BackgroundThe ANCA-associated vasculitides (AAV) are systemic autoimmune diseases affecting small and medium-sized blood vessels. Upper airways, lungs, and kidneys are variably involved in the different types of AAV, and the consequences of a missed or delayed diagnosis of renal vasculitis are potentially life-threatening. AAV may be classified into clinical syndromes or based on their serology (Anti-PR3 or Anti-MPO). Patient survival and the risk of end-stage renal disease are closely associated with renal functional status.ObjectivesThis study was undertaken to study the disease characteristics and outcomes in patients of AAV based on the presence or absence of renal involvement.MethodsThis was a longitudinal, observational study conducted at a tertiary care hospital in Northern India. Between February 2020 and December 2021, all consecutive adult patients diagnosed with AAV based on their autoantibody profiles (using indirect immunofluorescence and Line ImmunoAssay) and clinical features were included in this study after taking their informed consent. Demographic details, clinical features, laboratory parameters, disease activity, and mortality or morbidity outcomes of patients were analysed prospectively. All outcomes were compared between patients with and without renal involvement.ResultsA total of 112 patients were included in our study, with a median age of 51.5 years. 74 (66%) patients had renal involvement either in the form of Nephritic syndrome, Nephrotic syndrome, RPRF (Rapidly progressive renal failure), Nephritic nephrotic syndrome, or Asymptomatic Urinary Sediments. c-ANCA and PR3 positivity were seen in more than two-thirds of our population, without any significant correlation with organ involvement. Patients with renal disease had a significantly higher proportion with diffuse alveolar haemorrhage (32.4% vs 10.5%, p=0.05) and palpable purpura (19.6% vs 7.9%, p-0.025), but significantly lower occurrences of nasal pathology (14% vs 42%, p=0.001) and subglottic stenosis (1.4% vs 18.4%, p=0.001). Mean BVAS at enrolment was significantly higher in the renal group (20.9 vs 12.89). Remission was achieved in 50% and 47.4% of the patients with and without renal involvement respectively. Rates of relapse (19/74 vs 14/38), refractory disease, and mortality were not significantly different among the two subgroups. The commonest organ involvement in disease flare was pulmonary involvement. 21.6% of the patients developed CKD over a median follow-up period of 18 months.ConclusionKidney involvement is one of the commonest manifestations of AAV. Patients with renal involvement may have higher mean BVAS scores and an increased risk of developing alveolar haemorrhage and purpuric skin rash; while nasal pathology and subglottic stenosis occurred more frequently in patients without renal disease. Rates of remission, refractory disease, and mortality were almost similar, regardless of renal involvement, while relapses were numerically more in the non-renal AAV patients.References[1]Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, Kallenberg CG, St. Clair EW, Turkiewicz A, Tchao NK, Webber L. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. New England Journal of Medicine. 2010 Jul 15;363(3):221-32.[2]Córdova-Sánchez BM, Mejía-Vilet JM, Morales-Buenrostro LE, Loyola-Rodríguez G, Uribe-Uribe NO, Correa-Rotter R. Clinical presentation and outcome prediction of clinical, serological, and histopathological classification schemes in ANCA-associated vasculitis with renal involvement. Clinical rheumatology. 2016 Jul;35(7):1805-16.[3]Kronbichler A, Shin JI, Lee KH, Nakagomi D, Quintana LF, Busch M, Craven A, Luqmani RA, Merkel PA, Mayer G, Jayne DR. Clinical associations of renal involvement in ANCA-associated vasculitis. Autoimmunity Reviews. 2020 Apr 1;19(4):102495.Acknowledgements:NIL.Disclosure of InterestsNone Declared.

