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Primary objectives estimated prevalence of traditional cardiovascular disease (CVD) risk factors and compared different CVD risk prediction algorithms in an Indian rheumatoid arthritis (RA) population. Secondary objectives evaluated associations between carotid intima-media thickness (CIMT) and subclinical atherosclerosis (SCA) with CVD risk factors and CVD risk scores.The presence of CVD risk factors were recorded, and 10-year CVD risk was predicted using Framingham risk scoring (FRS) using lipids (FRS-Lipids), FRS using body mass index (FRS-BMI), QRISK-2, SCORE, and the algorithm recommended by ACC/AHA (ASCVD). CIMT was measured on the far-wall of the common carotid artery. Subclinical atherosclerosis was defined as CIMT 0.9 mm or the presence of carotid plaque.A total of 332 patents were enrolled, 12% had diabetes mellitus, 21.4% hypertension, and 6.9% were current/past smokers. Proportions of RA with predicted 10-year CVD risk 10% varied from 16.2 to 41.9% between scores. Highest magnitude of risk was predicted by FRS-BMI. Agreement between scores in predicting risk was moderate in general. Mean CIMT was 0.70 ± 0.15 mm. Age, male sex, and extra-articular manifestations associated with greater CIMT. All risk scores except SCORE moderately correlated with CIMT. About one-seventh had SCA defined as CIMT 0.9 mm or the presence of carotid plaques, associated with increasing age, male gender, or higher ratio of total cholesterol to high-density lipoprotein cholesterol. ASCVD and QRISK-2 scores had maximum area under curve for distinguishing SCA.Individual CVD risk scores predict 10-year CVD risk differently in Indian patients with RA, and require validation for predicting hard end points (CVD events, mortality). Key Points • Diabetes mellitus and hypertension are the most prevalent cardiovascular disease risk factors in Indian patients with RA. • Individual cardiovascular risk prediction scores predict risk differently in Indian patients with RA, highest risk being predicted by the FRS-BMI. • Carotid intima-media thickness in RA associated with increasing age, male sex and extra-articular manifestations. • 14% RA had subclinical atherosclerosis, associated with increasing age, male sex, and higher total cholesterol to HDL-C ratio, best distinguished by ASCVD and QRISK-2 scores.

BackgroundSystemic lupus erythematosus (SLE) is an autoimmune disorder affecting various organ systems with unknown etiology. Interleukin-6 (IL-6) and interferon-alpha (IFN-α) have been shown to have a major role in disease pathogenesis, and they correlate with SLE disease activity, but reports in the literature are conflicting. The present study aims to investigate the significance of IL-6 and IFN-α levels in SLE pathogenesis in an eastern Indian cohort.Material and Methods70 SLE patients fulfilled SLICC 2012 criteria, and 40 age- and gender-matched healthy controls (HC) were enrolled. Baseline characteristics along with disease activity were recorded for all patients. Levels of IL-6 and IFN-α were measured by using ELISA. For the meta-analysis, published articles were searched through different databases. Two independent researchers extracted data, and the meta-analysis was performed with CMA v3.1.ResultsThe plasma levels of IL-6 and IFN-α in SLE patients were significantly elevated compared to HC (IL-6: p < .0001, IFN-α: p = 0.01). SLEDAI score correlated positively with plasma IL-6 ( p < .0001, r = 0.46) and IFN-α levels ( p < .0001; r = 0.47). Meta-analysis of previous reports, including our case–control data, revealed higher IL-6 ( p < .0001) and IFN-α ( p = .005) in SLE patients compared to HC. Furthermore, IL-6 ( p < .0001, r = 0.526) and IFN-α ( p < .0001; r = 0.371) levels positively correlated with the disease activity.ConclusionIL-6 and IFN-α levels are elevated in SLE and they correlate with disease activity. Further studies with a larger sample size in different populations are required to validate our findings.

There is paucity of data on tuberculosis in Indian patients with systemic lupus erythematosus (SLE). We retrospectively studied clinical features and outcome of tuberculosis in SLE. Medical records of patients who developed tuberculosis simultaneous or after the diagnosis of SLE were retrospectively reviewed. All patients fulfilled 1997 ACR and/or SLICC 2012 classification criteria for SLE. A diagnosis of tuberculosis required bacteriological, histopathological or CT/MRI suggestive of tuberculosis and initiation of four drug antituberculous therapy. Baseline parameters were compared with the rest of cohort to identify predictors of tuberculosis. In our cohort of 1335 SLE patients, 48 (3.6%) developed tuberculosis. Incidence of tuberculosis was calculated to be 733 per 100,000 patient years and occurred after a mean disease duration of 3.0 ± 4.1 years. Extrapulmonary tuberculosis (n = 37) was commoner than pulmonary tuberculosis (n =11). Most common radiological pattern in pulmonary tuberculosis was miliary and musculoskeletal TB was most common extrapulmonary TB. A microbiological diagnosis was obtained in 52.1% patients. Male gender was associated with higher risk of tuberculosis [OR 3.30 (1.55-7.05)]. Mortality was 14.5% and all patients who died had either disseminated (n = 5) or central nervous system (CNS) tuberculosis (n = 2). Incidence of tuberculosis in SLE is higher than general population and is associated with different phenotype and higher mortality. Male gender was associated with increased risk of tuberculosis in SLE.

