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Modern Rheumatology May 2020Most patients with familial Mediterranean fever (FMF) have their first attack at age < 20 years. Information about late-onset (age ≥40 years) FMF is limited. We aimed...
Most patients with familial Mediterranean fever (FMF) have their first attack at age < 20 years. Information about late-onset (age ≥40 years) FMF is limited. We aimed to evaluate the demographic, clinical, and genetic characteristics of late-onset FMF patients in the Japanese population. We retrospectively analyzed 292 patients with FMF. Patients were divided into three groups according to age of disease onset: Group I, ≤19 years; Group II, 20-39 years; and Group III, ≥40 years. Of 292 patients, 44 (15.1%) experienced their first attack at age ≥40 years. While high fever (97.7%) and arthritis (45.5%) were common symptoms in Group III patients, peritonitis (40.9%) and pleuritis (25.0%) were significantly lower than in other groups. The frequency of patients carrying p.M694I (18.2%), which is the most representative mutation in Japan, was significantly lower in Group III than in Group I. The response to colchicine therapy was good (95.1%) and similar in all groups. In Japan, more patients than expected had late-onset FMF. They had a milder form of disease, with less frequent peritonitis and pleuritis. The response to colchicine treatment was good. Clinicians should consider FMF for patients with unexplained recurrent febrile episodes, regardless of age.
Topics: Adolescent; Adult; Age of Onset; Arthritis; Colchicine; Familial Mediterranean Fever; Female; Fever; Humans; Japan; Male; Mutation; Pleurisy; Pyrin
PubMed: 31116049
DOI: 10.1080/14397595.2019.1621440 -
Journal of Oncology 2021Pyothorax-associated lymphoma (PAL) is a rare disease developing from a long-term pleural cavity inflammation. Most reported PAL cases have a history of artificial...
Pyothorax-associated lymphoma (PAL) is a rare disease developing from a long-term pleural cavity inflammation. Most reported PAL cases have a history of artificial pneumothorax. However, the clinical features of artificial pneumothorax-unrelated PAL remain largely unknown. Here, we reported two PAL cases diagnosed from our center in the past ten years. One case developed from asymptomatic pyothorax after pneumonectomy with a latency of 28 years, while the other case showed a relatively short latency of one year. Then we reviewed the literature of artificial pneumothorax-unrelated PAL by searching PubMed and Google Scholar from 2007. In total, nine artificial pneumothorax-unrelated PAL cases were found, predominantly in old male with median age of 76 years (ranging from 51 to 88). Most cases were diagnosed with diffuse large B-cell lymphoma (DLBCL) ( = 8, 88.9%) and had evidence of Epstein-Barr virus (EBV) infection ( = 6, 66.7%) or tuberculous pleurisy ( = 5, 55.6%). Notably, four cases (44.4%) had short intervals (no more than two years) between pleuritis and PAL. Regarding the overall survival, one-third cases survived more than 5 years after the diagnosis of PAL. In conclusion, the features of artificial pneumothorax-unrelated PAL are comparable with the classic type of PAL, except for some patients with short duration of pleuritis, and need to be identified. Treatment guideline of DLBCL is recommended for the management of PAL.
PubMed: 33564306
DOI: 10.1155/2021/3869438 -
Respiratory Medicine Apr 2021Nitric oxide (NO) regulates various physiological and pathophysiological functions in the lungs. However, there is much less information about the effects of NO in the... (Review)
Review
Nitric oxide (NO) regulates various physiological and pathophysiological functions in the lungs. However, there is much less information about the effects of NO in the pleura. The present review aimed to explore the available evidence regarding the role of NO in pleural disease. NO, has a double-edged role in the pleural cavity. It is an essential signaling molecule mediating various physiological cell functions such as lymphatic drainage of the serous cavities, the immune response to intracellular multiplication of pathogens, and downregulation of neutrophil migration, but also induces genocytotoxic and mutagenic effects when present in excess. NO is implicated in the pathogenesis of asbestos-related or exudative pleural disease and mesothelioma. From a clinical point of view, the fraction of exhaled NO has been suggested as a potential non-invasive tool for the diagnosis of benign asbestos-related disorders. Under experimental conditions, NO-mimetics were found to attenuate hypoxia-induced therapy resistance in mesothelioma. Similarly, hybrid agents consisting of an NO donor coupled with a parent anti-inflammatory drug showed an enhancement of the anti-inflammatory activity of anti-inflammatory drugs. However, given the paucity of research work performed over the last years in this area, further research should be undertaken to establish reliable conclusions with respect to the feasibility of determining or targeting the NO signaling pathway for pleural disease diagnosis and therapeutic management.
