Calcinosis in Juvenile Dermatomyositis-Epidemiology, Pathogenesis, Clinical Features, and Treatment: A Systematic Review.

PURPOSE OF REVIEW: We performed a systematic review of the literature on the epidemiology, pathogenesis, clinical and laboratory characterization, and treatment of calcinosis in patients with juvenile dermatomyositis (JDM). A qualitative systematic review was conducted from January 1975 to April 2023 according to the PRISMA protocol using three electronic databases: PubMed, Web of Science, and Scopus. Studies were analyzed based on the following eligibility criteria: at least one combination of the terms described in the search strategy appeared in the title, written in English, Portuguese, or Spanish, and addressed the epidemiology, pathogenesis, diagnosis, and treatment of calcinosis in juvenile dermatomyositis. Systematic or scoping reviews, letters, clinical images, book chapters, abstracts, inflammatory myopathy in other connective tissue diseases, idiopathic inflammatory myopathies in adults, and purely qualitative studies were excluded. RECENT FINDINGS: Seventy-five studies were included. According to the literature, calcinosis is common in women, around five years old, with three years of disease in association with osteoarticular, cutaneous, pulmonary manifestations, and fever. The pathogenesis is still unknown, but the participation of interleukin 1 and 6, tumor necrosis factor alpha, and innate immunity dysregulation seem to be involved. Common autoantibodies are anti-NXP-2, anti-MDA-5, and anti-Mi-2, and their treatment remains controversial. Prospective, randomized, controlled studies are needed to evaluate treatment protocols and map the natural history of this serious complication. Calcinosis seems to be more common in White female children with muscle weakness, fever, arthritis, severe pulmonary, and skin involvement with anti-NXP-2, anti-MDA-5, and anti-Mi-2 autoantibodies. The multitargets and aggressive treatment is recommended.

Gastroenterological Manifestations of Immunoglobulin G Subclass 4-Related Disease-Epidemiology, Clinical Manifestations, Diagnosis and Treatment.

Despite causing high morbidity, IgG4-related disease (IgG4-RD) and its gastroenterological manifestations lack better and greater theoretical contributions. Therefore, the objective of this work was to evaluate the clinical-epidemiological, diagnostic and treatment aspects of the gastrointestinal manifestations of this disease. A systematic review was carried out using the PubMed, Scopus and Embase databases between January 2012 and March 2023 with the following descriptors: "Immunoglobulin G4-Related Disease" (MeSH) AND #2 "Gastrointestinal Tract" (MeSH). Our data collection grouped a total of 3607 patients from mostly epidemiological cohort studies and cross-sectional follow-ups. In the subgroup analysis, IgG4-RD was associated with male gender, with an estimated prevalence between 54% and 80%. In our findings, the prevalence by topography was presented in the following ranges: lacrimal glands (17-57%); salivary glands (28-72%); pancreas (19-60%); biliary tract (5-40%); retroperitoneal cavity (9-43%). Longitudinal studies are needed to better map the natural history of the gastrointestinal manifestations of IgG4-RD and enable the formulation of individualized treatments.

Patients with inflammatory myopathies overlapping with systemic sclerosis: A Brazilian-Japanese bicentric study.

Objectives: This study aims to describe and compare the demographic, clinical, and laboratory characteristics and follow-up of representative samples of patients with myopathies and systemic sclerosis overlap syndromes (Myo-SSc) from two tertiary centers. Patients and methods: This is a cross-sectional and retrospective study conducted between January 2000 and December 2020. Fourty-five patients were analyzed with Myo-SSc (6 males, 39 females; mean age: 50.2±15.4 years; range, 45 to 65 years) from two tertiary centers (n=30 from Brazil and n=15 from Japan). Results: The median follow-up was 98 (range, 37 to 168) months. Muscle impairment started simultaneously with the diagnosis of systemic sclerosis in 57.8% (26/45) of cases. Muscle involvement occurred before the onset of systemic sclerosis in 35.5% (16/45) of cases, and after in 6.7% (3/45). Polymyositis was observed in 55.6% (25/45) of cases, followed by dermatomyositis in 24.4% (11/45) and antisynthetase syndrome in 20.0% (9/45). Concerning systemic sclerosis, the diffuse and limited forms occurred in 64.4% (29/45) and 35.6% (16/45) of the cases, respectively. Comparing the subgroups, Myo or SSc onset was earlier in Brazilian patients, and they had a higher frequency of dysphagia (20/45, [66.7%]) and digital ulcers (27/45, [90%]), whereas Japanese patients had higher modified Rodnan skin scores (15 [9 to 23]) and prevalence of positive anti-centromere antibodies (4/15 [23.7%]). The current disease status and mortality were similar in both groups. Conclusion: In the present study, Myo-SSc affected middle-aged women, and its manifestation spectrum varied according to geographic distribution.

