Occupational silica exposure in an Australian systemic sclerosis cohort

Rheumatology ◽  
2020 ◽  
Vol 59 (12) ◽  
pp. 3900-3905 ◽  
Author(s):  
Shreeya Patel ◽  
Kathleen Morrisroe ◽  
Susanna Proudman ◽  
Dylan Hansen ◽  
Joanne Sahhar ◽  
...  

Abstract Objective To determine the frequency of self-reported occupational exposure to silica in SSc patients enrolled in the Australian Scleroderma Cohort Study, and to compare the disease characteristics of the silica-exposed patients with those of the non-exposed patients. Method Data collected over a 12-year period from 1670 SSc patients were analysed. We compared the demographic and clinical characteristics of those who reported occupational silica exposure with those who did not. A subgroup analysis of male patients was performed, as well as a multivariable analysis of correlates of silica exposure. Results Overall, 126 (7.5%) of the cohort reported occupational silica exposure. These individuals were more likely to be male (73 of 231, i.e. 31.6% males exposed) and to have worked in mining and construction industries. Those who reported silica exposure were younger at the onset of SSc skin involvement [odds ratio (OR) 0.9, P = 0.02], of male gender (OR 14.9, P < 0.001), have joint contractures (OR 1.8, P = 0.05) and have higher physical disability as defined by scleroderma HAQ (OR 1.4, P = 0.01). Conclusion The highest percentage of silica exposure was found in males. These patients were more likely to have the presence of certain clinical manifestations and Scl-70 antibody, which is known to confer a poor prognosis. These findings support the association between occupational silica exposure and the subsequent development of SSc. Further investigation is required to describe the range of clinical manifestations and disease course, including prognosis and treatment response, in those diagnosed with occupationally induced SSc compared with idiopathic SSc.

2017 ◽  
Vol 39 (1) ◽  
pp. 78-85 ◽  
Author(s):  
P Kumar ◽  
H Singh ◽  
N Khurana ◽  
A B Urs ◽  
J Augustine ◽  
...  

Background: Myeloid sarcomas (MS) are rare extramedullary tumors composed of blasts of myeloid lineage that either precede, follow or present concomitantly with acute myeloid leukaemia (AML) or myeloproliferative neoplasms. The diagnosis of MS is especially challenging in patients without an antecedent history of leukemia. Methods: We present 2 cases of intraoral MS that presented as de novo lesions. A detailed review of cases of intraoral MS that either preceded or presented along with leukemia has been done with emphasis on diagnostic criteria used. Results: Two male patients aged 28 and 5 years presented with MS with one patient presenting with concomitant AML. A combination of morphological and immunohistochemical methods was used for diagnosis. A thorough review of world literature revealed 44 cases of intraoral MS that presented as de novo lesions. Conclusion: Intraoral MS is a rare tumor with poor prognosis. It may be diagnostically challenging due to its protean clinical manifestations and histological overlap with other tumors.


2021 ◽  
Vol 49 (2) ◽  
pp. 030006052098565
Author(s):  
Cai-Fu Zhao ◽  
Su-Fen Zhao ◽  
Ze-Qing Du

Small cell carcinoma of the cervix is a rare malignant tumor in the clinical setting. Clinical manifestations of this tumor are mostly similar to those of normal types of cervical cancer. Small cell carcinoma of the cervix only shows symptoms of neuroendocrine tumors, such as syndrome of inappropriate antidiuretic hormone secretion (SIADH). Most of the hyponatremia caused by SIADH can be managed after removal of the cause. Hyponatremia is a predictor of poor prognosis and can be used as an indicator of partial recurrence. We report a case of small cell carcinoma of the cervix complicated by SIADH. Our patient presented with irregular vaginal bleeding after menopause. After one cycle of chemotherapy, there was trembling of the limbs, and a laboratory examination showed low Na+ and low Cl− levels. After limited water intake, intravenous hypertonic saline, and intermittent diuretic treatment, the patient’s blood Na+ levels returned to normal. After a radical operation, the above-mentioned symptoms disappeared.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 882.1-882
Author(s):  
C. Sengler ◽  
S. Eulert ◽  
M. Niewerth ◽  
T. Kallinich ◽  
H. Wittkowski ◽  
...  

