ABSTRACT
A 30-year-old woman visited the dermatology and venereology clinic with red rashes on her cheeks with spreading wounds to the ears present for 6 months. Similar ailments were also found on the chest and upper arms accompanying black spots on both palms. Initially, red rashes appeared intermittently, observed around the eyes and cheeks, especially at sun exposure. Tenderness or pruritus was not present; however, the patient had joints ache, sore fingers, hair loss as well as frequent fever.
Subject(s)
COVID-19 , Lupus Erythematosus, Cutaneous , Lupus Erythematosus, Systemic , Female , Humans , Adult , Pandemics , COVID-19/complications , Alopecia/complications , Lupus Erythematosus, Systemic/complicationsABSTRACT
OBJECTIVE: During the COVID-19 pandemic, many research studies were adapted, including our longitudinal study examining cognitive impairment (CI) in systemic lupus erythematosus (SLE). Cognitive testing was switched from in-person to virtual. This analysis aimed to determine if the administration method (in-person vs. virtual) of the ACR-neuropsychological battery (ACR-NB) affected participant cognitive performance and classification. METHODS: Data from our multi-visit, SLE CI study included demographic, clinical, and psychiatric characteristics, and the modified ACR-NB. Three analyses were undertaken for cognitive performance: (1) all visits, (2) non-CI group visits only and (3) intra-individual comparisons. A retrospective preferences questionnaire was given to participants who completed the ACR-NB both in-person and virtually. RESULTS: We analysed 328 SLE participants who had 801 visits (696 in-person and 105 virtual). Demographic, clinical, and psychiatric characteristics were comparable except for ethnicity, anxiety and disease-related damage. Across all three comparisons, six tests were consistently statistically significantly different. CI classification changed in 11/71 (15%) participants. 45% of participants preferred the virtual administration method and 33% preferred in-person. CONCLUSIONS: Of the 19 tests in the ACR-NB, we identified one or more problems with eight (42%) tests when moving from in-person to virtual administration. As the use of virtual cognitive testing will likely increase, these issues need to be addressed - potentially by validating a virtual version of the ACR-NB. Until then, caution must be taken when directly comparing virtual to in-person test results. If future studies use a mixed administration approach, this should be accounted for during analysis.
Subject(s)
COVID-19 , Lupus Erythematosus, Systemic , Rheumatology , Humans , United States , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/psychology , Retrospective Studies , Longitudinal Studies , Pandemics , COVID-19/complications , CognitionABSTRACT
BACKGROUND: Immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura are both causes of thrombocytopenia. Recognizing thrombotic thrombocytopenic purpura is crucial for subsequent treatment and prognosis. In clinical practice, corticosteroids and rituximab can be used to treat both immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura; plasma exchange therapy is the first-line treatment in thrombotic thrombocytopenic purpura, while corticosteroids are strongly recommended as first-line treatment in immune thrombocytopenic purpura. The differential diagnosis of immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura is essential in clinical practice. However, case reports have suggested that immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura can occur concurrently. CASE PRESENTATION: We report the case of a 32-year-old Asian female without previous disease who presented with pancytopenia, concurrent with immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura. The morphology of the megakaryocytes in the bone marrow indicated immune-mediated thrombocytopenia. The patient received glucocorticoid treatment, and her platelet count increased; however, schistocytes remained high during the course of the therapy. Further investigations revealed ADAMTS13 activity deficiency and positive ADAMTS13 antibodies. The high titer of antinuclear antibody and positive anti-U1-ribonucleoprotein/Smith antibody indicated a potential autoimmune disease. However, the patient did not fulfill the current criteria for systemic lupus erythematosus or mixed connective tissue disease. The patient responded well to plasma exchange therapy, and her platelet count remained normal on further follow-up. CONCLUSIONS: Concurrence of immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura is rare, but clinicians should be aware of this entity to ensure prompt medical intervention. Most of the reported cases involve young women. Human immunodeficiency virus infection, pregnancy, and autoimmune disease are the most common underlying conditions.
