- Fibro-osseous pseudotumor of the digit: Case report and surgical experience with extensive digital lesion abutting on neurovascular bundles. [Journal Article]
- AMAnn Med Surg (Lond) 2018; 35:158-162
- CONCLUSIONS: FOPD although benign, a soft tissue osteosarcoma is one of the differential diagnosis. Meticulous attention to the clinical, pathological and histological features of FOPD is required. Early diagnosis and treatment of FOPD is very crucial in optimizing the overall outcome. Pre-operative planning with various radiological modalities was of great help anticipating the surgical course.
- Update on the clinical management of juvenile dermatomyositis. [Journal Article]
- ERExpert Rev Clin Immunol 2018 Oct 11
- Juvenile dermatomyositis (JDM) is a rare, chronic autoimmune illness with primary features of symmetric, proximal muscle weakness and involvement of the skin with a number of identifiable rashes. Evi...
Juvenile dermatomyositis (JDM) is a rare, chronic autoimmune illness with primary features of symmetric, proximal muscle weakness and involvement of the skin with a number of identifiable rashes. Evidence to support treatment decisions is limited, given the paucity of clinical trials. Consensus based methods, informed by available data, play an important role in treatment recommendations. Areas covered: This review focuses on evidence and consensus opinion regarding therapeutic options in JDM and identifies gaps where future research is needed. Expert commentary: The combination of trial evidence (as limited as it is) and consensus opinion support standard initial management for children with JDM to consist of high dose corticosteroids, either intravenous or oral, and methotrexate. Several other agents have preliminary support, either through clinical trials or case series for their use in patients who either fail to respond adequately, have severe disease or have contraindications to standard initial therapy. One of the important goals of management in JDM will be to reduce the corticosteroid exposure experienced by patients. To meet this goal, progress in a number of key areas is needed: increased international collaboration, advances in study design and increased translational research.
- Imaging of joints in systemic lupus erythematosus. [Review]
- CEClin Exp Rheumatol 2018 Sep-Oct; 36 Suppl 114(5):68-73
- Musculoskeletal symptoms are among the most common manifestations in patients with systemic lupus erythematosus (SLE), being reported in up to 95% of patients; joint and tendon involvement can range ...
Musculoskeletal symptoms are among the most common manifestations in patients with systemic lupus erythematosus (SLE), being reported in up to 95% of patients; joint and tendon involvement can range from arthralgia to severe deforming arthropathy; while myositis a rare manifestation, comorbid fibromyalgia is reported in up to 40% of SLE patients. All these manifestations have a significant impact on the patients' quality of life, possibly leading to disability and functional impairment in daily living activities. In recent years, thanks to the availability of new imaging techniques for the assessment of tendon and joint pathologies, the approach to the definition and characterisation of these manifestations in SLE is constantly evolving. In this review we will therefore illustrate the state of the art of imaging techniques in the assessment of joint involvement in SLE, focusing on ultrasounds (US) and magnetic resonance (MRI), discussing their advantages, drawbacks and possible future developments. The main findings that emerge from the recent literature is that imaging studies may allow a more accurate definition of disease subtypes revealing an unexpected higher prevalence of joint and tendon involvement with respect to what known by clinical evaluation and standard radiography. Indeed, US and MRI also made possible the identification of joints and tendons pathologies in patients with no or very mild clinical symptoms. On the other hand, the interpretation of some findings remains uncertain, as well as the validity and feasibility of this analysis in clinical practice. Thus, further studies should clarify the clinical meaning of subclinical abnormalities detected in US and MRI scans and their impact on the long-term outcomes.
- [Recent Developments in Myositis Syndromes]. [Journal Article]
- DMDtsch Med Wochenschr 2018; 143(20):1472-1476
- Idiopathic inflammatory myopathies (IIM) are a rare and clinically polymorphic and heterogenous group of myositis syndromes. Myositis is part of a systemic autoimmune disorder with various extramuscu...
Idiopathic inflammatory myopathies (IIM) are a rare and clinically polymorphic and heterogenous group of myositis syndromes. Myositis is part of a systemic autoimmune disorder with various extramuscular manifestations affecting skin, lungs, joints, esophagus and other organ systems. Most myositis patients have autoantibodies against non organspecific antigens. More than 20 different autoantibodies are known. They are associated with different clinical phenotypes of adult or juvenile dermatomyositis or myositis-overlap syndromes and different genetic markers. Pure polymyositis, if not a monosymptomatic manifestation of a systemic disorder, so far has no marker antibody and is an exclusion diagnosis. Sporadic inclusion body myositis (sIBM) has no extramuscular manifestations, cN1A antibodies are directed against muscle-derived antigen and are not highly specific for IBM.Myositis syndromes differ in histopathology. Patients with anti-synthetase-syndrome frequently have necrotizing perifascicular myositis with myonuclear actin inclusions.New classification criteria have been developed by the European (EULAR) and American (ACR) rheumatology societies and different outcome measurements for clinical studies are now available.There is still a lack of controlled therapeutic trials. However there is good consensus that glucocorticosteroids (GC) are necessary and effective to treat active myositis usually in combination with methotrexate or azathioprine. Rituximab is effective in GC-resistant myositis as well as high dose i. v. immune globulin (IVIG) in certain conditions.
