239th ENMC International Workshop: Classification of dermatomyositis, Amsterdam, the Netherlands, 14–16 December 2018

Mammen, Andrew L.; Allenbach, Yves; Stenzel, Werner; Benveniste, Olivier

doi:10.1016/j.nmd.2019.10.005

Cited by 187 publications

(173 citation statements)

References 172 publications

(169 reference statements)

Supporting

Mentioning

155

Contrasting

Unclassified

Order By: Relevance

“…Moreover, we observed a dilatation of blood vessels in the perimysium in the majority of cases, as previously reported (19), as well as the capillaries enlargement which is usual in DM (8,32). Nevertheless, only a minority of edematous myositis patients met the other specific pathological criteria for DM classification (7,21) with only 12% of cases with a perifascicular atrophy. Finally, there was a predominance of CD8 + T cells, while DM has been traditionally associated with CD4 + T cells infiltrates (13,14).…”

Section: Discussionsupporting

confidence: 88%

“…The exclusion criteria were: severe kidney, liver or heart failure, as well as patients with systemic sclerosis since edema may be a feature at disease onset. IMNM, DM and anti-synthetase patients based on clinico-serological and/or clinico-pathological criteria (2,21,28) without clinical limb edema were included as controls.…”

Section: Patientsmentioning

confidence: 99%

“…Idiopathic inflammatory myopathies (IIM) are a heterogeneous condition involving the skeletal muscle but also displaying extra-muscular features. Recently, myositis-specific antibodies (MSA) have been identified and permitted to refine classification criteria (4,(21)(22)28). Using a clinico-serological and/or pathological approaches, IIM were classified into: dermatomyositis (DM), inclusion body myositis (IBM), and polymyositis (PM) (28) or immune-mediated necrotizing myopathy (IMNM), and anti-synthetase syndrome (ASyS) (22).…”

Section: Introductionmentioning

confidence: 99%

“…Using a clinico-serological and/or pathological approaches, IIM were classified into: dermatomyositis (DM), inclusion body myositis (IBM), and polymyositis (PM) (28) or immune-mediated necrotizing myopathy (IMNM), and anti-synthetase syndrome (ASyS) (22). Among the extra-muscular features, only the presence of a classical DM skin rash has been included as a classification criterion (21,28) yet other skin manifestations have been described in IIM.…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Edematous myositis: a clinical presentation first suggesting dermatomyositis diagnosis

et al. 2020

Self Cite

View full text Add to dashboard Cite

Aims: Edema of the limbs is uncommon in idiopathic inflammatory myopathies (IIM). The few reported cases have been associated with severe and refractory dermatomyositis (DM), sometimes in association with cancers. We aimed to determine if edematous myositis is a homogeneous subtype based on clinical, serological and pathological features. Methods: This is a retrospective observational study performed between 2008 and 2015 in the French national referral center for myositis. All adult patients with an inflammatory muscle biopsy and upper limbs edema were included as well as IIM cases without limb edema as controls. Clinical, biological and pathological features were collected. Results: Seventeen edematous myositis were included and compared to 174 IIM without edema, including 50 DM controls. Edema was the first manifestation in 23% of patients. Muscle weakness was severe and symmetric, 71% of patients presented dysphagia and a restrictive ventilatory pattern was found in 40%. Fifty‐two percent of patients had a typical DM skin rash and 23% had cancer within 3 years of diagnosing myositis. Fifty‐three percent of patients presented a myositis specific antibody and only DM‐specific antibodies were detected. Classic pathological DM features (perifascicular atrophy, perifascicular/perimysial perivascular inflammation) were uncommon but capillary C5b‐9 deposition and MxA expression were seen in 79% and 73% of cases, respectively. A perimysial edema was found in 82% of cases. Seventeen percent of patients died (median follow up of 18 months). Edematous myositis demonstrated more marked capillary C5b‐9 deposition compared to IIM controls. There was no clinical, biological or pathological difference with DM controls except for limb edema. Conclusion: Our study underlines that limb edema could be a symptom of IIM and that edematous myositis are mostly DM. The vasculopathy seems to play a key role in its pathophysiology. Limb edema associated with muscle impairment should suggest the diagnosis of DM in clinical settings.