Background/Aims Psoriatic Arthritis (PsA) is a type of chronic inflammatory arthritis with diverse extra-articular manifestations and complications, including an increased burden of cardiovascular (CV) disease. Traditional CV risk factors are more common in PsA patients, leading to not only overt but also subclinical CV disease. Endothelial dysfunction and altered handling of low-density lipoproteins (LDL) lead to atherogenesis. Oxidized LDL (Ox-LDL) and antibodies against it (Anti Ox-LDL) may reflect the in vivo inflammatory burden in systemic vasculature in PsA. This study aimed to assess subclinical atherosclerosis in PsA patients and compare them with healthy controls, by assessing carotid intimal medial thickness (CIMT) and laboratory surrogates of dyslipidemia. Methods This was a cross-sectional, case-control study. Between January 2019 and May 2020, adult patients classified as PsA as per the CASPAR criteria, with symptom duration of at least 2 years, were included in this study. An equal number of age- and gender-matched controls were enrolled. We excluded patients on lipid-lowering drugs for more than the past 3 months. Demographic details, clinical and laboratory parameters (including Ox-LDL and Anti Ox-LDL antibodies), and bilateral CIMT assessment using B-mode Doppler Ultrasonography by a single, blinded cardiologist, were assessed for each participant. The study was approved by the Institutional Ethics Committee. Results Fifty PsA patients consented to the study, with a median age of 43.92 ± 10.79 years. Fifty healthy controls were also enrolled. 57% of the cases were male, with plaque psoriasis in 94%, and polyarthritis in 54%. Compared to the controls, PsA patients had significantly higher mean body mass index (BMI, 26.5 vs 24.6, p = 0.004), mean VLDL (25.6 mg/dl vs 20.5 mg/dl, p = 0.01), mean bilateral CIMT (0.62 mm vs 0.46 mm, p < 0.001), and mean Anti Ox-LDL antibody levels (29 U/ml vs 22.4 U/ml, p < 0.001). There was a significantly greater proportion of PsA patients with total cholesterol to HDL ratio >3.5 (86% vs 68%, p = 0.03), obesity (18% vs 6%, p = 0.01), and Anti Ox-LDL >30 U/ml (32% vs 6%, p = 0.02). CIMT and Anti Ox-LDL levels did not show any significant correlation. Univariate and multivariate analyses revealed that older patient age, total cholesterol level, and presence of PsA were risk factors for increased mean CIMT as a proxy for subclinical atherosclerosis. Conclusion This study used a novel biomarker, the Anti-Ox-LDL antibody, as a proposed surrogate marker for atherosclerotic risk stratification in patients with PsA. To our knowledge, this is the first such study in PsA. We found an increased prevalence of subclinical atherosclerosis in PsA patients compared to age- and gender-matched healthy controls. Age, total cholesterol levels, and the presence of PsA were independent predictors of atherosclerotic risk. Anti-Ox-LDL antibodies are significantly elevated in PsA patients compared to controls, but they may not correlate well with CIMT values. Disclosure D. Yadavalli: None. S. Ghosh: None. S. Dhuria: None. K. Telang: None. L. Sharma: None. R. Bajaj: None. V. Singal: None. R. Gupta: None.

Background/Aims Studies in autoimmune inflammatory myositis (AIM) have shown a strong role of autoantibodies in the diagnosis and prognosis of patients with myositis. This ongoing study shares the preliminary data of 139 patients with AIM from a North Indian tertiary care centre. This prospective, observational study was conducted at a 1250-bedded hospital to assess the prevalence of myositis-specific antibodies (MSA) and myositis-associated antibodies (MAA) in patients from the Indian subcontinent with AIM and the correlation of these antibodies with clinical features. Methods From November 2016 to September 2022, patients with AIM (satisfying the Bohan and Peter criteria of 1975) attending the Rheumatology and Clinical Immunology Department of Medanta Hospital were included after taking their informed consent, and divided into subgroups as dermatomyositis (DM), polymyositis (PM), CTD-associated myositis (CTD-M), cancer-associated myositis (CAM), and juvenile myositis (JM). The Institutional Ethics Committee approved the study. Clinical data and sera from patients were collected and analysed. Results The study included 139 individuals with a mean age of 45.4 years. Fifty-two had DM, 49 had PM, 25 had CTD-M, 6 had CAM, and 7 had JM. ANA positivity was found in 67.70%, MSA in 22.3%, MAA in 25.4%, and both in 12.3%. Antibodies against Mi-2 were identified in 13 (28.9%) patients, Jo-1 in 14 (32.50%), PL-7 in 3 (6.7%), PL-12 in 4 (8.9%), SRP in 9 (20%), and MDA-5 and NXP2 in 1 (2.2%) patient each. Antibodies against Ro were found in 28 (57.1%) of the patients, RNP in 8 (16.3%), and PM-Scl in 9 (18.4%). Mi-2 antibodies were mostly identified in DM patients and were significantly associated with skin rash. SRP positivity was mostly found mainly in PM. Raynaud's and ILD were linked to the presence of Jo-1 and Ro antibodies. Malignancy screening was negative in NXP2 and TIF1γ antibody-positive patients. Conclusion MSA was found in 22.3% of cases and MAA in 25.4%. Mi-2 antibodies were linked to rash, but none had ILD, whereas Jo-1 antibodies were linked to mechanic hands, arthritis, and ILD. We aim to gather more strong results in the future as additional patients are recruited in this continuing trial. Disclosure S. Dhuria: None. P. Khatri: None. N. Negalur: None. K. Telang: None. S. Ghosh: None. D. Yadavalli: None. V. Singal: None. R. Gupta: None.