Background:Although children and adolescents are less likely to develop COVID-19 and generally show milder disease courses, it is unclear what impact the SARS-CoV2 infection has on children and adolescents with rheumatic and musculoskeletal disease (RMD). Due to their underlying disease as well as therapeutic immunosuppression these patients may be at higher risk of being more severely affected by SARS-CoV2. Furthermore, SARS-CoV2 infection might trigger a flare of the underlying disease.Objectives:To evaluate clinical characteristics and disease course of COVID-19 in children and adolescents with RMD and to analyze possible effects of SARS-CoV2 infection on the underlying disease under different therapeutic regimens.Methods:Data from juvenile patients with RMD recorded via the SARS-CoV2 questionnaire within the National Pediatric Rheumatology Database and the registry for hospitalized children and adolescents with COVID-19 of the German Society for Pediatric Infectious Diseases were analyzed. In addition to age, sex and diagnosis, information was collected about the date and method of a positive SARS-CoV2 testing, reason for testing, on clinical manifestations, disease course, treatment and outcome of COVID-19, on drug therapy at the time of virus detection, on disease activity (NRS 0 – 10, 0 = best) of the underlying disease at the last visit before and after the SARS-CoV2 infection.Results:From April 17th 2020 until January 25th 2021, data of 67 patients with RMD and confirmed SARS-CoV2 infection were collected. Mean age was 13.5 ± 3.9 years with equal sex distribution. The majority of patients were diagnosed with juvenile idiopathic arthritis (JIA, 64%), 12 (18%) patients had an autoinflammatory disease (FMF, CAPS, PFAPA, TRAPS) and 5 (7%) a connective tissue disease. Fifty-two patients (78%) were treated with a disease modifying antirheumatic drug (DMARD), 39% with a biological DMARD and 9% systemic glucocorticoids at the time of SARS-CoV-2 infection. Nineteen patients (28%) were tested for SARS-CoV-2 because of typical symptoms, the majority (67%) because of contact to an infected person. PCR was used most often (in 60 %).52 patients (78%) developed symptoms of COVID-19, 15 patients remained asymptomatic. The most common symptom of COVID-19 was rhinitis (42%) and fever (38%), followed by fatigue (34%), taste/smell disorder (33%), sore throat (27%) and cough (23%).Disease severity was graded as mild in 44 of 52 (85%) symptomatic patients, only two patients were hospitalized, one of whom required intensive care and died of cardiorespiratory failure 3 days after symptom onset. In 22 of 26 (85%) SARS-CoV2-positive patients, no relevant increase in disease activity (difference in NRS ≤ 1 before/after infection) of the underlying disease was observed 31 days after symptom onset (median, IQR 17-52 days). One patient, who had paused tocilizumab for 2 doses, experienced a flare of his seronegative polyarthritis 2 months after asymptomatic SARS-CoV-2 infection.Conclusion:In our cohort, the clinical picture of COVID-19 in children and adolescents with RMD was similar to that of healthy peers. The majority of patients showed mild disease course with good outcome under various medications, however, one patient with a severe course of COVID-19 died. In addition, SARS-CoV2 infection does not appear to have a relevant impact on the underlying disease activity, whereas discontinuation of therapy might pose a risk of flare.Disclosure of Interests:None declared.


2021 ◽  
Author(s):  
Hu Hei ◽  
Bin Zhou ◽  
Wenbo Gong ◽  
Chen Zheng ◽  
Jianwu Qin

Abstract Purpose: There is a sex disparity in papillary thyroid cancer (PTC). Male sex is associated with a higher likelihood of advanced stage disease. This study aimed to examine the significance of sex for extranodal extension (ENE) in PTC. Patients and Methods: We reviewed the data of PTC patients who had undergone initial surgical resection from July 2012 to December 2014 (N = 1531). The effects of sex and other clinicopathological factors on ENE were investigated.Results: Of 1531 patients identified, 377 (24.6%) were male, 816 (53.3%) had positive nodes, and 256 (16.7%) had ENE. Compared with female patients, male patients had a higher risk of ENE (P < 0.001). Multivariable analysis of clinicopathological factors revealed that male sex (odds ratio [OR], 1.98; 95% confidence interval [CI], 1.37 - 2.87; P < 0.001), age older than 60 years (OR, 1.93; 95% CI, 1.08 - 3.35; P = 0.023), extrathyroidal extension (OR, 3.52; 95% CI, 2.42 - 5.14; P < 0.001), bilateral multifocality (OR, 2.18; 95% CI, 1.53 - 3.13; P < 0.001), and more positive nodes were significantly associated with increased risk of ENE. Patients with 6-10 positive nodes were 16.45-fold higher to have ENE than patients with 5 positive nodes or less (95% CI, 11.07 - 24.68; P < 0.001).Conclusion: Male PTC patients had a higher risk of ENE than female. Sex was an independent predictor of ENE. The underlying mechanism needs to be investigated further.


