Dermatomyositis – Rokowania, prognozy i postęp choroby

Dermatomyositis
Rokowania, prognozy i postęp choroby

Dermatomyositis jest autoimmunologiczną miopatią o zmiennym rokowaniu, z 10% śmiertelnością, głównie w pierwszych latach choroby. Wskaźniki przeżycia wynoszą około 82,6% po 1 roku, 73,9% po 2 latach, 66,7% po 5 latach i 55,4% po 9 latach. Czynniki prognostyczne obejmują wiek powyżej 60 lat, współistniejące nowotwory (obecne u 25% pacjentów), zajęcie narządów wewnętrznych (serce, płuca, przełyk), dysfonię, śródmiąższową chorobę płuc oraz obecność specyficznych autoprzeciwciał, takich jak Jo-1 i anty-MDA5, które wiążą się z cięższym przebiegiem i gorszym rokowaniem. Przebieg choroby jest heterogenny – od spontanicznej remisji u 20% pacjentów do piorunującego przebiegu u 5%, a większość ma charakter przewlekły lub policykliczny.

Prognoza w dermatomyositis

Dermatomyositis jest poważną chorobą autoimmunologiczną, której rokowanie może być zróżnicowane i zależy od wielu czynników. Mimo że jest to schorzenie uznawane za potencjalnie możliwe do leczenia, dane dotyczące długoterminowego rokowania wykazują znaczną zmienność. W literaturze medycznej opisywane są różne czynniki prognostyczne oraz odległe wyniki leczenia, co wskazuje na złożoność przebiegu tej choroby.¹²

Śmiertelność i przeżywalność

Śmiertelność związana z dermatomyositis wynosi co najmniej 10%, przy czym większość zgonów występuje w pierwszych latach od wystąpienia objawów choroby. Główne przyczyny zgonów to nowotwory złośliwe, powikłania sercowo-naczyniowe oraz płucne.¹² Według niektórych badań, wskaźniki przeżycia wynoszą około:

82,6% po 1 roku
73,9% po 2 latach
66,7% po 5 latach
55,4% po 9 latach

Dla dermatomyositis, polymyositis i miopatii nekrotyzującej ponad 95% pacjentów przeżywa ponad 5 lat od diagnozy, jednak przebieg choroby może być trudny do przewidzenia. U niektórych pacjentów występuje tylko jeden ostry epizod choroby w ciągu życia, podczas gdy inni zmagają się z objawami przez wiele lat.¹

Czynniki prognostyczne wpływające na rokowanie

Zidentyfikowano szereg czynników, które wiążą się z gorszym rokowaniem u pacjentów z dermatomyositis:¹²

Wiek pacjenta – starszy wiek (powyżej 60 lat) jest najsilniejszym predyktorem niekorzystnego rokowania
Współistniejący nowotwór złośliwy – występuje u około 25% pacjentów
Zajęcie narządów wewnętrznych – szczególnie powikłania sercowe, płucne i przełykowe
Dysfonia (zaburzenia głosu) – marker ciężkiego przebiegu choroby
Śródmiąższowa choroba płuc – znacząco pogarsza rokowanie
Brak dysfagii (paradoksalnie)
Osłabienie i anoreksja

¹²³

Przebieg choroby

Dermatomyositis może przebiegać w różny sposób, a określenie konkretnego scenariusza dla danego pacjenta jest często trudne:¹²

U około 20% pacjentów może dojść do spontanicznej remisji
Około 5% pacjentów doświadcza piorunującego, postępującego przebiegu prowadzącego do zgonu
Większość osób, które przeżyły, ma przewlekły ciągły lub policykliczny przebieg choroby
W długoterminowej obserwacji około 65% pacjentów odzyskuje normalną siłę mięśniową
34% pacjentów nie ma lub ma jedynie niewielką niepełnosprawność
Tylko 16% osiąga normalne wyniki w skalach jakości życia

¹²³

Powikłania długoterminowe

Pacjenci, którzy przeżyją ostrą fazę dermatomyositis, mogą doświadczać różnych długoterminowych powikłań wpływających na jakość życia:¹²