Objectives This cross-sectional study was designed to assess the clinical profile and frequency of associated autoantibodies in all consecutive patients classified as systemic sclerosis (SSc) at Medanta—the Medicity Hospital, Gurgaon, India. Methods Between August 2017 and July 2019, we identified a total of 119 consecutive patients meeting the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) 2013 criteria for SSc and 106 patients consented to this study. Their clinical and serological data at the time of enrolment were analysed. Results Our cohort had a mean age at symptom onset of 40 ± 13 years with a median symptom duration of 6 years. We had 76 patients (71.7%) with interstitial lung disease (ILD), which was a higher proportion compared to European cohorts. 62 patients (58.5%) had diffuse cutaneous involvement which was significantly associated with anti-Scl70 antibodies (p < 0.001), digital ulcers (p = 0.039) and the presence of ILD (p = 0.004). 65 patients (61.3%) had anti-Scl70 and 15 patients (14.2%) had anti-centromere (anti-CENP) antibodies. Scl70 positivity was associated with the presence of ILD (p < 0.001) and digital ulcers (p = 0.01). Centromere antibodies had a negative association with ILD (p < 0.001), but was a risk factor for calcinosis (p < 0.001) and pulmonary arterial hypertension (PAH) (p = 0.01). The combination of diffuse cutaneous disease and Scl70 antibodies was the strongest predictor of ILD and digital ulcers (p = 0.015). sm/RMP, RNP68 and Ku antibodies correlated with musculoskeletal involvement (p < 0.01), while all seven of the patients with Pm/Scl antibodies had ILD. Renal involvement was noted in only two patients. Limitations A single-centre study may not capture the true prevalence of disease characteristics in the population. Referral bias for patients with diffuse cutaneous disease has been noted. Data on RNA-Polymerase antibodies have not been provided. Conclusion North Indian patients have some characteristic differences in disease phenotype as compared to their Caucasian counterparts with a larger proportion of patients presenting with ILD and Scl70 antibodies. Antibodies against Ku, RNP and Pm/Scl occur in a minority of patients, but may be associated with musculoskeletal features.

Background/Aims Systemic sclerosis (SSc) is a systemic autoimmune disease in which osteoporosis has been reported to be more prevalent than in healthy individuals. Bone loss is hypothesised to be related to vasculopathy in SSc, but the causative pathways are not very clear. This study was conducted to investigate the prevalence of osteoporosis in Indian patients with SSc and to assess its association with various disease-related characteristics. Methods This was a cross-sectional, case-control study performed in a 1,250 bedded tertiary care hospital. Between August 2017 and June 2019, all consecutive patients attending the outpatient and in-patient departments of the Department of Rheumatology & Clinical Immunology at our institute, classified as SSc as per the ACR/EULAR 2013 criteria, were included after taking their informed consent. Age and gender-matched healthy controls were included and all enrolled subjects were subjected to a dual energy X-ray absorptiometry (DEXA) scan. Demographic and clinical details of all patients were recorded. Osteoporosis was defined as per the WHO definition of a T score ≤ -2.5. The study was approved by the Institutional Review Board and the Institutional Ethics Committee. Results 90 patients with SSc and 45 age and gender-matched healthy controls were included in the study. Confounding factors for bone health, like smoking or alcohol intake, the proportion of post-menopausal women, and past or family history of fragility fractures, were matched between the two groups. The prevalence of osteoporosis among SSc cases was significantly higher than in the healthy controls (34.4% vs. 6.6%, p &lt; 0.001). In univariate analysis, age, low body mass index (BMI), post-menopausal status, diffuse cutaneous phenotype, gastrointestinal involvement, digital ulcers, and pulmonary arterial hypertension were found to be significantly associated with osteoporosis. Multivariate analysis revealed that the presence of osteoporosis was significantly associated with lower BMI, post-menopausal status, and the presence of digital ulcers in patients. Interestingly, glucocorticoid use did not appear to increase the risk of osteoporosis in our cohort. Conclusion Patients with systemic sclerosis and digital ulcers, low BMI, or post-menopausal status are at high risk of developing osteoporosis and therefore should be screened for osteoporosis by DEXA scan to prevent unforeseen fracture-related morbidity and mortality. Glucocorticoids may not be the major culprit as evidenced by this study, while control of disease activity may lead to better bone health status. Disclosure D. Tanna: None. S. Ghosh: None. K. Telang: None. G. Ekbote: None. V. Singal: None. R. Gupta: None.