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Review

Current and Emerging Biomarkers in Dermatomyositis: Clinical Utility and Future Directions

by
Fiona Jaederlund
1,2,
Ka Wei Katty Joo Hu
1,2,
Claudio Karsulovic
2,3,* and
Lia Hojman
2,4
1
Facultad de Medicina, Universidad del Desarrollo-Clinica Alemana de Santiago, Santiago 7550000, Chile
2
Laboratorio de Inmunomodulación Neuroendocrina, Instituto de Ciencias Biomédicas, Facultad de Medicina, Universidad de Chile, Santiago 8380453, Chile
3
Servicio de Reumatología, Clínica Alemana de Santiago, Universidad del Desarrollo, Santiago 7610315, Chile
4
Servicio de Dermatología, Clínica Alemana de Santiago, Universidad del Desarrollo, Santiago 7610315, Chile
*
Author to whom correspondence should be addressed.
Int. J. Transl. Med. 2026, 6(1), 4; https://doi.org/10.3390/ijtm6010004
Submission received: 20 November 2025 / Revised: 28 December 2025 / Accepted: 7 January 2026 / Published: 9 January 2026
Browse Figure
Versions Notes

Abstract

Idiopathic inflammatory myopathies (IIM) comprise a heterogeneous group of autoimmune disorders with variable systemic involvement. Among them, dermatomyositis (DM) is the subtype with the most extensive biomarker characterization due to its defined immunopathology and frequent association with interstitial lung disease (ILD). This narrative review summarizes studies retrieved from PubMed, Scopus, and Web of Science up to March 2025, focusing on non-autoantibody biomarkers in DM. Reported categories include soluble proteins, cytokines, chemokines, muscle-specific microRNAs, and transcriptomic signatures reflecting interferon activation, tissue injury, and fibrotic remodeling. Among the most validated molecules, interferon-stimulated genes, ferritin, KL-6, SP-D, and CXCL10 demonstrate diagnostic and prognostic value, particularly in anti-MDA5-positive DM, where they support early identification of patients at risk for rapidly progressive ILD. However, despite increasing evidence, most biomarkers lack disease specificity, standardized cutoffs, and multicenter validation, while molecular assays remain confined to specialized laboratories. Clinically accessible markers such as ferritin, KL-6, and CXCL10 currently offer the highest translational potential. Nevertheless, the heterogeneity of study designs and analytical methods continues to limit comparability and routine clinical integration. Future research should prioritize the validation of composite biomarker panels through standardized, multicentric studies to enhance diagnostic precision and enable precision medicine approaches in DM and related inflammatory myopathies.