Subject(s)
Lupus Erythematosus, Systemic , Purpura, Thrombocytopenic, Idiopathic , Purpura, Thrombotic Thrombocytopenic , Pregnancy , Female , Humans , Adult , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombocytopenic, Idiopathic/complications , Platelet Count , Rituximab/therapeutic use , Lupus Erythematosus, Systemic/complicationsABSTRACT
Pediatric systemic lupus erythematosus is a chronic autoimmune disorder with a highly variable course and prognosis. It results in functional abnormalities in the immune system due to intrinsic factors and the use of immunosuppressive therapies associated with underlying comorbidities seem to increase the risk of severe COVID-19 and poor outcomes of the disease in pediatric systemic lupus erythematosus (SLE) patients. The aim of this review is to obtain a better understanding of the existing link between this new viral infection and pediatric lupus. We have analyzed the characteristics of newly diagnosed cases of pediatric SLE following COVID-19 which have been reported in the literature and which describe the impact that COVID-19 has on patients already suffering with pediatric SLE.
Subject(s)
Autoimmune Diseases , COVID-19 , Lupus Erythematosus, Systemic , Humans , Child , Lupus Erythematosus, Systemic/complications , Immunosuppression TherapyABSTRACT
OBJECTIVE: To compare the risk of SARS-CoV-2 infection and its related severe sequelae between patients with systemic lupus erythematosus (SLE) and the general population according to COVID-19 vaccination status. METHODS: We performed cohort studies using data from The Health Improvement Network to compare the risks of SARS-CoV-2 infection and severe sequelae between patients with SLE and the general population. Individuals aged 18-90 years with no previously documented SARS-CoV-2 infection were included. We estimated the incidence rates and HRs of SARS-CoV-2 infection and severe sequelae between patients with SLE and the general population according to COVID-19 vaccination status using exposure score overlap weighted Cox proportional hazards model. RESULTS: We identified 3245 patients with SLE and 1 755 034 non-SLE individuals from the unvaccinated cohort. The rates of SARS-CoV-2 infection, COVID-19 hospitalisation, COVID-19 death and combined severe outcomes per 1000 person-months were 10.95, 3.21, 1.16 and 3.86 among patients with SLE, and 8.50, 1.77, 0.53 and 2.18 among general population, respectively. The corresponding adjusted HRs were 1.28 (95% CI: 1.03 to 1.59), 1.82 (95% CI: 1.21 to 2.74), 2.16 (95% CI: 1.00 to 4.79) and 1.78 (95% CI: 1.21 to 2.61). However, no statistically significant differences were observed between vaccinated patients with SLE and vaccinated general population over 9 months of follow-up. CONCLUSION: While unvaccinated patients with SLE were at higher risk of SARS-CoV-2 infection and its severe sequelae than the general population, no such difference was observed among vaccinated population. The findings indicate that COVID-19 vaccination provides an adequate protection to most patients with SLE from COVID-19 breakthrough infection and its severe sequelae.
Subject(s)
COVID-19 Vaccines , COVID-19 , Lupus Erythematosus, Systemic , Humans , COVID-19/complications , COVID-19/epidemiology , COVID-19/prevention & control , COVID-19 Vaccines/adverse effects , Disease Progression , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/epidemiology , SARS-CoV-2ABSTRACT
OBJECTIVES: We compared the outcomes of patients with or without systemic lupus erythematosus (SLE) who were diagnosed with coronavirus disease 19 (COVID-19) and evaluated factors within patients with SLE associated with severe outcomes. METHODS: This retrospective cohort study used the deidentified Optum COVID-19 electronic health record dataset to identify patients with COVID-19 from 1/1/2020 to 31/12/2020. Cases with SLE were matched with general controls at a ratio of 1:10 by age, sex, race and ethnicity and COVID-19 diagnosis date. Outcomes included 30-day mortality, mechanical ventilation, hospitalisation and intensive care unit admission. We evaluated the relationship between COVID-19-related outcomes and SLE using multivariable logistic regression. In addition, within SLE cases, we examined factors associated with COVID-19 related outcomes, including disease activity and SLE therapy. RESULTS: We included 687 patients matched with 6870 controls. Unadjusted rates of outcomes for patients with SLE were significantly worse than for matched controls including mortality (3.6% vs 1.8%), mechanical ventilation (6% vs 2.5%) and hospitalisation (31% vs 17.7%) (all p<0.001). After multivariable adjustment, patients with SLE had increased risks of mechanical ventilation (OR 1.81, 95% CI 1.16 to 2.82) and hospitalisation (OR 1.32, 95% CI 1.05 to 1.65). Among patients with SLE, severe disease activity was associated with increased risks of mechanical ventilation (OR 5.83, 95% CI 2.60 to 13.07) and hospitalisation (OR 3.97, 95% CI 2.37 to 6.65). Use of glucocorticoids, mycophenolate and tacrolimus before COVID-19 was associated with worse outcomes. CONCLUSION: Patients with SLE had increased risk of severe COVID-19-related outcomes compared with matched controls. Patients with severe SLE disease activity or prior use of corticosteroids experienced worse outcomes.
