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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 -
Cureus Mar 2022Although systemic lupus erythematosus (SLE) can manifest differently in each patient, ascites is a rare first sign. The diagnosis of SLE can be easily missed when the...
Although systemic lupus erythematosus (SLE) can manifest differently in each patient, ascites is a rare first sign. The diagnosis of SLE can be easily missed when the initial presentation is uncommon. A 39-year-old male presented with painless abdominal fullness and was found to have ascites, thrombocytopenia, and anemia. He was initially diagnosed with Evan's syndrome and treated with prednisone. Upon follow-up, he had worsening thrombocytopenia and was found to have a positive antinuclear antibody, anti-double-stranded DNA antibody, and low complement levels consistent with SLE. He was treated with methylprednisolone, intravenous immunoglobulin, and mycophenolate mofetil with improvement.
PubMed: 35449635
DOI: 10.7759/cureus.23231 -
The Turkish Journal of Pediatrics 2020Familial Mediterranean fever (FMF) is an autosomal-recessive auto-inflammatory disorder characterized by recurrent episodes of fever with serositis. Sacroiliitis... (Review)
Review
BACKGROUND AND OBJECTIVES
Familial Mediterranean fever (FMF) is an autosomal-recessive auto-inflammatory disorder characterized by recurrent episodes of fever with serositis. Sacroiliitis associated with FMF is very rare, especially in children. We aimed to describe the demographic, clinical, laboratory features, and treatment responses of pediatric patients with FMF -related sacroiliitis.
METHODS
The study consisted of seven pediatric patients younger than 16 years, diagnosed with sacroiliitis associated with FMF between 2010 and 2017. Medical records of patients were retrospectively evaluated. Sacroiliitis was diagnosed based on magnetic resonance imaging. We also reviewed previous studies of FMF related sacroiliitis.
RESULTS
Five of the seven patients (male:female ratio of 5:2) had a M694V (homozygous) mutation, one patient had a M694V (heterozygous) mutation, and one patient had a V726A (heterozygous) mutation. All patients were HLA-B27 negative. One of the cases achieved remission with colchicine plus non-steroidal anti-inflammatory drug treatment, and one patient`s symptoms were managed by the addition of sulfasalazine. Four patients responded to etanercept treatment, and one patient`s symptoms were suppressed with canakinumab.
CONCLUSION
Sacroiliitis can be seen in pediatric FMF patients suffering with inflammatory back pain. This manifestation generally occurs in FMF patients who have M694V mutation. Etanercept could likely show a beneficial effect in patients who are resistant to disease modifying anti-rheumatic drugs and non-steroidal anti-inflammatory drugs. In addition, canakinumab treatment should be considered as a successful alternative therapy in this rare group of patients.
Topics: Child; Colchicine; Familial Mediterranean Fever; Female; Homozygote; Humans; Male; Mutation; Pyrin; Retrospective Studies; Sacroiliitis
PubMed: 32419408
DOI: 10.24953/turkjped.2020.02.002 -
Saudi Medical Journal Mar 2023To determine the prevalence of selected single nucleotide polymorphisms (rs1080985, rs28624811, rs1065852, rs28371725, and rs1135840) in () gene among Saudi systemic...
OBJECTIVES
To determine the prevalence of selected single nucleotide polymorphisms (rs1080985, rs28624811, rs1065852, rs28371725, and rs1135840) in () gene among Saudi systemic lupus erythematosus (SLE) patients and to investigate the association between the genetic variants and clinical features of SLE.
METHODS
This cross-sectional study was carried out on adult Saudi patients at King Khalid University Hospital, Riyadh, Saudi Arabia. Patients with confirmed SLE based on the 2012 Systemic Lupus International Collaborating Clinics classification criteria were included in the study. Peripheral blood was collected for genomic deoxyribonucleic acid extraction and TaqMan technologies were used for target genotyping. For statistical analysis, differences in genotype frequencies were determined using the Chi-square test, and the association between the variant genotypes and SLE features was evaluated using logistical regression models.
