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Modern Rheumatology Apr 2024To identify potential predictors of the disease course of systemic juvenile idiopathic arthritis (sJIA) at the time of diagnosis.
PURPOSE
To identify potential predictors of the disease course of systemic juvenile idiopathic arthritis (sJIA) at the time of diagnosis.
METHODS
This retrospective observational study was conducted in patients diagnosed with sJIA in our hospital between April 2009 and October 2023. The relationship between the disease course of sJIA patients and demographic, clinical, laboratory findings and complications were analyzed.
RESULTS
Of the 51 patients diagnosed with sJIA, 26 (51%) patients had monocyclic, 7 (13.7%) polycyclic and 18 (35.2%) persistent disease course. 3 (5.8%) patients had a persistent disease course with persistent arthritis developed flares with systemic manifestations during follow-up. The presence of arthritis, polyarticular involvement, and hip involvement at the time of diagnosis were associated with persistent disease course (p=0.009, p=0.003, p=0.003). Serositis and higher white blood cell and neutrophil counts at the time of diagnosis were associated with a monocyclic disease course (p=0.034, p=0.002, p=0.008). However, no significant correlation was found between macrophage activation syndrome (MAS) and disease course (p=1).
CONCLUSIONS
Systemic JIA patients with polyarthritis and hip involvement at disease onset may develop a persistent course. Although MAS is an important complication of sJIA, its effect on the course of the disease was not found in this study.
PubMed: 38590038
DOI: 10.1093/mr/roae036 -
Clinical and Experimental Medicine Dec 2023This study aimed to elucidate the immune status of systemic lupus erythematosus (SLE) patients with infections. We enrolled 253 SLE patients including 77 patients with...
This study aimed to elucidate the immune status of systemic lupus erythematosus (SLE) patients with infections. We enrolled 253 SLE patients including 77 patients with infections. Clinical features and immunological parameters were analyzed, with particular reference to neutrophil CD64 (nCD64) expression, myeloid-derived suppressor cells (MDSCs), activated T cells and multiple cytokines. Among the 77 SLE patients with infections, 32 patients (41.56%) developed fever and 20 patients (25.97%) developed serositis, which were higher compared to the non-infection group. A considerably higher level of nCD64 was found in the infection group (4.65 vs 1.01, P < 0.001). In addition, the infection group exhibited higher percentages of total MDSCs (6.99 vs 4.30%, P = 0.003), polymorphonuclear MDSCs (PMN-MDSCs) (P = 0.032) and monocytic MDSCs (M-MDSCs) (P = 0.015). T cells were more activated during infections, with an elevated level of IL-2R (P < 0.001). Specifically, higher percentages of CD4CD38 T cells (55.73 vs 50.17%, P = 0.036), CD8HLA-DR T cells (59.82 vs 47.99%, P < 0.001) and CD8CD38 T cells (68.59 vs 63.90%, P = 0.044) were identified in the infection group. Furthermore, the serum levels of IL-6, IL-8 and IL-10 were elevated in the infection group (all P < 0.001). Higher proportions of neutrophils, CD4 and CD8 T cells, and MDSCs were activated during infections in SLE patients. Additionally, the serum cytokines altered during infections, with noticeably elevated levels of IL-6, IL-8 and IL-10. Infections may lead to the amplification of immune alterations in SLE.
Topics: Humans; Interleukin-10; CD8-Positive T-Lymphocytes; Interleukin-6; Interleukin-8; Lupus Erythematosus, Systemic; Cytokines
PubMed: 37938465
DOI: 10.1007/s10238-023-01220-z -
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 -
Romanian Journal of Internal Medicine =... Mar 2024Castleman's disease (CD), known as angiofollicular lymph node hyperplasia, is an uncommon condition. The two most common histological subtypes are hyaline vascular and... (Review)
Review
INTRODUCTION
Castleman's disease (CD), known as angiofollicular lymph node hyperplasia, is an uncommon condition. The two most common histological subtypes are hyaline vascular and plasma cell. We performed a retrospective analysis to define the clinic-pathological features and survival of CD, which is quite rare focusing on the particularities of our series with a review of the recent literature.
METHODS
This is a retrospective study conducted in the department of internal medicine of Hedi Chaker hospital in Sfax, Tunisia over 25 years. The disease was histologically confirmed in all patients. For each file, we collected a set of data by filling in a pre-designed form.
