ABSTRACT
OBJECTIVES: Treatment targets in systemic lupus erythematosus (SLE) have been validated in unselected-in terms of severity-cohorts, which limits their generalisability. We assessed remission (Definition of Remission in SLE (DORIS)) and Lupus Low Disease Activity State (LLDAS) in a historical cohort of 348 patients with active moderate-to-severe disease and median follow-up of 5 years. METHODS: Active SLE was defined as Physician Global Assessment ≥1.5 and/or SLE Disease Activity Index 2000 ≥6, requiring therapy intensification. DORIS/LLDAS, organ damage, flares and adverse events were monitored. Shared frailty survival, generalised linear models and K-means clustering were applied. RESULTS: Sustained DORIS and LLDAS for ≥6 months occurred in 41.1% and 80.4%, respectively, and resulted in reduced damage accrual (HR: 0.58; 95% CI 0.36 to 0.93 and 0.61; 0.43 to 0.86) and severe flares (HR: 0.14; 0.08 to 0.27 and 0.19; 0.13 to 0.27). LLDAS without DORIS was also protective (HR: 0.65; 0.43 to 0.98 for damage, 0.49; 0.36 to 0.67 for flares). Models fitting increasing duration of targets showed that DORIS ≥50% and LLDAS ≥60% of time, or alternatively, ≥24 and ≥36 months, achieved optimal balance between feasibility (20.2-41.7%) and specificity (73.3-86.1%) for damage-free outcome. These targets were linked to reduced serious adverse events (risk ratio (RR): 0.56-0.71), hospitalisation (RR: 0.70) and mortality (RR: 0.06-0.13). Patients with predominant arthritis and mucocutaneous disease experienced reduced DORIS/LLDAS, compared with counterparts with major organ involvement. Conventional drugs were more frequently used in the former group, whereas potent immunosuppressive/biological agents in the latter. CONCLUSIONS: In moderate-to-severe SLE, sustained DORIS/LLDAS for at least 6 months is sufficient, while attainment for at least 24 months ensures higher specificity for damage-free progression, thus facilitating treat-to-target strategies and clinical trials. Arthritis and skin disease represent unmet therapeutic needs that could benefit from novel biologics.
Subject(s)
Arthritis , Lupus Erythematosus, Systemic , Skin Diseases , Humans , Arthritis/drug therapy , Immunosuppressive Agents/therapeutic use , Lupus Erythematosus, Systemic/drug therapy , Remission Induction , Severity of Illness Index , Skin Diseases/drug therapy , Clinical Trials as TopicABSTRACT
PURPOSE OF THE REVIEW: This review provides an overview of medical conditions and risk factors associated with CPPD. RECENT FINDINGS: Recent studies have indicated that CPPD patients may have a higher risk for systemic conditions such as cardiovascular diseases. Calcium pyrophosphate deposition disease (CPPD) is a common crystal arthropathy that primarily affects older adults, and, in most cases, the aetiology is idiopathic. Age is the most remarkable risk factor and due to the aging population, the prevalence of this condition is expected to increase. Strong evidence supports an association between CPPD and several metabolic and endocrine conditions, including hemochromatosis, hyperparathyroidism, hypomagnesemia, and hypophosphatasia. Additionally, there is growing evidence of an increased risk for cardiovascular diseases among CPPD patients, alongside potential links to rheumatic disorders, gender, medications, and joint trauma. Further research is needed to explore the underlying mechanisms linking CPPD to associated conditions and to develop targeted therapies with the aim of improving patient outcomes.
ABSTRACT
Introduction: Systemic Lupus Erythematosus (SLE) is an autoimmune disease associated with an increased risk of cardiovascular diseases (CVDs), leading to elevated mortality rates among patients. We aimed to evaluate the levels of cardio-ankle vascular index (CAVI), global longitudinal strain (GLS), ventricular-arterial coupling (VAC), and high-sensitivity cardiac troponin I (hsTnI) in SLE patients and to explore their relationship with clinical parameters. Methods: This cross-sectional study enrolled 82 SLE patients without evident cardiac or kidney impairment and 41 age- and sex-matched healthy controls. We comparatively evaluated CAVI, GLS, VAC, and hsTnI between SLE patients and controls, and we assessed their association among SLE patients with disease activity based on the SELENA-SLEDAI Activity Index. Multivariate regression analysis was performed to identify independent predictors of CAVI and hsTnI within the SLE cohort. Results: In comparison to healthy controls, SLE patients presented with significantly higher CAVI, GLS, and hsTnI levels, while VAC was significantly reduced (p < 0.001). Furthermore, SLE patients with active disease (SELENA-SLEDAI ≥ 4) exhibited higher levels of CAVI and troponin than those with inactive disease (p < 0.001). SLEDAI was an independent predictor of CAVI, while VAC and SLEDAI were independent determinants of hsTnI in the SLE cohort. Conclusions: SLE patients displayed abnormal levels of CAVI, VAC, GLS, and troponin compared to healthy individuals. Our findings implicate the potential of those CV novel CVD risk factors to refine screening and therapeutic strategies for this specific population.