RESULTS
There were 107 participants included in this study. Overall, the most predominant (23.4%) recessive genotype was AA in rs28624811, and the least prevalent (1.9%) recessive genotype was TT in rs28371725. Moreover, the variant rs1080985 genotypes (GC or CC) were significantly associated with the presence of serositis manifestation (OR=3.15, =0.03), even after adjusting for age and gender. However, the dominant rs28624811 genotype (GG) was associated with renal involvement (OR=2.56, =0.03).
CONCLUSION
Systemic lupus erythematosus patients carrying variants might be considered at risk for certain manifestations of SLE. Further studies are needed to investigate the implication of these genetic variations in clinical outcomes and drug response.
Topics: Adult; Humans; Case-Control Studies; Cross-Sectional Studies; Cytochrome P-450 CYP2D6; Gene Frequency; Genetic Predisposition to Disease; Genotype; Lupus Erythematosus, Systemic; Polymorphism, Single Nucleotide; Saudi Arabia
PubMed: 36940959
DOI: 10.15537/smj.2023.44.3.20220581 -
BMC Pulmonary Medicine Nov 2021Chlamydia pneumoniae is a common cause of atypical community acquired pneumonia (CAP). The diagnostic approach of chlamydial infections remains a challenge. Diagnosis of...
BACKGROUND
Chlamydia pneumoniae is a common cause of atypical community acquired pneumonia (CAP). The diagnostic approach of chlamydial infections remains a challenge. Diagnosis of delayed chlamydial-associated complications, involving complex autoimmune pathophysiological mechanisms, is still more challenging. C. pneumoniae-related cardiac complications have been rarely reported, including cases of endocarditis, myocarditis and pericarditis.
CASE PRESENTATION
A 40-year old female was hospitalized for pleuropericarditis following lower respiratory tract infection. The patient had been hospitalized for CAP (fever, dyspnea, chest X-ray positive for consolidation on the left upper lobe) 5 weeks ago and had received ceftriaxone and moxifloxacin. Four weeks after her discharge, the patient presented with fever, shortness of breath and pleuritic chest pain and was readmitted because of pericardial and bilateral pleural effusions (mainly left). The patient did not improve on antibiotics and sequential introduction of colchicine and methylprednisolone was performed. The patient presented impressive clinical and laboratory response. Several laboratory and clinical assessments failed to demonstrate any etiological factor for serositis. Chlamydial IgM and IgG antibodies were positive and serial measurements showed increasing kinetics for IgG. Gold standard polymerase chain reaction of respiratory tract samples was not feasible but possibly would not have provided any additional information since CAP occurred 5 weeks ago. The patient was discharged under colchicine and tapered methylprednisolone course. During regular clinic visits, she remained in good clinical condition without pericardial and pleural effusions relapse.
CONCLUSIONS
C. pneumoniae should be considered as possible pathogen in case of pleuritis and/or pericarditis during or after a lower respiratory tract infection. In a systematic review of the literature only five cases of C. pneumoniae associated pericarditis were identified. Exact mechanisms of cardiovascular damage have not yet been defined, yet autoimmune pathways might be implicated.
Topics: Adult; Chlamydophila Infections; Chlamydophila pneumoniae; Female; Humans; Pericarditis
PubMed: 34809625
DOI: 10.1186/s12890-021-01743-9 -
Reumatologia 2020Familial Mediterranean fever (FMF) is characterized by recurrent, self-limiting attacks of fever and serositis. Nutrition is very important in the management of chronic...
OBJECTIVES
Familial Mediterranean fever (FMF) is characterized by recurrent, self-limiting attacks of fever and serositis. Nutrition is very important in the management of chronic diseases. Previous studies suggested that salty and fatty diet cause inflammation, therefore we aimed to investigate the effects of dietary self-efficacy and behavior about low-salt or low-fat diet on disease course in children with FMF.
MATERIAL AND METHODS
This cross-sectional study included patients aged between 10-18 years, diagnosed in our department and admitted between June 2019 and September 2019. Demographic and clinical properties were obtained from the medical files of the patients. Children's Dietary Self-Efficacy Scale (CDSS) and Health Behavior Questionnaire (HBQ) - Diet Behavior Scale (DBS) were performed for dietary self-efficacy and behavior about preferring low-salt or low-fat diet. Clinical features were compared between patients, which were grouped according to the sum of these two scales, with a cut-off score of 5.