Syringomyelia is an important etiology of Charcot arthropathy of the elbow. We present five interesting patients, along with a systematic literature review summarizing the clinical profile and management of syringomyelia-induced Charcot arthropathy of the elbow. PUBMED, SCOPUS, EMBASE, and Science Direct databases were screened for English articles published between 1980 and 2022 using the search query: "Syringomyelia" AND "elbow" AND ("arthropathy" OR "neuropathic" OR "Charcot"). Articles without full text and/or lack of conclusive evidence of elbow arthropathy due to syringomyelia were excluded. The reference lists of the selected articles were reviewed to identify additional articles describing syringomyelia-induced Charcot arthropathy of the elbow. All five patients in the current series had elbow arthritis with variable motor weakness and dissociated sensory loss. The literature review included 31 reports (45 patients) and five patients from our center (n = 50). The median age at presentation was 45 (13-77) years. The median duration of arthropathy was 24 (0.5-180) months. Thirty-three patients had isolated elbow arthropathies. The other joints affected included the shoulder (n = 13), wrist (n = 7), metacarpophalangeal joints (n = 3), and interphalangeal joints (n = 1). Chiari malformations were present in 33 (66%) patients. Sensory deficits, motor deficits, and ulnar neuropathies were described in 36 (72%), 31 (62%), and 14 (28%) patients, respectively. Surgical decompression for syringomyelia was performed in 13 (26%) patients. The presence of dissociated sensory loss, with or without motor weakness, is key to the suspicion of syringomyelia-induced Charcot arthropathy of elbow. Chiari malformation and ulnar neuropathy are frequently associated with this condition. Key Points • Charcot arthropathy of elbow is not so uncommon as believed • Syringomyelia is an important etiology of Charcot arthropathy of elbow • Therefore, all patients with elbow arthropathy of unknown etiology must be evaluated for dissociative sensory loss • Chiari malformation and ulnar neuropathy are commonly associated with syringomyelia-induced Charcot arthropathy of elbow joint.

Background Mortality in SLE has a bimodal peak with early deaths mainly related to disease activity and infection. Although mortality has reduced over years, it is still two to three folds compared to the general population. In India due to increased burden of infection and limited access to health care, the causes may be different. Methods Retrospective, review of records of all adult SLE patients fulfilling ACR 1997 criteria, who died in hospital between 2000-2019 at a teaching hospital in India was done. In addition, baseline clinical features were extracted for all adult SLE patients seen during this period. Infections were either bacteriologically proven or based on clinicradiological or serologic evidence. Active disease was defined as SLEDAI 2k ≥ 5. Logistic regression was performed to ascertain risk factors for mortality. Results A total of 1337 (92% females) patient records were reviewed .The mean age at presentation was 29.9 ± 9 years.60–75% of patients had fever, mucocutaneous disease and arthritis, while nephritis, hematologic, serositis and neurologic involvement was seen in 48.6%, 43.2%, 16% and 10.3% respectively as presenting mainfestations. There were 80 in hospital deaths .Infection was the most common cause of death, with 37 due to infection alone and in 24 disease activity also contributed. Only 18 deaths were due to active disease. Among bacterial infections lung was the most common site and gram negative organism were the most common pathogens. There were 10 deaths due to Tuberculosis(TB) and half of them had disseminated disease. Patients with disease activity had a SLEDAI of 14.8 ± 6.4, with neurological, renal and cardiovascular involvement being the major contributors to mortality in 11, 7 and 6 cases respectively. Higher age at onset, male gender, fever, myositis, neurological, cardiovascular, gastrointestinal involvement, vasculitis, elevated serum creatinine at baseline were independent predictors of death. Conclusion Infections are the most common cause of in-hospital mortality in SLE and TB still accounts for 15% of deaths related to infection. Vasculitis, myositis, cardiovascular and gastrointestinal involvement emerged as novel predictors of mortality in our cohort.