Subject(s)
COVID-19 , Lupus Erythematosus, Systemic , Humans , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/drug therapy , Lupus Erythematosus, Systemic/diagnosis , Retrospective Studies , COVID-19 Testing , COVID-19/complications , Immunosuppressive Agents/therapeutic useABSTRACT
BACKGROUND/AIMS: The recent coronavirus disease 2019 (COVID-19) pandemic has been associated with changes in the epidemiology of not only infectious diseases but also several non-infectious conditions. This study investigated changes in the recorded incidence of various rheumatic diseases during the COVID-19 pandemic. METHODS: The number of patients for each disease from January 2016 to December 2020 was obtained from the Korean Health Insurance Review and Assessment Service database. We compared the incidence of nine rheumatic diseases (seropositive rheumatoid arthritis, systemic lupus erythematosus [SLE], idiopathic inflammatory myositis [IIM], ankylosing spondylitis [AS], systemic sclerosis, Sjögren's syndrome, Behçet's disease [BD], polymyalgia rheumatica, and gout) and hypertensive diseases to control for changes in healthcare utilisation before and after the COVID-19 outbreak. The disease incidence before and after the COVID-19 outbreak was compared using the autoregressive integrated moving average (ARIMA) and quasi- Poisson analyses. RESULTS: Compared with the predicted incidence in 2020 using the ARIMA model, the monthly incidence of SLE, BD, AS, and gout temporarily significantly decreased, whereas other rheumatic diseases and hypertensive diseases were within the 95% confidence interval (CI) of the predicted values in the first half of 2020. In age- and sex-adjusted quasi-Poisson regression analysis, the annual incidences of IIM (rate ratio [RR], 0.473; 95% CI, 0.307 to 0.697), SLE (RR, 0.845; 95% CI, 0.798 to 0.895), and BD (RR, 0.850; 95% CI, 0.796 to 0.906) were significantly decreased compared with those in the previous 4 years. CONCLUSION: The recorded annual incidence of some rheumatic diseases, including IIM, SLE, and BD, decreased during the COVID-19 pandemic.
Subject(s)
COVID-19 , Gout , Lupus Erythematosus, Systemic , Rheumatic Diseases , Spondylitis, Ankylosing , Humans , Incidence , Pandemics , COVID-19/epidemiology , COVID-19/complications , Rheumatic Diseases/epidemiology , Spondylitis, Ankylosing/complications , Lupus Erythematosus, Systemic/complications , Gout/complicationsABSTRACT
INTRODUCTION: Patients with SLE (systemic lupus erythematosus) have a higher risk of infection due to dysregulated immune system as well as long-term use of immunosuppressants (IS). This could influence the risk of COVID-19 and its outcome. METHODS: We conducted a longitudinal prospective study across 15 rheumatology centres during the first wave of the pandemic to understand the risk factors contributing to COVID-19 in SLE patients. During the 6 months follow-up, those who tested positive for COVID-19, their clinical course and outcome information were recorded. RESULTS: Through the study period (April-December 2020), 36/1379 lupus patients (2.9%) developed COVID-19. On analysing the COVID-19 positive versus negative cohort during the study period, male gender (adjusted RR 3.72, 95% C.I. 1.85,7.51) and diabetes (adjusted RR 2.94, 95% C.I. 1.28, 6.79) emerged as the strongest risk factors for COVID-19, in the adjusted analysis. There was no significant influence of organ involvement, hydroxychloroquine, glucocorticoid dosage (prednisolone< 7.5 mg or ≥ 7.5 mg/day) or IS on the risk of COVID-19. There was only one death (1/36) among the lupus patients due to COVID-19. CONCLUSION: Traditional risk factors rather than lupus disease process or IS influenced the risk of COVID-19 in our cohort.