RESULTS
The mean age of 74 FMF patients (44 females, 34 males), included in the study, was 14.6 ±2.82 years. Median CDSS and DBS scores of the patients were 5 (minimum -6, maximum 14) and 0 (minimum -10, maximum 12), respectively. According to the sum of these two scales, 39 (52.7%) patients who had scored at least 5, had a statistically higher rate of complete response to colchicine. The remaining clinical parameters were similar between these two groups.
CONCLUSIONS
Low-salt or low-fat diet may be an adjuvant modification in the management of children with FMF. Further studies are needed to clarify the role of low-salt or low-fat diet in FMF pathogenesis.
PubMed: 32476680
DOI: 10.5114/reum.2020.95361 -
Frontiers in Microbiology 2022is an important duck pathogen responsible for septicemia and infectious serositis, which has caused great economic losses to the duck industry. Phenylalanine-arginine...
is an important duck pathogen responsible for septicemia and infectious serositis, which has caused great economic losses to the duck industry. Phenylalanine-arginine β-naphthylamide (PAβN) is an efflux pump inhibitor, which mainly reduces the efflux effect by competing with antibiotics for efflux pump channels. Here, we found that strain GD2019 showed resistances to gentamicin, amikacin, kanamycin, and neomycin. Notably, PAβN could significantly reduce the Minimal inhibitory concentrations (MICs) of neomycin on the GD2019 strain. Moreover, PAβN combined with neomycin significantly decreased bacterial loads, relieved pathological injury and increase survival rate ( < 0.05) for the ducks lethally challenged by the GD2019 strain. Therefore, our results suggested, and PAβN could reduce neomycin-resistant of Importantly, finding of this study provide a new approach for treating antibiotic-resistant infection.
PubMed: 36713163
DOI: 10.3389/fmicb.2022.985789 -
World Journal of Clinical Pediatrics Mar 2024Lung damage in systemic juvenile arthritis (sJIA) is one of the contemporary topics in pediatric rheumatology. Several previous studies showed the severe course and...
BACKGROUND
Lung damage in systemic juvenile arthritis (sJIA) is one of the contemporary topics in pediatric rheumatology. Several previous studies showed the severe course and fatal outcomes in some patients. The information about interstitial lung disease (ILD) in the sJIA is scarce and limited to a total of 100 cases.
AIM
To describe the features of sJIA patients with ILD in detail.
METHODS
In the present retrospective cohort study, information about 5 patients less than 18-years-old with sJIA and ILD were included. The diagnosis of sJIA was made according to the current 2004 and new provisional International League of Associations for Rheumatology criteria 2019. ILD was diagnosed with chest computed tomography with the exclusion of other possible reasons for concurrent lung involvement. Macrophage activation syndrome (MAS) was diagnosed with HLH-2004 and 2016 EULAR/ACR/PRINTO Classification Criteria and hScores were calculated during the lung involvement.
RESULTS
The onset age of sJIA ranged from 1 year to 10 years. The time interval before ILD ranged from 1 mo to 3 years. The disease course was characterized by the prevalence of the systemic features above articular involvement, intensive rash (100%), persistent and very active MAS (hScore range: 194-220) with transaminitis (100%), and respiratory symptoms (100%). Only 3 patients (60%) developed a clubbing phenomenon. All patients (100%) had pleural effusion and 4 patients (80%) had pericardial effusion at the disease onset. Two patients (40%) developed pulmonary arterial hypertension. Infusion-related reactions to tocilizumab were observed in 3 (60%) of the patients. One patient with trisomy 21 had a fatal disease course. Half of the remaining patients had sJIA remission and 2 patients had improvement. Lung disease improved in 3 patients (75%), but 1 of them had initial deterioration of lung involvement. One patient who has not achieved the sJIA remission had the progressed course of ILD. No cases of hyper-eosinophilia were noted. Four patients (80%) received canakinumab and one (20%) tocilizumab at the last follow-up visit.