1. Introduction

Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of autoimmune diseases that primarily affect skeletal muscle, with variable degrees of inflammation and tissue damage [1]. Within this spectrum, dermatomyositis (DM) represents a distinct subtype characterized by progressive and symmetric proximal muscle weakness accompanied by characteristic cutaneous manifestations such as heliotrope rash and Gottron’s papules, which are considered nearly pathognomonic [2]. However, DM exhibits marked clinical heterogeneity, as not all disease subtypes present with progressive muscle weakness, nor do all patients develop interstitial lung disease or significant extramuscular involvement. The clinical features of IIMs are classically described by the Bohan and Peter criteria, which include symmetric proximal muscle weakness, elevated serum muscle enzymes, electromyographic abnormalities, muscle biopsy showing inflammation, and characteristic skin lesions. More recently, the 2017 ACR/EULAR classification criteria have incorporated clinical, serological, and histopathological variables to improve diagnostic accuracy and disease stratification across IIM subtypes, providing a framework in which biomarkers may further refine classification and prognostication [3].
Among idiopathic inflammatory myopathies, dermatomyositis provides the richest evidence base for biomarker discovery due to its well-defined immunopathology, frequent association with interstitial lung disease (ILD), and the high prevalence of specific autoantibodies such as anti-MDA5 and anti-TIF1γ. Robust correlations between molecular markers and severe clinical outcomes, particularly rapidly progressive ILD, have positioned DM as an ideal model for exploring the translational potential of non-autoantibody biomarkers in inflammatory myopathies [1,4].
DM is also associated with an increased risk of malignancy, systemic vasculopathy, and higher mortality in rapidly progressive or treatment-refractory forms, underscoring the need for reliable biomarkers to guide early diagnosis and management [4]. Although myositis-specific autoantibodies remain central to classification and prognosis, other molecular markers, such as cytokines, chemokines, myokines, and muscle-specific microRNAs, offer complementary insights into inflammatory activity, fibrotic remodeling, and tissue injury.
This review provides a comprehensive analysis of the most relevant non-autoantibody biomarkers described in idiopathic inflammatory myopathies, with a particular focus on dermatomyositis (DM). These biomarkers are discussed according to their biological role and clinical applicability, encompassing three main domains: immunological, cellular or soluble, and prognostic/therapeutic response. This classification is not intended to be mutually exclusive, as most biomarkers may reflect multiple disease dimensions. Rather, it highlights the predominant context in which each marker has been studied and validated, while the prognostic and therapeutic response domain integrates biomarkers with longitudinal evidence supporting their utility in monitoring disease activity, predicting outcomes, or assessing treatment response across clinical settings.
  • Immunological (main mediators of inflammation or innate/adaptive immunity): IL-6, TNF-α, IFN-α/β, CXCL9/CXCL10, CCL2 (MCP-1), ISGs, neopterin, TGF-β1, MHC class I, C5b-9 (MAC).
  • Cellular or soluble (related to tissue damage, local inflammation, or cellular response): CRP/ESR, ferritin, S100A8/A9 (calprotectin), LDH, CK-MB, myoglobin, miR-1, miR-133a/b, miR-206, MMP-1/MMP-3, KL-6, SP-D.
  • Prognostic/therapeutic response (useful to monitor disease course, treatment response, or prognosis): ISGs, CXCL9/CXCL10, ferritin, KL-6, SP-D, miR-1, miR-133a/b, miR-206, MMP-1/MMP-3 [4].
By integrating immunological, biochemical, and molecular perspectives, this review aims to distinguish validated clinical biomarkers from emerging experimental candidates, providing a critical synthesis of their evidence base, methodological limitations, and translational relevance within precision medicine approaches for the heterogeneous clinical spectrum of DM and related myopathies.

2. Methodology

This narrative review was conducted through a comprehensive literature search aimed at identifying non-autoantibody biomarkers in dermatomyositis (DM) and related idiopathic inflammatory myopathies (IIMs). Articles published between January 2000 and March 2025 were retrieved from PubMed (National Center for Biotechnology Information, Bethesda, MD, USA), Web of Science (Clarivate Analytics, Philadelphia, PA, USA), Elsevier’s ScienceDirect database (Elsevier B.V., Amsterdam, The Netherlands), and SciELO (Scientific Electronic Library Online; Fundação de Amparo à Pesquisa do Estado de São Paulo, São Paulo, Brazil) to ensure broad coverage of relevant biomedical evidence. The search strategy combined free-text terms and Medical Subject Headings (MeSH) related to inflammatory myopathies and biomarkers. The key descriptors included Dermatomyositis, Idiopathic Inflammatory Myopathies, Myositis, Biological Markers, Proteomics, MicroRNAs, Gene Expression Profiling, Transcriptome, Cytokines, Chemokines, Interferons, Transforming Growth Factor beta1, Neopterin, Ferritin, C-Reactive Protein, Erythrocyte Sedimentation Rate, Creatine Kinase, Lactate Dehydrogenase, and Myoglobin. Inclusion criteria comprised original human studies, meta-analyses, and systematic reviews published in English or Spanish, addressing circulating, tissue, or transcriptomic biomarkers in DM or IIMs. Exclusion criteria included animal-only studies, case reports with fewer than five patients, and publications without measurable biomarker data. To enhance methodological transparency, each biomarker discussed was categorized by its biological function and clinical application. The level of evidence (High, Moderate, or Low) presented in Table 1 follows the Oxford Centre for Evidence-Based Medicine (OCEBM) guidelines, based on study design, reproducibility, and clinical validation. Although this review is narrative rather than systematic, deliberate efforts were made to apply structured search and selection procedures to ensure a coherent, comprehensive, and critically informed synthesis of current evidence on non-autoantibody biomarkers in dermatomyositis.

