SLE is associated with significant mortality, and data from South Asia is limited. Thus, we analysed the causes and predictors of mortality and hierarchical cluster-based survival in the Indian SLE ...Inception cohort for Research (INSPIRE).
Data for patients with SLE was extracted from the INSPIRE database. Univariate analyses of associations between mortality and a number of disease variables were conducted. Agglomerative unsupervised hierarchical cluster analysis was undertaken using 25 variables defining the SLE phenotype. Survival rates across clusters were assessed using non-adjusted and adjusted Cox proportional-hazards models.
Among 2072 patients (with a median follow-up of 18 months), there were 170 deaths (49.2 deaths per 1000 patient-years) of which cause could be determined in 155 patients. 47.1% occurred in the first 6 months. Most of the mortality (n = 87) were due to SLE disease activity followed by coexisting disease activity and infection (n = 24), infections (n = 23), and 21 to other causes. Among the deaths in which infection played a role, 24 had pneumonia. Clustering identified four clusters, and the mean survival estimates were 39.26, 39.78, 37.69 and 35.86 months in clusters 1, 2, 3 and 4, respectively (P < 0.001). The adjusted hazard ratios (HRs) (95% CI) were significant for cluster 4 2.19 (1.44, 3.31), low socio-economic-status 1.69 (1.22, 2.35), number of BILAG-A 1.5 (1.29, 1.73) and BILAG-B 1.15 (1.01, 1.3), and need for haemodialysis 4.63 (1.87,11.48).
SLE in India has high early mortality, and the majority of deaths occur outside the health-care setting. Clustering using the clinically relevant variables at baseline may help identify individuals at high risk of mortality in SLE, even after adjusting for high disease activity.
Objectives
Determine domain-based-outcomes and steroid-sparing efficacy of generic tofacitinib in IIM.
Methods
This is a multicenter retrospective study wherein clinical phenotype, autoantibody ...profile, prior immunosuppressives, and outcomes at 3, 6, and 12 months were retrieved for IIM patients prescribed tofacitinib. Overall clinical response was assessed as complete or partial remission as per physician judgment. Changes in cutaneous and calcinosis domain were recorded as per physician global assessment (PGA), lung domain as per medical research council (MRC) dyspnea scale, and muscle strength by Manual Muscle Testing-8 (MMT-8).
Results
Forty-two patients of IIM with mean age 38.7 ± 16 years; (76.2% (
N
= 32) women), median duration of illness 48 (19;88) months were included. Commonest indication for initiating tofacitinib was either for refractory or as steroid sparing for cutaneous domain (
N
= 25/42, 59.5%) followed by calcinosis (
N
= 16/42, 38%). Overall complete and/or partial remission was achieved in 23/37 (64.8%), 30/35 (85.7%), and 29/30 (96.6%) patients at 3, 6, and 12 months, respectively. At 12-month follow-up, there was a reduction in prednisolone dose, with absolute decrease from a daily dose of 17.5 mg (IQR 5;50) to 2.5 mg (IQR 0;5) (
p
< 0.001). Individual domain assessments revealed improvement in cutaneous domain 16/25 (64%) and calcinosis 6/15 (40%). Adverse effects included herpes zoster (
N
= 2/42, 4.8%) and dyslipidemia (
N
= 4/42, 9.5%).
Conclusions
Treatment with generic tofacitinib significantly reduces the daily dose of corticosteroids and is effective in cutaneous domain including calcinosis in IIM.
Key points
• This multicenter retrospective study is the first real-world data from India, elucidating steroid sparing efficacy of generic tofacitinib in patients with inflammatory myositis.
• Domain-based outcome assessment suggests good clinical improvement especially in cutaneous domain, even those with refractory disease.
• Modest benefits were evident in calcinosis, but its effect on the muscle and pulmonary domain appears limited.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
To study the clinical, pathological and prognostic profile of patients with temporal arteritis in India.
The study was conducted in a tertiary care center from south India from 2005 to 2010 in the ...departments of neurology and medicine. The details of all patients that satisfied the ACR 1990 criteria for diagnosis of temporal arteritis were reviewed. The clinical presentation, laboratory parameters and biopsy findings of the patients were analyzed and compared with other studies from India done over the last decade.
A total of 15 patients were diagnosed with temporal arteritis. The male:female ratio was 1.5:1. The mean age of onset was 67.58 years. Mean time for detection after onset of symptoms was 2.56 months. Typical manifestations included headache (100%), temporal artery tenderness (100%), jaw claudication (20%), polymyalgia rheumatica (53%) and visual manifestations (20%). The erythrocyte sedimentation rate was elevated in all patients. Biopsy was done in 13 patients, with 11 of them being positive. All patients responded to steroids well, with most patients being symptom-free within the first 48 h of treatment.
Temporal arteritis seems to be underdiagnosed in India, with all patients previously misdiagnosed, and with a mean time from symptom onset to diagnosis of 2.5 months. The clinical presentation of temporal arteritis in India appears to be similar to that of the West, with no gender preference and a slightly younger age group.
