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Arthritis Research & Therapy Jul 2021We sought to examine the disease course of High Disease Activity Status (HDAS) patients and their different disease patterns in a real-world longitudinal cohort. Disease...
BACKGROUND
We sought to examine the disease course of High Disease Activity Status (HDAS) patients and their different disease patterns in a real-world longitudinal cohort. Disease resolution till Lupus Low Disease Activity State (LLDAS) has been a general treatment goal, but there is limited information on this subset of patients who achieve this.
METHODS
All consenting patients of the Monash Lupus Cohort who had at least 12 months of observation were included. HDAS was defined as SLEDAI-2K ≥ 10 ever, and HDAS episode as the period from the first HDAS clinic visit until attainment of LLDAS. We examined the associations of different HDAS patterns with the likelihood of damage accrual.
RESULTS
Of 342 SLE patients, 151 experienced HDAS at least once, accounting for 298 HDAS episodes. The majority of HDAS patients (76.2%) experienced Recurrent HDAS (> 1 HDAS visit), and a smaller subset (47.7%) had Persistent HDAS (consecutive HDAS visits for longer than 2 months). Recurrent or Persistent HDAS patients were younger at diagnosis and more likely to experience renal or serositis manifestations; persistent HDAS patients were also more likely to experience neurological manifestations. Baseline SLEDAI greater than 10 was associated with longer HDAS episodes. Recurrent and Persistent HDAS were both associated with an increased likelihood of damage accrual. The total duration of HDAS episode greater than 2 years and experiencing multiple HDAS episodes (≥4) was also associated with an increased likelihood of damage accrual (OR 1.80, 95% CI 1.08-2.97, p = 0.02, and OR 3.31, 95% CI 1.66-13.26, p = 0.01, respectively).
CONCLUSION
HDAS episodes have a highly variable course. Recurrent and Persistent HDAS, and longer duration of HDAS episodes, increased the risk of damage accrual. In addition to a major signifier of severity in SLE, its resolution to LLDAS can determine the subsequent outcome in SLE patients.
Topics: Cohort Studies; Disease Progression; Humans; Lupus Erythematosus, Systemic; Severity of Illness Index; Time Factors
PubMed: 34261522
DOI: 10.1186/s13075-021-02572-1 -
Brazilian Journal of Microbiology :... Jun 2021Magellanic penguins (Spheniscus magellanicus) migrate to the continental shelf of southern-southeastern Brazil during austral winter. Stranded penguins are directed to...
Magellanic penguins (Spheniscus magellanicus) migrate to the continental shelf of southern-southeastern Brazil during austral winter. Stranded penguins are directed to rehabilitation centers, where they occasionally develop fungal diseases. Aspergillosis, a mycosis caused by Aspergillus spp., is one of the most important diseases of captive penguins, while Candida sp. has been detected in penguins undergoing rehabilitation. Nevertheless, their occurrence in the wild is poorly understood. This study surveyed the occurrence of mycoses in free-ranging Magellanic penguins wintering in southeastern Brazil. These penguins were either found dead or stranded alive and died during transport to a rehabilitation center. Overall, 61 fresh to moderate autolyzed carcasses were necropsied. Upon necropsy, three juvenile males (4.9%) presented mycotic-consistent gross lesions. Histopathology and panfungal PCRs confirmed the mycoses. Major microscopic findings were marked chronic necrotizing multifocal to coalescent pneumonia, airsacculitis, and esophageal/gastric serositis with two types of intralesional fungal structures: (a) septated acute-angled branching hyphae (n = 2) and (b) yeast structures (n = 1), both PAS- and Grocott-positive. Sequences identical to Aspergillus sp. were retrieved in two cases, while the third had sequences identical to Candida palmioleophila. This study describes two cases of aspergillosis and one of candidiasis in free-ranging Magellanic penguins, confirming the species' susceptibility in the wild. These mycoses could be associated with the animals' poor body condition, and/or impaired immunity, and natural and anthropogenic challenges related to migration. To the authors' knowledge, this is the first report of aspergillosis in free-ranging Magellanic penguins in the Atlantic Ocean and of candidiasis in penguins worldwide.