Subject(s)
Cardiovascular Diseases , Lupus Erythematosus, Systemic , Troponin I , Vascular Stiffness , Adult , Female , Humans , Male , Middle Aged , Biomarkers/blood , Cardio Ankle Vascular Index , Cardiovascular Diseases/blood , Cardiovascular Diseases/physiopathology , Cardiovascular Diseases/etiology , Case-Control Studies , Cross-Sectional Studies , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/blood , Lupus Erythematosus, Systemic/physiopathology , Troponin I/blood , Vascular Stiffness/physiologyABSTRACT
BACKGROUND AND PURPOSE: Primary Sjögren syndrome (pSS) is a chronic, systemic, autoimmune disorder characterized by lymphocytic infiltrates of the exocrine organs, leading to sicca symptoms and parotid enlargement. pSS has been linked to various neurological manifestations, including peripheral neuropathy (PN). We aimed to provide a comprehensive analysis of the currently available evidence regarding pSS-related PN. METHODS: A literature search in the PubMed database was performed, and 49 papers were eligible to be included in this systematic review and meta-analysis. RESULTS: The pooled prevalence of PN in pSS is estimated to be 15.0% (95% confidence interval = 10.7%-20.7%). The mean age of pSS patients at PN diagnosis is 59 years. Among the patients with pSS and PN, 83% are females. Neuropathic symptoms usually precede or lead to the pSS diagnosis at a 2:1 ratio in patients with pSS-related PN. The commonest type of pSS-related PN is distal axonal polyneuropathy (80% of patients with pSS-related PN), followed by sensory ganglionopathy. Peripheral and cranial mononeuropathies-particularly trigeminal-are also frequent. Risk factors for developing PN include increasing age and presence of vasculitis. Immune-mediated pathogenetic mechanisms are discussed. Glucocorticoids are the most commonly used treatment option for managing pSS-related PN, when associated with vasculitis, followed by the use of intravenous immunoglobulin. CONCLUSIONS: PN is very common in pSS patients. Evidence on long-term prognosis of PN in pSS is limited, and further research is needed. Research into the use of immunosuppressive medication in nonvasculitic neuropathies in the context of pSS merits further consideration.
Subject(s)
Peripheral Nervous System Diseases , Sjogren's Syndrome , Vasculitis , Female , Humans , Middle Aged , Male , Sjogren's Syndrome/complications , Sjogren's Syndrome/pathology , Peripheral Nervous System Diseases/etiology , Peripheral Nervous System Diseases/complications , Vasculitis/complications , Immunoglobulins, IntravenousABSTRACT
Granulomatous mastitis (GM) is a benign, inflammatory condition of the breast that mainly affects women of reproductive age. Although its pathogenesis remains unknown, previous studies revealed an association between autoimmune rheumatic diseases (ARDs) and GM in a subset of patients implicating immune-mediated mechanisms. The aim of this narrative review was to identify and describe the ARDs associated with GM to shed further light on disease pathogenesis. We conducted a comprehensive literature search of patients presenting with GM and coexisting ARDs using electronic databases. An association between GM and various ARDs has been reported, including sarcoidosis, systematic lupus erythematosus, granulomatosis with polyangiitis, psoriasis/psoriatic arthritis, familial Mediterranean fever, ankylosing spondylitis, Sjogren's syndrome, rheumatoid arthritis, and erythema nodosum, with the most common being granulomatous mastitis-erythema nodosum-arthritis syndrome (GMENA), granulomatosis with polyangiitis (Wegener's) and sarcoidosis. In addition, clinical characteristics, diagnostic and therapeutic approaches were recorded. Further research is warranted to better understand the association between GM and ARDs and raise awareness amongst rheumatologists.
