Epidemiology of the idiopathic inflammatory myopathies

Khoo, Thomas; Lilleker, James B.; Thong, Bernard Yu-Hor; Leclair, Valérie; Lamb, Janine A.; Chinoy, Hector

doi:10.1038/s41584-023-01033-0

Cited by 12 publications

(5 citation statements)

References 238 publications

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“…According to a study conducted by Khoo et al in 2023, the estimated incidence rate of this condition is, between 0.2 to 2 cases per 100,000 person per year, while the prevalence ranges from 2 to 25 cases per 100,000 people per year. These variations can be attributed to factors such as diagnostic criteria, population genetics and environmental influences (7). A recent report by Cho et al showed that the prevalence of IIM in Korea ranges from 2.3 to 4.0 per 100,000 person-years.…”

Section: Discussionmentioning

confidence: 99%

“…Using a comprehensive population database is crucial for conducting epidemiological studies on these diseases. This allows for the identification of disparities in terms of geography and demograph-ics, as well as gaining a thorough understanding of their characteristics and different causes (6)(7)(8)(9)(10)(11). In terms of epidemiological clinical trials, a recent study conducted in Norway determined that the point prevalence of PM and DM to be 8.7 per 100,000.…”

Section: Introductionmentioning

confidence: 99%

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The prevalence, epidemiological characteristics and mortality trends of inflammatory myopathies patients in Oman: the Prevision study

Al Adhoubi,

Liyanage,

Al Salmi

et al. 2024

Clinical and Experimental Rheumatology

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ObjectiveThis research aims to investigate the prevalence, epidemiological characteristics, mortality rates, survival rates and the rate of malignancy in patients diagnosed with inflammatory myopathies (IIM) in Oman. MethodsThis is a longitudinal study, that covered a span of 16 years at eight rheumatology centres in Oman. The study included all adults and paediatric patients diagnosed with different types of idiopathic inflammatory myopathies (IIM) and who fulfil either the Bohan classification criteria or the 2017 EULAR/ACR classification criteria. ResultsThe study included a total of 116 patient with an average age of 38.78 (±17.61 SD) years. The most prevalent form of myositis was found to be dermatomyositis (DM) 48 (41.38%), followed by polymyositis (PM) 36 (31.03%) and juvenile myositis (JDM) 18(15.52%). However, inclusion body myositis and necrotising myopathy were relatively rare conditions. The prevalence rates for DM, PM and JDM were determined as 2.2, 2.2, and 1.14 per 100,000 population respectively. Cardiac complications were observed in 14.66% of cases. Among the individuals studied, a history of malignancy was present in around 1.72% of cases. ANA antibodies were present in 71.55% of the cases, anti-Jo 1 and anti-RNP/SM antibodies were detected in 8.62%, and Anti-Ro antibodies in 24.14%. The overall mortality rate was found to be 6.90% with a rate of 11.1% among JDM cases. The five-year survival rates for PM, DM and JDM were found to be 94.4%, 91.7% and 89.0% respectively. These rates decline over a 10-year period to 67%, 69% and 83.3% respectively. ConclusionThe study highlights the prevalence, mortality, and survival rates of IIM in Oman. Patients with JDM had a higher mortality rate. This underscores the significance of using novel healthcare strategies to improve clinical outcomes and meet special requirements for this group of patients.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

The prevalence, epidemiological characteristics and mortality trends of inflammatory myopathies patients in Oman: the Prevision study

Al Adhoubi,

Liyanage,

Al Salmi

et al. 2024

Clinical and Experimental Rheumatology

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“…Idiopathic inflammatory myopathies (IIM), commonly known as myositis, is a heterogeneous group of muscle diseases with a global prevalence of 2–25 individuals per 100, 000 persons ( Khoo et al, 2023 ). Based on the clinical, serological, and histological examination results, IIM can be classified into 5 major subtypes: dermatomyositis (DM), polymyositis (PM), immune-mediated necrotizing myopathy (IMNM), overlap myositis (OM), and sporadic inclusion body myositis (sIBM).…”

Section: Introductionmentioning

confidence: 99%

Mitochondrial defects in sporadic inclusion body myositis—causes and consequences

Iu,

So,

Chan

2024

Front. Cell Dev. Biol.