Background/Aims Infections are a major cause of morbidity and mortality in juvenile systemic lupus erythematosus (SLE). We assessed the incidence and risk factors for major infections in juvenile SLE. Methods We carried out a retrospective review of 175 patients of juvenile SLE (ACR 1997 criteria) with age 1 week, resulting in disability or death) were recorded. Predictors of infection and their outcomes were determined using multivariate logistic regression and Kaplan Meier survival analysis. Results Among 175 patients (154 were girls) with a mean age of 14.79±3.1 years, there were a total of 52 major infections in 43 patients with an incidence rate of 8.83 per 100 person-years. The respiratory tract (19) was the most commonly involved site followed by skin and soft tissue (10), gastrointestinal tract (6), systemic bacteremia (5), ear nose and throat (4), disseminated infection (3), urinary tract (2), central nervous system (1), joint (1) and dengue shock syndrome (1). Organisms isolated were Mycobacterium tuberculosis (11) which was the most common and predominantly extrapulmonary, followed by enterobacteria (12), Staphylococcus aureus (5), cytomegalovirus (3), Herpes zoster (2), Herpes simplex (1), MRSA (1), and Candida (1). Gastrointestinal involvement (OR 4.21), major organ involvement (OR 2.5), use of cyclophosphamide (OR 7.63), higher baseline SLEDAI (16[11.5-21] vs 12[8.75-16.25]) and a higher daily dose of prednisolone (16.1±15.1mg vs 6.1±5.8mg) at the time of the infection were predictors of a major infection. On multivariate analysis, only higher daily doses of corticosteroids (OR1.09, 95% CI 1.05-1.14) and gastrointestinal involvement (OR 4.98, 1.05-23.65) were significant predictors. Major infection-free survival at 1 year and 5 years was 82.5% (95% CI 76.8-88.7) and 72.5% (95% CI 65-80.9) respectively. There were eight deaths with five directly attributable to infection. Overall survival was worse in those who had an episode of major infection. Conclusion The risk of major infections in juvenile SLE is significant and associated with higher daily corticosteroid use and gastrointestinal involvement at baseline. Disclosure R. Chatterjee: None. S. Pattanaik: None. L. Gupta: None. D.P. Misra: None. V. Agarwal: None. A. Lawrence: None. A. Aggarwal: None.

There is paucity of data on tuberculosis in Indian patients with systemic lupus erythematosus (SLE). We retrospectively studied clinical features and outcome of tuberculosis in SLE.Medical records of patients who developed tuberculosis simultaneous or after the diagnosis of SLE were retrospectively reviewed. All patients fulfilled 1997 ACR and/or SLICC 2012 classification criteria for SLE. A diagnosis of tuberculosis required bacteriological, histopathological or CT/MRI suggestive of tuberculosis and initiation of four drug antituberculous therapy. Baseline parameters were compared with the rest of cohort to identify predictors of tuberculosis.In our cohort of 1335 SLE patients, 48 (3.6%) developed tuberculosis. Incidence of tuberculosis was calculated to be 733 per 100,000 patient years and occurred after a mean disease duration of 3.0 ± 4.1 years. Extrapulmonary tuberculosis (n = 37) was commoner than pulmonary tuberculosis (n =11). Most common radiological pattern in pulmonary tuberculosis was miliary and musculoskeletal TB was most common extrapulmonary TB. A microbiological diagnosis was obtained in 52.1% patients. Male gender was associated with higher risk of tuberculosis [OR 3.30 (1.55-7.05)]. Mortality was 14.5% and all patients who died had either disseminated (n = 5) or central nervous system (CNS) tuberculosis (n = 2).Incidence of tuberculosis in SLE is higher than general population and is associated with different phenotype and higher mortality. Male gender was associated with increased risk of tuberculosis in SLE.

Interstitial lung disease (ILD) is a leading cause of mortality in systemic sclerosis (SSc). However, mortality is improving as pathogenesis is being better understood and new therapies emerge. The roles of the inflammasome and NETosis in fibrosis are being elucidated. Epigenetic targets like DNA methylation and microRNA show promise as new targets for anti-fibrotic agents. The IL17-23 pathway has been shown to be active in SSc-ILD. Newer biomarkers are being described like CCL18 and the anti-eIF2B antibody. Hypothesis-free approaches are identifying newer genes like the ALOX5AP and XRCC4 genes. Computer-aided interpretations of CT scans, screening with ultrasonography and magnetic resonance imaging (MRI) are gradually emerging into practice. Imaging can also predict prognosis. A plethora of studies has shown the benefit of immunosuppression in halting ILD progression. Extent of lung involvement and PFT parameters are used to initiate therapy. The best evidence is for cyclophosphamide and mycophenolate. Besides these, corticosteroids and rituximab are being used in cases refractory to the first line drugs. Stem cell transplant is also backed by evidence in SSc. Longer studies on maintenance therapy are awaited. The inflammation in SSc is mostly subclinical and there is great interest in developing anti-fibrotic drugs for SSc-ILD. Perfinidone and nintedanib are under trial. The last resort is lung transplantation.