Topics: Animals; Animals, Wild; Aspergillosis; Aspergillus; Bird Diseases; Brazil; Candida; Candidiasis; DNA, Fungal; Male; Respiratory Tract Infections; Spheniscidae
PubMed: 33566323
DOI: 10.1007/s42770-021-00438-x -
Reumatologia Clinica Oct 2022To evaluate the correlation of quantitative anti-dsDNA level with proteinuria levels in patients with lupus nephritis in a tertiary care hospital.
OBJECTIVE
To evaluate the correlation of quantitative anti-dsDNA level with proteinuria levels in patients with lupus nephritis in a tertiary care hospital.
STUDY DESIGN
In this prospective cross-sectional study, 76 patients of newly diagnosed SLE coming to Fatima Memorial Hospital were included in the study period between January 2020 to June 2020. Demographic data such as age, gender, lupus manifestations such as serositis, arthritis, mucocutaneous disease, and neuropsychiatric manifestations were recorded. Quantitative anti-dsDNA was measured by enzyme-linked immunosorbent assay and proteinuria was estimated by 24h urinary protein collection. Data was analyzed by SPSS 23. Association between categorical variables was assessed using chi-square test. For comparison of categorical independent and continuous dependent variable t-test or Mann-Whitney U test was applied.
RESULTS
The median age of the cohort was 29 (with inter quartile range - IQR - of 13) years. The female gender comprised of 68 (89.4%) of the cohort population. The median anti-dsDNA level was 54.9 (183.6 IQR) IU, and baseline proteinuria of the cohort was 520mg/dL (1.49 IQR). There was a significant association of anti-dsDNA level with systemic features such as arthritis (p=<0.01), serositis (p=<0.01) and, Raynaud's phenomenon (p=<0.01). NPSLE and mucocutaneous features did not show statistically significant association (p=0.91 and 0.14 respectively). Baseline anti-dsDNA showed a statistically significant correlation with baseline proteinuria levels (p=<0.01).
CONCLUSION
Quantitative anti-dsDNA is directly correlated with nephritis measured as proteinuria, and can be detected even before organ involvement. Hence, it can determine disease course and guide early treatment.
Topics: Adolescent; Antibodies, Antinuclear; Arthritis; Cross-Sectional Studies; DNA; Female; Humans; Lupus Erythematosus, Systemic; Prospective Studies; Proteinuria; Serositis
PubMed: 36210140
DOI: 10.1016/j.reumae.2021.06.005 -
Autoimmune Diseases 2022Bullous systemic lupus erythematosus (BSLE) is an uncommon cutaneous presentation that occurs even less frequent in the pediatric population. A retrospective review was...
Bullous systemic lupus erythematosus (BSLE) is an uncommon cutaneous presentation that occurs even less frequent in the pediatric population. A retrospective review was performed from January 2012 to December 2021 in all pediatric patients (aged <18 years) who fulfilled the diagnostic criteria for BSLE to evaluate the clinical characteristics, extracutaneous involvement, histopathologic features, immunofluorescence patterns, serological abnormalities, internal organ involvement, treatments, and outcomes. Among 1,415 patients with SLE, five patients were validated for the diagnosis of BSLE, accounting for 0.35%. The mean age at diagnosis was 12.2 years (standard deviation, 1.92). The clinical features of BSLE in the study population were generalized tense bullae and large extensive vesicles on the lips and perioral and mucosal areas. Pediatric BSLE in the study population revealed high SLE disease activity with multiple organ involvement. Hematologic abnormalities, serositis, and renal involvement were found in all patients, while polyarthritis (40%) and neurological abnormalities (40%) were less frequently observed. Systemic corticosteroids, intravenous immunoglobulin, immunosuppressants, antimalarials, and dapsone were prescribed in the study population. The cutaneous lesions subsided in all patients with a median clearance duration of 14 days (range, 5-56 days). BSLE in the pediatric population has auxiliary manifestations with high disease activity. Multiple organ involvement, especially hematologic abnormalities, serositis, and renal involvement, was frequently found in the study population. Although cutaneous lesions in BSLE subsided in all patients, involvement of other organs, especially renal impairment, required aggressive treatment, and long-term follow-up.