- Inhospital Complications of Patients With Neuromuscular Disorders Undergoing Total Joint Arthroplasty. [Journal Article]
- JAJ Am Acad Orthop Surg 2018 Oct 02
- CONCLUSIONS: Because more patients with NM disease become candidates of TJA, a team of neurologists, anesthesiologists, therapists, and orthopaedic surgeon is required to anticipate, prevent, and manage potential complications identified in this study.
- [Dermatomyositis associated with anti-MDA5 autoantibody]. [Case Reports]
- MMedicina (B Aires) 2018; 78(5):360-363
- Dematomyositis is an idiopathic inflammatory myopathy with a variable clinical spectrum. In recent years, a number of myositis-specific antibodies have been identified including anti-MDA5, which is u...
Dematomyositis is an idiopathic inflammatory myopathy with a variable clinical spectrum. In recent years, a number of myositis-specific antibodies have been identified including anti-MDA5, which is us eful for diagnosis, prognosis and classification of the diverse clinical forms of the disease. This antibody is associated with cutaneous ulcers, rapidly progressive interstitial lung disease, early mortality and poor prognosis, so the detection of this antibody in a suitable clinical context, raises the need for an aggressive immunosuppressive treatment. We describe a case of dermatomyositis classified as hypomyopathic (i.e. involving mild muscle weakness), presenting specific skin lesions, interstitial lung disease, and presence of anti-MDA5 antibody that had a favorable response to combined treatment with cyclophosphamide, gamma globulin and corticosteroids.
- The association of sociodemographic and disease variables with hand function: a Scleroderma Patient-centered Intervention Network cohort study. [Journal Article]
- CEClin Exp Rheumatol 2018 Jul-Aug; 36 Suppl 113(4):88-94
- CONCLUSIONS: Multiple factors are associated with hand function in SSc. The presence of moderate or severe small joint contractures, the presence of digital ulcers, and severity of Raynaud's phenomenon had the largest associations. Effective interventions are needed to improve the management of hand function in patients with SSc.
- Magnetic resonance imaging findings in dogs with orbital inflammation. [Journal Article]
- JSJ Small Anim Pract 2018 Oct 02
- CONCLUSIONS: MRI outlines the extent of retrobulbar inflammation. Clinical findings were associated with imaging findings. MRI overestimated the presence of foreign bodies.
- Calcium increases titin N2A binding to F-actin and regulated thin filaments. [Journal Article]
- SRSci Rep 2018 Oct 01; 8(1):14575
- Mutations in titin are responsible for many cardiac and muscle diseases, yet the underlying mechanisms remain largely unexplained. Numerous studies have established roles for titin in muscle function...
Mutations in titin are responsible for many cardiac and muscle diseases, yet the underlying mechanisms remain largely unexplained. Numerous studies have established roles for titin in muscle function, and Ca2+-dependent interactions between titin and actin have been suggested to play a role in muscle contraction. The present study used co-sedimentation assays, dynamic force spectroscopy (DFS), and in vitro motility (IVM) assays to determine whether the N2A region of titin, overlooked in previous studies, interacts with actin in the presence of Ca2+. Co-sedimentation demonstrated that N2A - F-actin binding increases with increasing protein and Ca2+ concentration, DFS demonstrated increased rupture forces and decreased koff in the presence of Ca2+, and IVM demonstrated a Ca2+-dependent reduction in motility of F-actin and reconstituted thin filaments in the presence of N2A. These results indicate that Ca2+ increases the strength and stability of N2A - actin interactions, supporting the hypothesis that titin plays a regulatory role in muscle contraction. The results further support a model in which N2A - actin binding in active muscle increases titin stiffness, and that impairment of this mechanism contributes to the phenotype in muscular dystrophy with myositis. Future studies are required to determine whether titin - actin binding occurs in skeletal muscle sarcomeres in vivo.
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- Diagnostic potential of sarcoplasmic MxA expression in subsets of dermatomyositis. [Journal Article]
- NANeuropathol Appl Neurobiol 2018 Sep 28
- CONCLUSIONS: Sarcoplasmic MxA expression is more sensitive than PFA and RIG-I expression for a pathological diagnosis of DM, regardless of the autoantibody-related subgroup. In light of its high sensitivity and specificity, it may be considered a pathological hallmark of DM per se. Also, lack of MxA expression in ASS supports the idea that ASS is a distinct entity from DM. This article is protected by copyright. All rights reserved.