Prevalence and clinical characteristics of adult polymyositis and dermatomyositis; data from a large and unselected Norwegian cohort

Dobloug, Cecilie; Garen, Torhild; Bitter, Helle; Stjärne, Johan; Stenseth, Guri; Grøvle, Lars; Sem, Marthe; Gran, Jan Tore; Molberg, Øyvind

doi:10.1136/annrheumdis-2013-205127

Cited by 118 publications

(88 citation statements)

References 27 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The estimated annual incidence of dermatomyositis/polymyositis ranges from 6 to 10 per 1 000 000 [6]. Cutaneous signs, the hallmark of dermatomyositis, often are the initial presentation of disease and may or may not be associated with muscle manifestations.…”

Section: Introductionmentioning

confidence: 99%

Idiopathic inflammatory myopathies and the lung

et al. 2015

View full text Add to dashboard Cite

Idiopathic inflammatory myositis (IIM) is a group of rare connective tissue diseases (CTDs) characterised by muscular and extramuscular signs, in which lung involvement is a challenging issue. Interstitial lung disease (ILD) is the hallmark of pulmonary involvement in IIM, and causes morbidity and mortality, resulting in an estimated excess mortality of 50% in some series. Except for inclusion body myositis, these extrapulmonary disorders are associated with the general and visceral involvement frequently found in other CTDs including fever, Raynaud's phenomenon, arthralgia, nonspecific cutaneous modifications and ILD, for which the prevalence is estimated to be up to 65%. Substantial heterogeneity exists within the spectrum of IIMs, and each condition is associated with various frequencies and subtypes of pulmonary involvement. This heterogeneity is partly related to the presence of various autoantibodies encompassing anti-synthetase, anti-MDA5 and anti-PM/Scl. ILD is present in all subsets of IIM including juvenile myositis, but is more frequent in dermatomyositis and overlap myositis. IIM can also be associated with other presentations of respiratory involvement, namely pulmonary arterial hypertension, pleural disease, infections, drug-induced toxicity, malignancy and respiratory muscle weakness. Here, we critically review the current knowledge about adult and juvenile myositis-associated lung disease with a detailed description of therapeutics for chronic and rapidly progressive ILD. @ERSpublications Interstitial lung disease is a leading cause of morbidity in dermatomyositis/polymyositis

show abstract

Section: Introductionmentioning

confidence: 99%

Idiopathic inflammatory myopathies and the lung

et al. 2015

View full text Add to dashboard Cite

show abstract

“…Similar to our patients, pneumonia has been reported as the main cause of death in other IIM studies. 6,12,13 Long-term prognosis of patients with IIM and lung involvement is poor, with a mortality rate of 27% 34,45 and a 5-year survival of approximately 50%, 46 especially when it is associated to infections or complications such as aspiration pneumonia/ pneumonitis, muscle weakness, drug-induced disease, pulmonary congestion secondary to heart failure, pulmonary hypertension, pneumomediastinum, pneumothorax, or massive subcutaneous emphysema. 47 Factors associated with mortality in IIM are diverse.…”

Section: Discussionmentioning

confidence: 99%

Clinical and Prognostic Factors Associated With Survival in Mexican Patients With Idiopathic Inflammatory Myopathies

Galindo-Feria

Rojas-Serrano

Hinojosa‐Azaola

2016

JCR: Journal of Clinical Rheumatology

View full text Add to dashboard Cite

show abstract

“…The autoimmune myopathies are rare diseases, with a prevalence of just 9–14 cases per 100,000 people [14, 15], and only ~ 10% of these have either anti-SRP or anti-HMGCR myopathy [9, 16••]. As will be discussed in more detail, both anti-SRP and anti-HMGCR myopathies can occur in children [17••, 18••, 19•, 20].…”

Section: Epidemiologymentioning

confidence: 99%

Immune-Mediated Necrotizing Myopathy

2018

View full text Add to dashboard Cite

Purpose of Review Immune-mediated necrotizing myopathy (IMNM) is a type of autoimmune myopathy characterized by relatively severe proximal weakness, myofiber necrosis with minimal inflammatory cell infiltrate on muscle biopsy, and infrequent extra-muscular involvement. Here, we will review the characteristics of patients with IMNM. Recent Findings Anti-signal recognition particle (SRP) and anti-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) autoantibodies are closely associated with IMNM and define unique subtypes of patients. Importantly, the new European Neuromuscular Centre criteria recognize anti-SRP myopathy, anti-HMGCR myopathy, and autoantibody-negative IMNM as three distinct subtypes of IMNM. Anti-SRP myopathy patients have more severe muscle involvement, have more common extra-muscular features, and may respond best to immunosuppressive regimens that include rituximab. In contrast, anti-HMGCR myopathy is often associated with statin exposure and intravenous immunoglobulin treatment may be an effective treatment, even as monotherapy. Both anti-SRP and anti-HMGCR myopathy tend to be most severe in younger patients. Furthermore, children with these forms of IMNM may present with dystrophy-like features which are potentially reversible with immunosuppressant treatment. IMNM patients with either autoantibody may experience fatty replacement of muscle soon after disease onset, suggesting that intense and early immunosuppressant therapy may provide the best chance to avoid long-term disability. Summary IMNM is composed of anti-SRP myopathy, anti-HMGCR myopathy, and autoantibody-negative IMNM. Both anti-SRP and anti-HMGCR myopathy can cause severe weakness, especially in younger patients. Anti-SRP myopathy patients tend to have the most severe weakness and most prevalent extra-muscular features. Autoantibody-negative IMNM remains poorly described.

show abstract

Prevalence and clinical characteristics of adult polymyositis and dermatomyositis; data from a large and unselected Norwegian cohort

Abstract: Our data indicate that the population prevalence of PM/DM in Caucasians is quite low, but underscores the complexity and severity of the disorders.

Cited by 118 publications

References 27 publications

Idiopathic inflammatory myopathies and the lung

Idiopathic inflammatory myopathies and the lung

Clinical and Prognostic Factors Associated With Survival in Mexican Patients With Idiopathic Inflammatory Myopathies

Immune-Mediated Necrotizing Myopathy

Contact Info

Product

Resources

About