Resztkowe osłabienie mięśni – około 25% pacjentów (szczególnie starsi mężczyźni) pozostaje z istotną niepełnosprawnością lub osłabieniem mięśni
Kontynuacja farmakoterapii – około połowa pacjentów długoterminowo przyjmuje niskie dawki leków
Uogólnione wapnienie (kalcynoza) – występuje głównie w dziecięcej postaci dermatomyositis
Przykurcze – szczególnie u dzieci z ciężką postacią choroby
Obniżona jakość życia – mimo odzyskania siły mięśniowej, zapalenie mięśni ma znaczący wpływ na postrzeganą niepełnosprawność i jakość życia

¹²³

Znaczenie autoprzeciwciał w rokowaniu

Obecność specyficznych autoprzeciwciał może mieć istotne znaczenie prognostyczne:¹²

Przeciwciała Jo-1 – prognozują długotrwałe przyjmowanie leków (OR 4,4; 95% CI 1,3 do 15,0)
Przeciwciała anty-MDA5 – wiążą się z nadmiernym stanem zapalnym i gorszym rokowaniem
Określone autoprzeciwciała krążące we krwi mogą być powiązane z ryzykiem wystąpienia nowotworu złośliwego u pacjentów z dermatomyositis

¹²³

Markery zapalenia a prognoza

Badania wykazały, że specyficzne markery zapalne mogą być powiązane z aktywnością choroby i jej rokowaniem, szczególnie w przypadku dermatomyositis z przeciwciałami anty-MDA5:¹

Nadmierne zapalenie jest szczególnie podwyższone u pacjentów z DM z przeciwciałami anty-MDA5 w porównaniu z grupą kontrolną i pacjentami bez tych przeciwciał
U pacjentów z DM z przeciwciałami anty-MDA5 obserwuje się wzmożoną aktywację ścieżek: interakcji cytokin i receptorów cytokin, szlaków sygnałowych chemokin, szlaku sygnałowego IL-17, szlaku sygnałowego TNF oraz szlaku sygnałowego JAK-STAT
Białka CX3CL1, IL-17C, IL-18R1, CCL20 i TNF zostały zidentyfikowane jako potencjalne biomarkery DM z przeciwciałami anty-MDA5

Różnice prognostyczne między podtypami zapalenia mięśni

Chociaż nie znaleziono istotnych różnic rokowniczych między podtypami zapalenia mięśni w niektórych badaniach, inne wskazują na pewne różnice:¹²

W dermatomyositis ogólne cechy, takie jak zwłóknienie płuc, nowotwór złośliwy czy osłabienie-anoreksja, mają największy wpływ na rokowanie
W polymyositis objawy mięśniowe są najważniejszym czynnikiem prognostycznym
Rokowanie w różnych formach zapalenia mięśni jest bardzo zróżnicowane i często zależy od obecności innych schorzeń, takich jak śródmiąższowa choroba płuc lub określonych autoprzeciwciał

¹²

Monitorowanie aktywności choroby a prognoza

Dokładna ocena aktywności choroby jest kluczowa dla określenia rokowania. W badaniach nad młodzieńczym dermatomyositis (JDM) wykazano, że istnieje istotna korelacja między różnymi parametrami oceny aktywności choroby:¹

Istnieje znacząca korelacja między wynikami skal CMAS (Childhood Myositis Assessment Scale) i MMT8 (Manual Muscle Testing 8)
Pacjenci z wyższym wynikiem CMAS zwykle mają wyższy wynik MMT8
Proponuje się zwiększenie wagi oceny lekarskiej (PGA) w ocenie aktywności choroby JDM, aby uwzględnić aktywność zmian skórnych

Główne przyczyny zgonów

Zgony związane z dermatomyositis mogą wynikać z różnych powikłań:¹²³⁴

Nowotwór złośliwy – pacjenci z towarzyszącym nowotworem mogą umrzeć z powodu choroby nowotworowej
Powikłania sercowo-naczyniowe – jedna z głównych przyczyn zgonów
Powikłania płucne – szczególnie śródmiąższowa choroba płuc
Osłabienie mięśni – prowadzące do niewydolności oddechowej
Dysfagia (zaburzenia połykania) prowadząca do aspiracji i zachłystowego zapalenia płuc
Powikłania jatrogenne – związane z leczeniem immunosupresyjnym