3. Immunological Biomarkers

3.1. Pro-Inflammatory Cytokines (IL-6, TNF-α, IFN-α/β)

Pro-inflammatory cytokines such as interleukin-6 (IL-6), tumor necrosis factor-alpha (TNF-α), and type I interferons (IFN-α/β) play central roles in the immune dysregulation that drives idiopathic inflammatory myopathies (IIMs), including dermatomyositis (DM). IL-6 is a pleiotropic cytokine involved in the acute-phase response, hematopoiesis, and B-cell maturation, and it acts as a key mediator of both systemic and muscle inflammation [5]. It activates STAT3 signaling, promoting muscle damage and inflammation, particularly in anti-MDA5-positive DM with interstitial lung disease [6,7]. Elevated IL-6 levels correlate with disease activity and progression, especially in juvenile forms [8,9]. TNF-α regulates both innate and adaptive immunity by promoting cytokine cascades, apoptosis, and lymphocyte differentiation [10]. In IIMs, increased TNF-α expression in muscle tissue enhances immune activation and MHC-I overexpression, fostering antigen presentation and chronic tissue injury [11,12]. Genetic variants such as the 308A promoter polymorphism further associate TNF-α with DM susceptibility [13]. However, anti–TNF-α therapies have shown inconsistent outcomes, underscoring the cytokine’s complex role [14]. Type I interferons (IFN-α/β) amplify inflammatory pathways via interferon-stimulated genes (ISGs), mediating NK cell activation, antigen presentation, and chronic immune activation [15,16]. In DM, excessive IFN production by plasmacytoid dendritic cells drives persistent ISG expression in skin and muscle, correlating with disease severity and clinical activity [17]. JAK–STAT inhibitors targeting this pathway have shown promising results in refractory DM by reducing ISG signatures [18].
Clinical use: Moderate evidence; useful in DM-ILD and disease activity monitoring but lacks specificity.
Comparative summary: IL-6 and TNF-α mainly reflect tissue-level inflammation, whereas IFN-α/β signals broader transcriptional immune activation. All three have strong biological rationale as biomarkers but lack disease specificity, supporting the use of combined biomarker panels rather than single-cytokine measurements.

3.2. Chemokines (CXCL9, CXCL10, CCL2)

Chemokines orchestrate leukocyte trafficking and play crucial roles in perpetuating inflammation in IIMs. CXCL9 and CXCL10, both IFN-γ–induced chemokines, recruit CD4+ and CD8+ T lymphocytes via CXCR3 interaction, maintaining inflammatory infiltrates in muscle tissue [19]. Their overexpression in DM muscle, vessels, and skin correlates with greater disease activity and presence of anti-MDA5 or anti-TIF1γ autoantibodies [20]. Elevated serum levels are associated with ILD and severe disease manifestations, particularly in juvenile or anti-MDA5-positive DM [21,22]. CCL2 (MCP-1) recruits monocytes and macrophages through CCR2 binding, sustaining local inflammation [23]. Its overexpression in DM muscle vasculature promotes cellular infiltration and correlates with clinical activity and cumulative organ damage [24,25]. Experimental CCL2/CCR2 inhibition has reduced inflammatory cell recruitment and improved outcomes in animal models [26].
Clinical use: Moderate evidence; serum CXCL9/10 and CCL2 are promising for activity monitoring and prognosis in DM, especially ILD forms.
Comparative summary: CXCL9/10 reflect IFN-driven responses, whereas CCL2 links NF-κB activation to monocyte recruitment. Their combined measurement may enhance specificity for DM-associated ILD compared with cytokine levels alone.

3.3. Interferon-Stimulated Genes (ISGs)

ISGs represent transcriptional outputs of interferon signaling via the JAK–STAT pathway, mediating antiviral defense, apoptosis, and antigen presentation [27]. In IIMs, ISG-enriched signatures in muscle, skin, and blood strongly correlate with disease activity and severity [28]. ISG15 overexpression has been proposed as a diagnostic marker for DM [28]. Persistent ISG activation perpetuates inflammation and tissue injury, suggesting a pathogenic loop [28]. JAK inhibitors such as ruxolitinib and tofacitinib effectively reduce ISG expression and clinical symptoms in refractory DM [29].
Clinical use: High evidence; ISG signature serves as a reliable indicator of disease activity and therapeutic response in DM.

