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Objective: Systemic lupus erythematosus (SLE) has become the most prevalent autoimmune condition requiring admission in the intensive care units (ICU) in the last two decades. Here we analysed the clinical outcomes of SLE patients admitted to our ICU between 2011 and 2021, and studied the prognostic role of high-density lipoprotein (HDL) and procalcitonin in those enrolled after August 2019. Methods: Systemic lupus erythematosus (ACR/SLICC 2012) were enrolled, 72 retrospectively and 30 prospectively. Data on indications for ICU admission, complications, infections, and disease activity were recorded. Outcome was mortality at 90 days (prospective) whereas in the retrospective analysis outcome was hospital discharge or death in hospital. Serum HDL and procalcitonin (PCT) was estimated in the prospectively enrolled 30 patients and compared with 30 non ICU-SLE patients. Results: Indications for ICU admissions were respiratory causes in 78/102 (76.5%) patients; for haemodynamic monitoring and for invasive procedures in the remaining. Pneumonia was the primary reason for mechanical ventilation, followed by diffuse alveolar haemorrhage (DAH). Eighty-three (81.3%) patients died; infections (n = 54) and SLE related causes (n = 29). APACHE-II >16 (p =.026), lymphopenia (p =.021), infection (p =.002), creatinine >1.3 mg/dL (p =.023), and hypotension requiring vasopressor support (p =.006) emerged as significant predictors of non-survival on multivariable analysis. HDL (mg/dL) day 1 was significantly lower in SLE-ICU patients compared to non ICU-SLE (31.8 ± 14.3 vs 38.8 ± 11.4 mg/dl); p =.045. On day 1, PCT (ng/mL) in SLE-ICU was significantly higher when compared to non-ICU SLE; median (IQR): 0.53 (0.26–5.27) versus 0.13 (0.05–0.47), p <.001), respectively. It was also significantly higher on day 5 in SLE-ICU than non-ICU SLE (median (IQR): 4.18 (0.20–14.67) versus 0.10 (0.08–0.46), p =.004. Conclusion: The mortality of SLE patients admitted to the ICU in this study is high, and infections were the principal reason for death. Baseline low HDL and higher procalcitonin are potential biomarkers to identify critically ill SLE patients. [ABSTRACT FROM AUTHOR]

Background: Systemic Lupus Erythematosus (SLE) is an autoimmune disease with multiorgan involvement presenting with a myriad of symptoms, including neuropsychiatric symptoms. Although many studies have evaluated screening questionnaires based psychiatric morbidity, very few studies have used contemporary diagnostic criteria. Objective: This study aimed to evaluate the prevalence of psychiatric disorders in patients with SLE admitted to a tertiary care hospital. Methods: A total of 79 patients diagnosed with SLE for at least for 1 year, who were not in delirium were assessed by a qualified psychiatrist for psychiatric morbidity as per the International Classification of Diseases, 10th Revision (ICD-10) criteria. Additionally, these patients were assessed on Patient Health Questionnaire-9 (PHQ-9) item version, Patient Health Questionnaire-15 (PHQ-15) item version, Generalized Anxiety Disorder-7 item scale and Montreal Cognitive Assessment (MoCA). Results: 51% (n = 40) of the participants were diagnosed with a psychiatric diagnosis, with depressive disorders being the most common, seen in 36.7% (n = 29) of the participants. Additionally, 10% (n = 8) participants were diagnosed with adjustment disorder and 2.5% (n = 2) were diagnosed with anxiety (not otherwise specified). Only one patient was diagnosed with organic psychosis. On PHQ-9, 39.8% (n = 33) were diagnosed with depression. 44.3% (n = 35) expressed death wishes and/or suicidal ideations. On PHQ-15, 17.7% (n = 14) of the participants scored for severe somatic distress (score >15). On GAD-7, 55.7% (n = 44) screened positive for anxiety symptoms, but only 7.6% (n =) had a score of 15 or more to indicate severe anxiety. Nearly half (n = 43; 52%) of the participants also had cognitive impairment as assessed on MoCA, with 13.3% (n = 11) of the participants having scores indicating severe dementia. Conclusions: Patients with SLE have a high prevalence of psychiatric comorbidities and should be routinely screened for psychiatric morbidity. They should be appropriately treated, to improve the overall treatment outcomes. [ABSTRACT FROM AUTHOR]

