The evaluation of myocarditis in patients with Still's disease; clinical findings from the multicentre international AIDA Network Still's Disease Registry.

Objective: To evaluate the cardiac involvement in patients with Still's disease with a focus on myocarditis included in the multicenter AIDA (AutoInflammatory Disease Alliance) network Still's disease registry. To exploit the predictive factors for myocarditis in deriving a clinical risk patient profile for this severe manifestation.

Methods: A multicenter observational study was built up assessing consecutive patients with Still's disease characterized by the cardiac involvement among those included in the AIDA Network Still's Disease Registry.

Rare diseases: What rheumatologists need to know?

Although the terms "rare diseases" (RD) and "orphan diseases" (OD) are often used interchangeably, specific nuances in definitions should be noted to avoid misconception. RD are characterized by a low prevalence within the population, whereas OD are those inadequately recognized or even neglected by the medical community and drug companies. Despite their rarity, as our ability on discovering novel clinical phenotypes and improving diagnostic tools expand, RD will continue posing a real challenge for rheumatologists.

IgG4-related disease-rare but you should not forget it.

Immunoglobulin G4-related disease is a systemic immune-mediated disease with insidious evolution characterized by fibroinflammatory lesions over virtually any organ system. Despite the remarkable progression of knowledge, its etiology remains undefined. Due to its relapse-remitting pattern, it could accumulate irreversible damage, increasing comorbidities and mortality.

Assessing workers with fibromyalgia: what should occupational physicians know?

Fibromyalgia is a chronic pain syndrome with a complex multifactorial etiopathogenesis that more frequently affects women. Although widespread pain is the dominant feature, fibromyalgia incorporates a wide variety of symptoms, such as fatigue, unrefreshed sleep, and cognitive and mood disorders. Central sensitization to pain is a key element in the pathophysiology of this syndrome.

Shrinking lung syndrome in primary Sjögren's syndrome: a case-based review.

Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease that affects exocrine glands, mainly the salivary and lacrimal glands, leading to the development of sicca symptoms. Patients with pSS may also present with extraglandular manifestations, including lung involvement, estimated to occur in 9-24% of cases. Shrinking lung syndrome (SLS) is an uncommon respiratory complication primarily associated with systemic lupus erythematosus, with a prevalence of approximately 1% in these patients.

The Role of MRI in Differentiating Demyelinating and Inflammatory (not Infectious) Myelopathies.

Demyelinating and inflammatory myelopathies represent a group of diseases with characteristic patterns in neuroimaging and several differential diagnoses. The main imaging patterns of demyelinating myelopathies (multiple sclerosis, neuromyelitis optica spectrum disorder, acute disseminated encephalomyelitis, and myelin oligodendrocyte glycoprotein antibody-related disorder) and inflammatory myelopathies (systemic lupus erythematosus-myelitis, sarcoidosis-myelitis, Sjögren-myelitis, and Behçet's-myelitis) will be discussed in this article, highlighting key points to the differential diagnosis.

Immune-Mediated Hypertrophic Pachymeningitis and its Mimickers: Magnetic Resonance Imaging Findings.

Hypertrophic pachymeningitis (HP) is a rare and chronic inflammatory disorder presenting as localized or diffuse thickening of the dura mater. It can be idiopathic or an unusual manifestation of immune-mediated, infectious, and neoplastic conditions. Although some cases may remain asymptomatic, HP can lead to progressive headaches, cranial nerve palsies, hydrocephalus, and other neurological complications, which makes its recognition a fundamental step for prompt treatment.

Work situation, work ability and expectation of returning to work in patients with systemic autoimmune myopathies.

Objectives: To document the work situation, the work ability and the expectation of returning to work among adult patients with systemic autoimmune myopathies (SAMs), and to identify the factors associated with each of these outcomes.

Methods: Cross-sectional study. The work situation (performing paid work vs out of work) was ascertained via a structured questionnaire.

Psoriatic arthritis mutilans: a descriptive study from a Brazilian tertiary center.

In this paper, we sought to determine the prevalence of arthritis mutilans in a single cohort of Brazilian psoriatic arthritis patients followed at a tertiary university reference center. Our study demonstrated a high prevalence of arthritis mutilans associated to comorbidities and biologic therapy. In addition, our data suggest that axial involvement may be an intriguing aspect of psoriatic arthritis mutilans and that rheumatologists should be aware of axial disease, even if the phenotype is marked by peripheral joint severity.

Systemic autoimmune diseases and work outcomes in Brazil: a scoping review.

Objective: To review articles that assessed work-related outcomes such as workability, work productivity, presenteeism, absenteeism, sick leave, return to work, and employment status of Brazilian patients with rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, Sjögren's syndrome, and systemic autoimmune myopathies.

Methods: This study was conducted in Medline databases (PubMed), SciELO, and Lilacs through a combination of descriptors of interest. Studies published until December 2020 were considered in the search strategy.

Analysis of patient-physician discrepancy in global assessment of systemic autoimmune myopathy disease activity.

Objectives: To compare the perception of disease activity (DA) between adult patients with systemic autoimmune myopathies (SAMs) and their physicians, and analyse possible sources of discordance.

Methods: This cross-sectional study included 75 patients with SAMs. Patients and physicians rated the global DA on a 0-10 cm visual analogue scale.

Raynaud's phenomenon in the occupational context.

Objective: To review articles that evaluated the prevalence of Raynaud's phenomenon of occupational origin.

Methods: The search for articles was carried out in the Medline (via PubMed), Embase, Web of Science, Scientific Electronic Library Online (SciELO), and Latin America and Caribbean Health Sciences Literature (Lilacs) databases.

Results: 64 articles were obtained from the electronic search; 18 articles met the eligibility criteria.

Peliosis hepatis and systemic lupus erythematosus: A rare condition identified by magnetic resonance imaging.

Peliosis hepatis is a rare benign disorder characterized by the presence of multiple cavities filled with blood with no preferential localization in the liver parenchyma. It may be related to several etiologic conditions, especially infections and toxicity of immunosuppressive drugs. To our knowledge, there are only three articles reporting the association between peliosis hepatis and systemic lupus erythematosus.

A morphometric study of the intestinal mucosa of rats submitted to omentoenteropexy.

Context: The omentoenteropexy technique was developed as an alternative method for intestinal neovascularization, due to the angiogenic properties of factors from the omentum.

Objectives: This study investigated changes in intestinal villi heights and crypts depths due to surgical techniques: seromiotomy with and without omentoenteropexy.

Method: Thirty rats were operated on, after being divided into three groups, namely GI, GII and GIII with 10 rats each.