2022 ◽  
Vol 12 ◽  
Author(s):  
Dianying Liu ◽  
Shaohua Liu ◽  
Meihong Xiu ◽  
Hongdong Deng ◽  
Huiyun Guo ◽  
...  

BackgroundSexual dysfunction is a common symptom in patients with schizophrenia, especially in chronically medicated patients. However, the relationship between sexual dysfunction and emotional response to sexual arousal in male patients with schizophrenia remains unclear. This study aimed to assess the incidence, risk factors of sexual dysfunction in males, and their clinical correlations to sexual arousal in male patients with schizophrenia in China.MethodsA total of 162 male patients, aged 18–50 years, with schizophrenia were recruited from a psychiatric hospital in Ganzhou. The clinical symptoms were assessed by the Positive and Negative Syndrome Scale (PANSS). The Arizona Sexual Experience Scale was utilized to evaluate sexual dysfunction. Erotic images were selected from International Affective Picture System (IAPS). Sixty-eight out of the 162 subjects completed the erotic pictures reactivity task.ResultsOverall, 48 (29.6%) patients were measured as having global sexual dysfunction, 72 (44.4%) patients as having strength of sex drive dysfunction, 51 (31.5%) patients as having sexual arousal dysfunction, 55 (34.0%) patients as having penile erection dysfunction, 60 (37.0%) patients as having reached orgasm dysfunction, and 60 (37.0%) patients as having satisfaction with orgasm dysfunction. The sexual dysfunction patients had significantly higher scores on the negative symptoms of the PANSS. The only important predictor of sexual dysfunction was the severity of PANSS negative factor. The sense of pleasure and arousal post viewing erotic images in the sexual dysfunction group were lower compared to the non-sexual dysfunction group. The sense of pleasure and approach motivation were significantly negatively correlated with the severity of sexual dysfunction.ConclusionsThis study shows that nearly one-third of young and middle-aged chronically medicated male inpatients with schizophrenia suffer from sexual dysfunction. The negative factor of the PANSS can be regarded as the risk factor of sexual dysfunction. Schizophrenia patients with sexual dysfunction experienced lower pleasure and higher avoidance motivation than non-sexual dysfunction patients when exposed to erotic stimuli.


2021 ◽  
Author(s):  
Ningning Yang ◽  
Qingqing Wang ◽  
Fengxia Chen ◽  
Haibin Ou ◽  
Yangyang Zeng ◽  
...  

Abstract Background: Metastatic adrenocortical carcinomas (MAC) have a poor prognosis. Advanced adrenocortical carcinoma often metastasizes to lung and liver. Prognostic factors of MAC have been rarely reported. This study aims to identify the association between specific metastasis and overall survival (OS) in MAC and determine the survival predictors for MAC patients.Methods: MAC patients' data was obtained from Surveillance, Epidemiology, and End Results (SEER) database between 2010 and 2016. Survival differences were analyzed by Kaplan–Meier analysis and log-rank tests. Cox proportional hazard model was used to identify the prognostic factors associated with overall survival.Results: A total of 152 MAC patients were selected, among whom 77 patients (50.7%) were diagnosed with one metastatic site, 75 (49.3%) diagnosed with more than one distant metastasis. For the whole MAC cohort, multivariable analysis showed that year of diagnosis between 2013 and 2016, without liver metastasis, surgery and chemotherapy were significantly favorable predictors of OS. For patients with one metastatic site, lung metastases had a better survival outcome than liver metastases(p=0.037). Besides, compared with patients who didn’t received surgery, patients underwent surgery were correlated with longer OS (p=0.004). For patients with more than one site of distant metastases, married status, surgery, and chemotherapy predicted a better OS. Radiotherapy did not improve overall survival outcomes in the three cohort.Conclusion: Liver metastasis has a poor prognosis. Year of diagnosis, metastatic sites, surgery and chemotherapy were significant prognostic factors for OS in MAC patients. For patients with single metastasis, surgery was a favorable prognostic factor, while married patients, surgery and chemotherapy predicted a better survival outcome in patients with more than one metastasis. Based on the collective findings, surgery can be regarded as the preferred treatment option for all MAC patients. Besides, chemotherapy is also a good choice for patients with multiple metastases.