3.4. Neopterin

Neopterin, produced by macrophages in response to IFN-γ, reflects cellular immune activation and oxidative stress [30]. Elevated neopterin levels in DM correspond to heightened immune activation and may contribute to tissue injury via reactive oxygen species generation [31,32]. Its concentration correlates with disease activity, especially in autoantibody-specific subtypes [33]. Although not yet standardized, neopterin monitoring could complement disease severity and treatment-response assessment [31]. However, its clinical translation is limited by the absence of disease-specific cut-off values, heterogeneous study populations, and the lack of multicenter validation studies specifically focused on dermatomyositis, which currently restricts its evidence level and routine applicability.
Clinical use: Limited evidence; potential adjunct marker for immune activation and severity assessment but lacks specificity in DM. Its greatest potential may lie in composite inflammatory panels rather than isolated measurement.

3.5. TGF-β1

Transforming growth factor beta 1 (TGF-β1) modulates immune regulation and fibrotic remodeling. In IIMs, it marks the shift from inflammation to fibrosis, promoting collagen deposition and myofibroblast activation [34]. Overexpression in DM muscle, particularly in fibrotic areas, indicates a role in chronic remodeling rather than acute inflammation [35]. TGF-β1 regulates microRNAs such as miR-21 and miR-424, enhancing extracellular matrix accumulation, while miR-663 acts as a negative regulator [36].
Clinical use: Emerging evidence; may serve as a marker of chronicity and fibrosis progression in DM, but lacks specificity. Its clinical applicability is further limited by variability in detection methods, inconsistent correlations with clinical outcomes across studies, and the absence of longitudinal and multicenter data validating its role as a standalone biomarker in dermatomyositis. At present, TGF-β1 remains more informative as a mechanistic marker of chronic remodeling rather than as a clinically actionable biomarker.

3.6. Complement and Antigen Presentation (MHC-I, C5b-9)

Aberrant antigen presentation and complement activation are central mechanisms linking innate and adaptive immunity in IIMs. MHC class I molecules, responsible for presenting endogenous antigens to CD8+ T cells, are aberrantly overexpressed in DM muscle fibers even in the absence of inflammation, suggesting early immune activation [37,38]. This induces endoplasmic reticulum stress and muscle fiber damage [39]. Experimentally, sustained MHC-I expression reproduces autoimmune myopathy features and correlates with early diagnostic stages [38,40]. The complement membrane attack complex (C5b-9) represents terminal complement activation leading to cell lysis. Its deposition in capillary endothelium and muscle fibers indicates intense complement-mediated damage, especially in DM and necrotizing myopathies [41,42]. In DM, C5b-9 localization in perifascicular capillaries constitutes a hallmark feature, differentiating it from other IIMs [43,44]. However, its specificity remains limited, requiring clinical correlation [44]. Beyond MHC class I overexpression on muscle fibers, which is a well-established hallmark of dermatomyositis, accumulating evidence indicates that MHC class II expression on antigen-presenting cells, along with CD4+ T-cell–mediated immune responses, also plays a central role in disease pathophysiology. These mechanisms contribute to cytokine-driven inflammation, type I interferon signaling, and vascular and perifascicular involvement characteristic of DM.
Clinical use: High evidence; MHC-I overexpression is an early diagnostic marker, while C5b-9 deposition supports DM histopathologic confirmation.
Comparative summary: MHC-I and C5b-9 mark immune effector mechanisms at the tissue level. MHC-I upregulation represents an early immunopathogenic trigger, while complement deposition reflects downstream cytotoxicity. Together, they provide histopathological specificity that complements circulating biomarkers.

3.7. Overall Synthesis of Immunological Biomarkers

Immunological biomarkers in DM reflect a continuum from cytokine-driven immune activation to chemokine-mediated recruitment and effector-mediated tissue injury. Proinflammatory cytokines and chemokines capture disease activity and systemic inflammation, whereas ISGs and MHC-I/C5b-9 represent molecular and structural footprints of immune dysregulation. Despite advances, individual biomarkers lack sufficient specificity; thus, integrated multi-marker panels combining cytokines, ISG signatures, and tissue markers may offer superior diagnostic and prognostic accuracy.