CONCLUSION
ILD is a severe life-threatening complication of sJIA that may affect children of different ages with different time intervals since the disease onset. Extensive rash, serositis (especially pleuritis), full-blown MAS with transaminitis, lymphopenia, trisomy 21, eosinophilia, and biologic infusion reaction are the main predictors of ILD. The following studies are needed to find the predictors, pathogenesis, and treatment options, for preventing and treating the ILD in sJIA patients.
PubMed: 38596441
DOI: 10.5409/wjcp.v13.i1.88912 -
Case Reports in Nephrology 2022A 32-year-old female was admitted to our institution with thrombocytopenia, fever, serositis, hepatosplenomegaly, diffuse lymphadenopathy, and renal insufficiency. A...
A 32-year-old female was admitted to our institution with thrombocytopenia, fever, serositis, hepatosplenomegaly, diffuse lymphadenopathy, and renal insufficiency. A diagnosis of systemic lupus erythematosus was made. Due to recalcitrant thrombocytopenia, serositis, and renal insufficiency methylprednisolone was prescribed in high doses. In addition to proteinuria and hematuria, she was found to have uric acid crystals in her urinalysis. A serum uric acid was found elevated at 18 mg/dL. Rasburicase infusions were started. Within 5 days of commencing rasburicase and continuing high-dose methylprednisolone, her serum creatinine normalized and proteinuria resolved. The microhematuria disappeared within 2 weeks of beginning rasburicase. The rapid reversal of renal insufficiency and all urinary abnormalities after the start of rasburicase infusions suggests that the renal injury was most likely due to uric acid-mediated renal injury and not lupus nephritis. Our case illustrates the co-occurrence of 2 distinct clinical entities, one common for the patient's age, sex, and foremost clinical findings, while the other uncommon and unexpected, but both associated to kidney injury. Clinicians must be aware that careful evaluation of symptoms and laboratory tests is needed to make a thorough differential diagnosis and provide the right treatment at the most opportune moment.
PubMed: 35676927
DOI: 10.1155/2022/3833649 -
Asian Pacific Journal of Allergy and... Dec 2021Juvenile systemic lupus erythematosus (JSLE) and adult SLE (ASLE) patients present with different clinical manifestations, but it is unknown if there are differences in...
BACKGROUND
Juvenile systemic lupus erythematosus (JSLE) and adult SLE (ASLE) patients present with different clinical manifestations, but it is unknown if there are differences in their antinuclear autoantibody (ANA) profiles or if staining patterns are associated with specific autoantibodies and clinical manifestations.
OBJECTIVE
To determine whether distinct types and numbers of ANA-staining patterns are associated with specific autoantibodies and clinical manifestations in JSLE and ASLE patients.
METHODS
A retrospective study was performed in Thai children (n = 146) and adults (n = 180) diagnosed with SLE using the Systemic Lupus International Collaborating Clinics classification criteria.
RESULTS
JSLE patients with a homogeneous pattern of staining and anti-dsDNA or anti-nucleosome antibodies in serum, developed renal involvement, leukopenia and acute/subacute cutaneous LE. Coarse speckled pattern with anti-RNP or anti-Sm showed thrombocytopenia and renal involvement in JSLE patients, but leukopenia in both groups. JSLE patients with fine-coarse speckled pattern and anti-RNP, anti-Sm, anti-Ro-52 or anti-SSA developed leukopenia, thrombocytopenia and renal involvement, whilst hemolytic anemia and serositis were commonly found in those with anti-Ro-52. Median SLEDAI score was higher in JSLE than ASLE patients.
CONCLUSIONS
Detailed ANA-staining patterns with specific autoantibodies show particular clinical manifestations and hence prompt further clinical investigations in both JSLE and ASLE patients. Therefore, this study demonstrates that distinct patterns of ANA staining and specific autoantibodies are clinically important in both children and adults with SLE.
Topics: Adult; Antibodies, Antinuclear; Autoantibodies; Child; Humans; Lupus Erythematosus, Systemic; Retrospective Studies
PubMed: 31012595
DOI: 10.12932/AP-211218-0465