RESULTS
18 patients were included. There were 8 men and 10 women with a mean age of 42.8 years. CD was monocentric in 5 cases (28%) and multicentric in 13 cases (72%). Clinically, peripheral adenopathy was present in 77.7% of patients and deep adenopathy in 72.2%. Systemic signs were found in 13 patients, including general condition (4.4%), fever (16.6%), serositis (27.7%), and skin involvement (33.3%). A biological inflammatory syndrome accompanied the clinical picture in 66% of patients. Abnormalities in the blood count were found in 12 cases (66%), with anemia in 11 cases, thrombocytosis in 3 cases, and hypereosinophilia in 3 cases. Cutaneous Kaposi's sarcoma was associated with Castleman's disease in 2 cases, Hodgkin's lymphoma, angioimmunoblastic T-cell lymphoma, and lymph node T-cell lymphoma were found in 1 case respectively. 3 of the patients had associated connective tissue diseases such as Sjögren's syndrome in 2 cases and rheumatoid arthritis in 1 case. HHV8 serology was positive in 1 case with a multicentric plasma cell form. Histologically, the plasma cell form represented 50% of cases, hyaline-vascular (39% of cases), and mixed (11% of cases). Therapeutically, high-dose corticosteroid therapy was initiated in 13 cases. As a second-line treatment, MOPP chemotherapy was used in 1 case due to transformation into Hodgkin's lymphoma, and biotherapy (rituximab) was used in 2 cases in the multicentric form. Surgical removal of superficial adenopathy was performed in 2 patients with monocentric CD.
CONCLUSION
: Castleman's disease (CD) is a non-malignant lymphoproliferation of localized or multicentric form with a wide and heterogeneous clinical spectrum. Diagnosis can be difficult due to the lack of clinical and radiological specificity. Management depends on the clinical form involving surgical and/or medical management.
Topics: Male; Humans; Female; Adult; Castleman Disease; Retrospective Studies; Hodgkin Disease; Tunisia; Lymphadenopathy; Lymphoma, T-Cell; HIV
PubMed: 37948573
DOI: 10.2478/rjim-2023-0028 -
Exertional leg pain represents a severe disease phenotype in childhood familial Mediterranean fever.Postgraduate Medicine Aug 2023Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease. Recurrent fever, serositis, and arthritis are common findings of the disease....
OBJECTIVES
Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease. Recurrent fever, serositis, and arthritis are common findings of the disease. In addition, musculoskeletal complaints such as exertional leg pain can be overlooked, although they are common and affect patients' quality of life. The aim of this study was to evaluate the frequency of exertional leg pain in pediatric FMF patients and to analyze the association of this finding with other characteristics of FMF.
METHODS
The files of FMF patients were retrospectively evaluated. The clinical characteristics and disease severity of the patients with exertional leg pain were compared with the patients without exertional leg pain. International severity scoring system for FMF (ISSF) and Mor severity score were used for assessment.
RESULTS
The study included 541 FMF patients (287 females), 149 (27.5%) with exertional leg pain. The median colchicine dosage was significantly higher in patients with exertional leg pain ( = 0.02), arthritis ( = 0.001) and arthralgia (p˂0.001) were encountered more frequently in the attacks of these patients. The median disease severity scores calculated by both Mor severity scale and ISSF were significantly higher in patients with exertional leg pain compared to those without (p˂0.001). In the group of patients with exertional leg pain, the mutation, either in one allele or in two alleles, was found to be significantly more common ( = 0.006 and p˂0.001, respectively).
CONCLUSIONS
Exertional leg pain in pediatric FMF patients is the component of moderate-to-severe disease course, and this may be considerably associated with the presence of mutation.
Topics: Female; Humans; Familial Mediterranean Fever; Retrospective Studies; Leg; Quality of Life; Phenotype; Arthralgia; Arthritis; Mutation
PubMed: 37309906
DOI: 10.1080/00325481.2023.2224649 -
Rheumatology (Oxford, England) Dec 2023To study the prevalence, correlates, and outcomes of GI manifestations in a prospectively enrolled nationwide cohort of SLE in India (INSPIRE).
OBJECTIVES
To study the prevalence, correlates, and outcomes of GI manifestations in a prospectively enrolled nationwide cohort of SLE in India (INSPIRE).
METHODS
It is an observational cohort study with analysis of the baseline database of the INSPIRE cohort with early outcomes assessed till April 10, 2023. Cases with GI manifestations as per the BILAG index were selected, pertinent clinical and laboratory data were retrieved for analysis. Patients with GI manifestations were compared with the rest of the cohort and factors associated with death were determined.