¹²³

Dermatomyositis a nowotwory złośliwe

Związek między dermatomyositis a nowotworami złośliwymi jest dobrze udokumentowany i ma istotne znaczenie prognostyczne:¹²

Szacuje się, że około 25% pacjentów z dermatomyositis ma lub rozwinie nowotwór złośliwy
Ryzyko wystąpienia nowotworu pozostaje podwyższone przez 3-5 lat od rozpoznania dermatomyositis
Starszy wiek jest najsilniejszym predyktorem wystąpienia nowotworu u pacjentów z dermatomyositis
Obecność specyficznych autoprzeciwciał we krwi może być powiązana z ryzykiem nowotworu

¹²

Znaczenie właściwej opieki dla rokowania

Biorąc pod uwagę ciężkość i obciążenie związane z dermatomyositis oraz możliwe długoterminowe powikłania, odpowiednie leczenie ma kluczowe znaczenie dla prognozy. Wczesna i adekwatna interwencja terapeutyczna może wpłynąć na przebieg choroby i zapobiec niektórym długoterminowym powikłaniom.¹ Nadal trwają badania nad określeniem biomarkerów, które mogłyby przewidzieć przyszłą aktywność choroby, co mogłoby umożliwić opracowanie spersonalizowanych strategii leczenia.²

