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SAGE Open Medical Case Reports 2023Familial Mediterranean fever is a rare autosomal recessive autoinflammatory disorder prevalent in Middle Eastern populations, characterized by episodic abdominal pain....
Familial Mediterranean fever is a rare autosomal recessive autoinflammatory disorder prevalent in Middle Eastern populations, characterized by episodic abdominal pain. This case report presents a 34-year-old Egyptian man with severe lower abdominal pain, chest discomfort, and joint pain, along with a positive family history of familial Mediterranean fever but had no previous personal history of this condition. Blood work revealed leukocytosis with neutrophilia and elevated C-reactive protein and erythrocyte sedimentation rate. The patient received intravenous fluids, antiemetics, and analgesics before further evaluation. Diagnosis of familial Mediterranean fever relies on clinical symptoms, ethnic origin, and family history, supported by specific criteria. Typical familial Mediterranean fever attacks involve serositis-induced pain, recurrent episodes, short-duration fever (12 h to 3 days), and arthritis. Familial Mediterranean fever may mimic other acute abdominal conditions, warranting consideration, particularly in individuals from Mediterranean regions. Genetic testing is valuable in confirming familial Mediterranean fever diagnosis.
PubMed: 37654544
DOI: 10.1177/2050313X231195964 -
Clinical Case Reports Aug 2023Adult-onset Still's disease is a rare inflammatory condition with diverse clinical features. Yamaguchi criteria aid diagnosis, and pleural effusion and elevated ferritin...
KEY CLINICAL MESSAGE
Adult-onset Still's disease is a rare inflammatory condition with diverse clinical features. Yamaguchi criteria aid diagnosis, and pleural effusion and elevated ferritin levels are important markers. Steroids are the first-line treatment.
ABSTRACT
Adult-onset Still's disease (AOSD) is a rare systemic inflammatory condition with an unknown etiology. It is characterized by, spiking fever, arthritis, evanescent rash, sore throat, serositis, hepatomegaly, splenomegaly, and lymphadenopathy. It is a diagnosis of exclusion and has infections, systemic autoimmune and inflammatory rheumatic diseases, malignancy, and adverse drug reactions as its differential diagnosis. Because of these characteristics, diagnosis is frequently delayed, posing a significant challenge for physicians. While several classification criteria can be used to diagnose Still's disease, they have limitations in terms of sensitivity and specificity. The Yamaguchi criteria are considered the most sensitive and commonly used, requiring the presence of at least five characteristics, with at least two being major diagnostic criteria. Steroid therapy is the first-line treatment for AOSD patients. In this case report, we present a 56-year-old female patient who developed pleurisy a few months after a car accident, subsequently diagnosed with adult-onset Still's disease.
PubMed: 37614293
DOI: 10.1002/ccr3.7510 -
Frontiers in Microbiology 2023is an emerging swine pathogen with high prevalence worldwide. The main lesions caused are arthritis and polyserositis, and the clinical manifestation of the disease may...
INTRODUCTION
is an emerging swine pathogen with high prevalence worldwide. The main lesions caused are arthritis and polyserositis, and the clinical manifestation of the disease may result in significant economic losses due to decreased weight gain and enhanced medical costs. We aimed to compare two challenge routes to induce infection using the same clinical isolate.
METHODS
Five-week-old, Choice hybrid pigs were inoculated on 2 consecutive days by intravenous route (Group IV-IV) or by intravenous and intraperitoneal routes (Group IV-IP). Mock-infected animals were used as control (control group). After the challenge, the clinical signs were recorded for 28 days, after which the animals were euthanized. Gross pathological and histopathological examinations, PCR detection, isolation, and genotyping of the re-isolated sp. and culture of bacteria other than sp. were carried out. The ELISA test was used to detect anti- immunoglobulins in the sera of all animals.
RESULTS
Pericarditis and polyarthritis were observed in both challenge groups; however, the serositis was more severe in Group IV-IV. Statistically significant differences were detected between the challenged groups and the control group regarding the average daily weight gain, pathological scores, and ELISA titers. Additionally, histopathological scores in Group IV-IV differed significantly from the scores in the control group. All re-isolated strains were the same or a close genetic variant of the original challenge strain.
DISCUSSION
Our results indicate that both challenge routes are suitable for modeling the disease. However, due to the evoked more severe pathological lesions and the application being similar to the hypothesized natural route of infection in Group IV-IV, the two-dose intravenous challenge is recommended by the authors to induce serositis and arthritis associated with infection.