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IZUM, KILJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
Thrombocytopenia in patients with systemic lupus erythematosus (SLE) is associated with higher morbidity and mortality. We report frequency, associations and short-term outcome of moderate-severe ...thrombocytopenia in a prospective inception cohort from India (INSPIRE). We evaluated consecutive SLE patients classified per SLICC2012 for the occurrence of thrombocytopenia and its associations. The outcomes assessed included bleeding manifestations, kinetics of thrombocytopenia recovery, mortality and recurrence of thrombocytopenia. Among a total of 2210 patients in the cohort, 230 (10.4%) had incident thrombocytopenia, of whom moderate (platelet count PC 20–50 × 10
9
/L) and severe thrombocytopenia (PC < 20 × 10
9
/L) were noted in 61 (26.5%) and 22 (9.5%), respectively. Bleeding manifestations were generally limited to the skin. Compared to controls, cases had a higher proportion of autoimmune haemolytic anaemia (
p
< 0.001), leukopenia (
p
< 0.001), lymphopenia (
p
< 0.001), low complement (
p
< 0.05), lupus anticoagulant (
p
< 0.001), higher median SLEDAI 2 K (
p
< 0.001) and lower proportion of anti-RNP antibody (
p
< 0.05). There was no significant difference in these variables between moderate and severe thrombocytopenia. There was a sharp rise in PC by 1 week that was sustained in the majority through the period of observation. There was three times higher mortality in the severe thrombocytopenia group as compared to moderate thrombocytopenia and controls. The thrombocytopenia relapse and lupus flare rates were similar across categories. We report a low occurrence of major bleeds and higher mortality in those with severe thrombocytopenia as compared to moderate thrombocytopenia and controls.
Key Points
•
Severe thrombocytopenia occurs in 1% of patients with SLE; however, major bleeds are uncommon.
•
Thrombocytopenia has a strong association with other lineage cytopenias and lupus anticoagulants.
•
Response to initial glucocorticoids therapy is quick and is well sustained with additional immunosuppressants.
•
Severe thrombocytopenia increases mortality threefold in SLE.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
The World Health Organization Quality of Life-Bref (WHOQOL-Bref) scale was designed to measure quality of life (QOL) in both medical and psychiatric illnesses. There have been a few studies to date ...that compare aspects of QOL in medical and psychiatric illnesses.
The aim of the study was to compare QOL in patients with systemic lupus erythematosus (SLE), a chronic medical illness and schizophrenia, a chronic psychiatric disorder.
In a prospective design, 50 patients with SLE and 50 patients with schizophrenia were assessed on measures of QOL by using the WHOQOL-Bref scale, demographic factors, disease severity, and psychiatric comorbidity.
There was a significant difference between the SLE group and the schizophrenia group on the social domain of the WHOQOL-Bref scale but not on other domains. Patients with SLE had lower scores, except on social domain. Disease severity correlated with scores on the physical domain and environmental domain in both illnesses. The presence of psychiatric comorbidity was associated with significantly lower QOL scores in SLE. The presence of insight was associated with nonsignificantly lower QOL scores in schizophrenia. There was a significant association between QOL scores and both income and religious belief system in SLE, while age and duration of illness correlated with QOL scores in schizophrenia.
Although the QOLs in schizophrenia and SLE were comparable on all domains except the social domain, the factors that mediate QOL in both these illnesses are different.
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IZUM, KILJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
There is uncertainty regarding the effect of the SARS-CoV-2 infection on patients with autoimmune rheumatic diseases (AIRD) who are on immunosuppressive drugs. We did a multicity cross-sectional ...seroprevalence study conducted in five different cities in India before COVID-19 immunization. Patients with a diagnosis of AIRD and DMARDs were included. Relatives of the patients, preferably staying in the same household with no known rheumatic diseases served as controls. Serum IgG antibodies to SARS-CoV-2 Receptor Binding Domain (RBD) of the spike protein and nucleoprotein (NP) were assayed in eight hundred and eighty nine sera (subjects with disease = 379 and in subjects without disease = 510). IgG antibodies to either RBD and/or NP were positive in 135 (36%) subjects with AIRD as compared to 196 (38%) controls. The seroprevalence of anti-RBD and anti-NP varied between different cities but was not significantly different between subjects with and without disease in Mumbai, Ahmedabad, Bengaluru and Bhubaneswar. However, the occurrence of IgG antibodies to RBD was significantly (
p
< 0.05) lower in subjects with disease (28/65;43%) as compared to subjects without disease (42/65;65%) in Kolkata, where the positivity rate was lower in connective tissue disease group than in inflammatory arthritis group. Overall, patients with rheumatic diseases on DMARDs have IgG antibodies to RBD and NP of SARSCoV-2 at a comparable level with that of subjects without disease, but the level of antibodies to RBD is lower in patients with connective tissue disease on immunosuppressive drugs in one centre.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Abstract An exhilarating era for autoimmune inflammatory rheumatic diseases (AIRD) has arrived; new therapeutics are emerging that not only control symptoms, but also may allow a chance for ...remission. However, enthusiasm must be tempered with judicious caution as gaps in our knowledge remain regarding long-term safety data especially with respect to new onset infections and reactivation of latent infections such as tuberculosis (TB). Chronic vigilance and appropriate anti-infective measures such as trimethoprim/sulphamethaxozole and TB chemoprophylaxis should be instituted whenever indicated to minimise risk. Vaccination is an appropriate choice to prevent substantial number of these infections. In this context, pneumococcal and influenza vaccines are the best evaluated and are recommended by standard vaccination guidelines by most of experts. Some studies have found mildly impaired immune responses to vaccines among patients receiving long-term immunosuppressive therapy and tumour necrosis factor (TNF) antagonists, but post vaccination antibody titres are frequently adequate to provide shield for the majority of immunised individuals. The accumulated data on the safety and effectiveness of vaccines warrant immunisation with the majority of vaccines for patients with AIRD with the exception of live vaccines. In India, however there is a concern about futility of influenza vaccine, as it is feared that the serotypes targeted in this vaccine may not be the prevailing in our geographic area. Vaccination status assessment as soon as diagnosis of any of AIRDs is established and updating to appropriate vaccination status should compulsorily be implemented in daily clinical practice by rheumatologists.