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Materiały źródłowe

#1 Longâterm 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. […] Dermatomyositis and polymyositis are serious diseases with a diseaserelated mortality of at least 10%. In the long term, myositis has a major effect on perceived disability and quality of life, despite the regained muscle strength. […] 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. In the long term, half the patients are still taking a low dosage of drugs, and one quarter (especially older men) are left with significant disability or muscle weakness.
#1 Long-term prognosis of 69 patients with dermatomyositis or polymyositis – PubMed
https://pubmed.ncbi.nlm.nih.gov/8809440/
Objectives: To assess the long-term prognosis of dermatomyositis and polymyositis. […] Results: 30 deaths (43.5%) occurred mainly due to cardiovascular (8), pulmonary (8), carcinomatous (5) and iatrogenic complications (5). Survival rates were 82.6% at 1 year, 73.9% at 2.66, 7% at 5 and 55.4% at 9. Significant prognostic factors for death (Cox model with time-dependent covariates) were old age (p 0.0001), dysphonia (p 0.001), pulmonary interstitial fibrosis (p 0.02), absence of dysphagia (p 0.02) and asthenia-anorexia (p 0.05). […] Conclusions: High mortality occurred in the first year, and the survival rate decreased continually up to 9 years. The main prognostic factor for death is old age, but dermatomyositis and polymyositis must be considered separately. General features (pulmonary fibrosis, cancer, asthenia-anorexia) are involved in dermatomyositis, whereas muscular symptoms are the most significant in polymyositis. The long-term functional prognosis was fairly good, except for generalized calcinosis, which tended to occur in childhood dermatomyositis.
#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. […] 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. Many experience only one period of acute illness in their lifetime; others struggle with symptoms for years. […] Complications contributing to mortality include myositis-associated cancer, dysphagia (trouble swallowing), interstitial lung disease, and heart problems.
#1 Dermatomyositis: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/332783-overview
Dermatomyositis may spontaneously remit in as many as 20% of affected patients. About 5% of patients have a fulminant progressive course with eventual death. However, patients who survive the disease may experience residual weakness and disability. Children with severe dermatomyositis may develop contractures. Therefore, many patients require long-term therapy. […] Risk factors for a poorer prognosis in patients with dermatomyositis include the following: an associated malignancy, cardiac, pulmonary, or esophageal involvement, older age (ie, 60 years). […] Dermatomyositis may cause death because of muscle weakness or cardiopulmonary involvement. Patients with an associated cancer may die of the malignancy. […] The association between malignancy and dermatomyositis has long been recognized. An estimated 25% of patients with dermatomyositis have or will develop an associated malignancy, and the risk appears to remain elevated for 3-5 years.
#1 Longâterm outcome in polymyositis and dermatomyositis
https://pmc.ncbi.nlm.nih.gov/articles/PMC1798355/
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. […] The presence of Jo1 autoantibodies predicted the persistent use of drugs (OR 4.4; 95% CI 1.3 to 15.0).
#1 Shared and distinctive inflammation-related protein profiling in idiop | JIR
https://www.dovepress.com/shared-and-distinctive-inflammation-related-protein-profiling-in-idiop-peer-reviewed-fulltext-article-JIR
Plasma profiling revealed that inflammation and inflammatory pathways were extremely elevated in patients with IIM, especially in patients with anti-MDA5 autoantibodies. The shared and distinctive inflammation-related protein signature was demonstrated in patients with/without anti-MDA5 autoantibodies. Cytokine-cytokine receptor interaction, chemokine signaling pathway, IL-17 signaling pathway, TNF signaling pathway, JAK-STAT signaling pathway were enriched in anti-MDA5+ DM patients compared with anti-MDA5- IIM patients. CX3CL1, IL-17C, IL-18R1, CCL20 and TNF were screened as biomarkers of anti-MDA5+ DM. […] Excessive inflammation was prominently elevated in anti-MDA5+ DM patients compared with HCs and patients without anti-MDA5+ autoantibodies. […] The excessive and distinctive inflammation condition in anti-MDA5+ DM may be associated with the poor prognosis of this disorder. […] We previously revealed that plasma KRT19 was a potential biomarker in anti-MDA5+ DM. In this study, we first demonstrated that IL-18R1, CCL20, CX3CL1, TNF and CXCL5 could be new diagnostic biomarkers for anti-MDA5+ DM.
#1 Clinical signs and symptoms in a joint model of four disease activity parameters in juvenile dermatomyositis: a prospective, longitudinal, multicenter cohort study | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-018-1687-8
The substantial correlation between the subject-specific random intercepts for CMAS and MMT8 indicated that patients with a higher CMAS tended to have a higher MMT8, as reported previously. […] This implied that information captured by CMAS was also partially captured by MMT8 and vice versa. […] In conclusion, our observations supported the previously made proposal to increase the weight of PGA in the evaluation of JDM disease activity, to account for cutaneous disease activity. […] Future work may investigate the predictive ability of these signs and symptoms in the assessment of disease activity. […] Follow up studies may attempt to predict future disease activity using the clinical signs and symptoms that were associated with higher disease activity found in the current study.