2020 ◽  
Author(s):  
Wenzhi ZHANG ◽  
Hui LI ◽  
Jingzhen LIU ◽  
Jiawei XU ◽  
Jinjin HAO ◽  
...  

Abstract The knowledge of clinical characteristics and prognosis of pediatric acute megakaryocytic leukemia (AMKL) with or without acquired +21 was limited. We reported 15 AMKL pediatric patients without Down Syndrome (four cases with acquired +21 and 11 cases without acquired +21) with the clinical manifestations, laboratory data, and prognosis. The clinical features and laboratory data between patients with acquired +21 and patients without acquired +21 are similar. As for prognosis, three of the 11 cases without acquired +21 obtained complete remission (CR) after 1st induction. The median follow-up time of the 11 cases was 9 months. Among four cases with acquired +21, one case gave up treatment during 1st induction, one obtained CR after 1st induction and was still alive after 49 months of follow-up. One case obtained CR after 2nd induction and was still alive for 15 months of follow-up after bone marrow transplantation, the other patient was planning for allogeneic hematopoietic stem cell transplantation (HSCT) without CR. The median follow-up time of the four cases was 12 months. None relapsed in our study. In conclusion, acquired trisomy 21 may not be an indicator for poor prognosis. Cytogenetics analysis can help us for diagnosis stratification, prognostic judgment and individualized treatment of AMKL.


2022 ◽  
Vol 8 ◽  
Author(s):  
Han Zhang ◽  
Yingying Wu ◽  
Yuqing He ◽  
Xingyuan Liu ◽  
Mingqian Liu ◽  
...  

Objective: To study the differences in clinical characteristics, risk factors, and complications across age-groups among the inpatients with the coronavirus disease 2019 (COVID-19).Methods: In this population-based retrospective study, we included all the positive hospitalized patients with COVID-19 at Wuhan City from December 29, 2019 to April 15, 2020, during the first pandemic wave. Multivariate logistic regression analyses were used to explore the risk factors for death from COVID-19. Canonical correlation analysis (CCA) was performed to study the associations between comorbidities and complications.Results: There are 36,358 patients in the final cohort, of whom 2,492 (6.85%) died. Greater age (odds ration [OR] = 1.061 [95% CI 1.057–1.065], p &lt; 0.001), male gender (OR = 1.726 [95% CI 1.582–1.885], p &lt; 0.001), alcohol consumption (OR = 1.558 [95% CI 1.355–1.786], p &lt; 0.001), smoking (OR = 1.326 [95% CI 1.055–1.652], p = 0.014), hypertension (OR = 1.175 [95% CI 1.067–1.293], p = 0.001), diabetes (OR = 1.258 [95% CI 1.118–1.413], p &lt; 0.001), cancer (OR = 1.86 [95% CI 1.507–2.279], p &lt; 0.001), chronic kidney disease (CKD) (OR = 1.745 [95% CI 1.427–2.12], p &lt; 0.001), and intracerebral hemorrhage (ICH) (OR = 1.96 [95% CI 1.323–2.846], p = 0.001) were independent risk factors for death from COVID-19. Patients aged 40–80 years make up the majority of the whole patients, and them had similar risk factors with the whole patients. For patients aged &lt;40 years, only cancer (OR = 17.112 [95% CI 6.264–39.73], p &lt; 0.001) and ICH (OR = 31.538 [95% CI 5.213–158.787], p &lt; 0.001) were significantly associated with higher odds of death. For patients aged &gt;80 years, only age (OR = 1.033 [95% CI 1.008–1.059], p = 0.01) and male gender (OR = 1.585 [95% CI 1.301–1.933], p &lt; 0.001) were associated with higher odds of death. The incidence of most complications increases with age, but arrhythmias, gastrointestinal bleeding, and sepsis were more common in younger deceased patients with COVID-19, with only arrhythmia reaching statistical difference (p = 0.039). We found a relatively poor correlation between preexisting risk factors and complications.Conclusions: Coronavirus disease 2019 are disproportionally affected by age for its clinical manifestations, risk factors, complications, and outcomes. Prior complications have little effect on the incidence of extrapulmonary complications.


