Materiały źródłowe

• #1 Prognosis – The Myositis Association

  https://www.myositis.org/about-myositis/treatment-disease-management/prognosis/

  Prognosis for the different forms of myositis vary greatly and often depend on the presence of other conditions, such as interstitial lung disease or certain autoantibodies. […] While inclusion body myositis is a progressive disease, life expectancy for those with IBM is usually the same as for those without the disease. In fact, IBM patients usually dont die from the disease, but from complications (often preventable) that are associated with it. […] For dermatomyositis, polymyositis, and necrotizing myopathy, the progression of the disease is more complicated and harder to predict. More than 95 percent of those with DM, PM, and NM are still alive more than five years after diagnosis. […] Of those who die early in the disease course, the most frequent cause is not the myositis itself, but infection due to suppressed immunity as a result of treatment. […] Complications contributing to mortality include myositis-associated cancer, dysphagia (trouble swallowing), interstitial lung disease, and heart problems.

• #1 Long‐term outcome in polymyositis and dermatomyositis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1798355/

  Although polymyositis and dermatomyositis are regarded as treatable disorders, prognosis is not well known, as in the literature longterm outcome and prognostic factors vary widely. […] In the long term, myositis has a major effect on perceived disability and quality of life, despite the regained muscle strength. […] Mortality ranges from 4% to 45% of patients, and favourable longterm outcome varies between 18% and 90%. […] Predictors of poor outcome include old age, male sex, dysphagia, longstanding symptoms before diagnosis or treatment, various types of myositis, pulmonary or cardiac involvement, and the presence of antisynthetase or antisignal recognition particle (SRP) autoantibodies. […] Death was regarded as diseaserelated if caused by cancer diagnosed 2years before or after the onset of myositis, pulmonary complications, cardiac complications in patients 40years without cardiac history, complications of prednisone or other immunosuppressive treatment, or complications specifically related to a CTD.

• #1 Myositis – USZ

  https://www.usz.ch/en/disease/myositis/

  Myositis is a chronic disease that usually progresses without treatment. However, muscle inflammation can often be stopped by timely and sufficient treatment. The course of myositis varies widely and depends on the type, severity of muscle damage and involvement of other organs. […] Often, we can achieve significant improvement with medication in the majority of cases, sometimes even freedom from symptoms. Then those affected can live an almost normal everyday life and the quality of life is maintained. […] Inclusion body myositis, on the other hand, has a less favorable prognosis. After five years, many sufferers are dependent on walking aids, and after about ten years on a wheelchair. It can bring many other problems, such as difficulty swallowing and shortness of breath.

• #1 Juvenile Dermatomyositis: what comes next? Long-term outcomes in childhood myositis from a patient perspective | Pediatric Rheumatology | Full Text

  https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-022-00754-y

  Of 190 questionnaires sent, 84 (44%) were returned. Average age of respondents was 20.6 years (SD 3.9), time since diagnosis was 12.4 years (SD 5.0), age at onset was 9.2 years (SD 4.3), female to male ratio 4.25:1. Forty-nine (59%) self-reported persistently active disease, 54 (65%) were still taking immunosuppressive medication. […] After 12.4 years, questionnaire responders reported self-perceived high rates of persistently active disease and medication use, reduced rates of employment and were more likely to live with a parent/guardian. Perceived persistently active muscle disease appeared to affect quality of life in these patients and was the most significant contributor to long-term outcomes. […] These data suggest that active muscle disease was perceived to be an important long-term outcome from a patient perspective in this cohort.

• #1 Myositis | Diagnosis & Disease Information

  https://www.rheumatologyadvisor.com/ddi/myositis/

  Myositis, particularly anti-SS-OM, is associated with interstitial lung disease, which can be life-threatening. A 2023 study found that the 1-year survival rate for patients with myositis-associated interstitial lung disease was 86.7%. However, patients with the amyopathic subtype involving dermatologic manifestations but not substantial muscle weakness had higher all-cause mortality compared with those with myopathy. The presence of specific autoantibodies can be used as a prognostic biomarker and predict interstitial lung disease and cancer. […] The most serious myositis complication is interstitial lung disease, which occurs in 20% to 80% of patients with myositis and is a frequent cause of death. The anti-SS-OM subtype is associated with a higher risk for interstitial lung disease, and patients with this subtype or other high-risk features should undergo frequent lung screening. According to the BSR guidelines, high-dose steroids; cyclosporin or tacrolimus; and cyclophosphamide or rituximab are recommended for patients with rapidly progressing interstitial lung disease.