#1 Clinical signs and symptoms in a joint model of four disease activity parameters in juvenile dermatomyositis: a prospective, longitudinal, multicenter cohort study | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-018-1687-8
It is currently impossible to predict the prognosis of patients with juvenile dermatomyositis (JDM). […] The aim of the current study was to find clinical signs and symptoms associated with higher disease activity as measured by four widely used continuous outcome variables, assessed longitudinally in a large, multicenter cohort of patients with JDM. Such associations with high disease activity could be used in follow up studies to predict disease outcome in patients with JDM as a first step towards tailor-made treatment. […] Given the severity and burden of the disease and possible long-term complications, adequate treatment is of utmost importance. […] Previous studies have revealed some clinical factors associated with a worse prognosis. […] The results showed as expected that dysphonia, already known to be a marker of severe disease activity, was associated with higher disease activity.
#2 Long-term prognosis of 69 patients with dermatomyositis or polymyositis – PubMed
https://pubmed.ncbi.nlm.nih.gov/8809440/
Objectives: To assess the long-term prognosis of dermatomyositis and polymyositis. […] Results: 30 deaths (43.5%) occurred mainly due to cardiovascular (8), pulmonary (8), carcinomatous (5) and iatrogenic complications (5). Survival rates were 82.6% at 1 year, 73.9% at 2.66, 7% at 5 and 55.4% at 9. Significant prognostic factors for death (Cox model with time-dependent covariates) were old age (p 0.0001), dysphonia (p 0.001), pulmonary interstitial fibrosis (p 0.02), absence of dysphagia (p 0.02) and asthenia-anorexia (p 0.05). […] Conclusions: High mortality occurred in the first year, and the survival rate decreased continually up to 9 years. The main prognostic factor for death is old age, but dermatomyositis and polymyositis must be considered separately. General features (pulmonary fibrosis, cancer, asthenia-anorexia) are involved in dermatomyositis, whereas muscular symptoms are the most significant in polymyositis. The long-term functional prognosis was fairly good, except for generalized calcinosis, which tended to occur in childhood dermatomyositis.
#2 Dermatomyositis: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/332783-overview
Dermatomyositis may spontaneously remit in as many as 20% of affected patients. About 5% of patients have a fulminant progressive course with eventual death. However, patients who survive the disease may experience residual weakness and disability. Children with severe dermatomyositis may develop contractures. Therefore, many patients require long-term therapy. […] Risk factors for a poorer prognosis in patients with dermatomyositis include the following: an associated malignancy, cardiac, pulmonary, or esophageal involvement, older age (ie, 60 years). […] Dermatomyositis may cause death because of muscle weakness or cardiopulmonary involvement. Patients with an associated cancer may die of the malignancy. […] The association between malignancy and dermatomyositis has long been recognized. An estimated 25% of patients with dermatomyositis have or will develop an associated malignancy, and the risk appears to remain elevated for 3-5 years.
#2 Longâterm outcome in polymyositis and dermatomyositis
https://pmc.ncbi.nlm.nih.gov/articles/PMC1798355/
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. […] The presence of Jo1 autoantibodies predicted the persistent use of drugs (OR 4.4; 95% CI 1.3 to 15.0).
#2 Dermatomyositis: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/332783-overview
The presence of specific circulating autoantibodies has been linked with risk of malignancy in patients with dermatomyositis. […] Older age appears to be the strongest predictor of malignancy in patients with dermatomyositis. […] Overall, data suggest that the mortality rate in persons with dermatomyositis is higher than that in the general population.
#2 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. […] 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. Many experience only one period of acute illness in their lifetime; others struggle with symptoms for years. […] Complications contributing to mortality include myositis-associated cancer, dysphagia (trouble swallowing), interstitial lung disease, and heart problems.
#2 Clinical signs and symptoms in a joint model of four disease activity parameters in juvenile dermatomyositis: a prospective, longitudinal, multicenter cohort study | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-018-1687-8
The substantial correlation between the subject-specific random intercepts for CMAS and MMT8 indicated that patients with a higher CMAS tended to have a higher MMT8, as reported previously. […] This implied that information captured by CMAS was also partially captured by MMT8 and vice versa. […] In conclusion, our observations supported the previously made proposal to increase the weight of PGA in the evaluation of JDM disease activity, to account for cutaneous disease activity. […] Future work may investigate the predictive ability of these signs and symptoms in the assessment of disease activity. […] Follow up studies may attempt to predict future disease activity using the clinical signs and symptoms that were associated with higher disease activity found in the current study.