Topics: Anti-Inflammatory Agents; Asbestos; Biomarkers; Humans; Mesothelioma; Nitric Oxide; Nitric Oxide Donors; Pleura; Pleural Diseases; Signal Transduction
PubMed: 33662805
DOI: 10.1016/j.rmed.2021.106350 -
The Journal of Medical Investigation :... 2021The incidence rate of pulmonary nontuberculous mycobacterial disease (PNTMD) in Japan is the highest among major industrialized nations. Although the typical clinical... (Review)
Review
The incidence rate of pulmonary nontuberculous mycobacterial disease (PNTMD) in Japan is the highest among major industrialized nations. Although the typical clinical course and radiological manifestations of PNTMD are different from those of pulmonary tuberculosis (TB), confusion about these mycobacterial diseases leads to a diagnostic pitfall. Diagnostic challenges include the coexistence of Mycobacterium tuberculosis (MTB) and nontuberculous mycobacteria (NTM), false positives for NTM in MTB nucleic acid amplification tests, microbial substitution, and abnormal radiological manifestations caused by NTM. Features of extrapulmonary NTM diseases, such as pleurisy, vertebral osteomyelitis, and disseminated disease, are different from the corresponding tuberculous diseases. Moreover, the immunological background of the patient (status of human immunodeficiency virus infection with or without antiviral therapy, continuation or discontinuation of immunosuppressive therapy, use of immune checkpoint inhibitor, pregnancy and delivery, etc.) influences the pathophysiology of mycobacterial diseases. This review describes the varying clinical presentations of NTM disease with emphasis on the differences from TB. J. Med. Invest. 68 : 220-227, August, 2021.
Topics: Humans; Mycobacterium Infections, Nontuberculous; Mycobacterium tuberculosis; Nontuberculous Mycobacteria; Tuberculosis; Tuberculosis, Pulmonary
PubMed: 34759134
DOI: 10.2152/jmi.68.220 -
International Journal of Infectious... Mar 2023Extrapulmonary tuberculosis (EPTB) is not a notifiable infectious disease in China but is a heavy burden on public health. However, the epidemic situation of EPTB...
OBJECTIVES
Extrapulmonary tuberculosis (EPTB) is not a notifiable infectious disease in China but is a heavy burden on public health. However, the epidemic situation of EPTB nationwide is unclear. This study aimed to investigate the magnitude and main subtypes of EPTB in China.
METHODS
We conducted a national cross-sectional study with multistage, stratified cluster random sampling during 2020-2021. We calculated proportions of EPTB in all patients with TB by organs. Logistic regression models were used to estimate odds ratios by characteristics.
RESULTS
A total of 6843 patients with TB were included. Of them, 24.6% were patients with EPTB, and the proportion of EPTB solo was 21.3%. Higher EPTB burden was observed in children, female patients, clinically diagnosed patients, provincial-level and prefectural-level health facilities, and Central and West China. EPTB occurred most frequently in respiratory (35.5%), musculoskeletal (15.8%), and peripheral lymphatic (15.8%) systems with top three subtypes, including tuberculous pleurisy (35.0%), spinal TB (9.8%) and cervical tuberculous lymphadenopathy (7.9%). With the increase of age, proportion of peripheral lymphatic TB decreased, and proportion rank of genitourinary TB rose.
CONCLUSION
It is essential to strengthen the diagnosis and treatment capacity for EPTB in primary medical facilities. EPTB should be added to the National Tuberculosis Program as a notifiable disease.
Topics: Child; Humans; Female; Tuberculosis, Extrapulmonary; Cross-Sectional Studies; Tuberculosis, Pleural; Tuberculosis, Lymph Node; China
PubMed: 36509333
DOI: 10.1016/j.ijid.2022.12.005 -
Tuberkuloz Ve Toraks Sep 2020Behcet's disease is a chronic inflammatory multi-systemic vasculitis. Recurrent oral and genital aphthous ulcers, uveitis, arthritis, arterial aneurysms, venous...