• #1 The predictive prognostic factors for polymyositis/dermatomyositis-associated interstitial lung disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5765702/

  Interstitial lung disease (ILD) is the principal cause of death in polymyositis/dermatomyositis (PM/DM). Here we investigated prognostic factors for death and serious infection in PM/DM-ILD using the multicenter database. […] As independent risk factors for early death, extended ILD lesions in upper lung fields (odds ratio (OR) 8.01, p=0.016) and hypocapnia (OR 6.85, p=0.038) were identified. […] The present study shows the progression of ILD at baseline is the most critical for survival and that infection, especially respiratory infection, is an additive prognostic factor under the potent immunosuppressive treatment. […] These data indicate that ILD is a critical prognostic factor in patients with PM/DM-ILD and that infection additively contributes to unfavorable clinical outcomes. […] We found both local and extrapulmonary findings, including immunosuppressive therapies, were risk factors for fatal events and serious infections in patients with PM/DM-ILD, based on clinical findings.

• #1 Myositis | Diagnosis & Disease Information

  https://www.rheumatologyadvisor.com/ddi/myositis/

  Patients diagnosed with myositis have an increased risk for cancer. One study found that 21% of patients with myositis developed cancer, usually within 1 year of their myositis diagnosis. The presence of the following risk factors may indicate a greater likelihood of cancer: Anti-TIF1- autoantibodies; Anti-NXP2 autoantibodies; Male sex; Dysphagia; Cutaneous necrosis; Resistance to immunosuppressive therapy; Older age; Rapid onset; and Negative test results for known myositis-specific autoantibodies.

• #1 A Novel Scoring System to Predict a Cancer Development in Patients with Idiopathic Inflammatory Myopathy – ACR Meeting Abstracts

  https://acrabstracts.org/abstract/a-novel-scoring-system-to-predict-a-cancer-development-in-patients-with-idiopathic-inflammatory-myopathy/

  A Novel Scoring System to Predict a Cancer Development in Patients with Idiopathic Inflammatory Myopathy […] Previous studies have demonstrated that idiopathic inflammatory myopathy (IIM) is associated with an elevated cancer risk. However, there is limited evidence on the specific risk factors and effective risk-stratification for cancer in patients with IIM. […] The primary outcome was occurrence of myositis-associated cancer, defined as that occurred within 3 years before and after the diagnosis of IIM. […] Patients with moderate- and high-risk groups showed significantly higher incidence of myositis-associated cancer compared to those in the low-risk groups (adjusted HR 9.13 [3.81 to 21.91] and 21.99 [11.13 to 43.45], respectively). […] Our scoring system accurately quantified the risk of myositis-associated cancer in patients with IIM.

• #1 Predictive value of myositis antibodies: role of semiquantitative classification and positivity for more than one autoantibody | RMD Open

  https://rmdopen.bmj.com/content/11/1/e005007

  The PPV for a myositis diagnosis (ever during data collection) was highest for anti-HMGCR antibodies (94%), followed by anti-MDA5, anti-Jo-1 and anti-TIF1- (4954%). […] The PPV of myositis antibodies for diagnoses of myositis or other myositis spectrum diseases vary considerably between individual autoantibodies. Higher PPVs can be expected with stronger band intensities and with the presence of 2 overlapping myositis antibodies. […] The PPV for malignancy was highest for anti-TIF1- (38%), followed by anti-PL-7 (32%). […] Stronger antibody band intensity was associated with higher PPVs for myositis and CTD but not for ILD or malignancies. Simultaneous positivity for 2 antibodies compared with single antibody was associated with higher PPVs for myositis, CTD and ILD. […] Higher PPVs for myositis can be expected with anti-HMGCR antibodies, stronger antibody band intensities and simultaneous positivity for multiple myositis antibodies.