Subject(s)
Arthritis, Psoriatic , Arthritis, Rheumatoid , Autoimmune Diseases , Erythema Nodosum , Granulomatosis with Polyangiitis , Granulomatous Mastitis , Respiratory Distress Syndrome , Rheumatic Diseases , Sarcoidosis , Humans , Female , Granulomatosis with Polyangiitis/complications , Granulomatous Mastitis/complications , Erythema Nodosum/complications , Autoimmune Diseases/complications , Arthritis, Rheumatoid/complications , Rheumatic Diseases/complications , Arthritis, Psoriatic/complications , Respiratory Distress Syndrome/complicationsABSTRACT
The neutrophil-to-lymphocyte ratio (NLR) emerged as a potential biomarker in SLE, but its association with several outcomes remains unclear. We aimed to evaluate the relationship between NLR and SLE disease activity, damage, depression, and health-related quality of life. A cross-sectional study was conducted, including 134 patients with SLE who visited the Division of Rheumatology between November 2019 and June 2021. Demographics and clinical data including NLR, Safety of Estrogens in Lupus Erythematosus National Assessment-Systemic Lupus disease activity index (SELENA-SLEDAI), Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI), physician global assessment (PhGA), patient global assessment (PGA), patient health questionnaire (PHQ)-9, patient self-rated health, and lupus quality of life (LupusQoL) scores, were collected. Patients were stratified into two groups and compared using the NLR cut-off of 2.73, the 90th percentile value of healthy individuals. The analysis included t-test for continuous variables, χ2-test for categorical variables, and logistic regression adjusting for age, sex, BMI, and glucocorticoid use. Among the 134 SLE patients, 47 (35%) had an NLR ≥ 2.73. The NLR ≥ 2.73 group had significantly higher rates of severe depression (PHQ ≥ 15), poor/fair self-rated health, and the presence of damage (SDI ≥ 1). These patients also scored significantly lower in LupusQoL domains (physical health, planning, and body image), and higher in SELENA-SLEDAI, PhGA, and PGA. Logistic regression confirmed that high NLR is associated with severe depression (PHQ ≥ 15) (OR:7.23, 2.03-25.74), poor/fair self-rated health (OR:2.77,1.29-5.96), high SELENA-SLEDAI score(≥ 4) (OR:2.22,1.03-4.78), high PhGA (≥ 2) (OR:3.76, 1.56-9.05), and presence of damage (SDI ≥ 1) (OR:2.67, 1.11-6.43). High NLR in SLE may indicate depression, worse quality of life, active disease, and the presence of damage.
Subject(s)
Lupus Erythematosus, Systemic , Quality of Life , Humans , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/complications , Cross-Sectional Studies , Depression , Neutrophils , Severity of Illness IndexABSTRACT
Pulmonary Hypertension (PH) is a common manifestation in patients with Systemic Lupus Erythematosus (SLE) and varies from asymptomatic to life-threatening disease. PH can result not only from immune system dysregulation, but also from various conditions, including cardiorespiratory disorders and thromboembolic diseases. Most commonly, SLE-related PH presents with non-specific symptoms, such as progressive dyspnea on exertion, generalized fatigue and weakness and eventually dyspnea at rest. Prompt diagnosis of SLE-related PH and early identification of the underlying pathogenetic mechanisms is demanded in order to introduce targeted therapy to prevent irreversible pulmonary vascular damage. In most cases the management of PH in SLE patients is similar to idiopathic pulmonary arterial hypertension (PAH). Furthermore, specific diagnostic tools like biomarkers or screening protocols, to establish early diagnosis seem to be not available yet. Although, the survival rates for patients with SLE-related PH vary between studies, it is evident that PH presence negatively affects the survival of SLE patients.
Subject(s)
Hypertension, Pulmonary , Lupus Erythematosus, Systemic , Thromboembolism , Humans , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Familial Primary Pulmonary Hypertension , DyspneaABSTRACT
To evaluate the rheumatic diseases patient expert program and assess the students' perspective on its implementation into the medical school's curriculum. During the 4th year, small groups of medical students participated in a 2-h session with a rheumatic disease patient expert and a rheumatologist. The students had the opportunity to learn about the patient's journey, manifestations, shared thoughts, and asked questions. The patient demonstrated the hand musculoskeletal exam. At the end of the academic year, an online survey was sent to 88 students and they were asked to fill out a 19-item questionnaire using a Likert-scale response. The voluntary response rate was 67%, and 64.4% were females. Overall, most participants had a favorable experience with the program (strongly agree/agree response). 93% were satisfied with the communication they had with the patient, 93% felt the patient was active in their teaching, and 89% were engaged in meaningful learning. The vast majority of the students would recommend the program to their fellow students and they strongly believe that it should be a part of the medical school's curriculum. The findings of this study indicate the patient expert program is a novel educational activity that had a favorable impact on medical students' education and a positive perspective of implementing it to the medical school curriculum. The program's implementation will raise awareness for RMD, attract more students in the field of rheumatology, and promote the patient-centered care approach.