Assessment of cardiovascular risk factors in patients with idiopathic inflammatory myopathies: a systematic review.

We performed a systematic review of cardiovascular risk factors in idiopathic inflammatory myopathies (IIMs) and their cardiovascular outcomes, including acute coronary syndrome and stroke. A qualitative systematic review was conducted from January 1956 to December 2022 according to the PRISMA protocol using three electronic databases: PubMed, Web of Science, and Scopus. The studies were analyzed based on the following eligibility criteria: at least one combination of the terms described in the search strategy appeared in the title, written in English, Portuguese, or Spanish, and addressed risk factors for cardiovascular diseases in IIMs. Brief reports, reviews, papers addressing juvenile IIMs, congress proceedings, monographs, and dissertations were excluded. Twenty articles were included. According to the literature, most patients with IIMs are middle-aged North American or Asian women, with dyslipidemia and hypertension. The prevalence of the cardiovascular risk factors was generally low in IIMs, but with a high incidence of acute myocardial infarction. Further theoretical and prospective studies are needed to define the actual impact of each variable (e.g., hypertension, diabetes, smoking, alcoholism, obesity, and dyslipidemia) on the cardiovascular risk of patients with IIMs.

The Impact of the COVID-19 Pandemic on the Mental Health of Teachers and Its Possible Risk Factors: A Systematic Review.

(1) Objective: The objective was to analyze the development of psychiatric pathologies/burnout syndrome and their possible risk factors in teachers in the context of the COVID-19 pandemic. (2) Methods: A qualitative systematic review was carried out, according to the PRISMA protocol, in the PubMed, Scopus, and Web of Science databases using a combination of the following descriptors [MeSH]: "mental health", "mental disorders'', "covid-19" and "school teachers''. Articles selected were written in English, Portuguese and Spanish, published between November 2019 and December 2022. (3) Results: The most common psychiatric pathologies were generalized anxiety disorders and depression. Burnout syndrome was also quite prevalent. Of the 776 articles identified, 42 were selected after applying the eligibility criteria. Although there is variability among the analyzed studies, the risk factors most correlated with increased morbidity in teachers were: (i) being female; (ii) age below the fifth decade of life; (iii) pre-existence of chronic or psychiatric illnesses before the pandemic; (iv) difficulty in adapting to the distance education model; (v) family/work conflicts; (vi) negative symptoms caused by the pandemic. (4) Conclusions: Therefore, the COVID-19 impact on mental health appears to be more common in female teachers in their fifth decade of life and with pre-existing psychiatric comorbidities. However, prospective studies are needed to better map this situation.

Characteristics of Older Patients with Takayasu's Arteritis: A Two-Center, Cross-Sectional, Retrospective Cohort Study. / Particularidades dos Pacientes com Arterite de Takayasu em Idade Mais Avançada: Estudo Coorte, Retrospectivo e Transversal.

BACKGROUND: Few studies have assessed elderly patients with Takayasu's arteritis (TAK). OBJECTIVES: To evaluate the progression of TAK in different age groups and its possible effects on drug treatment and disease activity. METHODS: This cross-sectional and retrospective cohort study included 66 TAK patients. Patients were interviewed and data of the 12 preceding months were collected from electronic medical records. The patients were divided into four quartiles according to current age and compared for clinical and laboratory data, treatment, comorbidities, disease status, and functional status. Statistical significance was set at p<0.05. RESULTS: The groups were Q1(22-36 years, n=16), Q2(37-42 years, n=18), Q3(43-49 years, n=17), and Q4(51-66 years, n=15). The frequency of patients with disease activity, fatigue, comorbidities and vascular impairments, and the TAK disease extent index were also comparable between the groups. With age, disease duration was longer (p=0.001), fewer patients used prednisone (Q1:43.8%, Q2:33.3%, Q3:11.8%, and Q4:6.7%; p=0.049) and immunosuppressive drugs [Q1:100.0%, Q2:66.7%, Q3:58.8%, and Q4:46.7%; Q1 versus Q3 (p=0.043), and Q1 versus Q4 (p=0.005) in post-hoc analyses], and patients had greater functional status impairment (Q2 versus Q3, p=0.003). In addition, the levels of disease damage, new TAK symptoms, and complications in the preceding 12 months were not different between the groups. CONCLUSIONS: Older patients with TAK require minimal drug treatment, and have greater impairment of functional status, which may be attributed to aging-related factors.