Introduction: Identifying factors predicting adverse pregnancy outcomes involving systemic lupus erythematosus (SLE) is a research priority. The aims of this study were to investigate (a) the maternal and fetal outcomes of pregnant lupus patients and the factors associated with adverse pregnancy outcomes, and (b) the effect of pregnancy on lupus disease activity of these patients. Methods: This was an ambi-directional study collecting information from five multi-specialist referral centres across the state of Karnataka, India over 5 years (2013–2018). Clinical details of pregnancies and outcomes that were temporally associated with lupus disease were recorded using a structured pro forma. The Safety of Estrogen in SLE National Assessment-SLE Disease Activity Index (SELENA-SLEDAI) was used to assess lupus activity during the 6 months prior to pregnancy and the intra- and post-partum periods. Modifications suggested in the SLE Pregnancy Disease Activity Index were considered while scoring. Results: A total of 121 pregnancies in 80 SLE patients with a mean age of 27.1 (±4.5) years and with a mean disease duration of 4.6 (±4.1) years were reviewed. Largely patients were in clinical remission (109/121; 90.1%). Antiphospholipid antibody positivity was seen in 45/121 (37.2%) patients. A history of lupus nephritis was noted in 29/121 (24%) patients. Maternal complications (32%) were mainly due to hypertensive disorders of pregnancy (HDP; 19/121; 15.7%). Adverse fetal outcomes (58%) were mainly in the form of spontaneous first-trimester abortions (21/121; 16%), stillbirth (14/121; 11.6%) and prematurity (24/121; 20%). HDP is strongly associated with stillbirth and prematurity and is independent of active lupus. Disease activity was associated with a three-fold increased risk of adverse fetal outcome in univariate analysis. The risk of major flare during pregnancy is low (4.1%) when conception occurs during stable disease. Hydroxychloroquine (HCQ) use was associated with reduced risk of flare (p = 0.001) in patients in remission at the time of conception. Conclusions: The risk of major flare during pregnancy is low when conception happens during stable disease. HCQ use was associated with reduced risk of flare in patients in remission at the time of conception. HDP was strongly associated with stillbirth and prematurity and are independent of active lupus in our cohort. [ABSTRACT FROM AUTHOR]

Lupus panniculitis is usually difficult to treat, and the patient is often put on multiple immunosuppressives with variable clinical response and relapses, notwithstanding the long-term side effects. We describe two cases of refractory lupus panniculitis which have been treated successfully with rituximab which is a chimeric anti-CD20 antibody. It reduces the number of circulating mature B-cells, thereby reducing the autoantibodies and the mediators of inflammation. Rituximab is a good alternative to patients who are not responsive to conventional treatment options for lupus panniculitis. There have been few side effects reported by the patients, but the clinical response and psychological well-being clearly outweigh them. [ABSTRACT FROM AUTHOR]

Prospective data on infections in systemic lupus erythematosus (SLE) from India are scarce. We studied the frequency and predictors of infections in hospitalized SLE patients. All data on infections were prospectively recorded. During the study period, 212 SLE patients (91% women) were hospitalised. Sixty-three (29.7%) had infections. The most common infections were pneumonia, skin and soft-tissue infections and urinary-tract infections. Mortality was higher in the infection group compared to the no infection group (11.1% vs. 0.7%; p =0.01). At three months, 10/63 developed another episode of infection. On logistic regression, the predictors of infection were fever [odds ratio (OR) = 4.17], vasculitis (OR = 2.64), thrombocytopaenia (OR = 3.59), presence of co-morbidities (OR = 3.59) and duration of hospital stay >11 days (OR = 3.55); p<0.001 for all. High-sensitivity CRP (hsCRP) and procalcitonin were measured in 95 patients. hsCRP was significantly higher in the infection group compared to the no infection group (median 27 vs. 6.5 mg/L; p <0.001). Procalcitonin was similar in both groups. A hsCRP of 11.5 mg/L had a sensitivity of 66.7% and specificity of 72.9% to diagnose infection. To conclude, 29.7% of Indian SLE patients who are hospitalized have infections, resulting in a significantly increased duration of hospital stay. The presence of fever and co-morbidities are predictors of infection. The role of high hsCRP to support the diagnosis of infection in SLE is once again confirmed. [ABSTRACT FROM AUTHOR]

Introduction: Systemic Lupus Erythematosus (SLE) is an auto immune disorder affecting mainly adolescent females and young women of reproductive age. The disease is characterised by widespread inflammation of blood vessels and connective tissues due to the presence of anti-nuclear antibodies (ANA). There are limited number of studies from South India on paediatric lupus. Our objectives were to study the clinical and immunological features of childhood SLE along with treatment modalities and its outcome at the end of one year follow up. The correlation between various auto-antibodies and systemic involvement was also assessed. Methods: This was a retrospective observational study carried out in paediatric unit at a tertiary care centre in South India. Data was obtained through patient's medical records. From April 2003 to April 2019, 32 children were diagnosed to have SLE as per the American college of Rheumatology 1997 criteria. Results: The study population included 32 children fulfilling the criteria. Female to male ratio was 4.3:1. The mean age at diagnosis was 11.52 years. The most common clinical manifestations were renal (87.5%) followed by haematological (81.3%), musculoskeletal (59.4%), mucocutaneous (53.1%) and nervous system (31.3%) involvement. All patients were positive for anti-nuclear antibodies. Anti-double stranded DNA (78.1%) was the most common auto-antibody profile followed by anti-ribosomal p protein (37.5%) and anti-nucleosome antibody (37.5%). During the follow up, 13 (40.6%) children attained complete remission, 10 (31.2%) went into partial remission and nine (28.1%) had persisting active disease. Conclusion: The clinical spectrum and outcome of paediatric SLE depends upon the age of presentation and number of organ systems involved at the time of diagnosis. Our study throws light on various aspects of SLE in children from developing countries like India. [ABSTRACT FROM AUTHOR]