PubMed: 35911475
DOI: 10.1155/2022/9388745 -
Frontiers in Immunology 2022Familial Mediterranean fever (FMF) is a monogenic autoinflammatory disease characterized by recurrent episodes of fever and serositis. Colchicine (Col) has a crucial...
Familial Mediterranean fever (FMF) is a monogenic autoinflammatory disease characterized by recurrent episodes of fever and serositis. Colchicine (Col) has a crucial role in the prevention of amyloidosis and FMF attacks. The effect of Col on innate immune cells is based on the inhibition of the microtubule system. The microtubule system is also very important for neurosecretory functions. The inhibitory effect of Col on neurosecretory functions is an overlooked issue. Considering that the neuroimmune cross-talk process plays a role in the development of inflammatory diseases, the effect of Col on the neuronal system becomes important. FMF attacks are related to emotional stress. Therefore, the effect of Col on stress mediators is taken into consideration. In this hypothetical review, we discuss the possible effects of Col on the central nervous systems (CNS) and peripheral nervous systems (PNS) in light of mostly experimental study findings using animal models. Studies to be carried out on this subject will shed light on the pathogenesis of FMF attacks and the other possible mechanisms of action of Col apart from the anti-inflammatory features.
Topics: Amyloidosis; Animals; Anti-Inflammatory Agents; Colchicine; Familial Mediterranean Fever
PubMed: 35251026
DOI: 10.3389/fimmu.2022.834769 -
Rheumatology International Jun 2022Most of the published data relate to classical forms of rheumatic diseases (RD) and information on rare inflammatory disorders such as Behçet's syndrome (BS) and...
Safety of SARS-CoV-2 vaccination in patients with Behcet's syndrome and familial Mediterranean fever: a cross-sectional comparative study on the effects of M-RNA based and inactivated vaccine.
Most of the published data relate to classical forms of rheumatic diseases (RD) and information on rare inflammatory disorders such as Behçet's syndrome (BS) and familial Mediterranean fever (FMF) is limited. We studied the frequency of side effects and disease flares after COVID-19 vaccination with either Pfizer/BioNTech or Sinovac/CoronaVac in 256 patients with BS, 247 with FMF, and 601 with RD. Telephone interviews were conducted using a questionnaire survey in a cross-sectional design in patients with BS, FMF, and RD followed by a single university hospital. Study participants were vaccinated either with CoronaVac (BS:109, FMF: 90, and RD: 343,) or BioNTech (BS: 147, FMF: 157 and RD: 258). The majority have received double dose (BS: 94.9%, FMF 92.3% and RD: 86.2%). BioNTech ensured a significantly better efficacy than CoronaVac against COVID-19 in all patient groups (BS: 1.4% vs 10.1%; FMF: 3.2% vs 12.2%, RD:2.7% vs 6.4%). Those with at least one adverse event (AE) were significantly more frequent among those vaccinated with BioNTech than those with CoronaVac (BS: 86.4% vs 45%; FMF: 83.4% vs 53.3%; and RD: 83.3% vs 45.5%). The majority of AEs were mild to moderate and transient and this was true for either vaccine. There were also AEs that required medical attention in all study groups following CoronaVac (BS: 5.5%, FMF: 3.3%, and RD:2.9%) or BioNTech (BS: 5.4%, FMF: 1.9%, and RD: 4.7%). The main causes for medical assistance were disease flare and cardiovascular events. Patients with BS (16.0%) and FMF (17.4%) were found to flare significantly more frequently when compared to those with RD (6.0%) (p < 0.001). This was true for either vaccine. BS patients reported mainly skin-mucosa lesions; there were however, 11 (4.3%) who developed major organ attack such as uveitis, thrombosis or stroke. Flare in FMF patients were associated mainly with acute serositis with or without fever. Arthralgia/arthritis or inflammatory back pain were observed mainly in the RD group. Our study demonstrates that BS and FMF patients vaccinated with either CoronaVac or BioNTech demonstrated similar AE profile and frequency compared to RD patients. AEs that required physician consultation or hospitalization occurred in all study groups after either CoronaVac or BioNTech. Increased frequency of flares in BS and FMF compared to that seen in RD might reflect defects in innate immunity and deserves further investigation. Caution should be required when monitoring these patients after vaccination.