Behcet's disease is a chronic inflammatory multi-systemic vasculitis. Recurrent oral and genital aphthous ulcers, uveitis, arthritis, arterial aneurysms, venous thrombosis, gastrointestinal system lesions and skin lesions can be seen. Large, medium or small arteries and veins may be involved. While venous lesions cause occlusion, arterial lesions can cause both occlusion and aneurysm. Major forms of pulmonary involvement include pulmonary artery aneurysm, arterial and venous thrombosis, pulmonary infarction, recurrent pneumonia, and pleurisy. In Behcet's disease, pulmonary thromboembolism (PE) is often associated with endothelial damage. Neutrophils play an important role in the pathogenesis of thrombosis. Coagulation components such as fibrinogen, thrombin, factor Xa and factor VIIa activate the inflammatory cascade and induce vascular events. It is important to understand the pathogenesis of vascular events in determining the effective treatment strategy. Here we present a patient with Behcet's disease who was presented with pulmonary embolism and was investigated for the etiology of thrombosis.
Topics: Behcet Syndrome; Humans; Male; Middle Aged; Pulmonary Artery; Pulmonary Embolism; Tomography, X-Ray Computed; Ultrasonography, Doppler; Venous Thrombosis
PubMed: 33295733
DOI: 10.5578/tt.69502 -
Irish Veterinary Journal Nov 2020Respiratory disease is one of the most important factors impacting pig production worldwide. There is no available information on the prevalence of key pathogens...
BACKGROUND
Respiratory disease is one of the most important factors impacting pig production worldwide. There is no available information on the prevalence of key pathogens implicated in Irish pig production. The objective of this study was to describe the prevalence of pleurisy, pneumonia, lung abscesses, pericarditis and liver milk spots in finisher pigs of a cohort of Irish pig farms, and to describe the seroprevalence of: influenza A virus (IAV), porcine reproductive and respiratory syndrome virus (PRRSv), Mycoplasma hyopneumoniae (Mhyo) and Actinobacillus pleuropneumoniae (APP).
RESULTS
In brief, 56 farrow-to-finish farms (29% of the Irish breeding herd) were enrolled in the study in 2017. Data on lungs, heart, and liver lesions were assessed for each farm at slaughter. An average of 417 (range 129-1154) plucks per farm were assessed for pleurisy, pneumonia, lung abscesses, pericarditis, and liver milk spots. Blood samples from 32 finisher pigs were collected at slaughter for each farm. The observed prevalence of pleurisy and pneumonia was one of the lowest reported in similar studies in Europe (13 and 11% estimated average within farm, respectively). Pleurisy lesions were mostly moderate and severe. Pneumonia lesions affected a low level of lung surface (5.8%). Prevalence of pericarditis was mid-high (8%) and the prevalence of liver milk spots was high, with an average of 29% of the livers affected. For serology, 78.6% of the farms were positive for IAV, 50% were positive for PRRSv, 71.4% were positive for Mhyo, and 98.2% were positive for APP. Influenza virus was the main pathogen associated with pleurisy (P < 0.001) and Mhyo was the main pathogen associated with pneumonia (P < 0.001) and pericarditis (P = 0.024).
CONCLUSIONS
Farms affected with pleurisy had moderate to severe lesions. Farms affected with pneumonia had mild lesions, which could be the effect of the generalised use of Mhyo vaccination in piglets. The seroprevalence of IAV, PRRSv, Mhyo and APP in the present study sample is similar to or lower than in other European countries. Further research on the PRRSv and APP strains circulating in Ireland is necessary to support the design of national or regional control plans.
PubMed: 33319705
DOI: 10.1186/s13620-020-00176-w -
Porcine Health Management Jun 2023Across the European Union (EU), efforts are being made to achieve modernisation and harmonisation of meat inspection (MI) code systems. Lung lesions were prioritised as...
BACKGROUND
Across the European Union (EU), efforts are being made to achieve modernisation and harmonisation of meat inspection (MI) code systems. Lung lesions were prioritised as important animal based measures at slaughter, but existing standardized protocols are difficult to implement for routine MI. This study aimed to compare the informative value and feasibility of simplified lung lesion scoring systems to inform future codes for routine post mortem MI.