Subject(s)
Education, Medical, Undergraduate , Rheumatology , Students, Medical , Cross-Sectional Studies , Curriculum , Female , Humans , Male , Surveys and QuestionnairesABSTRACT
OBJECTIVE: Cutaneous polyarteritis nodosa (CPAN) is a necrotizing vasculitis of the middle-size vessels, confined to the skin. We conducted a systematic review in order to identify studies evaluating the different treatment modalities used in CPAN. METHODS: This systematic review was conducted according to PRISMA guidelines, registered in PROSPERO: CRD42020222195. PubMed/Medline databases were searched from inception to December of 2020 using the terms: (Polyarteritis nodosa[Title/Abstract]) AND ((therapy[Title/Abstract]) OR (management[Title/Abstract]) OR (treatment[Title/Abstract]))' and 'Cutaneous arteritis [Title/Abstract]'. Articles evaluating pertaining to the management of CPAN in adults were eligible for inclusion. RESULTS: A total of seven eligible case series with 325 unique patients were included. No study included a control population. In general, systemic corticosteroids were widely used as induction treatment. Immunosuppressive agents combined with corticosteroids were AZA, hydroxychloroquine, sulfasalazine, sulphapyridine, CYC, MTX, mycophenolate, tacrolimus, rituxima and thalidomide. Other agents utilized in the studies were dapsone, colchicine, non-steroid anti-inflammatory drugs, salicylates, warfarin and clopidogrel. In some studies, the presence of ulcerations was associated with an increased risk of relapse. CONCLUSION: The evidence available regarding the management of patients with CPAN is limited at best. Further studies are needed in order to evaluate the effect of treatment on disease remission, relapses and mortality.
Subject(s)
Polyarteritis Nodosa/drug therapy , Drug Therapy, Combination , HumansABSTRACT
OBJECTIVE: To determine the contributing factors associated with major depressive disorder (MDD) in SLE patients and examine the association between disease-specific health-related quality of life [lupus quality of life (LupusQoL)] domains and MDD. METHODS: Depression was assessed by the patient health questionnaire (PHQ)-9, and scores ≥10 indicate MDD. Demographic data, LupusQoL domains, clinical and other features of the SLE patients were described and compared between MDD (PHQ-9 ≥10) and non-MDD (PHQ-9 <10) groups using χ2 tests for categorical variables and Wilcoxon rank sum tests for non-normal continuous variables. The risk of MDD was evaluated for the patient and physician-reported features individually using log-binomial models to estimate relative risks and 95% confidence limits. RESULTS: Eighty-eight patients with SLE met eligibility criteria, with a mean (range) age of 48.6 (19-80), mostly female (80%) and with a mean disease duration of 13.2 years. Compared with the non-MDD group, patients with MDD (n = 32, 36%) were more likely to have the following SLE manifestations: mucocutaneous, vascular, ocular, pulmonary and musculoskeletal involvement. Self-rated health described as poor/fair was markedly associated with MDD (P < 0.001, relative risk = 0.48). Based on relative risks, higher pain visual analogue score, and patient and physician global assessment scores were also linked to MDD. The LupusQoL domain scores were notably lower in the MDD patients, with a statistically significant reduction in all LupusQoL domains. CONCLUSION: Predictors of MDD in SLE patients include higher scores in pain and global assessment, poor or fair self-reported health, and specific organ involvement. These findings may help clinicians to recognize and manage MDD promptly.