Author(s):  
Alla Ishchenko ◽  
Johan Joly ◽  
Neerinckx Barbara ◽  
Rik Lories ◽  
Kurt de Vlam

Abstract Objectives Biological treatments revolutionized the management of psoriatic arthritis (PsA) by significantly improving clinical manifestations and preventing structural damage. Both result in better quality of life and improved physical functioning. Since the introduction of the first TNF inhibitor (TNFi) in the early 2000s, therapeutic options for PsA are increasing steadily and a new generation of biologicals, including anti interleukin (IL)-17 and anti IL23 strategies, allows distinct targeted approaches. The purpose of this study was to investigate whether the demographic, clinical and disease characteristics of PsA patients that are selected for first-line biological treatment, changed over time since the introduction of biologicals. Methods Patients with a clinical diagnosis of PsA were included in the KU Leuven BioSPAR registry, a prospective cohort of spondyloarthritis (SpA) and PsA patients treated with biologicals and targeted synthetic disease modifying anti-rheumatic drugs (tsDMARDs) such as apremilast and JAK inhibitors. Demographics, prior DMARD use, disease characteristics and disease activity parameters were recorded at the initiation of biological treatment and subsequently every 3-months for the first 2 years and later every 6 months. The patient data were compared in three treatment periods, corresponding with availability of the first and the second generation of TNF inhibitors and the third generation of biologicals. Results Analysis of 185 Caucasian patients with PsA from our prospective cohort showed longer disease duration and higher disease activity with higher tender joint count (TJC), swollen joint count (SJC) and higher CRP in the first as compared with the later time periods. The demographic characteristics and prior DMARD use did not change over time. Skin and nail psoriasis were more frequent in earlier compared with the later treatment periods. The bio-DMARD survival rate was similar in the early and later treatment periods. Conclusion The population of patients, selected for treatment escalation, changed over time since the introduction of biologicals. Our results suggest that with years of experience, PsA patients might be considered earlier and for therapy intensification in patients with less active disease as compared with profiles in the early days of biological treatment.


2021 ◽  
Vol 12 ◽  
Author(s):  
Shan Qiao ◽  
Huai-kuan Wu ◽  
Ling-ling Liu ◽  
Ran-ran Zhang ◽  
Mei-ling Wang ◽  
...  

Objective: This study aimed to investigate epidemiological characteristics, clinical manifestations, and long-term outcomes of patients with autoimmune encephalitis (AE) in the east of China.Methods: From January 2015 to December 2019, 226 potential AE patients were recruited from five clinical centers, and a total of 185 patients who met the diagnostic criteria were included in the study. We retrospectively reviewed clinical features, auxiliary examinations, details of treatments, and outcomes of AE, and identified risk factors of poor prognosis. Modified Rankin Scale scores were used to evaluate neurological function, and scores of 3–6 indicated a poor-prognosis.Results: Patients with five main subtypes of AE were enrolled in the study, as follows: anti-NMDAR (79), anti-LGI1 (55), anti-CASPR2 (30), anti-GABABR (16), and anti-AMPAR (5). Among 185 patients, 58.38% (108/185) were male and 41.62% (77/185) were female. The median age at disease onset was 41 years (interquartile range, 17–62). The most common clinical manifestations of AE were seizures (146, 78.92%) and memory deficit (123, 66.49%). A total of 95 (51.35%) patients had abnormal brain magnetic resonance imaging results. Electroencephalographic findings were abnormal in 131 (70.81%) patients, and 168 (90.81%) and 26 (14.05%) patients were treated with first- and second-line immunotherapies, respectively. All surviving patients were followed-up for at least 1 year (range 12–36 months). Good clinical outcomes were achieved in 117 (63.24%), while 68 (36.76%) patients had a poor prognosis. Further, 33 (17.84%) patients relapsed and 10 (5.41%) died within 1 year post-discharge. Older patients tended to have a poorer prognosis, and the occurrence of mental behavioral disorders, movement disorders, disturbance of consciousness, central hypoventilation, and tumors were overrepresented in the poor-prognosis group.Conclusions: AE is a treatable disease, and most patients have a good prognosis. There are differences in the clinical manifestations of patients with different AE subtypes. Some with AE will relapse, and long-term follow-up is of great significance for further research.


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