Topics: Behcet Syndrome; COVID-19; COVID-19 Vaccines; Cross-Sectional Studies; Familial Mediterranean Fever; Humans; Pain; RNA; Rheumatic Diseases; SARS-CoV-2; Vaccination; Vaccines, Inactivated
PubMed: 35376962
DOI: 10.1007/s00296-022-05119-y -
BMC Rheumatology May 2024Patients with rheumatoid arthritis (RA) are at risk of developing interstitial lung disease (ILD), which is associated with high mortality. Screening tools based on risk...
BACKGROUND
Patients with rheumatoid arthritis (RA) are at risk of developing interstitial lung disease (ILD), which is associated with high mortality. Screening tools based on risk factors are needed to decide which patients with RA should be screened for ILD using high-resolution computed tomography (HRCT). The ANCHOR-RA study is a multi-national cross-sectional study that will develop a multivariable model for prediction of RA-ILD, which can be used to inform screening for RA-ILD in clinical practice.
METHODS
Investigators will enrol consecutive patients with RA who have ≥ 2 of the following risk factors for RA-ILD: male; current or previous smoker; age ≥ 60 years at RA diagnosis; high-positive rheumatoid factor and/or anti-cyclic citrullinated peptide (titre > 3 x upper limit of normal); presence or history of certain extra-articular manifestations of RA (vasculitis, Felty's syndrome, secondary Sjögren's syndrome, cutaneous rheumatoid nodules, serositis, and/or scleritis/uveitis); high RA disease activity in the prior 12 months. Patients previously identified as having ILD, or who have had a CT scan in the prior 2 years, will not be eligible. Participants will undergo an HRCT scan at their local site, which will be assessed centrally by two expert radiologists. Data will be collected prospectively on demographic and RA-related characteristics, patient-reported outcomes, comorbidities and pulmonary function. The primary outcomes will be the development of a probability score for RA-ILD, based on a multivariable model incorporating potential risk factors commonly assessed in clinical practice, and an estimate of the prevalence of RA-ILD in the study population. It is planned that 1200 participants will be enrolled at approximately 30 sites in the USA, UK, Germany, France, Italy, Spain.
DISCUSSION
Data from the ANCHOR-RA study will add to the body of evidence to support recommendations for screening for RA-ILD to improve detection of this important complication of RA and enable early intervention.
TRIAL REGISTRATION
clinicaltrials.gov NCT05855109 (submission date: 3 May 2023).
PubMed: 38773593
DOI: 10.1186/s41927-024-00389-4 -
Poultry Science Jan 2023Duck infectious serositis is an acute and infectious disease caused by Riemerella anatipestifer (R. anatipestifer) that leads to perihepatitis, pericarditis, meningitis,...
Duck infectious serositis is an acute and infectious disease caused by Riemerella anatipestifer (R. anatipestifer) that leads to perihepatitis, pericarditis, meningitis, and airbag inflammation in ducks, which causes serious economic losses to the global duck industry. The phoP/phoR is a novel 2-component signal transduction system first reported in gram-negative bacteria, of which phoP acts as a global regulator and virulence factor. In this study, the phoP gene from the R. anatipestifer YM strain was knocked out using homologous recombination technology and replaced with the spectinomycin resistance gene (Spec). The virulence of the R. anatipestifer YMΔphoP strain was reduced by approximately 47,000 times compared to that of the wild-type R. anatipestifer YM strain. Ducks were immunized with live R. anatipestifer YMΔphoP strain by subcutaneous inoculation at a dose of 10 to 10 CFU (0.2 mL per duck) and challenged with the wild-type R. anatipestifer YM strain 14 days later. The protection rate in the immunized group was 100%. The growth characteristics of ducks in the immunized and negative control groups were normal, and the research demonstrated R. anatipestifer YMΔphoP strain have suitable immunogenicity and protective effects. Thus, the study findings suggest that the novel R. anatipestifer YMΔphoP strain may provide a candidate for the development of a gene deletion activated vaccine against duck infectious serositis.