PubMed: 37601388
DOI: 10.3389/fmicb.2023.1209119 -
Cureus Jun 2023Introduction Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with multisystemic involvement. The clinical presentation and immunological findings of...
Introduction Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with multisystemic involvement. The clinical presentation and immunological findings of SLE patients from different regions in Saudi Arabia have been studied. There have been no studies on the clinical manifestations of SLE in patients in Saudi Arabia's southern region. This article aims to explore the clinical manifestations of SLE in a tertiary center in the southern region of Saudi Arabia. Methods A retrospective study was carried out on 108 SLE patients who were seen in the rheumatology clinic at Aseer Central Hospital over six months from January 2022 to June 2022. Patients' demographics, clinical and serological characteristics, and therapeutic data were reviewed. Results The male-to-female ratio was 1:12.5, with a mean age at presentation of 28.6 ± 10 years. The mean disease duration was 9.06 ± 5.96 years. Mucocutaneous and musculoskeletal manifestations were the most common, accounting for 76% and 57% of all cases, respectively. Neuropsychiatric involvement and lupus nephritis were present in 29% and 31% of patients, respectively. The hematological abnormalities that were present included anemia (60%), leukopenia (37%), and thrombocytopenia (15%). Antinuclear antibody (ANA) was detected in 100%, anti-double-stranded DNA (anti-dsDNA) antibody in 55%, anti-Smith antibody in 13%, and hypocomplementemia in 52% of patients. Hydroxychloroquine was received by 98% and oral steroids by 41% of the patients. Other drugs include azathioprine (23%), mycophenolate mofetil (15%), methotrexate (23%), belimumab (9%), cyclophosphamide (10%), and rituximab (6%). Conclusion The main clinical features of our patients were in parallel with previous studies in Saudi Arabia as well as in Arab countries. We found a lower prevalence of lupus nephritis, serositis, and anti-dsDNA antibody. Further multicenter studies are required to investigate the long-term outcome and survival of SLE patients.
PubMed: 37525814
DOI: 10.7759/cureus.41215 -
Scientific Reports Jul 2023The aim of this study was to investigate the characteristics of CD4CD40 T cells (Th40 cells) in Chinese systemic lupus erythematosus (SLE) patients. Flow cytometry was...
The aim of this study was to investigate the characteristics of CD4CD40 T cells (Th40 cells) in Chinese systemic lupus erythematosus (SLE) patients. Flow cytometry was used to identify the percentage of Th40 cells in peripheral blood from 24 SLE patients and 24 healthy individuals and the level of IL-2, IL-4, IL-6, IL-10, IFN-r, and TNF-α in serum (22 cases) from the SLE patients. Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2000) was used to assess the SLE disease active state. The percentage of Th40 cells in T cells from SLE patients (19.37 ± 17.43) (%) was significantly higher than that from healthy individuals (4.52 ± 3.16) (%) (P < 0.001). The percentage of Th40 cells was also positively associated with SLEDAI-2000 (P = 0.001) and negatively associated with complement C3 (P = 0.007). The Th40 cell percentage was different in SLE patients with different organs involved. The Th40 cell percentage in SLE patients with lupus serositis (29.29 ± 22.19) was significantly higher than that in patients without serositis (13.41 ± 10.79; P = 0.040), and the percentage in SLE patients with lupus pneumonia involvement (29.11 ± 11.88) was significantly higher than that in patients without lupus pneumonia (16.80 ± 17.99; P = 0.043). After 4 weeks treatment, the Th40 cell percentage decreased significantly (P = 0.005). However, Th40 cell expression was not related to cytokines (IL-2, IL-4, IL-6, IL-10, IFN-r, and TNF-α; P > 0.05). A significantly higher percentage of Th40 cells was found in SLE patients, and the Th40 cell percentage was associated with SLE activity. Thus, Th40 cells may be used as a predictor for SLE disease activity and severity and therapeutic efficacy.
Topics: Humans; Interleukin-10; Interleukin-6; Tumor Necrosis Factor-alpha; Interleukin-2; Interleukin-4; Serositis; Lupus Erythematosus, Systemic; Pneumonia
PubMed: 37400575
DOI: 10.1038/s41598-023-37749-y -
Poultry Science Jul 2023Infectious serositis is a common disease caused by Riemerella anatipestifer (R. anatipestifer) in ducks, characterized by respiratory distress, septicemia, and...