Subject(s)
COVID-19 , Lupus Erythematosus, Systemic , Humans , Male , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/drug therapy , Prospective Studies , COVID-19/complications , Longitudinal Studies , Immunosuppressive Agents/adverse effects , Risk FactorsABSTRACT
We described the profile and outcome of Filipino patients with inflammatory rheumatic diseases (IRDs) who developed COVID-19 (IRD-C19) during the onset of the pandemic, prior to vaccinations and variants. We obtained de-identified data of Filipino patients with IRD-C19 from the Global Rheumatology Alliance registry from March 2020 to August 2021. Descriptive statistics and multivariate analyses were applied. Registered were 164 patients (mean age 44 years; 70% female). The most common IRDs were systemic lupus erythematosus (SLE, 41.4%), rheumatoid arthritis (RA, 15.2%), and gout (14.6%). Majority were receiving conventional DMARDs (59.1%) and/or glucocorticoid therapy (GC, 51.2%). Half (58.5%) were hospitalized, with risk higher in active IRD (OR 3.7), heart disease (8.52), and hypertension (8.73); and lower in SLE patients (0.15). Among hospitalized patients, 76% needed supplemental oxygen. Heart disease (6.28), hypertension (7.6), and moderate-to-high IRD activity (3.37) were associated with higher odds of requiring oxygen supplementation. Hypertension was associated with mechanical ventilation (8.23). Twenty-four (15%) patients died, with odds lower if on prednisone ≥ 10 mg/day (0.17) and with other autoimmune IRDs aside from SLE and RA (0.05). Among patients with IRD-C19 prior to vaccinations and variants, higher disease activity, hypertension, and heart disease were associated with poorer outcomes. Prednisone ≥ 10 mg/day was associated with lower odds of death. This study provides valuable historical information, emphasizing the need for continued data collection to clarify COVID-19's impact.
Subject(s)
Arthritis, Rheumatoid , COVID-19 , Heart Diseases , Hypertension , Lupus Erythematosus, Systemic , Rheumatic Diseases , Rheumatic Fever , Humans , Female , Adult , Male , COVID-19/complications , Prednisone , Arthritis, Rheumatoid/complications , Lupus Erythematosus, Systemic/drug therapy , Lupus Erythematosus, Systemic/complications , Hypertension/complications , Vaccination , Rheumatic Diseases/drug therapy , Rheumatic Diseases/complicationsABSTRACT
OBJECTIVE: To determine if SARS-CoV-2 mRNA vaccination has an impact on the clinical course of systemic lupus erythematosus (SLE). METHODS: Puerto Ricans with SLE who received mRNA COVID-19 vaccines were studied. Demographic parameters, clinical manifestations, disease activity (per Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), disease damage (per Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index), emergency room visits, hospitalizations, and pharmacologic therapy were determined. Baseline variables (prior to vaccination) were compared between patients with and without exacerbation after SARS-CoV-2 vaccination. Among those with exacerbation, clinical outcomes were determined up to 1 year after vaccination. RESULTS: Of the entire cohort (n = 247), 14 (5.7%) had post-vaccination exacerbations. Photosensitivity, oral ulcers, anti-Ro antibodies, higher SLEDAI score, and corticosteroids exposure were associated with post-vaccination flares. Among those with post-vaccination flares, 10 (71.4%) had major organ involvement. No significant differences were observed for mean SLEDAI scores, emergency room visits, hospitalizations, disease damage, and exposure to immunosuppressive drugs before and after SARS-CoV-2 mRNA vaccination. At 12 months of follow-up, all patients were fully controlled without evidence of active disease. CONCLUSION: In our group of SLE patients, 5.7% had a disease flare after SARS-CoV-2 mRNA vaccination. Most had exacerbations involving major organs/systems. Mucocutaneous manifestations, anti-Ro antibodies, disease activity, and corticosteroids were associated with flares. Awareness of these factors and the possibility of a major lupus flare after vaccination with COVD-19 vaccines is critical to provide timely and effective therapy.
