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Medicine Jan 2024The European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for idiopathic inflammatory myopathies (IIM) have been widely...
The European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for idiopathic inflammatory myopathies (IIM) have been widely used in recent times. However, no studies have focused on electromyography (EMG) findings of IIM, considering the criteria. This study aimed to elucidate the frequency of EMG abnormalities, particularly fibrillation potentials and positive sharp waves (Fib/PSW), the most objective EMG findings of IIM. Clinical and EMG records of adult patients who were clinically diagnosed with polymyositis (PM), dermatomyositis (DM), amyopathic DM (ADM), or inclusion body myositis (IBM) were retrospectively reviewed and classified according to the EULAR/ACR classification criteria. The frequency of Fib/PSW in EMG was investigated in the recruited cases. Seventy-nine patients with clinically diagnosed IIM (44 with PM, 17 with DM, 7 with ADM, and 11 with IBM) were recruited. After classification using EULAR/ACR, 75 satisfied definite or probable IIM (61 and 14, respectively), and the frequency of Fib/PSW in this group was 95%. Furthermore, the remaining 4 patients with insufficient IIM probability also showed Fib/PSW. Fib/PSW may also be seen in cases with insufficient IIM probability not satisfying the criteria. EMG may help detect muscle involvement in these cases through Fib/PSW.
Topics: Adult; Humans; Rheumatology; Retrospective Studies; Myositis; Dermatomyositis; Rheumatic Diseases; Collagen Diseases; Myositis, Inclusion Body
PubMed: 38277547
DOI: 10.1097/MD.0000000000037105 -
Immunity, Inflammation and Disease Jan 2024Little is known about the features of macrophage activation syndrome (MAS) in dermatomyositis, especially the association between rapidly progressive interstitial lung...
BACKGROUND
Little is known about the features of macrophage activation syndrome (MAS) in dermatomyositis, especially the association between rapidly progressive interstitial lung disease (RP-ILD) and MAS.
OBJECTIVE
To determine the characteristics of MAS in patients with dermatomyositis and their association with RP-ILD.
METHODS
This was a retrospective cohort study of 201 dermatomyositis patients at the First Affiliated Hospital of Zhejiang University over a 10-year period.
RESULTS
A total of 22 (10.9%) patients were diagnosed with MAS. The rate of RP-ILD was significantly higher in patients with MAS than in those without MAS (81.8% vs. 17.4%, respectively, p < .001). Multivariate analysis indicated that RP-ILD (p = .019), ferritin level > 1685 ng/mL (p = .007) and hemoglobin < 100 g/L (p = .001) were independent risk factors for MAS. Furthermore, RP-ILD patients with MAS presented more cardiac injury (50.0% vs. 13.3%, respectively, p < .009), central nervous system dysfunction (42.8% vs. 3.4%, respectively, p < .001) and hemorrhage (38.9% vs. 3.3%, respectively, p = .003) than RP-ILD patients without MAS. The 90-day cumulative survival rate for patients with MAS was significantly lower than for those without MAS (18.2% vs. 82.1%, respectively, p < .001).
CONCLUSION
MAS was a common and fatal complication of dermatomyositis in our cohort. MAS is closely related to RP-ILD in patients with dermatomyositis. When RP-ILD is present in dermatomyositis patients with abnormal laboratory findings, such as cytopenia and hyperferritinemia, the presence of MAS should be considered.
Topics: Adult; Humans; Retrospective Studies; Case-Control Studies; Dermatomyositis; Macrophage Activation Syndrome; Lung Diseases, Interstitial
PubMed: 38270325
DOI: 10.1002/iid3.1141 -
Briefings in Bioinformatics Nov 2023Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of muscle disorders including adult and juvenile dermatomyositis, polymyositis, immune-mediated... (Review)
Review
Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of muscle disorders including adult and juvenile dermatomyositis, polymyositis, immune-mediated necrotising myopathy and sporadic inclusion body myositis, all of which present with variable symptoms and disease progression. The identification of effective biomarkers for IIMs has been challenging due to the heterogeneity between IIMs and within IIM subgroups, but recent advances in machine learning (ML) techniques have shown promises in identifying novel biomarkers. This paper reviews recent studies on potential biomarkers for IIM and evaluates their clinical utility. We also explore how data analytic tools and ML algorithms have been used to identify biomarkers, highlighting their potential to advance our understanding and diagnosis of IIM and improve patient outcomes. Overall, ML techniques have great potential to revolutionize biomarker discovery in IIMs and lead to more effective diagnosis and treatment.