Epidemiological investigation and drug resistance characteristics of Riemerella anatipestifer strains from large-scale duck farms in Shandong Province, China from March 2020 to March 2022.
Infectious serositis is a common disease caused by Riemerella anatipestifer (R. anatipestifer) in ducks, characterized by respiratory distress, septicemia, and neurological symptoms. In this study, 1,020 samples (brain and liver) were collected from ducks with suspected R. anatipestifer infection from March 2020 to March 2022 in Shandong Province, of which 171 R. anatipestifer strains were identified by PCR and isolation culture. The serotype of all strains was analyzed, and 74 strains were subjected to drug sensitivity tests and drug resistance genes detection. The results showed that the overall prevalence rate of R. anatipestifer in Shandong Province was 16.7% (171/1,020), with most strains coming from brain samples of ducklings under 3-mo old collected from September to December each year. Histopathological examination showed that heart vessels of the diseased duck were highly dilated and filled with red blood cells, with obvious fibrin exudates outside the pericardium, and fatty degeneration of liver cells. There were 45 strains of serotype 1, 45 strains of serotype 2, 2 strains of serotype 4, 33 strains of serotype 6, 44 strains of serotype 7, and 2 strains of serotype 10. The minimum inhibitory concentration (MIC) of 10 common antibiotics against 74 representative strains was determined by the agar dilution method. It was found that 74 strains had the most severe resistance to gentamicin (77%) and fully susceptible to ceftriaxone, but the 81.1% isolated strains were multidrug resistant. Resistance genes testing of 74 R. anatipestifers showed that tetracycline resistance gene tet X had the highest detection rate of 95.9%, followed by macrolide resistance gene ermF with 77%, and the rate of β-lactam resistance gene bla is the lowest (10.8%). The animal experiment of 4 R. anatipestifer strains with different serotypes showed that they had strong pathogenicity to 7-day-old ducklings, which could cause nervous symptoms, and the mortality rate was 58% to 70%. The autopsy showed obvious pathological changes. These findings of this study on R. anatipestifer will help us to understand the latest prevalence, drug resistance characteristics, and pathogenicity of R. anatipestifer in Shandong, China, and provide a scientific guide for the treatment and control of the disease.
Topics: Animals; Anti-Bacterial Agents; Chickens; Drug Resistance, Bacterial; Ducks; Farms; Flavobacteriaceae Infections; Macrolides; Poultry Diseases; Riemerella
PubMed: 37209657
DOI: 10.1016/j.psj.2023.102759 -
Joint Bone Spine Sep 2023To evaluate the impact of obesity in patients with adult-onset Still's disease (AOSD) and to assess their clinical characteristics and disease outcomes.
OBJECTIVES
To evaluate the impact of obesity in patients with adult-onset Still's disease (AOSD) and to assess their clinical characteristics and disease outcomes.
METHODS
The clinical features of AOSD patients with a body mass index (BMI)≥30 were assessed among those included in the multicentre Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale (GIRRCS) cohort.
RESULTS
Out of 139 AOSD patients, who had BMI registered in our database, 26 (18.7%) had a BMI≥30. A lower rate of sore throat (P<0.05), pericarditis (P<0.05), and pleuritis (P<0.05) was shown in obese patients. Additionally, obese patients showed higher values of C-reactive protein (CRP) (P<0.05) and ferritin (P<0.05) than others. Furthermore, obese patients were characterised by biologic disease-modifying antirheumatic drug (bDMARD) failure in subsequent follow-up (P<0.05). They also presented higher rate of comorbidity than non-obese patients (P<0.05). Finally, obesity predicted the presence of a chronic disease course in both univariate (HR: 1.72, 95%CI: 1.03-2.51, P<0.05) and multivariate analyses (HR: 1.85, 95%CI: 1.45-2.89, P<0.05). Obesity was also a significant predictor of bDMARD failure in AOSD patients in both univariate (HR: 3.03, 95%CI: 1.42-6.45, P<0.01) and multivariate analyses (HR: 3.59, 95%CI: 1.55-8.27, P<0.01).
CONCLUSION
Obese patients at the time of diagnosis of the disease were characterised by a lower prevalence of sore throat, serositis, as well as by higher values of CRP and ferritin. Obesity was also a predictive factor for a chronic disease course and bDMARD failure, thus highlighting a subset of patients with AOSD to be carefully managed.