Subject(s)
COVID-19 Vaccines , COVID-19 , Lupus Erythematosus, Systemic , Humans , Cohort Studies , COVID-19/prevention & control , COVID-19/complications , COVID-19 Vaccines/adverse effects , Disease Progression , Lupus Erythematosus, Systemic/complications , Puerto Rico , RNA, Messenger , SARS-CoV-2 , Severity of Illness Index , Symptom Flare Up , Vaccination/adverse effectsABSTRACT
Recently, due to the coronavirus disease 2019 (COVID-19) pandemic, much concern has been raised about patients with chronic diseases who may become more susceptible to the disease. The present cross-sectional study aimed to characterize the clinical course of COVID-19 in patients with systemic lupus erythematosus (SLE). In addition, a possible correlation between the immunosuppression state and the incidence of COVID-19 is investigated. In May 2020, 500 SLE patients registered in the database of Golestan Rheumatology Research Center (Golestan province, Iran) were selected for this cross-sectional study. Using a questionnaire, patients were contacted by telephone to collect data including demographic characteristics, disease status, drug use, and new clinical symptoms. Data were analyzed using SPSS software version 24.0. Of the 500 selected patients, 355 responded to the phone calls and subsequently enrolled in the study. Among the enrolled patients, 25 were classified as COVID-19 positive, including eight hospitalized patients, of which two required intensive care and subsequently died. COVID-19 incidence was significantly lower in the immunosuppressed patients (2.2% vs. 10%, P=0.01). There was no significant correlation between hydroxychloroquine consumption and the incidence of COVID-19 in SLE patients. Fever, fatigue, dyspnea, and dry cough were the most common clinical symptoms. Our results showed that COVID-19 incidence was lower in immunosuppressed than the non-immunosuppressed SLE patients. Further studies are required to substantiate the role of immunosuppression in the development of COVID-19. A preprint version of this study was published at https://www.researchsquare.com/article/rs-78704/v1 with doi: https://doi.org/10.21203/rs.3.rs-78704/v1.
Subject(s)
COVID-19 , Lupus Erythematosus, Systemic , Humans , COVID-19/complications , COVID-19/epidemiology , Cross-Sectional Studies , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/epidemiology , Lupus Erythematosus, Systemic/diagnosis , Hydroxychloroquine , Immunosuppression TherapyABSTRACT
Autoimmune diseases are known to be a risk factor for severe COVID-19 infection. This is the first case series of patients with autoimmune disease suffering from COVID-19 infection in Jakarta, Indonesia. There were 12 confirmed cases of COVID-19 infection in autoimmune patients from March 2020 until February 2021. We select 5 patients in this case series. Three of them had systemic lupus erythematous (SLE), one of them had rheumatoid arthritis, and one of them had ankylosing spondylitis. Three of them had high BSR Risk Stratification. Most of them had used daily steroid therapy. Fatigue, abdominal pain, diarrhea, and cough were the common symptoms found. None of the patients were admitted to ICU, used mechanical ventilators, and all of them survived. Most of the patients were prescribed anti-coagulant therapy. This first comprehensive case series can provide valuable information regarding the clinical characteristics of COVID-19 infection in the Indonesian autoimmune disorder patient population.
Subject(s)
Arthritis, Rheumatoid , Autoimmune Diseases , COVID-19 , Lupus Erythematosus, Systemic , Spondylitis, Ankylosing , Humans , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/drug therapy , Autoimmune Diseases/complications , Autoimmune Diseases/epidemiology , Arthritis, Rheumatoid/drug therapy , Arthritis, Rheumatoid/epidemiology , Spondylitis, Ankylosing/drug therapy , Spondylitis, Ankylosing/epidemiologyABSTRACT
Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection may present with some systemic lupus erythematosus (SLE) manifestations intermingled with Kawasaki disease features. These emerging presentations were dubbed under the umbrella term 'multisystem inflammatory syndrome in children (MIS-C)'. A one and half-year-old girl, admitted to Mansoura University Children's Hospital (MUCH) with fever, bad general condition, vomiting, widespread maculopapular, vasculitic rash, hands and feet oedema, oral ulceration, arthralgia and lymphadenopathy. Moreover, bicytopenia, positive antinuclear, anti-double-stranded DNA antibodies and low C3 qualified her as a case of juvenile SLE. Despite the child received the initial therapy of immunosuppressive medication, her general condition deteriorated with fever persistence and rash exacerbation. At that time, the skin of her hands and feet started to peel. Thus, an expanded study for other alternatives was obligatory; SARS-CoV-2 infection testing revealed positive IgG serology, and retesting for lupus autoantibodies turned negative. HRCT chest showed bilateral basal consolidation with ground-glass appearance. Furthermore, Echo exhibited coronary artery dilation with thrombus inside. This evolution raised the concern for COVID-related MIS-C syndrome. This report provides a model of COVID-19 heterogeneity with protean immune-related manifestations. This case has a unique presentation that necessities its description, in order to provide a nidus for future studies in this new entity.
