of clinical disease activity and angiographic progression in Takayasu arteritis (TA). Serum MRP8/14 levels were assayed by commercial ELISA for 85 TA patients and 24 healthy controls at baseline, and for 56 and 21 TA patients during follow-up visits R1 and R2, respectively. Disease was categorised as active, indeterminate and stable according to Indian Takayasu Arteritis score (ITAS 2010), ITAS-A(CRP) and angiography. Patients were divided into responders and non-responders/relapsers based on treatment response. Non-parametric tests were used for inter-group comparisons at baseline and during follow-up time points. Generalised Estimating Equation was used to study association between changes in serial MRP8/14 levels and disease activity. At baseline, median MRP8/14 levels were higher in patients with TA than healthy controls [7353 (4524 to11283) vs 4896 (3194 to 8474.5) ng/ml, p = 0.011]. Patients with active disease had higher levels [8552 (5463-12488)] than stable disease [5292.5 (3140.5-7310)], p = 0.002, and healthy controls [4896 (3194-8474.5)], p = 0.001. Changes in serial MRP8/14 level were associated with changes in disease activity, independent of steroid dose, p = 0.000. At R1, MRP 8/14 levels were lower than baseline in responders (n = 38) [9146.0 (6296.8-13693.8) vs 6501 (4314.8-8304.5), p = 0.004], but did not change in non-responders/relapsers (n = 14) [6693.5(4210.8-10516.3) vs 7755.0(5342-10741.0), p = 0.42]. Similar trend was observed at R2. MRP8/14 levels increased during follow-up in 66% and 26.3% of angiographic progressors and non-progressors, respectively. MRP8/14 in TA may act as a novel biomarker with prognostic implications. [ABSTRACT FROM AUTHOR]

Abstract: Background/Purpose: Normal C‐reactive protein (CRP) in active Takayasu arteritis (TA) is a dilemma. We attempted to validate our pilot study finding of rs1205 in CRP gene being protective against TA. Methods: Genomic DNA of 104 patients and 185 sex‐matched healthy controls were genotyped for rs1205 by Taqman assay. Clinical details, demography, angiographic and activity scores (Indian Takayasu arteritis score 2010) were recorded prospectively at baseline and during follow‐up visits for 12 months. Minor allele frequency (MAF) and genotype distribution between patients and controls as well as patient subgroups were compared using χ2 test with Bonferroni correction (pc) and logistic regression was performed to determine independent associations. Results: The majority of patients (n = 84) and controls (n = 166) were females. MAF of T allele of rs1205 was less frequent in patients (27%) as compared to controls (37.6%), P = 0.013, pc = 0.026 with an odds ratio of 0.632 irrespective of gender. Frequency of CC genotype was higher in cases (53.8%) than controls (37.3%), P = 0.006, pc = 0.018. A dominant model of genotype‐phenotype association revealed CC to be associated with more frequent coronary arterial and ascending aorta involvement than the other genotypes clubbed together (P = 0.01 and P = 0.014, respectively). Blunted CRP response seems to be less frequent in patients with CC genotype (P = 0.064). Conclusion: T allele of rs1205 in CRP gene was less frequent in TA. CC genotype was associated with involvement of coronary arteries and ascending aorta. CC genotype was less commonly associated with blunted CRP response (CT + TT > CC). [ABSTRACT FROM AUTHOR]

Objective: Anti-C1q antibodies (Anti-C1q Ab) are seen in hypocomplementemic urticarial vasculitis syndrome (HUVS), infection-associated vasculitis such as hepatitis C virusrelated vasculitis and in autoimmune diseases such as rheumatoid vasculitis, polyarteritis nodosa, giant cell arteritis, vascular Behcet’s disease, and cryoglobulin associated vasculitis. Aim of this study is to evaluate the presence of Anti-C1q Ab in vasculitis and to determine if any difference exists between primary and secondary vasculitis in relation to this antibody. Patients and Methods: Consecutive patients with diagnosis of either a primary or secondary vasculitis were recruited. Primary vasculitis were diagnosed by the American College of Rheumatology 1990 criteria. Clinical features and serological markers were noted. Anti-C1q Ab was assayed by commercially available ELISA kit (Demeditec Diagnostics GmbH, Germany). Results: Sixty-four patients were recruited for the study comprising of 41 primary vasculitis and 23 secondary vasculitis cases. No difference in Anti-C1q Ab levels between primary and secondary vasculitis was noted. Four patients were positive for Anti-C1q Ab out of the 64 patients. Of the four, one patient was diagnosed as HUVS, 2 patients as systemic lupus erythermatosus with vasculitis (16.7%) and another patient was diagnosed as rheumatoid arthritis with vasculitis (14.28%). Anti-C1q Ab negatively correlated with age and C3, but it correlated positively with erythrocyte sedimentation rate (ESR) in vascultic patients. Conclusion: Presence of anti-C1q Ab did not differ between the patients with primary and secondary vasculitis. Anti-C1q Ab titers correlated with younger age, high ESR, and low C3 in patients with vasculitis in our study. [ABSTRACT FROM AUTHOR]

Assessment of disease activity in Takayasu arteritis (TA) is challenging. We aimed to study utility of serum amyloid A (SAA) to assess disease activity and its association with SAA gene polymorphisms, if any, in our TA patients. Serum of 99 consecutive adult TA patients and 40 healthy controls were assayed for SAA. Depending on the ITAS2010 and ITAS-CRP score, patients were designated as having active disease if ITAS2010 ≥ 2 or ITAS-CRP ≥ 3 and stable disease if ITAS2010 = 0 or ITAS-CRP is ≤1. Clinical ITAS of 0 with raised inflammatory markers scoring a ITAS-CRP of 2 was considered as indeterminate for disease activity assessment. Repeat SAA levels for active group was measured after 6 months from baseline. SAA levels between active and stable disease as well as serial levels were compared. DNA of 40 patients and controls were genotyped for SAA polymorphisms (rs12218, rs2468844) and the allele frequencies were compared. At baseline, SAA levels were higher in patients as compared to controls (137.4 vs 100.8 ng/ml, p = 0.001) and higher in patients with active disease (166.4 ng/ml) than those with stable disease (98.2 ng/ml), p = 0.001. SAA decreased during follow-up in treatment responders (189.9 ng/ml at baseline vs 119.0 ng/ml at follow-up, p = 0.008); in contrast, there was no significant change among non-responders during follow-up. Allelic frequencies of SAA gene polymorphisms did not differ between cases and controls. SAA may be a reliable biomarker to assess disease activity and treatment response in TA. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]