Topics: Animals; Bacterial Proteins; Ducks; Flavobacteriaceae Infections; Gene Deletion; Poultry Diseases; Riemerella; Serogroup; Serositis
PubMed: 36436377
DOI: 10.1016/j.psj.2022.102294 -
Clinical and Experimental Rheumatology 2020We occasionally encounter patients with familial Mediterranean fever (FMF) whose attacks are triggered by specific factors; however, information regarding these factors...
OBJECTIVES
We occasionally encounter patients with familial Mediterranean fever (FMF) whose attacks are triggered by specific factors; however, information regarding these factors is limited. Our purpose was to identify the factors that trigger febrile attacks in Japanese patients with FMF.
METHODS
Our retrospective study included 372 patients (229 women, 143 men) with FMF, who were diagnosed between April 2007 and June 2018. We retrospectively investigated clinical features, genetic variants, and the factors that the patients perceived to have triggered their attacks. Patients completed a questionnaire that included the following triggering factors, anxiety, psychological stress, tiredness, excitement, environmental change, and menstruation.
RESULTS
Of 372 patients, 180 (49.4%) reported some triggering factors. Psychological stress and tiredness were commonly reported factors regardless of sex; however, menstruation (39.7%, n=91) was the most commonly reported triggering factor in female patients with FMF. Menstrual-related patients had a younger age of onset and diagnosis, a higher frequency of peritonitis, and a higher rate of patients with endometriosis compared with the non-menstrual-related patients.
CONCLUSIONS
Gaining an understanding of these triggering factors could help to reduce attacks and educate the patients. Clinicians may need to consider FMF for patients who have fever and serositis that occurs with every menstrual period.
Topics: Colchicine; Familial Mediterranean Fever; Female; Fever; Humans; Japan; Male; Menstruation; Retrospective Studies
PubMed: 33200741
DOI: No ID Found -
Therapeutic Advances in Chronic Disease 2022This study evaluated the prognostic value of the multivariable risk assessment for systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH).
OBJECTIVE
This study evaluated the prognostic value of the multivariable risk assessment for systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH).
METHODS
A multicenter prospective cohort of SLE-associated PAH (CSTAR-PAH cohort) diagnosed based on right heart catheterization (RHC) was established. Baseline and follow-up records were collected. Three methods of risk assessment, including (1) the number of low-risk criteria, based on World Health Organization functional class (WHO FC), 6-min walking distance (6MWD), right atrial pressure (RAP), and cardiac index (CI); (2) the three-strata stratification based on the average risk score of four variables (WHO FC, 6MWD, RAP, and CI); and (3) the four-strata stratification based on COMPARE 2.0 model were applied. A risk-assessment method using three noninvasive low-risk criteria was applied at the first follow-up visit. Survival curves between patients with different risk groups were compared by Kaplan-Meier's estimation and log-rank test.
RESULTS
Three-hundred and ten patients were enrolled from 14 PAH centers. All methods of stratification at baseline and first follow-up significantly discriminated long-term survival. Survival rates were also significantly different based on the noninvasive risk assessment in first follow-up visit. Survival deteriorated with the escalation of risk from baseline to first follow-up. Patients with baseline serositis had a higher rate of risk improvement in their follow-up.
CONCLUSION
The risk assessment has a significant prognostic value at both the baseline and first follow-up assessment of SLE-associated PAH. A noninvasive risk assessment can also be useful when RHC is not available during follow-up. Baseline serositis may be a predictor of good treatment response in patients with SLE-associated PAH.
PubMed: 35898921
DOI: 10.1177/20406223221112528