Subject(s)
Depressive Disorder, Major/epidemiology , Depressive Disorder, Major/etiology , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/psychology , Adult , Aged , Aged, 80 and over , Cross-Sectional Studies , Cyprus/epidemiology , Female , Humans , Male , Middle Aged , Young AdultABSTRACT
Systemic sclerosis (SSc) is an autoimmune rheumatic disease characterised by vasculopathy, inflammation and fibrosis in multiple organs and occasionally coexists with rheumatic conditions, such as axial spondyloarthritis (axSpA). The aim of this study was to demonstrate the successful use of tofacitinib in SSc and axial spondylarthritis (axSpa), as a novel therapeutic agent; however, further studies are needed to elucidate the role of JAK inhibition in the treatment of both conditions. In this paper, we report a case of a 58-year-old woman with diffuse SSc who developed axSpA. The patient had a 10-year history of SSc and was treated with tocilizumab before the onset of lower back pain. The diagnostic evaluation included MRI that demonstrated bone marrow oedema in the sacroiliac joints, the HLA-B27 was positive, and given the inflammatory features of her back pain, she was diagnosed with axSpa. The biologic agent was switched into etanercept with an improvement of the back pain; however, the patient had a relapse of her skin manifestations. Given the treatment failure, we aim to treat the 2 coexisting conditions concurrently with tofacitinib, which led to symptom improvement. This case report is noteworthy, given the rarity of the coexistence of both conditions and the therapeutic challenges faced by the clinician. The online database MEDLINE/PubMed and Scopus where searched for articles published from inception to June 2020, using the terms "ankylosing spondylitis" AND "systemic sclerosis" OR "axial spondyloarthritis" AND "systemic sclerosis". Also, we searched the American College of Rheumatology and European League Against Rheumatism annual meeting abstracts from 2015 to 2019 using the same terms. As a result, we found 9 similar case reports. 9 case reports of patients with both conditions were identified through a literature review and we highlighted the differences and similarities between them. Also, we emphasise on intracellular signalling inhibitors as novel therapeutic targets in treating both conditions. Tofacitinib might be a novel therapeutic agent in the management of SSc and axSpA.
Subject(s)
Scleroderma, Diffuse/drug therapy , Spondylitis, Ankylosing/drug therapy , Female , Humans , Low Back Pain/etiology , Magnetic Resonance Imaging , Middle Aged , Piperidines , Protein Kinase Inhibitors , Pyrimidines , Sacroiliac Joint/diagnostic imaging , Sacroiliac Joint/pathology , Scleroderma, Diffuse/complications , Spondylitis, Ankylosing/complications , Spondylitis, Ankylosing/diagnostic imagingABSTRACT
Granulomatous mastitis (GM) is a rare form of inflammatory breast condition associated with unilateral or bilateral breast pain, swelling and mass formation. Although the disease pathogenesis remains unknown, several reports have associated GM with manifestations such as erythema nodosum and occasionally with arthritis, suggesting that GM might have an autoimmune disease component. We aim to describe two cases of coexistence of GM, erythema nodosum, and arthritis. We also conducted a literature review to comprehensively assess and describe the characteristics of patients with GM, erythema nodosum, and arthritis, and identify effective treatment options. A literature review was conducted using PubMed and EMBASE, and 14 case reports/series were retrieved, with a total number of 29 patients. All patients are women and unilateral breast involvement was evident in the majority of patients. Nine patients (31%) presented with arthritis, 6 patients (20.7%) had a fever, and 6 patients (20.7%) developed the symptoms during pregnancy. All patients had normal chest radiograph and tissue cultures were negative. In most of the cases (n = 25, 86.2%), symptom improvement was observed with glucocorticoids and four patients (13.8%) underwent surgical treatment for the GM. Given the clinical characteristics of patients with GM, with erythema nodosum, with or without arthritis, and the positive response to glucocorticoids, we propose that the described phenotype represents an underrecognized systemic autoimmune disease that could be designated by the acronym "GMENA" (granulomatous mastitis, erythema nodosum, arthritis) syndrome. Further studies are needed to elucidate the pathogenesis of the syndrome.