• #1 Long‐term outcome in polymyositis and dermatomyositis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1798355/

  Significant muscle weakness was arbitrarily defined as an MRC sum score of 128, thus allowing for one muscle group to be rated bilaterally with an MRC grade 4. […] The presence of Jo1 autoantibodies predicted the persistent use of drugs (OR 4.4; 95% CI 1.3 to 15.0). […] Our study shows that patients with dermatomyositis and polymyositis have a mortality risk of 10% to die of a cause related to their disease, mostly cancer, especially during the first years after onset of myositis. […] Most of the survivors have a chronic continuous or polycyclic disease course. […] At longterm followup, 65% of patients who survived had normal strength, 34% had no or slight disability, and only 16% had normal scores on the qualityoflife scale. […] Not surprisingly, diseaserelated death was associated with age and associated cancer, but no other prognostic factors, especially no differences between myositis subtypes, were found. […] We could not confirm several factors often reported to be a determinant of unfavourable outcome, such as duration of symptoms before treatment. […] Finally, we also found no indication that type of myositis determines the outcome.

• #1 Myositis: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/24170-myositis

  Theres no cure for myositis, but in most cases, treatment can put it into remission. […] Some cases of myositis can be fatal. Around 5% of people with dermatomyositis die within a year of their diagnosis. This is why its important to get your symptoms examined by a healthcare provider as soon as you notice them.

• #2 Long‐term outcome in polymyositis and dermatomyositis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1798355/

  Although polymyositis and dermatomyositis are regarded as treatable disorders, prognosis is not well known, as in the literature longterm outcome and prognostic factors vary widely. […] In the long term, myositis has a major effect on perceived disability and quality of life, despite the regained muscle strength. […] Mortality ranges from 4% to 45% of patients, and favourable longterm outcome varies between 18% and 90%. […] Predictors of poor outcome include old age, male sex, dysphagia, longstanding symptoms before diagnosis or treatment, various types of myositis, pulmonary or cardiac involvement, and the presence of antisynthetase or antisignal recognition particle (SRP) autoantibodies. […] Death was regarded as diseaserelated if caused by cancer diagnosed 2years before or after the onset of myositis, pulmonary complications, cardiac complications in patients 40years without cardiac history, complications of prednisone or other immunosuppressive treatment, or complications specifically related to a CTD.

• #2 Long‐term outcome in polymyositis and dermatomyositis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC1798355/

  Significant muscle weakness was arbitrarily defined as an MRC sum score of 128, thus allowing for one muscle group to be rated bilaterally with an MRC grade 4. […] The presence of Jo1 autoantibodies predicted the persistent use of drugs (OR 4.4; 95% CI 1.3 to 15.0). […] Our study shows that patients with dermatomyositis and polymyositis have a mortality risk of 10% to die of a cause related to their disease, mostly cancer, especially during the first years after onset of myositis. […] Most of the survivors have a chronic continuous or polycyclic disease course. […] At longterm followup, 65% of patients who survived had normal strength, 34% had no or slight disability, and only 16% had normal scores on the qualityoflife scale. […] Not surprisingly, diseaserelated death was associated with age and associated cancer, but no other prognostic factors, especially no differences between myositis subtypes, were found. […] We could not confirm several factors often reported to be a determinant of unfavourable outcome, such as duration of symptoms before treatment. […] Finally, we also found no indication that type of myositis determines the outcome.

• #2 Myositis: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/24170-myositis

  Theres no cure for myositis, but in most cases, treatment can put it into remission. […] Some cases of myositis can be fatal. Around 5% of people with dermatomyositis die within a year of their diagnosis. This is why its important to get your symptoms examined by a healthcare provider as soon as you notice them.

• #2 Myositis – USZ

  https://www.usz.ch/en/disease/myositis/

  Myositis is a chronic disease that usually progresses without treatment. However, muscle inflammation can often be stopped by timely and sufficient treatment. The course of myositis varies widely and depends on the type, severity of muscle damage and involvement of other organs. […] Often, we can achieve significant improvement with medication in the majority of cases, sometimes even freedom from symptoms. Then those affected can live an almost normal everyday life and the quality of life is maintained. […] Inclusion body myositis, on the other hand, has a less favorable prognosis. After five years, many sufferers are dependent on walking aids, and after about ten years on a wheelchair. It can bring many other problems, such as difficulty swallowing and shortness of breath.

