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Iranian Journal of Immunology : IJI Jun 2022Adult-onset Still's disease (AOSD), which presents many non-specific symptoms, such as rash leukocytosis, spiking fever, and sore throat, is a rare auto inflammatory... (Review)
Review
BACKGROUND
Adult-onset Still's disease (AOSD), which presents many non-specific symptoms, such as rash leukocytosis, spiking fever, and sore throat, is a rare auto inflammatory disease. Other clinical features that are frequently observed include lymphadenopathy, arthralgia, serositis, splenomegaly, and hepatomegaly. Laboratory tests show high levels of C-reactive protein, ferritin, and erythrocyte sedimentation rate reflecting the systemic inflammatory process in AOSD patients.
CASE PRESENTATION
The patient was a middle-aged woman with a high fever (39.8 C), sore throat, rashes on limbs with pruritus, mainly at the joints (elbow, knee, and ankle), muscle aches, dizziness, infirmity, weakness, and poor appetite without arthralgia. The ferritin level was above 1500 (normal value: 14-233) ng/L. Antineutrophil, antinuclear antibodies, and rheumatoid factor were negative. Combining the symptoms such as fever, rash, stress-induced acute inflammation, arthritis, and ferritin levels, the patient was eventually diagnosed with adult Still's disease. She received methylprednisolone 40mg intravenously every 12 hours for one week. On the second week, the dose was reduced to 40mg in the morning and 20mg in the evening, and finally, the dose was reduced to 40mg oral intake in the morning and 8mg in the evening. After half a month of treatment, the patient's high fever and skin rashes subsided, and the other symptoms also gradually relieved.
CONCLUSIONS
A case of a middle-aged woman diagnosed with adult Still's disease is reported, and the possible pathogenesis and treatment of the disease are discussed. This case highlights the importance of early diagnosis and timely treatment of adult Still's disease to prevent potentially fatal complications.
Topics: Adult; Arthralgia; Exanthema; Female; Ferritins; Humans; Middle Aged; Pharyngitis; Still's Disease, Adult-Onset
PubMed: 35767894
DOI: 10.22034/iji.2022.92228.2137 -
Mediterranean Journal of Hematology and... 2023
PubMed: 38028394
DOI: 10.4084/MJHID.2023.063 -
The Journal of Veterinary Medical... Oct 2020Murine coronavirus (CoV) is a beta-CoV that infects mice by binding to carcinoembryonic antigen-related cell adhesion molecule 1. Intraperitoneal infection with the... (Review)
Review
Murine coronavirus (CoV) is a beta-CoV that infects mice by binding to carcinoembryonic antigen-related cell adhesion molecule 1. Intraperitoneal infection with the murine CoV strain JHM (JHMV) induces acute mild hepatitis in mice. While both innate and acquired immune responses play a significant role in the protection against murine CoV infection in mice, CD8 cytotoxic T lymphocytes (CTLs) and interferon-γ are essential for viral clearance in JHMV-induced hepatitis. In addition, CoVs are characterized by high diversity, caused by mutations, recombination, and gene gain/loss. 25V16G is an immune-escape JHMV variant, which lacks a dominant CTL epitope. By evading immune responses, 25V16G establishes persistent infections, leading to granulomatous serositis in interferon-γ-deficient mice. These examples of CoV-associated pathogenesis in mice might provide useful information on other CoV infections, including coronavirus disease 2019 (COVID-19).
Topics: Animals; Coronavirus Infections; Interferon-gamma; Mice; Murine hepatitis virus; T-Lymphocytes, Cytotoxic
PubMed: 32759577
DOI: 10.1292/jvms.20-0313 -
European Journal of Medical Research Oct 2022Familial Mediterranean fever (FMF) is an autosomal recessive disease associated with mutations in the Mediterranean fever gene (MEFV) that manifests with recurrent...
BACKGROUND
Familial Mediterranean fever (FMF) is an autosomal recessive disease associated with mutations in the Mediterranean fever gene (MEFV) that manifests with recurrent episodes of febrile serositis. Fabry's disease (FD) is an X-linked lysosomal storage disease caused by mutations in the alpha-galactosidase A gene and presents with a wide range of gastrointestinal, skin, vascular, renal and neurological manifestations. FMF and FD share similar manifestations, which may lead to misdiagnosis of one as the other; mostly FD is misdiagnosed as FMF. Moreover, various overlapping manifestations may stem from comorbidities, commonly coupled to FMF (such as Behcet's disease, inflammatory bowel disease, glomerulonephritis, fibromyalgia, and multiple sclerosis), as well as from colchicine adverse effects, which may add to the diagnostic confusion. Thus, we postulated that screening FMF for FD will lead to the identification of patients falsely diagnosed with FMF or who, in addition to FMF, suffer from FD that was previously missed.