#3 Clinical signs and symptoms in a joint model of four disease activity parameters in juvenile dermatomyositis: a prospective, longitudinal, multicenter cohort study | Arthritis Research & Therapy | Full Text
https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-018-1687-8
It is currently impossible to predict the prognosis of patients with juvenile dermatomyositis (JDM). […] The aim of the current study was to find clinical signs and symptoms associated with higher disease activity as measured by four widely used continuous outcome variables, assessed longitudinally in a large, multicenter cohort of patients with JDM. Such associations with high disease activity could be used in follow up studies to predict disease outcome in patients with JDM as a first step towards tailor-made treatment. […] Given the severity and burden of the disease and possible long-term complications, adequate treatment is of utmost importance. […] Previous studies have revealed some clinical factors associated with a worse prognosis. […] The results showed as expected that dysphonia, already known to be a marker of severe disease activity, was associated with higher disease activity.
#3 Dermatomyositis: Practice Essentials, Background, Pathophysiology
https://emedicine.medscape.com/article/332783-overview
Dermatomyositis may spontaneously remit in as many as 20% of affected patients. About 5% of patients have a fulminant progressive course with eventual death. However, patients who survive the disease may experience residual weakness and disability. Children with severe dermatomyositis may develop contractures. Therefore, many patients require long-term therapy. […] Risk factors for a poorer prognosis in patients with dermatomyositis include the following: an associated malignancy, cardiac, pulmonary, or esophageal involvement, older age (ie, 60 years). […] Dermatomyositis may cause death because of muscle weakness or cardiopulmonary involvement. Patients with an associated cancer may die of the malignancy. […] The association between malignancy and dermatomyositis has long been recognized. An estimated 25% of patients with dermatomyositis have or will develop an associated malignancy, and the risk appears to remain elevated for 3-5 years.
#3 Long-term prognosis of 69 patients with dermatomyositis or polymyositis – PubMed
https://pubmed.ncbi.nlm.nih.gov/8809440/
Objectives: To assess the long-term prognosis of dermatomyositis and polymyositis. […] Results: 30 deaths (43.5%) occurred mainly due to cardiovascular (8), pulmonary (8), carcinomatous (5) and iatrogenic complications (5). Survival rates were 82.6% at 1 year, 73.9% at 2.66, 7% at 5 and 55.4% at 9. Significant prognostic factors for death (Cox model with time-dependent covariates) were old age (p 0.0001), dysphonia (p 0.001), pulmonary interstitial fibrosis (p 0.02), absence of dysphagia (p 0.02) and asthenia-anorexia (p 0.05). […] Conclusions: High mortality occurred in the first year, and the survival rate decreased continually up to 9 years. The main prognostic factor for death is old age, but dermatomyositis and polymyositis must be considered separately. General features (pulmonary fibrosis, cancer, asthenia-anorexia) are involved in dermatomyositis, whereas muscular symptoms are the most significant in polymyositis. The long-term functional prognosis was fairly good, except for generalized calcinosis, which tended to occur in childhood dermatomyositis.
#3 Shared and distinctive inflammation-related protein profiling in idiop | JIR
https://www.dovepress.com/shared-and-distinctive-inflammation-related-protein-profiling-in-idiop-peer-reviewed-fulltext-article-JIR
Plasma profiling revealed that inflammation and inflammatory pathways were extremely elevated in patients with IIM, especially in patients with anti-MDA5 autoantibodies. The shared and distinctive inflammation-related protein signature was demonstrated in patients with/without anti-MDA5 autoantibodies. Cytokine-cytokine receptor interaction, chemokine signaling pathway, IL-17 signaling pathway, TNF signaling pathway, JAK-STAT signaling pathway were enriched in anti-MDA5+ DM patients compared with anti-MDA5- IIM patients. CX3CL1, IL-17C, IL-18R1, CCL20 and TNF were screened as biomarkers of anti-MDA5+ DM. […] Excessive inflammation was prominently elevated in anti-MDA5+ DM patients compared with HCs and patients without anti-MDA5+ autoantibodies. […] The excessive and distinctive inflammation condition in anti-MDA5+ DM may be associated with the poor prognosis of this disorder. […] We previously revealed that plasma KRT19 was a potential biomarker in anti-MDA5+ DM. In this study, we first demonstrated that IL-18R1, CCL20, CX3CL1, TNF and CXCL5 could be new diagnostic biomarkers for anti-MDA5+ DM.
#3 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. […] 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. Many experience only one period of acute illness in their lifetime; others struggle with symptoms for years. […] Complications contributing to mortality include myositis-associated cancer, dysphagia (trouble swallowing), interstitial lung disease, and heart problems.
#4 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. […] 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. Many experience only one period of acute illness in their lifetime; others struggle with symptoms for years. […] Complications contributing to mortality include myositis-associated cancer, dysphagia (trouble swallowing), interstitial lung disease, and heart problems.