Subject(s)
COVID-19 , Exanthema , Lupus Erythematosus, Systemic , Antibodies, Viral , Autoantibodies , COVID-19/complications , COVID-19/diagnosis , Child , DNA , Exanthema/etiology , Female , Fever , Humans , Immunoglobulin G , Infant , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , SARS-CoV-2 , Systemic Inflammatory Response Syndrome/diagnosisABSTRACT
BACKGROUND: Systemic lupus erythematosus (SLE) is a disease that can lead to damage of multiple organs and, along with certain treatments, increase the risk of developing cancer, cardiovascular disease, diabetes, osteoporosis, and infections. Preventive services are particularly important in patients with SLE to mitigate the aforementioned risks. We aimed to evaluate the trends of preventive services utilization in patients with systemic lupus erythematosus, compared with non-SLE population. METHODS: All ≥19-year-old patients in the Lupus Midwest Network (LUMEN) registry, a population-based cohort, with SLE on January 1, 2015, were included and matched (1:1) by sex, age, race, and county to non-SLE comparators. Among both groups, we compared the rates of screenings for breast and cervical cancer, hypertension, hyperlipidemia, diabetes mellitus, and osteoporosis as well as immunizations. RESULTS: We included 440 SLE patients and 430 non-SLE comparators. The probability of breast cancer screening among women with SLE was similar to comparators (hazard ratio [HR] 1.09, 95% CI 0.85-1.39), while cervical cancer screening was lower (HR 0.75, 95% CI 0.58-0.96). Hypertension screening was higher among patients with SLE (HR 1.35, 95% CI 1.13-1.62); however, hyperlipidemia screening was similar to comparators (HR 1.16, 95% CI 0.96-1.41). Diabetes and osteoporosis screenings were more likely to be performed for SLE patients than for comparators (HR 2.46, 95% CI 2.11-2.87; and HR 3.19, 95% CI 2.31-4.41; respectively). Influenza and pneumococcal immunizations were higher among SLE patients (HR 1.31, 95% CI 1.12-1.54; and HR 2.06, 95% CI 1.38-3.09; respectively), while zoster vaccination was similar (HR 1.17, 95% CI 0.81-1.69). CONCLUSIONS: The trends of utilization of preventive services by SLE patients vary according to screening or vaccine compared with the general population. Considering these differences, we demonstrate an opportunity for improvement, particularly in cervical cancer, hyperlipidemia, and osteoporosis screenings and vaccinations.
Subject(s)
Hyperlipidemias , Hypertension , Lupus Erythematosus, Systemic , Osteoporosis , Uterine Cervical Neoplasms , Adult , Early Detection of Cancer , Female , Humans , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/epidemiology , Risk Factors , Uterine Cervical Neoplasms/diagnosis , Uterine Cervical Neoplasms/epidemiology , Uterine Cervical Neoplasms/prevention & control , Young AdultABSTRACT
Haemophagocytic lymphocytic histiocytosis (HLH) is a rare, life-threatening condition caused by abnormal activation of cytotoxic T lymphocytes, natural killer cells and macrophages resulting in hypercytokinaemia and immune-mediated injury of multiple organ systems. Secondary HLH occurs in the setting of a malignant, infectious or autoimmune stimulus. Macrophage activation syndrome (MAS) is the term used to describe HLH that develops secondary to rheumatological diseases such as lupus and juvenile idiopathic arthritis, among others. Commonly observed and documented symptoms include fever, organomegaly and lymphadenopathy. Given the potential for multiorgan failure in HLH/MAS, early identification, diagnosis and initiation of treatment is essential. We present a case of secondary HLH/MAS with acute inflammatory gastroenteritis in a middle-aged woman with a history of systemic lupus erythematosus.