show abstract

Section: Discussionsupporting

confidence: 88%

Section: Patientsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Edematous myositis: a clinical presentation first suggesting dermatomyositis diagnosis

et al. 2020

Self Cite

View full text Add to dashboard Cite

show abstract

“…The term "myositis" is often used interchangeably with "idiopathic inflammatory myopathy" (IIM), referring to primary autoimmune diseases of muscle including dermatomyositis, inclusion body myositis (IBM), antisynthetase syndrome, and necrotizing autoimmune myopathy [1][2][3][4][5][6]. However, there are also known causes of inflammatory myopathies which must be considered in the evaluation of patients with acute or subacute myopathy, including infections, drugs, mixed connective tissue disease, and malignancies.…”

Section: Introductionmentioning

confidence: 99%

Secondary Causes of Myositis

Berth

Lloyd

2020

Curr Treat Options Neurol

View full text Add to dashboard Cite

show abstract

Association Between SARS-CoV-2 Infection and Immune-Mediated Myopathy in Patients Who Have Died

et al. 2021

Self Cite

View full text Add to dashboard Cite

IMPORTANCE Myalgia, increased levels of creatine kinase, and persistent muscle weakness have been reported in patients with COVID-19.OBJECTIVE To study skeletal muscle and myocardial inflammation in patients with COVID-19 who had died. DESIGN, SETTING, AND PARTICIPANTSThis case-control autopsy series was conducted in a university hospital as a multidisciplinary postmortem investigation. Patients with COVID-19 or other critical illnesses who had died between March 2020 and February 2021 and on whom an autopsy was performed were included. Individuals for whom informed consent to autopsy was available and the postmortem interval was less than 6 days were randomly selected. Individuals who were infected with SARS-CoV-2 per polymerase chain reaction test results and had clinical features suggestive of COVID-19 were compared with individuals with negative SARS-CoV-2 polymerase chain reaction test results and an absence of clinical features suggestive of COVID-19. MAIN OUTCOMES AND MEASURES Inflammation of skeletal muscle tissue was assessed by quantification of immune cell infiltrates, expression of major histocompatibility complex (MHC) class I and class II antigens on the sarcolemma, and a blinded evaluation on a visual analog scale ranging from absence of pathology to the most pronounced pathology. Inflammation of cardiac muscles was assessed by quantification of immune cell infiltrates. RESULTS Forty-three patients with COVID-19 (median [interquartile range] age, 72 [16] years; 31 men [72%]) and 11 patients with diseases other than COVID-19 (median [interquartile range] age, 71 [5] years; 7 men [64%]) were included. Skeletal muscle samples from the patients who died with COVID-19 showed a higher overall pathology score (mean [SD], 3.4 [1.8] vs 1.5 [1.0]; 95% CI, 0-3; P < .001) and a higher inflammation score (mean [SD], 3.5 [2.1] vs 1.0 [0.6]; 95% CI, 0-4; P < .001). Relevant expression of MHC class I antigens on the sarcolemma was present in 23 of 42 specimens from patients with COVID-19 (55%) and upregulation of MHC class II antigens in 7 of 42 specimens from patients with COVID-19 (17%), but neither were found in any of the controls. Increased numbers of natural killer cells (median [interquartile range], 8 [8] vs 3 [4] cells per 10 high-power fields; 95% CI, 1-10 cells per 10 high-power fields; P < .001) were found. Skeletal muscles showed more inflammatory features than cardiac muscles, and inflammation was most pronounced in patients with COVID-19 with chronic courses. In some muscle specimens, SARS-CoV-2 RNA was detected by reverse transcription-polymerase chain reaction, but no evidence for a direct viral infection of myofibers was found by immunohistochemistry and electron microscopy. CONCLUSIONS AND RELEVANCEIn this case-control study of patients who had died with and without COVID-19, most individuals with severe COVID-19 showed signs of myositis ranging from mild to severe. Inflammation of skeletal muscles was associated with the duration of illness and was more pronounced than cardiac inflammation. Det...

show abstract

239th ENMC International Workshop: Classification of dermatomyositis, Amsterdam, the Netherlands, 14–16 December 2018

Cited by 187 publications

References 172 publications

Edematous myositis: a clinical presentation first suggesting dermatomyositis diagnosis

Edematous myositis: a clinical presentation first suggesting dermatomyositis diagnosis

Secondary Causes of Myositis

Association Between SARS-CoV-2 Infection and Immune-Mediated Myopathy in Patients Who Have Died

Contact Info

Product

Resources

About