• #2 Juvenile Dermatomyositis: what comes next? Long-term outcomes in childhood myositis from a patient perspective | Pediatric Rheumatology | Full Text

  https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-022-00754-y

  Our findings also highlight the importance of including the patient perspective in the assessment of long term outcomes, to ensure not only that future research priorities can facilitate desired outcomes for patients but that we can also start to target initial management strategies more effectively based on a combination of clinical and patient-reported data.

• #2 Myositis | Diagnosis & Disease Information

  https://www.rheumatologyadvisor.com/ddi/myositis/

  Patients diagnosed with myositis have an increased risk for cancer. One study found that 21% of patients with myositis developed cancer, usually within 1 year of their myositis diagnosis. The presence of the following risk factors may indicate a greater likelihood of cancer: Anti-TIF1- autoantibodies; Anti-NXP2 autoantibodies; Male sex; Dysphagia; Cutaneous necrosis; Resistance to immunosuppressive therapy; Older age; Rapid onset; and Negative test results for known myositis-specific autoantibodies.

• #2 The predictive prognostic factors for polymyositis/dermatomyositis-associated interstitial lung disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5765702/

  Interstitial lung disease (ILD) is the principal cause of death in polymyositis/dermatomyositis (PM/DM). Here we investigated prognostic factors for death and serious infection in PM/DM-ILD using the multicenter database. […] As independent risk factors for early death, extended ILD lesions in upper lung fields (odds ratio (OR) 8.01, p=0.016) and hypocapnia (OR 6.85, p=0.038) were identified. […] The present study shows the progression of ILD at baseline is the most critical for survival and that infection, especially respiratory infection, is an additive prognostic factor under the potent immunosuppressive treatment. […] These data indicate that ILD is a critical prognostic factor in patients with PM/DM-ILD and that infection additively contributes to unfavorable clinical outcomes. […] We found both local and extrapulmonary findings, including immunosuppressive therapies, were risk factors for fatal events and serious infections in patients with PM/DM-ILD, based on clinical findings.

• #2 Myositis | Diagnosis & Disease Information

  https://www.rheumatologyadvisor.com/ddi/myositis/

  Myositis, particularly anti-SS-OM, is associated with interstitial lung disease, which can be life-threatening. A 2023 study found that the 1-year survival rate for patients with myositis-associated interstitial lung disease was 86.7%. However, patients with the amyopathic subtype involving dermatologic manifestations but not substantial muscle weakness had higher all-cause mortality compared with those with myopathy. The presence of specific autoantibodies can be used as a prognostic biomarker and predict interstitial lung disease and cancer. […] The most serious myositis complication is interstitial lung disease, which occurs in 20% to 80% of patients with myositis and is a frequent cause of death. The anti-SS-OM subtype is associated with a higher risk for interstitial lung disease, and patients with this subtype or other high-risk features should undergo frequent lung screening. According to the BSR guidelines, high-dose steroids; cyclosporin or tacrolimus; and cyclophosphamide or rituximab are recommended for patients with rapidly progressing interstitial lung disease.

• #2 Biologic predictors of clinical improvement in rituximab-treated refractory myositis | BMC Musculoskeletal Disorders | Full Text

  https://bmcmusculoskeletdisord.biomedcentral.com/articles/10.1186/s12891-015-0710-3

  To examine the longitudinal utility of a biomarker signature in conjunction with myositis autoantibodies (autoAbs) as predictors of disease improvement in refractory myositis patients treated with rituximab. […] Biomarker signatures in conjunction with autoAbs help predict response to rituximab in refractory myositis. Biomarker and clinical responses are greatest at 16 weeks after rituximab. […] We found that biomarker signatures in conjunction with autoAbs prior to treatment help guide response to BCD in refractory myositis. […] Therefore, results of our current study indicate that autoAbs, especially anti-synthetase, anti-Mi-2, non-MAA, and undefined autoAbs in conjunction to IFNCK high scores, are strong predictors of response in rituximab treated myositis patients in the RIM trial. […] Our study is the first to comprehensively demonstrate that biomarkers in conjunction with autoAbs are major predictive factors of response in myositis patients treated with BCD.

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