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Purpose of the study Myositis is strongly associated with malignancy with a prevalence varying between 6.7 and 32%. This association is predominantly found in dermatomyositis (DM) and in immune-mediated necrotizing myopathy (IMNM). Anti-transcriptional intermediary factor 1 gamma (TIF-1γ) antibody, a myositis specific autoantibody, is associated with a high malignancy rate in adults with DM. The pathogenesis behind this association remains unclear. Recent findings TIF-1γ is a ubiquitous molecule, involved in various biological pathways of which the TGF-β pathway is the most important. It mediates transcription through posttranslational modifications. Furthermore, TIF-1γ can either act as a tumor suppressor or as a tumor promoter, depending on the cellular context and cancer stage. Using anti-TIF-1γ antibodies to predict cancer-associated DM has a specificity of 89% [95% CI: 82-93%] and a sensitivity of 78% [95% CI: 45-94%] and is useful for diagnosis and malignancy risk stratification in myositis. Expression of TIF-1γ is elevated in affected muscle and skin tissue in myositis patients and in several cancers associated with myositis, suggesting that these proteins share antigenic similarity. When cancerous tissues express tumor-specific antigens due to mutations or loss-of-heterozygosity in TIF-1γ alleles, the immune system promotes antibody production against these neoantigens. Hypothetically, the newly formed antibodies cross-react with antigens expressed in myoblasts during regeneration, resulting in myositis. Conclusion Anti-TIF-1γ autoantibodies are strongly associated with the presence of cancer. Increasing data provide potential pathophysiological insights, linking anti-TIF-1γ autoantibodies to the anti-tumor response.

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Objectives: The benefits of rituximab as a therapy for idiopathic inflammatory myopathies (IIMs) remain unclear. The Rituximab-In-Myositis trial suggested that antisynthetase antibody-positive IIM patients could benefit from rituximab treatment. This paper aims to gather, assess and summarize all articles that report on ≥5 rituximab-treated antisynthetase antibody-positive IIM patients. Methods: A search of the Pubmed, Embase, and Web of Science databases for IIM and rituximab was performed. All results obtained were screened. Reports that were judged to possibly contain data on 5 or more rituximab-treated antisynthetase patients were further examined. Every article that met the inclusion criteria was included in the summary table. Results: The search, performed on the 3rd of November 2022, netted 2717 original papers. After the initial screening, 216 full papers were assessed for eligibility. Twenty-two studies were included in the summary table. These papers were primarily retrospective cohorts. A total of 326 patients had outcomes reported. Conclusion: The post hoc analysis of the RIM trial (N=30) remains the largest least-biased group of reported patients that meet the inclusion criteria. In this trial, over 90% of antisynthetase patients treated with RTX met the definition of improvement, and presence of antisynthetase antibodies predicted a shorter time to improvement (HR 3.08). The rest of the included reports suggest that rituximab as a third-line treatment can net good results, even in refractory patients. Biases of the included studies should be considered. An individual risk-benefit assessment should be made when considering rituximab in IIM patients.

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Systemic sclerosis (SSc) affects the upper gastro-intestinal (GI) system in up to 90% of patients. This systematic review investigates the role of high-resolution manometry (HRM) in systemic sclerosis, by evaluating correlation between HRM abnormalities and upper gastro-intestinal symptoms or other organ involvement, and reports on effects of therapeutic interventions on HRM findings. A literature search was performed in PubMed and Embase to identify relevant articles using the keywords “HRM” and “systemic sclerosis”. Of 43 articles, 17 met all study selection criteria and were included. The majority of studies assessed correlation between esophageal symptoms and esophageal dysmotility as primary outcome. A limited number of studies assessed the effectiveness of buspirone in SSc patients. Study quality assessment shows that most (11/18) studies were of very good or good quality. Studies that assessed upper GI symptoms using validated questionnaires such as the UCLA SCLC GIT 2.0 or GISSI score found an association between absent contractility on HRM and upper GI symptoms. Asymptomatic patients often have oesophageal body dysmotility when assessed by HRM. Oesophageal body dysmotility on HRM appears positively correlate with presence of interstitial lung disease (ILD) on high-resolution computed tomography (HRCT) while also correlating with DLCO <0.8 of predicted value on pulmonary function testing. There is contradictory evidence on the association between autoantibody profiles and skin involvement with HRM abnormalities. Buspirone appears beneficial as it increases lower oesophageal sphincter (LES) resting pressure and reduces upper GI symptoms. In conclusion, patients describing severe upper GI symptoms using the UCLA SCLC GIT 2.0 or GISSI scores can be expected to have severe oesophageal body dysmotility on HRM. Oesophageal body dysmotility on HRM seems to positively correlate with presence of ILD. Buspirone shows promising benefits as add-on therapy for upper GI symptoms in systemic sclerosis.

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Dermatomyositis (DM) is an auto-immune myopathy characterized by muscle weakness and skin involvement. Oral glucocorticoids and immune suppressive agents are considered to be first line treatment for muscular involvement, but skin lesions are often refractory. The majority of DM studies focus on muscle disease. However, skin involvement has a substantial negative impact on quality of life. Evidence on therapy for recalcitrant cutaneous DM is limited. In this article we report a case of refractory cutaneous DM patient that responded favorably to thalidomide. In addition, we reviewed the literature on the available treatment options. Only four cases reported on the efficacy of thalidomide in DM. In general, studies on second line drugs are limited, with only one valuable randomized controlled trial demonstrating efficacy of rituximab in skin dominated DM. As such, we can conclude that evidence is lacking for treating recalcitrant skin involvement in DM and that further randomized controlled trials are needed to validate current low-level evidence.