FUNDAMENTOS: Poucos estudos avaliaram pacientes idosos com Arterite de Takayasu (AT). OBJETIVO: Avaliar o progresso de AT em diferentes grupos etários em seus possíveis efeitos sobre o tratamento medicamentoso e atividade da doença. MÉTODOS: este estudo transversal, retrospectivo, do tipo coorte incluiu 66 pacientes com AT. Os pacientes foram entrevistados, e dados dos 12 meses anteriores foram coletados dos prontuários médicos eletrônicos. Os pacientes foram divididos em quatro quartis de acordo com idade atual, e comparados quanto aos dados clínicos e laboratoriais, tratamento, comorbidades, status da doença, e status funcional. Um p<0,05 foi estabelecido como estatisticamente significativo. RESULTADOS: Os grupos foram definidos como Q1(22-36 anos, n=16), Q2(37-42 anos, n=18), Q3(43-49 anos, n=17), e Q4(51-66 anos, n=15). A frequência de pacientes com atividade da doença, fadiga, comorbidades e comprometimentos vasculares, e o índice de extensão da doença (DEI. Tak) foram comparáveis entre os grupos. Pacientes com idade mais avançada apresentaram maior duração da doença (p=0,001) e maior comprometimento do status funcional (Q2 versus Q3, p=0,003); menos pacientes usaram prednisona (Q1:43,8%; Q2:33,3%; Q3:11,8%; e Q4:6,7%; p=0,049) e agentes imunossupressores [Q1:100,0%; Q2:66,7%; Q3:58,8% e Q4:46,7%; Q1 versus Q3 (p=0,043) e Q1 versus Q4 (p=0,005) nas análises post-hoc]. Além disso, os níveis de danos da doença, sintomas de uma nova ocorrência de AT, e complicações nos 12 meses precedentes não foram diferentes entre os grupos. CONCLUSÃO: Pacientes com AT e idade mais avançada requerem mínima intervenção medicamentosa e apresentam maior comprometimento no status funcional, o que pode ser atribuído a fatores relacionados ao envelhecimento.

Particularidades dos Pacientes com Arterite de Takayasu em Idade Mais Avançada: Estudo Coorte, Retrospectivo e Transversal / Characteristics of Older Patients with Takayasu's Arteritis: A Two-Center, Cross-Sectional, Retrospective Cohort Study

Resumo Fundamentos Poucos estudos avaliaram pacientes idosos com Arterite de Takayasu (AT). Objetivo Avaliar o progresso de AT em diferentes grupos etários em seus possíveis efeitos sobre o tratamento medicamentoso e atividade da doença. Métodos este estudo transversal, retrospectivo, do tipo coorte incluiu 66 pacientes com AT. Os pacientes foram entrevistados, e dados dos 12 meses anteriores foram coletados dos prontuários médicos eletrônicos. Os pacientes foram divididos em quatro quartis de acordo com idade atual, e comparados quanto aos dados clínicos e laboratoriais, tratamento, comorbidades, status da doença, e status funcional. Um p<0,05 foi estabelecido como estatisticamente significativo. Resultados Os grupos foram definidos como Q1(22-36 anos, n=16), Q2(37-42 anos, n=18), Q3(43-49 anos, n=17), e Q4(51-66 anos, n=15). A frequência de pacientes com atividade da doença, fadiga, comorbidades e comprometimentos vasculares, e o índice de extensão da doença (DEI. Tak) foram comparáveis entre os grupos. Pacientes com idade mais avançada apresentaram maior duração da doença (p=0,001) e maior comprometimento do status funcional (Q2 versus Q3, p=0,003); menos pacientes usaram prednisona (Q1:43,8%; Q2:33,3%; Q3:11,8%; e Q4:6,7%; p=0,049) e agentes imunossupressores [Q1:100,0%; Q2:66,7%; Q3:58,8% e Q4:46,7%; Q1 versus Q3 (p=0,043) e Q1 versus Q4 (p=0,005) nas análises post-hoc]. Além disso, os níveis de danos da doença, sintomas de uma nova ocorrência de AT, e complicações nos 12 meses precedentes não foram diferentes entre os grupos. Conclusão Pacientes com AT e idade mais avançada requerem mínima intervenção medicamentosa e apresentam maior comprometimento no status funcional, o que pode ser atribuído a fatores relacionados ao envelhecimento.