Antiphospholipid antibodies (APLA) are present in one-third of systemic lupus erythematosus (SLE) patients, and they are associated with both criteria and non-criteria manifestations. We studied the ...prevalence, clinical associations, and impact on mortality of APLA in SLE patients from India. Among the Indian SLE inception cohort (INSPIRE), patients who had data on all five routinely performed APLAs lupus anticoagulant (LA), IgG and IgM anticardiolipin antibody (aCL) and anti-β2-glycoprotein I(β2GPI) at enrolment were selected. Patients were divided into four categories based on the presence/absence of APLA associated manifestations and presence/absence of the APLA viz SLE-APS, SLE-APLA, SLE: events but no APLA, and SLE: no events, no APLA (reference group). 1035 SLE patients at least 1 APLA antibody was detected in 372 (35.9%). LA was present in 206 (19.9%), aCL in 126 (12.2%) and β2-GPI in 178 (17.2%). There were 88 thrombotic events in 83 patients (8.0%); 73 (82.9%) being arterial; APLA positivity was present in 37 (44.6%) AOR 1.70 (1.054, 2.76). SLE-APS patients were younger and had higher mortality AOR 4.11 (1.51, 11.3), neuropsychiatric and hematologic disease. SLE-APLA also had a higher mortality rate AOR 2.94 (1.06, 8.22) than the reference group. The mortality was highest in the subset of patients with thrombotic events in the presence of APLA AOR 7.67 (1.25, 46.9). The mere presence of APLA also conferred higher mortality even in the absence of thrombotic events AOR 3.51 (1.43, 8.63). Hematologic manifestations (36.1%) were the most common non-criteria-manifestation. One-third of SLE patients have APLA and its presence is associated with non-criteria hematologic manifestations, arterial thrombosis and higher mortality rate.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Objective:
To conduct an exploratory cluster analysis of systemic sclerosis patients from the baseline data of the Indian systemic sclerosis registry.
Methods:
Patients satisfying American College of ...Rheumatology-European League Against Rheumatism classification criteria for systemic sclerosis were included. The clusters formed using clinical and immunological parameters were compared.
Results:
Of the 564 systemic sclerosis registry participants, 404 patients were included. We derived four clusters of which three were anti-topoisomerase I predominant and one was anti-centromere antibody 2 dominant. Cluster 1 (n-82 (20.3%)) had diffuse cutaneous systemic sclerosis patients with the most severe skin disease, anti-topoisomerase I positivity, males, younger age of onset and high prevalence of musculoskeletal, vasculopathic and gastrointestinal features. Cluster 2 (n-141 (34.9%)) was also diffuse cutaneous systemic sclerosis and anti-topoisomerase I predominant but with less severe skin phenotype than cluster 1 and a lesser prevalence of musculoskeletal, vasculopathic and gastrointestinal features. Cluster 3 (n-119 (29.5%)) had limited cutaneous systemic sclerosis patients with anti-topoisomerase I positivity along with other antibodies. The proximal muscle weakness was higher and digital pitting scars were lower, while other organ involvement was similar between clusters 2 and 3. Cluster 4 (n-62 (15.30%)) was the least severe group with limited cutaneous systemic sclerosis and anti-centromere antibody predominance. Age of onset was higher with low musculoskeletal disease and a higher presence of upper gastrointestinal features. The prevalence of interstitial lung disease was similar in the three anti-topoisomerase I predominant clusters.
Conclusion:
With exploratory cluster analysis, we confirmed the possibility of subclassification of systemic sclerosis along a spectrum based on clinical and immunological characteristics. We also corroborated the presence of anti-topoisomerase I in limited cutaneous systemic sclerosis and the association of interstitial lung disease with anti-topoisomerase I.
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NUK, OILJ, SAZU, UKNU, UL, UM, UPUK
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