Topics: Adult; Humans; Still's Disease, Adult-Onset; Antirheumatic Agents; C-Reactive Protein; Obesity; Disease Progression; Ferritins
PubMed: 37080283
DOI: 10.1016/j.jbspin.2023.105576 -
Clinical and Experimental Medicine Sep 2023Despite an essential differential diagnosis for fever of unknown origin (FUO) in young adults, adult-onset Still's disease (AOSD) is infrequently considered and remained...
Despite an essential differential diagnosis for fever of unknown origin (FUO) in young adults, adult-onset Still's disease (AOSD) is infrequently considered and remained underdiagnosed in low-middle-income countries. The present study analyzed the clinical, serological, radiological, and pathological characteristics of AOSD presented as FUO in India. A hospital-based retrospective study of patients aged > 13 years admitted with FUO and later diagnosed with AOSD in Postgraduate Institute of Medical Education and Research, Chandigarh (India), was conducted between January 2014 and December 2020. Petersdorf and Beeson's criteria were used to define FUO. The diagnosis of AOSD was made based on Yamaguchi's criteria. Twenty-seven patients (median age 26 years, 14 females) were enrolled. All presented with intermittent fever with a median duration of 10 weeks. The typical features of AOSD at admission were arthralgia (n = 24), hepatosplenomegaly (n = 21), spiking fever ≥ 39 °C (n = 19), lymphadenopathy (n = 18), typical rash (n = 17), and sore throat (n = 11). Leukocytosis (n = 25) and neutrophilia (n = 19) were frequent. Hyperferritinemia was universal (range, 700-145,003 ng/ml; ≥ 2000, n = 23). At admission, AOSD was suspected in only nine FUO cases, while tuberculosis (n = 16), undifferentiated connective tissue disorder (n = 14), and lymphoproliferative disorder (n = 11) were common diagnostic possibilities. Crispin et al. clinical scale detected AOSD in only 15 (55.5%) FUO patients. Whole-body imaging (n = 27), including fluorodeoxyglucose positron emission tomography (n = 12), demonstrated reticuloendothelial organ-system involvement and serositis. Seventeen (63%) patients had macrophage activation syndrome at the time of AOSD diagnosis. AOSD FUO presents with typical but nonspecific features; thus, early differentiation from common causes (e.g., tuberculosis, lymphoma) is difficult. Macrophage activation syndrome is common in AOSD with FUO presentation.
Topics: Female; Young Adult; Humans; Adult; Fever of Unknown Origin; Retrospective Studies; Still's Disease, Adult-Onset; Macrophage Activation Syndrome; Diagnosis, Differential
PubMed: 36178600
DOI: 10.1007/s10238-022-00903-3 -
Irish Journal of Medical Science Aug 2023Familial Mediterranean fever (FMF) is the most common autoinflammatory disease characterized by short, repeated, and self-limiting attacks of fever and serositis....
BACKGROUND
Familial Mediterranean fever (FMF) is the most common autoinflammatory disease characterized by short, repeated, and self-limiting attacks of fever and serositis. Subclinical inflammation can persist in the periods with no symptoms and result in amyloidosis even with colchicine treatment. Neopterin and calprotectin have been considered essential players in inflammation and immune response.
AIM
The study was aimed to measure serum levels of neopterin and calprotectin in patients with FMF in the attack-free period.
METHODS
A total of 160 participants were recruited from the rheumatology department in this single-center, case-control study. Individuals having the inclusion criteria were divided into healthy controls (n = 80) and FMF (n = 80). The laboratory data were acquired from the electronic registration database. Serum calprotectin and neopterin were measured with ELISA test kits. FMF patients and healthy controls' laboratory findings were compared.
RESULTS
FMF patients' serum red cell distribution width (RDW), calprotectin, and neopterin values were significantly higher compared to healthy controls. There were no statistically significant differences between calprotectin and neopterin regarding gender, family history, and colchicine response of the FMF patients.
CONCLUSIONS
Calprotectin, neopterin, and RDW can be valuable marker candidates to be used in the follow-up of subclinical inflammation in FMF patients.
Topics: Humans; Familial Mediterranean Fever; Case-Control Studies; Neopterin; Leukocyte L1 Antigen Complex; Inflammation; Biomarkers; Colchicine
PubMed: 36163581
DOI: 10.1007/s11845-022-03173-w