Topics: Adult; Humans; Myositis; Dermatomyositis; Autoimmune Diseases; Biomarkers; Disease Progression
PubMed: 38243695
DOI: 10.1093/bib/bbad514 -
RMD Open Jan 2024To evaluate the effectiveness and safety of upadacitinib in treatment-refractory inflammatory myositis.
OBJECTIVES
To evaluate the effectiveness and safety of upadacitinib in treatment-refractory inflammatory myositis.
METHODS
Patients with refractory inflammatory myositis treated with upadacitinib from a single urban centre in Vancouver, British Columbia, Canada, were included from September 2020 to June 2023. The medical records of these patients were retrospectively reviewed.
RESULTS
10 total patients were identified for review, including 5 classic dermatomyositis (DM), 3 amyopathic DM (ADM) and 2 antisynthetase syndrome. The patients failed an average of four immunosuppressants before initiation of upadacitinib. Three had prior Janus kinase inhibitor therapy with tofacitinib. In the classic DM and ADM aggregate group, upadacitinib offered clinically and statistically significant cutaneous improvement. Lack of active muscle disease at baseline precluded analysis of the effect of upadacitinib on muscle weakness. Nine patients remained on upadacitinib at the end of the study period. One patient discontinued upadacitinib due to severe facial acne.
CONCLUSION
Upadacitinib appears to be effective in targeting cutaneous manifestations of refractory inflammatory DM. Further research is still needed to validate its efficacy on a broader population scale.
Topics: Humans; Retrospective Studies; Dermatomyositis; Myositis; Heterocyclic Compounds, 3-Ring
PubMed: 38242552
DOI: 10.1136/rmdopen-2023-003837 -
Arthritis Research & Therapy Jan 2024Dermatomyositis is an idiopathic inflammatory myopathy characterised by rashes and progressive muscle weakness. The recent ProDERM (Progress in DERMatomyositis) study is... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
Dermatomyositis is an idiopathic inflammatory myopathy characterised by rashes and progressive muscle weakness. The recent ProDERM (Progress in DERMatomyositis) study is the first large randomised, placebo-controlled trial to establish the efficacy and safety of intravenous immunoglobulin (IVIg) in adult patients with dermatomyositis. Objectives of this analysis were to closely examine the safety and tolerability of IVIg in patients from the ProDERM study.
METHODS
ProDERM was a double-blind, randomised, placebo-controlled, multicentre, phase 3 study. In the first period (weeks 0-16), adults with active dermatomyositis received 2.0 g/kg IVIg (Octagam 10%; Octapharma AG) or placebo every 4 weeks. In the open-label extension period (weeks 16-40), all patients received IVIg for 6 additional cycles; dose reduction (1.0 g/kg) was permitted if patients were stable. Treatment-emergent adverse events (TEAEs) were documented.
RESULTS
The 95 patients enrolled were randomised to receive IVIg (N = 47) or placebo (N = 48) in the first period, with 5 switching from placebo to IVIg. Overall, 664 IVIg infusion cycles were administered. During the first period, 113 TEAEs were possibly/probably related to treatment in 30/52 patients (57.7%) receiving IVIg and 38 in 11 patients (22.9%) on placebo. Eight patients discontinued therapy due to IVIg-related TEAEs. Eight thromboembolic events (TEEs) occurred in six patients on IVIg; six in five patients were deemed possibly/probably related to IVIg. Patients with TEEs exhibited more baseline TEE risk factors than those without TEEs (2.4-15.2-fold higher). Lowering infusion rate reduced the rate of TEEs, and none occurred at the lower IVIg dose. No haemolytic transfusion reactions or deaths occurred.