We report the clinicopathological features, treatment and outcome of 54 Indian children (14 boys) with biopsy-proven lupus nephritis followed over a 10-year period. The mean age (SD) at onset of disease was 9.6 ± 2.6 (range 2.5-14.4) years. Twenty-six (48.1%) patients had class IV nephritis, 7 (13.0%) had class V, whereas class I, II and III nephritis were present in 3 (5.6%), 10 (18.5%) and 6 (11.1%) patients, respectively. Hypertension, haematuria and nephrotic range proteinuria were present in 30 (55.6%), 31 (57.4%) and 28 (51.8%) patients, respectively. Compared with all the other classes combined, there were more boys among patients with class IV nephritis, and hypertension, haematuria, nephrotic syndrome and decreased glomerular filtration rate at presentation were more common. The mean duration of follow-up was 3.1 ± 2.9 years (median 2.5, range 0.2-10.3 years). Of the 39 patients who were followed-up for at least 1 year, 33 (84.6%) were in complete or partial remission, whereas six (15.4%) had no response to therapy. The incidence of serious infection was 1.5 episodes per 10 patient-years. Nine patients died, of whom four had serious infections or septicaemia, and three developed end-stage renal failure (ESRF). The patient survival rate at 3 years and at last follow-up visit was 88% and 83.3%, respectively, whereas the renal survival rates (without ESRF) were 92% and 94.4% respectively. Cox regression analysis showed no relation of gender, age of onset, presence of hypertension, haematuria and proteinuria, estimated glomerular filtration rate, renal histology and response to therapy to the outcome of death or ESRF. We found lower patient survival rate as compared with data from the developed countries but similar to that seen in developing countries. Serious infections were an important cause of mortality besides renal failure. [ABSTRACT FROM AUTHOR]

SLE has a range of fluctuating symptoms affecting individuals and their ability to work. Although South Asian (SA) patients are at increased risk of developing SLE there is limited knowledge of the impact on employment for these patients in the UK. Understanding ethnicity and disease-specific issues are important to ensure patients are adequately supported at work. Semi-structured interviews were conducted with patients of SA origin to explore how SLE impacted on their employment. Thematic analysis was used to analyse the data which are reported following COREQ guidelines. Ten patients (8 female; 2 male) were recruited from three rheumatology centres in the UK and interviewed between November 2019 and March 2020. Patients were from Indian (n = 8) or Pakistani (n = 2) origin and worked in a range of employment sectors. Four themes emerged from the data: (1) Disease related factors; (2) Employment related factors; (3) Cultural and interpersonal factors impacting on work ability; (4) Recommendations for improvement. Patients' ability to work was affected by variable work-related support from their hospital clinicians, low awareness of SLE and variable support from their employers, and cultural barriers in their communities that could affect levels of family support received. These findings highlight the need for additional support for SA patients with SLE in the workplace.

Systemic lupus erythematosus (SLE) and other autoimmune rheumatic diseases (AIRD) tend to co-aggregate in families, making positive familial history a risk factor. We aimed to estimate familial aggregation of AIRD in SLE patients and to compare between ones having a positive and negative family history of autoimmunity in our cohort. We included families of 157 consecutive SLE patients in a hospital-based, cross-sectional design for a three-generation pedigree study. Clinical and laboratory parameters of these patients were recorded. AIRD was seen in families of 39 SLE patients amounting to a familial prevalence of 24.8% [95% confidence interval (CI) 18.1, 31.6] with a relative risk (λ) of 4.3 for first-degree relatives (FDRs) and 1.1 for second-degree relatives (SDRs). SLE was the commonest AIRD seen in families of 19 patients with a familial prevalence of 12.1% (95% CI 7.0, 17.2) and λ of 78.2 for FDRs and 18.1 for SDRs. AIRD as a whole and SLE alone were seen more commonly with parental consanguinity (p < 0.05). Familial aggregation in SLE patients also showed a relatively higher percentage of affected males and lesser presentation with constitutional features (p < 0.05) than sporadic SLE patients. Rheumatoid arthritis (RA) was the second most common AIRD seen in 16/39 (41%) families with a RR of 3.1 in FDRs of SLE patients. In conclusion, Asian Indian SLE patients seem to have a high familial aggregation of AIRD, which is more pronounced in the background of parental consanguinity. SLE is the commonest AIRD seen amongst FDRs and SDRs of SLE patients, followed by RA, with FDRs being at highest risk.