RESULTS
Of the 2503 patients with SLE enrolled in the INSPIRE cohort, 243(9.7%) had GI manifestations observed early in the disease course(1,0-3 months). Ascites(162,6.5%), followed by enteritis(41,1.6%), pancreatitis(35,1.4%) and hepatitis(24,0.9%) were the most prevalent manifestations.All patients received immunosuppressive therapy, and four patients required surgery. Twenty-nine patients died(11.9%), with uncontrolled disease activity(17,58.6%) and infection(6,20.7%) accounting for the majority of deaths. Low socioeconomic class[lower(Hazard Ratio (95% Confidence intervals- CI) 2.8(1.1-7.9); upper lower 7.5(2-27.7); reference as upper class] and SLEDAI 2K[1.06(1.02-1.11)] were associated with death in the GI group.GI manifestations were significantly associated with age[Odds Ratio & 95% CI 0.97(0.96-0.99)], pleural effusion[4.9(3.6-6.7)], thrombocytopenia[1.7(1.2-2.4)], myositis[1.7(1.1-2.7)], albumin[0.7(0.5-0.8)], alkaline phosphatase(ALP)[1.01(1.0-1.002)], low C3[1.9(1.3-2.5)], total bilirubin[1.2(1.03-1.3)], alopecia[0.62(0.5-0.96], elevated anti-dsDNA[0.5(0.4-0.8)], and anti-U1RNP antibody[0.8(0.5-0.7)] in model one; and age[0.97(0.96-0.99)], creatinine[1.2(1.03-1.4)], total bilirubin[1.2(1.03-1.3)], ALP[1.01(1.0-1.002)], albumin[0.6(0.5-0.7)], andanti-U1RNP antibody[0.6(0.5-0.8)] in model two in multivariate analysis compared with patients without GI features. The mortality was higher in the GI group(11.9% and 6.6%, p= 0.01) as compared with controls.
CONCLUSION
GI manifestations were observed in 9.7% of the cohort and were always associated with systemic disease activity and had higher mortality.
PubMed: 38059606
DOI: 10.1093/rheumatology/kead653 -
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 -
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 -
International Ophthalmology Jul 2023Familial Mediterranean fever (FMF) is an auto-immune disease characterized by fever and serositis. With this study, we aimed at comparing the retinal vascular structures...
INTRODUCTION
Familial Mediterranean fever (FMF) is an auto-immune disease characterized by fever and serositis. With this study, we aimed at comparing the retinal vascular structures of FMF patients and healthy individuals using optical coherence tomography angiography (OCTA) device.
MATERIALS AND METHODS
Sixty-nine patients with FMF and 52 healthy controls who were matched by age and sex, didn't have any comorbidities and were admitted to our clinic for routine ophthalmic examination were enrolled in the study. Sixty-nine eyes of 69 patients and 52 eyes of 52 healthy controls that were not disqualified due to the exclusion criteria were included in the study. OCTA images of the patients and controls were analyzed.
RESULTS
The mean average age (± SD) of 69 FMF patients included in the study was 39.86 ± 13.16. Forty-nine patients were female, and twenty patients were male. Total vascular density of deep capillary plexus (DCP) and vascular density of DCP parafovea of the FMF patients were found to be statistically significantly lower than those of the controls (p = 0.045, p = 0.034, respectively), while total retinal parafoveal thickness, total retinal perifoveal thickness, outer retinal parafoveal thickness and outer retinal perifoveal thickness were statistically significantly higher in the patient group compared with the control group (p = 0.012, p = 0.014, p = 0.013, p = 0.009, respectively).
CONCLUSIONS
This study found reduced vascular density and increased retinal thickness in patients with FMF compared to controls. Larger studies are required to further explore the effect of FMF on the retina.
Topics: Humans; Male; Female; Optic Disk; Familial Mediterranean Fever; Tomography, Optical Coherence; Retina; Retinal Vessels; Angiography; Fluorescein Angiography
PubMed: 36525225
DOI: 10.1007/s10792-022-02613-x -
Cureus Dec 2023Familial Mediterranean fever (FMF) is a hereditary, autosomal recessive auto-inflammatory disorder characterized by recurrent attacks of fever and serositis. While...
Familial Mediterranean fever (FMF) is a hereditary, autosomal recessive auto-inflammatory disorder characterized by recurrent attacks of fever and serositis. While arthritis, pleuritis, peritonitis, and pericarditis are common in FMF, large pericardial effusions with cardiac tamponade as a sequelae of FMF are considered rare. We report a case of an 83-year-old female with a history of FMF who presented with chest pain. She was found to have acute pericarditis complicated by hemodynamically significant pericardial tamponade that was subsequently treated with an urgent pericardiocentesis followed by colchicine.
PubMed: 38186433
DOI: 10.7759/cureus.50137