Subject(s)
Arthritis/complications , Erythema Nodosum/complications , Granulomatous Mastitis/complications , Adult , Female , Humans , Pregnancy , SyndromeABSTRACT
OBJECTIVES: In late July, Cyprus experienced the second epidemic wave of COVID-19. We present the steps taken by the government and evaluate their effect on epidemic trends. MATERIALS: Cyprus Press and Information Office data were analysed. Using an R-based forecasting program, two models were created to predict cases up to 01/09/2020: Model 1, which utilised data up to 09/06/2020, when airports reopened to foreign travelers with COVID-19 screening; and Model 2, which utilised data until 24/06/2020, when screening for passengers from low-transmission countries was discontinued. RESULTS: PIO data revealed no significant policy changes between 24/06/2020 and 31/07/2020. Prediction models were robust and accurate (Model 1, R2 = 0.999, P < .001; Model 2, R2 = 0.998, P < .001). By August 30th, recorded cases exceeded those predicted by Model 1 by 24.47% and by Model 2 by 20.95%, with P values <.001 for both cases. CONCLUSIONS: The significant difference between recorded cases and those projected by Models 1 and 2 suggests that changes in epidemic trends may have been associated with policy changes after their respective dates. Discontinuation of major restrictions such as airport reopening, can destabilise the control of the epidemic, and may concomitantly necessitate a reevaluation of the current epidemic status. In the face of an evolving situation such as the COVID-19 pandemic, states are forced to balance the imposing of restrictions against their impact on the economy.
Subject(s)
COVID-19 , Pandemics , Public Policy , COVID-19/epidemiology , Cyprus/epidemiology , Humans , SARS-CoV-2ABSTRACT
ABSTRACT: Paraneoplastic arthritides are a group of inflammatory rheumatic syndromes induced by an occult and manifest malignancy, characterized by a wide range of musculoskeletal signs and symptoms that masquerade other rheumatic diseases such as rheumatoid arthritis. Although the pathogenesis of paraneoplastic arthritides is unknown, immune-mediated mechanisms can induce a paraneoplastic syndrome, with a dominant feature the polyarthritis. Common entities of paraneoplastic arthritides include paraneoplastic polyarthritis, hypertrophic osteoarthropathy, remitting seronegative symmetrical synovitis with pitting edema, palmar fasciitis and polyarthritis, and polyarthritis and panniculitis associated with pancreatic carcinoma. The electronic databases PubMed and Scopus were scrutinized using the following terms: paraneoplastic arthritis, paraneoplastic polyarthritis, or paraneoplastic rheumatic diseases. Abstracts, full articles, and selected references were reviewed. The aim of the present narrative review article was to describe the clinical characteristics, diagnostic evaluation, and management of paraneoplastic arthritides, and highlight the challenges that health care providers may encounter, distinguishing those conditions from other autoimmune rheumatic disorders. Future studies are needed to give insight into the mechanisms associated with paraneoplastic arthritides, leading to the development of novel diagnostic biomarkers.
Subject(s)
Arthritis, Rheumatoid , Fasciitis , Paraneoplastic Syndromes , Rheumatic Diseases , Synovitis , Edema , Humans , Paraneoplastic Syndromes/diagnosis , Paraneoplastic Syndromes/etiology , Paraneoplastic Syndromes/therapyABSTRACT
OBJECTIVE: Patients with autoimmune rheumatic diseases (ARDs) have a higher risk of developing organ failure, and they may require admission to the intensive care unit (ICU). The aim of our study is to determine the reasons for admission to the ICU, identify potential risk factors associated with mortality, and assess the outcomes of patients with ARD diseases admitted to the ICU. METHODS: We conducted a medical records review study of patients with ARD admitted to the ICU from 2012 to 2018. Patient data included demographic and clinical characteristics, ICU admission diagnoses, length of stay, complications, and immunosuppressive regimen. Short-term and long-term outcomes were assessed. RESULTS: A total of 80 ARD patients were identified with the mean age of 48.8, 67% were female, and 56% were Hispanic. The most common disease associated with ICU admission was systemic lupus erythematosus (42%), followed by rheumatoid arthritis (26%), and 12% of patients had systemic vasculitis. Sepsis was the leading cause of ICU admission, accounting for 31%, followed by respiratory failure due to pneumonia (10%) and congestive heart failure (10%). Twenty percent of patients died in the ICU, 5% died 30 days after ICU admission, and 7.5% died within 1 year after the ICU stay, resulting in overall mortality of 33% by the end of 1 year. Nonsurvivors were more likely to need mechanical ventilation (p = 0.001), vasopressor support (p < 0.001), had renal (p = 0.041) or cardiovascular (p < 0.001) involvement on admission, APACHE II score higher than 19 (p = 0.001), and 4 days or longer stay in the ICU (p = 0.001). CONCLUSIONS: Our findings indicated that systemic lupus erythematosus is the most common ARD associated with ICU admission, and sepsis was the most frequent cause. Predictors associated with higher mortality were the requirement for mechanical ventilation, vasopressor support, increase length of ICU stay, and renal and cardiovascular involvement on admission.