RESULTS
Data on lung lesions in finisher pigs were collected at slaughter targeting 83 Irish pig farms, with 201 batches assessed, comprising 31,655 pairs of lungs. Lungs were scored for cranioventral pulmonary consolidations (CVPC) and pleurisy lesions using detailed scoring systems, which were considered the gold standard. Using the data collected, scenarios for possible simplified scoring systems to record CVPC (n = 4) and pleurisy (n = 4) lesions were defined. The measurable outcomes were the prevalence and (if possible) severity scoring at batch level for CVPC and pleurisy. An arbitrary threshold was set to the upper quartile (i.e., the top 25% of batches with high prevalence/severity of CVPC or pleurisy, n = 50). Each pair of measurable outcomes was compared by calculating Spearman rank correlations and assessing if batches above the threshold for one measurable outcome were also above it for their pairwise comparison. All scenarios showed perfect agreement (k = 1) when compared among themselves and the gold standard for the prevalence of CVPC. The agreement among severity outcomes and the gold standard showed moderate to perfect agreement (k = [0.66, 1]). The changes in ranking were negligible for all measurable outcomes of pleurisy for scenarios 1, 2 and 3 when compared with the gold standard (rs ≥ 0.98), but these changes amounted to 50% for scenario 4.
CONCLUSIONS
The best simplified CVPC scoring system is to simply count the number of lung lobes affected excluding the intermediate lobe, which provides the best trade-off between value of information and feasibility, by incorporating information on CVPC prevalence and severity. While for pleurisy evaluation, scenario 3 is recommended. This simplified scoring system provides information on the prevalence of cranial and moderate and severe dorsocaudal pleurisy. Further validation of the scoring systems at slaughter and by private veterinarians and farmers is needed.
PubMed: 37391833
DOI: 10.1186/s40813-023-00324-y -
JACC. Case Reports Jun 2021A 53-year-old man with a background of acute myelomonocytic leukemia in remission presented with pleurisy. Repeat transthoracic echocardiography over several weeks...
A 53-year-old man with a background of acute myelomonocytic leukemia in remission presented with pleurisy. Repeat transthoracic echocardiography over several weeks revealed thickening left ventricular walls and decreasing systolic function. He died of decompensated heart failure due to cardiac myeloid sarcoma, with autopsy revealing an enlarged heart weighing >1 kg. ().
PubMed: 34317666
DOI: 10.1016/j.jaccas.2021.04.033 -
Microbiology Spectrum Feb 2022Actinobacillus pleuropneumoniae causes porcine pleuropneumonia, an important disease in the pig industry. Accurate and sensitive diagnostics such as DNA-based...
Actinobacillus pleuropneumoniae causes porcine pleuropneumonia, an important disease in the pig industry. Accurate and sensitive diagnostics such as DNA-based diagnostics are essential for preventing or responding to an outbreak. The specificity of DNA-based diagnostics depends on species-specific markers. Previously, an insertion element was found within an A. pleuropneumoniae-specific gene commonly used for A. pleuropneumoniae detection, prompting the need for additional species-specific markers. Herein, 12 marker candidates highly conserved (99 - 100% identity) among 34 A. pleuropneumoniae genomes (covering 13 serovars) were identified to be A. pleuropneumoniae-specific , as these sequences are distinct from 30 genomes of 13 other and problematic [] species and more than 1700 genomes of other bacteria in the family. Five marker candidates are within the gene, a known A. pleuropneumoniae-specific gene, validating our marker discovery method. Seven other A. pleuropneumoniae-specific marker candidates within the , , , , and genes were validated by polymerase chain reaction (PCR) to be specific to 129 isolates of A. pleuropneumoniae (covering all 19 serovars), but not to four closely related species, four [] species, or seven other bacterial species. This is the first study to identify A. pleuropneumoniae-specific markers through genome mining. Seven novel A. pleuropneumoniae-specific DNA markers were identified by a combination of and molecular methods and can serve as additional or alternative targets for A. pleuropneumoniae diagnostics, potentially leading to better control of the disease. Species-specific markers are crucial for infectious disease diagnostics. Mutations within a marker sequence can lead to false-negative results, inappropriate treatment, and economic loss. The availability of several species-specific markers is therefore desirable. In this study, 12 DNA markers specific to A. pleuropneumoniae, a pig pathogen, were simultaneously identified. Five marker candidates are within a known A. pleuropneumoniae-specific gene. Seven novel markers can be used as additional targets in DNA-based diagnostics, which in turn can expedite disease diagnosis, assist farm management, and lead to better animal health and food security. The marker discovery strategy outlined herein requires less time, effort, and cost, and results in more markers compared with conventional methods. Identification of species-specific markers of other pathogens and corresponding infectious disease diagnostics are possible, conceivably improving health care and the economy.
Topics: Actinobacillus pleuropneumoniae; Animals; Bacterial Proteins; Genetic Markers; Genome, Bacterial; Pathology, Molecular; Pleuropneumonia; Polymerase Chain Reaction; Swine; Swine Diseases
PubMed: 34985298
DOI: 10.1128/spectrum.01311-21