Abstract Background Few studies have assessed elderly patients with Takayasu's arteritis (TAK). Objectives To evaluate the progression of TAK in different age groups and its possible effects on drug treatment and disease activity. Methods This cross-sectional and retrospective cohort study included 66 TAK patients. Patients were interviewed and data of the 12 preceding months were collected from electronic medical records. The patients were divided into four quartiles according to current age and compared for clinical and laboratory data, treatment, comorbidities, disease status, and functional status. Statistical significance was set at p<0.05. Results The groups were Q1(22-36 years, n=16), Q2(37-42 years, n=18), Q3(43-49 years, n=17), and Q4(51-66 years, n=15). The frequency of patients with disease activity, fatigue, comorbidities and vascular impairments, and the TAK disease extent index were also comparable between the groups. With age, disease duration was longer (p=0.001), fewer patients used prednisone (Q1:43.8%, Q2:33.3%, Q3:11.8%, and Q4:6.7%; p=0.049) and immunosuppressive drugs [Q1:100.0%, Q2:66.7%, Q3:58.8%, and Q4:46.7%; Q1 versus Q3 (p=0.043), and Q1 versus Q4 (p=0.005) in post-hoc analyses], and patients had greater functional status impairment (Q2 versus Q3, p=0.003). In addition, the levels of disease damage, new TAK symptoms, and complications in the preceding 12 months were not different between the groups. Conclusions Older patients with TAK require minimal drug treatment, and have greater impairment of functional status, which may be attributed to aging-related factors.

Impacts of COVID-19 on children and adolescents: A systematic review analyzing its psychiatric effects.

OBJECTIVE: To summarize the most relevant data from a systematic review on the impact of COVID-19 on children and adolescents, particularly analyzing its psychiatric effects. METHODS: This review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines and included experimental studies (randomized-individually or pooled-and non-randomized controlled trials), observational studies with a group for internal comparison (cohort studies-prospective and retrospective-and case-control) and qualitative studies in the period from 2021 to 2022. RESULTS: The search identified 325 articles; we removed 125 duplicates. We selected 200 manuscripts, chosen by title and selected abstracts. We excluded 50 records after screening titles and abstracts, as they did not meet the inclusion criteria. We retrieved 150 records selected for a full reading. We excluded 90 text articles and we selected 25 records for the (n) final. Limitations: Due to the short period of data collection, from 2021 to 2022, there is a possibility of lack of relevant studies related to the mental health care of children and adolescents. In addition, there is the possibility of publication bias, such as only significant findings being published. CONCLUSION: The impact of COVID-19 on the mental health of children and adolescents is of great concern to child and youth psychiatry. Situations such as fear, anxiety, panic, depression, sleep and appetite disorders, as well as impairment in social interactions caused by psychic stress, are punctual markers of pain and psychic suffering, which have increasing impacts on the mental health panorama of children and adolescents globally, particularly in vulnerable and socially at-risk populations.

Reflections on the prevalence of human leukocyte antigen-B27 and human leukocyte antigen-B51 co-occurrence in patients with spondylarthritis.

We performed a literature mini-review of the clinical profile of patients with spondylarthritis who are also human leukocyte antigen (HLA)-B51-positive. It seems to us that patients with HLA-B27 and HLA-B51 are more common in men, Asians and between the third and ninth decades of life. They are more likely to develop peripheral joint conditions, with cutaneous manifestations (e.g., oral ulcers) and uveitis. Therefore, more robust epidemiological studies with more accurate methodology and multicenter locations are needed to better map the role of the interaction between HLA-B51 in patients with spondylarthritis.

COVID-19, mental health and Indigenous populations in Brazil: The epidemic beyond the pandemic.

The aim of this paper was to report on factors contributing to the deterioration of the mental health of Indigenous populations (IP) in Brazil. Five factors seem to have a direct impact on the mental health of IP in Brazil: (1) The absence of public policies; (2) Intellectual production; (3) Psychiatric medical care for remote areas (e.g., telemedicine) aimed at promoting the mental health of Brazil's IP, which causes a huge gap in the process of assistance and social, psychological, economic and cultural valorization of native peoples; (4) The dissemination of fake news, which exposed, above all, older IP to risk behaviors in the pandemic, such as refusal of vaccination; and (5) The violence carried out on IP lands due to economic interests with mining/agribusiness.