Subject(s)
Arthritis, Rheumatoid , Intensive Care Units , APACHE , Female , Humans , Prognosis , Respiration, Artificial , Retrospective StudiesABSTRACT
BACKGROUND: We conducted a systematic review to identify cases of infection-induced anti-myeloperoxidase (MPO) antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: PubMed/Medline databases were searched from inception to July of 2020, according to PRISMA guidelines. RESULTS: Among the 618 abstracts identified, 18 articles describing 23 patients (60.9% female, mean age 50.5 years) were included. Median time between infection and vasculitis development was 3 months. Five (21.7%) patients expired during follow-up. Vasculitis regressed after the resolution of infection in 12/23 (52.2%). ANCA titers decreased significantly on follow-up in 14/16 patients and in all survivors in which they were measured. Pathogens reported included Mycobacterium spp., Coccidioides spp., Rickettsia rickettsii, Staphylococcus spp., EBV, CMV and Dengue virus. CONCLUSIONS: MPO-AAV can occur after infection and may regress after its resolution. Infection should be considered in cases of MPO-AAV, as immunosuppressive treatment can have catastrophic results if the infection is not adequately treated.
Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/etiology , Infections/complications , Peroxidase/immunology , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/immunology , Humans , Immunosuppressive Agents/therapeutic use , Infections/immunologyABSTRACT
Osteitis condensans ilii is a noninflammatory condition of an uncertain etiology, characterized by sclerotic bone lesions located mainly in the iliac region of the sacroiliac joints. In many patients, osteitis condensans ilii remains an incidental imaging finding; however, it has been associated with lower back pain and may mimic inflammatory rheumatic conditions such as axial spondyloarthritis. The diagnosis is based on the presence of the characteristic sclerotic lesions on radiographs and the exclusion of other conditions that are associated with back pain. Management is usually conservative with the use of physical therapy and analgesics, and it is associated with a favorable prognosis. Herein, we conducted a narrative literature review using the terms osteitis condensans ilii, and we identified case reports, case series, reviews, and original studies associated with the condition. The aim of this article is to raise the awareness of this underrecognized clinicoradiological condition and to enable the health-care providers to recognize clinical and radiological features that should raise suspicion of the osteitis condensans illi, and to describe the treatment options.
Subject(s)
Ilium/diagnostic imaging , Low Back Pain/physiopathology , Osteitis/diagnostic imaging , Sacroiliac Joint/diagnostic imaging , Decision Trees , Diagnosis, Differential , Humans , Ilium/pathology , Low Back Pain/therapy , Osteitis/physiopathology , Osteitis/therapy , Osteitis Deformans/diagnosis , Physical Therapy Modalities , Radiography , Sclerosis , Spondylarthropathies/diagnosis , Tomography, X-Ray ComputedABSTRACT
The ojective of this study was to describe the reasons for admission to the burn center of patients with autoimmune rheumatic diseases (ARD), identify their clinical characteristics, and assess their outcomes relative to the non-ARD patients. We conducted a retrospective study of ARD patients admitted to a burn center from 2011 to 2018, and they were compared with a non-ARD group of patients. Medical records were reviewed for patients' clinical characteristics, including demographics, ARD diagnosis, laboratory studies, and APACHE II score. Additionally, we evaluate the reason for admission in the burn center, management during the burn center stay, complications, outcomes including length of stay, and mortality during the hospital stay. Among the 1094 adult patients admitted during the study period, 30 (2.7%) had a new or prior diagnosis of ARD. The most common ARD associated with admission in the burn center was rheumatoid arthritis (RA) (37%, n = 11) followed by systemic lupus erythematosus (SLE) (33%, n = 10). Burn injuries (47%, n = 14), and Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) (30%, n = 9) were the most frequent admission reasons. Compared with the non-ARD group (n = 52), ARD patients were more likely to be females (60% vs. 24%, P = 0.004), to receive glucocorticoids (50% vs. 6.9%; P < 0.05), require renal replacement (20% vs. 5%, P < 0.05) and enteral nutrition (63% vs. 24%; P < 0.05) during their burn stay. The non-ARD group was more likely to be admitted for burn injuries (81% vs 46%, P < 0.01). RA and SLE were the most common ARD, and burn injuries, followed by SJS/TEN, the most frequent causes associated with burn admissions. ARD patients were more likely to be female, received glucocorticoids, require renal replacement, and enteral nutrition during the burn stay.