CONCLUSIONS
Results from this study demonstrate that IVIg has a favourable safety profile for treatment of adult dermatomyositis patients and provides evidence that will help to inform treatment choice for these patients. Dermatomyositis patients receiving high-dose IVIg should be monitored for TEEs, and a low rate of infusion should be used to minimise TEE risk, particularly in those with pre-existing risk factors.
TRIAL REGISTRATION
ProDERM study (NCT02728752).
Topics: Adult; Humans; Immunoglobulins, Intravenous; Dermatomyositis; Infusions, Intravenous; Myositis; Double-Blind Method; Treatment Outcome
PubMed: 38233885
DOI: 10.1186/s13075-023-03232-2 -
Annals of Medicine and Surgery (2012) Jan 2024Mixed connective tissue disease (MCTD) is characterized by high titres of distinct antibodies: U1 ribonucleoprotein with variable clinical features seen in rheumatoid...
BACKGROUND
Mixed connective tissue disease (MCTD) is characterized by high titres of distinct antibodies: U1 ribonucleoprotein with variable clinical features seen in rheumatoid arthritis, systemic lupus erythematosus, scleroderma, polymyositis, and dermatomyositis. Limited case reports revealed the association between MCTD and cancer, like lymphoma, lung cancers, and others.
CASE PRESENTATION
A 22-year-old female presented with enlargement of the abdomen and oedema of the lower extremities, gradually started 25 days The patient had been diagnosed to have rheumatoid arthritis. She was treated with 7.5 mg/week MTX for 6 months. Physical examination revealed: pallor, lower limb oedema, with synovitis and deformities of hands. The laboratory tests showed anaemia, elevated levels of creatine phosphokinase ESR, positivity of antinuclear antibody, anti-ds DNA, and antinuclear ribonucleoprotein. Urinary protein excretion was 1625 mg/24 h. Chest X-ray showed bilateral pleural effusion. Echocardiography revealed pericardial effusion Thoracic-abdominal and pelvic tomography showed a heterogeneous mass with a diameter of 5 × 6 cm at the expense of the right ovary. The mass was removed surgically, and a biopsy was taken, and was compatible with ovarian high-grade serous adenocarcinoma. A course of solumedrol 1 g/IV/3 days was applied, and then continue with 60 mg/day oral predlone. Later on discharge, she was taken 25 mg/day predlone, and methotrexate 10 mg.
CONCLUSIONS
Our case showed that the patient had no risk factors for developing ovary cancer. On the contrary, our patient was a young, non-smoker, without any previous treatment before the RA diagnosis was taken, and finally, she had 3 children with full-term pregnancy, and well health. This case highlights the importance of maintaining a high index of suspicion for malignancy in MCTD patients. However, further investigation on the role of the immune system in the development of ovarian cancer in women with autoimmune diseases including MCTD remains necessary.
PubMed: 38222766
DOI: 10.1097/MS9.0000000000001462 -
Severe gastrointestinal involvements in patients with adult dermatomyositis with anti-NXP2 antibody.RMD Open Jan 2024Gastrointestinal (GI) involvements were scarcely reported in adult anti-nuclear matrix protein 2 (NXP2) dermatomyositis (NXP2DM). In this study, we investigated the...
OBJECTIVE
Gastrointestinal (GI) involvements were scarcely reported in adult anti-nuclear matrix protein 2 (NXP2) dermatomyositis (NXP2DM). In this study, we investigated the clinical, pathological and molecular features as well as treatment options of this rare yet life-threatening disease.
METHODS
We retrospectively collected the data of the cohort of NXP2 DM from 2012 to 2022 in our hospital. RNA sequencing was performed in intestinal samples of perforated patients compared with healthy controls data set.
RESULTS
A total of 56 patients with adult NXP2DM were collected including 10 cases with GI involvements. Abdominal pain and melena were the initial manifestations for GI involvements with a median 10-month time lag after the diagnosis of NXP2DM when myositis largely subsided. Within weeks, GI perforation occurred in 8 of 10 patients, while five patients underwent eight surgical interventions subsequently. The short-term mortality was observed in four patients. NXP2DM with GI involvements presented with more extramuscular systemic manifestations such as interstitial lung disease and subcutaneous calcinosis. The GI pathological features encompassed vasculitis/vasculopathy with high MxA expression, intestinal smooth muscle necrosis and serosal calcinosis. Gene expression profile validated the type-I interferon activation and revealed that epithelial mesenchymal transition and focal adhesion pathway may also contribute. Finally, vedolizumab, an anti-α4β7-integrin monoclonal antibody, exhibited promising therapeutic signals which should be further investigated.