METHODS
To identify missed FD among the FMF population, we performed chemical and genetic analyses for FD in blood samples obtained from a cohort of FMF patients followed in the specialized FMF center of our institution. To increase the likelihood of detecting patients with FD, we enriched the surveyed FMF population with patients exhibiting manifestations shared by patients with FD or who deviate from the typical FMF presentation.
RESULTS AND CONCLUSIONS
Of 172 surveyed FMF patients in a cohort derived from a clinic dedicated to FMF, none had FD. Thus, the postulation of increased odds for detecting FD in patients with FMF was not confirmed. Further exploration for FD in FMF population, is nevertheless recommended.
Topics: Humans; Familial Mediterranean Fever; Pyrin; Fabry Disease; alpha-Galactosidase; Colchicine; Mutation
PubMed: 36271470
DOI: 10.1186/s40001-022-00846-1 -
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 -
Journal of Surgical Case Reports Jul 2022We present two patients with right lower quadrant pain during the 36th week of pregnancy. In both cases, the challenges in diagnosing acute appendicitis in late...
We present two patients with right lower quadrant pain during the 36th week of pregnancy. In both cases, the challenges in diagnosing acute appendicitis in late pregnancy is underlined by misleading imaging results, revealing fluid in the lower abdomen, suggesting an appendicitis. Surgery was performed. Pre- and intraoperative gynecological examinations showed no signs of fetal distress. In patient 1, surgery revealed a torsion and necrosis of the right ovary and a 7-cm cyst of the fallopian tube. Open ovariectomy and appendectomy were performed. In patient 2, we saw a perforated appendicitis and cloudy ascites. Histology after appendectomy showed spots of endometriosis and serositis infiltrating into the appendix with signs of perforation at the tip. Patient 1 recovered after a short period of bowel paralysis. Patient 2 needed Caesarean section due to severe deceleration in the cardiotocograph and irregular uterine contractions. The newborn was kept in the neonatal ICU for 10 days.
PubMed: 35903664
DOI: 10.1093/jscr/rjac200 -
Viruses Mar 2024Feline infectious peritonitis (FIP) is a multisystemic, generally lethal immuno-inflammatory disease of domestic cats caused by an infection with a genetic variant of...
Feline infectious peritonitis (FIP) is a multisystemic, generally lethal immuno-inflammatory disease of domestic cats caused by an infection with a genetic variant of feline coronavirus, referred to as the FIP virus (FIPV). We leveraged data from four different antiviral clinical trials performed at the University of California, Davis. Collectively, a total of 60 client-owned domestic cats, each with a confirmed diagnosis of naturally occurring FIP, were treated with a variety of antiviral compounds. The tested therapies included the antiviral compounds GS-441524, remdesivir, molnupiravir and allogeneic feline mesenchymal stem/stroma cell transfusions. Four client-owned cats with FIP did not meet the inclusion criteria for the trials and were not treated with antiviral therapies; these cats were included in the data set as untreated FIP control cats. ELISA and Western blot assays were performed using feline serum/plasma or ascites effusions obtained from a subset of the FIP cats. Normalized tissue/effusion viral loads were determined in 34 cats by a quantitative RT-PCR of nucleic acids isolated from either effusions or abdominal lymph node tissue. Twenty-one cats were PCR "serotyped" (genotyped) and had the S1/S2 region of the coronaviral gene amplified, cloned and sequenced from effusions or abdominal lymph node tissue. In total, 3 untreated control cats and 14 (23.3%) of the 60 antiviral-treated cats died or were euthanized during (13) or after the completion of (1) antiviral treatment. Of these 17 cats, 13 had complete necropsies performed (10 cats treated with antivirals and 3 untreated control cats). We found that anticoronaviral serologic responses were persistent and robust throughout the treatment period, primarily the IgG isotype, and focused on the viral structural Nucleocapsid and Membrane proteins. Coronavirus serologic patterns were similar for the effusions and serum/plasma of cats with FIP and in cats entering remission or that died. Viral RNA was readily detectable in the majority of the cats in either abdominal lymph node tissue or ascites effusions, and all of the viral isolates were determined to be serotype I FIPV. Viral nucleic acids in cats treated with antiviral compounds became undetectable in ascites or abdominal lymph node tissue by 11 days post-treatment using a sensitive quantitative RT-PCR assay. The most common pathologic lesions identified in the necropsied cats were hepatitis, abdominal effusion (ascites), serositis, pancreatitis, lymphadenitis, icterus and perivasculitis. In cats treated with antiviral compounds, gross and histological lesions characteristic of FIP persisted for several weeks, while the viral antigen became progressively less detectable.