Subject(s)
Arthritis, Juvenile , Gastroenteritis , Lupus Erythematosus, Systemic , Lymphohistiocytosis, Hemophagocytic , Macrophage Activation Syndrome , Arthritis, Juvenile/complications , Female , Gastroenteritis/complications , Gastroenteritis/diagnosis , Humans , Lupus Erythematosus, Systemic/complications , Lymphohistiocytosis, Hemophagocytic/complications , Lymphohistiocytosis, Hemophagocytic/diagnosis , Macrophage Activation Syndrome/complications , Macrophage Activation Syndrome/etiology , Middle AgedABSTRACT
OBJECTIVES: To study the rate of SARS-CoV-2 vaccination and post-vaccination disease flares in patients with systemic lupus erythematosus (SLE). METHODS: Patients who fulfilled ≥ 4 of the ACR criteria for SLE were identified and their SARS-CoV-2 vaccination status was traced. Flares of SLE at 6-week post-vaccination were reviewed retrospectively. Clinical characteristics of patients with and without vaccination, and those who did or did not experience post-vaccination flares were compared by statistical analyses. RESULTS: 914 adult patients with SLE were studied (92.5 % women, age 48.6 ± 14.0 years; SLE duration 14.5 ± 8.6 years). Two doses of the SARS-Cov-2 vaccines (61.5 % BioNTech; 38.5 % CoronaVac) were received by 449 (49.1 %) patients. The vaccination rate in SLE was significantly lower than that of the adult general population (77.8 %; p < 0.001) at the time of data analysis. Patients who were hesitant for vaccination were more likely to be hypertensive, have a history of neuromuscular manifestations, and a significantly higher organ damage score (1.10 ± 1.45 vs 0.74 ± 1.15; p < 0.001). However, none of these factors were significantly associated with vaccine hesitancy on multivariate analysis. Among 449 vaccinated patients, 37(8.2 %) experienced SLE flares: mild/moderate in 34; severe in 3. In an equal number of unvaccinated SLE controls randomly matched for the post-vaccination observation period, 28(6.2 %) had SLE flares: mild/moderate in 17; severe in 11 (odds ratio [OR] for flare in vaccinated patients 1.40[0.81-2.43]; p = 0.23, adjusted for age, sex, active serology, SLE duration and prednisolone use). In vaccinated patients, logistic regression revealed that active lupus serology before vaccination (OR 2.63[1.05-6.62]; p = 0.04) and a history of arthritis (OR 2.71[1.05-7.00]; p = 0.04) or discoid skin lesion (OR 4.73[1.90-11.8]; p = 0.001) were associated with SLE flares following vaccination, adjusted for confounders. CONCLUSION: Hesitancy for COVID-19 vaccination is common in SLE patients. Vaccination against SARS-CoV-2 is not significantly associated with increased SLE flares. Patients with active SLE serology or a history of arthritis/discoid lesion are more likely to flare after vaccination.
Subject(s)
Arthritis , COVID-19 , Lupus Erythematosus, Systemic , Adult , COVID-19/prevention & control , COVID-19 Vaccines/adverse effects , Female , Humans , Lupus Erythematosus, Systemic/complications , Male , Middle Aged , Prednisolone , Retrospective Studies , SARS-CoV-2 , VaccinationABSTRACT
OBJECTIVE: SLE is associated with increased cardiovascular risk (CVR). High serum concentrations of triglyceride-rich lipoproteins and apolipoprotein B-rich particles constitute the characteristic dyslipidaemia of SLE. METHODS: A cross-sectional study was conducted to study the relationship between genetic variants involved in polygenic hypertriglyceridaemia, subclinical atherosclerosis and lipoprotein abnormalities. 73 women with SLE and 73 control women age-matched with the case group were recruited (age range 30-75 years). Serum analysis, subclinical atherosclerosis screening studies for the detection of plaque, and genetic analysis of the APOE, ZPR1, APOA5 and GCKR genes were performed. RESULTS: Triglyceride concentrations and the prevalence of hypertension, dyslipidaemia and carotid atherosclerosis were higher in women with SLE than in the control group. Multivariate logistic regression showed that CC homozygosity for the GCKR rs1260326 gene (OR=0.111, 95% CI 0.015 to 0.804, p=0.030) and an increase of 1 mmol/L in triglyceride concentrations were associated with a greater risk of carotid plaque in women with SLE (OR=7.576, 95% CI 2.415 to 23.767, p=0.001). CONCLUSIONS: GCKR CC homozygosity (rs1260326) and serum triglyceride concentrations are independently associated with subclinical carotid atherosclerosis in women with SLE. Subclinical carotid atherosclerosis is also more prevalent in these women compared with the control group. The study of GCKR rs1260326 gene variants may contribute to more precise assessment of CVR and modulation of the intensity of lipid-lowering treatment in patients with SLE.