CONCLUSIONS
GI involvement is a unique complication in patients with adult NXP2+DM. Timely recognition and targeted therapy may turn out to be lifesaving.
Topics: Adult; Humans; Dermatomyositis; Retrospective Studies; Myositis; Calcinosis; Interferon Type I
PubMed: 38199847
DOI: 10.1136/rmdopen-2023-003901 -
Journal of Clinical Neurology (Seoul,... Jan 2024Advances in serological tests are transforming the classification of idiopathic inflammatory myopathy (IIM). The new criteria suggested by the 119th European...
BACKGROUND AND PURPOSE
Advances in serological tests are transforming the classification of idiopathic inflammatory myopathy (IIM). The new criteria suggested by the 119th European Neuromuscular Center international workshop divide IIM cases into four main diseases according to clinical and pathological findings, adding immune-mediated necrotizing myositis and nonspecific myositis to the classic categories of polymyositis and dermatomyositis.
METHODS
Seventy one cases of IIM with sufficient available clinical and pathological data were reviewed to be reclassified according to the new criteria.
RESULTS
Most of the cases previously classified as polymyositis (77.8%, 35/45) were reclassified as immune-mediated necrotizing myopathy. The results of myositis-specific antibodies matched well with the new clinicopathological classification.
CONCLUSIONS
This new clinicopathological classification for IIM in combination with serological test results could be applied to our previous case series. Adoption of the new criteria will lead to a better understanding of the disease and hence new therapeutic insights.
PubMed: 38179634
DOI: 10.3988/jcn.2022.0432 -
RMD Open Jan 2024To explore the toxicity of low-dose methotrexate (MTX), an uncommon, but life-threatening event.
OBJECTIVES
To explore the toxicity of low-dose methotrexate (MTX), an uncommon, but life-threatening event.
METHODS
We analysed the presentation, course and risk factors of all patients admitted to the rheumatology ward with severe low-dose MTX toxicity. These patients were compared with patients without signs of relevant MTX toxicity.
RESULTS
The 12 patients admitted for MTX toxicity included 7 patients with rheumatoid arthritis, 2 with psoriatic arthritis or psoriasis, 2 patients with giant cell arteritis and 1 with myositis. 1 patient died from infections, while 11 survived under folinic acid administration. All patients suffering from severe MTX toxicity were older than 70 years and were therefore compared with 400 patients who were also older than 70 years, but without MTX toxicity. Of these 400 control patients, the group of patients not on MTX (n=232) had more renal impairment than the group of patients on MTX (n=168). Compared with the 168 MTX-treated patients without toxicity, the 12 patients with life-threatening toxic events had a lower median estimated glomerular filtration rate (eGFR) at the routine visit preceding the acute event (64 (range 32-77) vs 69 (range 8 to >90) mL/min x 1.73, p=0.0251). A multivariate analysis found that patients with toxicity were more frequently treated with diuretics (6/12 vs 24/168), proton pump inhibitors (PPIs; 10/12 vs 70/168) and levetiracetam (2/12 vs 1/168).
CONCLUSIONS
Patients older than 70 years with lower eGFR and being on diuretics, but also on PPIs and levetiracetam, have a significantly higher risk for MTX toxicity.
Topics: Humans; Aged; Methotrexate; Diuretics; Levetiracetam; Arthritis, Rheumatoid; Psoriasis
PubMed: 38176739
DOI: 10.1136/rmdopen-2023-003827 -
Juvenile dermatomyositis with scleroderma features - when skin thickening is not systemic sclerosis.ARP Rheumatology 2023
Topics: Humans; Dermatomyositis; Sclerosis; Scleroderma, Systemic; Scleroderma, Localized; Skin Abnormalities
PubMed: 38174756
DOI: No ID Found