Topics: Humans; Cats; Animals; Feline Infectious Peritonitis; Ascites; Coronavirus Infections; Coronavirus, Feline; RNA, Viral; Antiviral Agents
PubMed: 38543827
DOI: 10.3390/v16030462 -
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 -
JGH Open : An Open Access Journal of... Jun 2020Anti-Tumor Necrosis Factor (TNF)-induced lupus (ATIL) is a distinct clinical entity, increasingly recognized in patients with inflammatory bowel disease treated with...
BACKGROUND AND AIMS
Anti-Tumor Necrosis Factor (TNF)-induced lupus (ATIL) is a distinct clinical entity, increasingly recognized in patients with inflammatory bowel disease treated with anti-TNF therapy. Our aims were to evaluate the incidence and clinical and serological markers of ATIL in this population.
METHODS
This observational cohort study reviewed 454 patient treatment courses with anti-TNF therapy (300 infliximab and 154 adalimumab). A diagnosis of ATIL was based on the most widely accepted diagnostic criteria: (i) a temporal relationship between symptoms and anti-TNF therapy and resolution of symptoms following cessation of the offending medication; (ii) at least one serologic American College of Rheumatology (ACR) criterion of Systemic Lupus Erythematosus (SLE); and (iii) at least one nonserological criterion such as arthritis, serositis, or rash. Clinical, demographic, and serological predictors were evaluated.
RESULTS
The incidence rate of ATIL was 5.7% for infliximab and 0.6% for adalimumab, which are much higher than previously reported postmarketing estimates. The median duration to diagnosis following commencement of anti-TNF therapy was 15 months (3-62 months). ATIL occurs more commonly patients that commence therapy at an older age (46.47 years ± 13.79 years vs. 38.85 years ± 14.75 years, = 0.033).
CONCLUSIONS
ATIL is a significant complication of anti-TNF therapy, affecting 1 in every 20 patients who commence infliximab. A panel of serological markers is useful to confirm the diagnosis and exclude other conditions that may mimic ATIL. Clinicians using anti-TNF medications should counsel patients about this potential risk and monitor for clinical manifestations of lupus during routine follow up.
PubMed: 32514462
DOI: 10.1002/jgh3.12291 -
Frontiers in Immunology 2020In a previous study, we have reported an increased plasma midkine (MK) and pleiotrophin (PTN) concentrations in patients with systemic lupus erythematosus (SLE) and the...
In a previous study, we have reported an increased plasma midkine (MK) and pleiotrophin (PTN) concentrations in patients with systemic lupus erythematosus (SLE) and the increase in MK and PTN associated with inflammatory cytokines interleukin (IL)-17 level and some clinical manifestations, suggesting the underlying association of MK and PTN with SLE. This study was conducted to investigate the association between common single-nucleotide polymorphisms (SNPs) in the and gene and SLE susceptibility. A total of 989 subjects (496 SLE patients and 493 healthy controls) were included and genotyped for three SNPs and seven SNPs in using improved multiple ligase detection reaction (iMLDR). Results have demonstrated no significant differences for genotype and allele frequencies in all 10 SNPs between SLE patients and healthy controls. Case-only analysis in SLE revealed that, in gene, the genotype frequency of AA/AG (rs35324223) was significantly lower in patients with photosensitivity than those without; the allele frequency of A/G (rs20542) was significantly higher in patients without serositis. In gene, the A/G allele frequency (rs322236), C/T allele frequency, and TT/CT genotype frequency (rs6970141) showed significantly increased results in patients with immunological disorder compared to those without. Furthermore, no significant differences in plasma MK and PTN concentrations with its SNPs genotypes were found. and SNPs showed no associations with SLE genetic susceptibility, but it may be associated with the course of this disease; further studies are needed to focus on the mechanism of and genes in the pathogenesis of SLE.
Topics: Adult; Asian People; Carrier Proteins; Case-Control Studies; China; Cohort Studies; Cytokines; Female; Gene Frequency; Genetic Association Studies; Genetic Predisposition to Disease; Genotype; Healthy Volunteers; Humans; Lupus Erythematosus, Systemic; Male; Middle Aged; Midkine; Polymorphism, Single Nucleotide
PubMed: 32153561
DOI: 10.3389/fimmu.2020.00110