The impact of "the war that drags on" in Ukraine for the health of children and adolescents: Old problems in a new conflict?

The early months of 2022 have already included several distressing world events. From the ongoing COVID-19 pandemic, to protests against vaccine mandates and COVID-19 restrictions, to the Russian invasion of Ukraine. Escalating conflict in Ukraine poses an immediate and growing threat to the lives and well-being of the country's 7.5 million children. Humanitarian needs are multiplying - and spreading by the hour. Children have been killed. Children have been wounded. They are being profoundly traumatized by the violence all around them. Hundreds of thousands of people are on the move, and family members are becoming separated from their loved ones.

COVID-19, liver dysfunction and pathophysiology: A conceptual discussion.

The intra and extracellular pathways of hepatic injury by coronavirus disease 2019 (COVID-19) are still being studied. Understanding them is important to treat this viral disease and other liver and biliary tract disorders. Thus, this paper aims to present three hypotheses about liver injury caused by COVID-19: (1) The interactions between severe acute respiratory syndrome coronavirus 2 spike protein and membrane receptors in the hepatocyte; (2) The dysbiosis and "gut-liver axis" disruption in patients with serious clinical presentations of COVID-19; and (3) The inflammatory response exacerbated through the production of interleukins such as interleukin-6. However, despite these new perspectives, the pathophysiological process of liver injury caused by COVID-19 is still complex and multifactorial. Thus, understanding all these variables is a challenge to science but also the key to propose individualized and effective patient therapies.

Rituximab as a treatment for human immunodeficiency virus-associated nemaline myopathy: What does the literature have to tell us?

We presented a letter about a case of a 37-year-old Black female with a history of human immunodeficiency virus and an undetectable viral load. She was evaluated with weakness in the scapular (grade III) and pelvic girdles (grade II), elevation of creatine phosphokinase levels and muscle biopsy compatible with nemaline myopathy. She was treated with rituximab showing improvement of the condition.

Domestic violence against Latin American women and female mental health during the new coronavirus (SARS-CoV-2) outbreak.

We aimed to discuss the impact of the coronavirus pandemic on the VAW scenario and how it affects women's mental health. The short communication from secondary data collected from the official websites of seven countries in Latin America was performed. The sample was non-probabilistic, for convenience. The collection took place in January 2021. During the COVID-19 pandemic, the increased contact of the victim with the aggressor (if he/she is the spouse), deepening of economic disparities, stressful environment, and loss of social support mechanisms are some of the factors that explain the possible increase in VAW rates. VAW is a sociological phenomenon whose bases are rooted in historical, political, religious, and economic conditions. In addition to the biological dimension of the pandemic, it is necessary to reflect on the long-term impacts of this new social configuration, especially on more vulnerable groups such as women in underdeveloped countries.

Pathophysiology and molecular mechanisms of liver injury in severe forms of COVID-19: An integrative review.

BACKGROUND AND AIMS: SARS-CoV-2 has primary pulmonary impairment, but other organs such as the liver can also be affected. This implies a worsening of patient's prognosis and an increase in morbidity and mortality. The metabolic pathways and molecular factors involved in the genesis of this injury are still unknown. Therefore, we aimed to carry out an integrative review about the pathophysiology and possible molecular mechanisms of liver injury by COVID-19. METHODS: We carried out an integrative literature review in the following databases: PubMed, Scopus, and Embase from December 2020 to March 2021 using the following descriptors: # 1 "COVID-19" (MeSH) AND / OR # 2 "Liver injury" (MeSH) AND / OR # 3 "Pathophysiology" (MesH). RESULTS: The data were extracted and divided into two main themes, for heuristic purposes: "Hepatotropism and SARS-CoV-2", and "Pathophysiological hypotheses for liver injury associated with SARS-CoV-2". CONCLUSIONS: The virus seems to promote liver damage through five mechanisms: direct injury, humoral and cellular inflammatory response, hypoxemia caused by a decrease in the effective circulating volume, reinfection through the portal system, and use of drugs in the treatment. The literature also points out that the expression of the angiotensin-converting enzyme II and transmembrane serine protease 2 receptors is expressive in cholangiocyte and is present in hepatocytes, which is a risk factor for the virus to enter these cells. Finally, patients with previous liver disease appear to be more susceptible to liver injury by COVID-19.