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Sporadic inclusion body myositis (sIBM) is a distinct subcategory of Idiopathic Inflammatory Myopathies (IIM), characterized by unique pathological features such as muscle inflammation, rimmed vacuoles, and protein aggregation within the myofibers. Although hyperactivation of the immune system is widely believed as the primary cause of IIM, it is debated whether non-immune tissue dysfunction might contribute to the disease’s onset as patients with sIBM are refractory to conventional immunosuppressant treatment. Moreover, the findings that mitochondrial dysfunction can elicit non-apoptotic programmed cell death and the subsequent immune response further support this hypothesis. Notably, abnormal mitochondrial structure and activities are more prominent in the muscle of sIBM than in other types of IIM, suggesting the presence of defective mitochondria might represent an overlooked contributor to the disease onset. The large-scale mitochondrial DNA deletion, aberrant protein aggregation, and slowed organelle turnover have provided mechanistic insights into the genesis of impaired mitochondria in sIBM. This article reviews the disease hallmarks of sIBM, the plausible contributors of mitochondrial damage in the sIBM muscle, and the immunological responses associated with mitochondrial perturbations. Additionally, the potential application of mitochondrial-targeted chemicals as a new treatment strategy to sIBM is explored and discussed.

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“…Incidence and prevalence of DM vary geographically, with the highest adult incidence rate seen in the United States (US; 15.2/million person-years) and highest prevalence rates seen in the US (21.4/100,000 people) and Spain (19/100,000). 1 DM incidence demonstrates a bimodal distribution, with juvenile DM commonly diagnosed between ages 4-14 years and adult DM commonly diagnosed between ages 40-60 years. 2 The pathogenesis of DM is multifactorial and not completely understood, with contributions from genetic, environmental, and immunological factors.…”

Section: Introductionmentioning

confidence: 99%

Dermatomyositis: Practical Guidance and Unmet Needs

Cassard,

Seraly,

Riegert

et al. 2024

ITT

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Dermatomyositis is a heterogeneous idiopathic inflammatory myopathy associated with various cutaneous manifestations and variable presence of myositis, interstitial lung disease, and other visceral organ involvement. An accurate diagnosis of dermatomyositis requires correlating clinical examination findings with serological and histological findings. Familiarity with pathognomonic and common cutaneous manifestations of dermatomyositis, which are highlighted here, can be especially helpful in making an accurate diagnosis. Additionally, evaluating patients for presence of myositis-specific autoantibodies can further support or refute a dermatomyositis diagnosis. When present, myositis-specific autoantibodies can also help guide workups for various dermatomyositis-associated manifestations, as each is associated with relatively distinct clinical characteristics. Evaluating patients for various systemic manifestations often relies on expert opinion recommendations; however, societal guideline statements concerning the evaluation of some manifestations have recently been described. Although malignancy-associated dermatomyositis is a well-accepted subtype, there is limited evidence to support extensive malignancy screening has a favorable benefit–risk ratio in most dermatomyositis patients. However, recent research has uncovered novel associations between dermatomyositis and malignancy, suggesting the possibility of identifying high-risk subsets of dermatomyositis patients in whom malignancy screening may have a high value. Treatment for dermatomyositis has remained largely unchanged over the past several decades. Although many dermatomyositis patients can be effectively treated with current options, either as monotherapy or with combination regimens, there is a need for more targeted and effective DM therapies, in general, and for MDA5(+) dermatomyositis-associated rapidly progressive interstitial lung disease. Fortunately, significant current and emerging research activities evaluating various novel medications for dermatomyositis provide hope for exciting future advances in patients with this intriguing immune-mediated disease.

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Epidemiology of the idiopathic inflammatory myopathies

Cited by 12 publications

References 238 publications

The prevalence, epidemiological characteristics and mortality trends of inflammatory myopathies patients in Oman: the Prevision study

The prevalence, epidemiological characteristics and mortality trends of inflammatory myopathies patients in Oman: the Prevision study

Mitochondrial defects in sporadic inclusion body myositis—causes and consequences

Dermatomyositis: Practical Guidance and Unmet Needs

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