4. Cellular/Soluble Biomarkers

4.1. CRP/ESR

C-reactive protein (CRP) is an acute-phase reactant synthesized by the liver in response to interleukin-6 during inflammation. It binds to damaged cell components and pathogens, activating complement and facilitating phagocytosis, thus mediating innate immune defense [45]. Erythrocyte sedimentation rate (ESR) reflects the plasma’s inflammatory protein load, indirectly measuring systemic inflammation [46]. In idiopathic inflammatory myopathies (IIMs), both CRP and ESR are broadly used for assessing disease activity. In dermatomyositis (DM), ESR tends to rise moderately, while CRP often remains normal unless inflammation is pronounced [47]. However, in anti-MDA5-positive DM, CRP elevations correlate with systemic inflammation and poorer outcomes, particularly in rapidly progressive interstitial lung disease (RP-ILD) [48]. Persistently elevated ESR values have also been associated with ILD development and mortality risk [49].
Clinical use: High evidence for longitudinal monitoring and risk assessment in DM. Although nonspecific, their combined interpretation helps identify active or severe disease phenotypes.

4.2. Ferritin and S100A8/A9 (Calprotectin)

Ferritin is a 24-subunit protein complex that stores iron in its ferric form, limiting oxidative stress. It is mainly synthesized by hepatocytes and macrophages and also functions as an acute-phase reactant upregulated by cytokines such as IL-6 [50]. In IIMs, elevated ferritin correlates with heightened systemic inflammation and poor prognosis [51]. In DM, particularly in anti-MDA5-positive subtypes, ferritin levels are strongly associated with RP-ILD and higher mortality [52]. S100A8/A9, or calprotectin, is a heterodimeric calcium-binding protein complex abundant in neutrophils and monocytes [53]. Upon activation, it is secreted into the extracellular space, promoting leukocyte chemotaxis, cytokine release, and oxidative stress through NADPH oxidase activation [54]. In IIMs, calprotectin is overexpressed in inflamed muscle tissues and correlates with disease activity [55]. In DM-ILD, serum S100A8/A9 levels are linked to reduced pulmonary function, extensive lung lesions, and poor outcomes, as well as with elevated IL-4 and IL-6 levels [56].
Clinical use: Moderate evidence; Ferritin provides prognostic value in identifying high-risk anti-MDA5 phenotypes, while calprotectin correlates more closely with disease activity and ILD severity, but both lack specificity.
Comparative summary: Ferritin primarily signals macrophage activation and iron dysregulation, whereas calprotectin reflects neutrophil and monocyte-driven inflammation. Both demonstrate prognostic potential in DM-ILD, supporting their combined assessment for early risk stratification and therapeutic monitoring.

4.3. LDH and CK-MB

Lactate dehydrogenase (LDH) catalyzes the interconversion of lactate and pyruvate during anaerobic glycolysis [57]. It is ubiquitously expressed and released into circulation following tissue damage. In IIMs, LDH elevation indicates ongoing muscle necrosis and correlates with inflammatory activity, especially when creatine phosphokinase (CPK) levels are normal [58]. In DM, high LDH levels are strongly associated with ILD presence and severity, often preceding RP-ILD onset [59]. It is also incorporated into the FLAIR score to predict pulmonary complications [60]. CK-MB, an isoenzyme of creatine kinase composed of muscle (M) and brain (B) subunits, is typically used for cardiac assessment but can also increase in skeletal muscle injury [61]. In IIMs, elevated CK-MB can appear in the absence of cardiac pathology, reflecting active muscle inflammation [62,63]. Persistent elevation, particularly when total CK is normal, may indicate subclinical disease activity [64].
Clinical use: Moderate evidence; combined interpretation improves diagnostic accuracy in muscle-dominant and pulmonary forms of DM, but are highly unspecific for DM alone.
Comparative summary: LDH reflects overall metabolic stress and inflammatory burden, including pulmonary involvement, while CK-MB refines the interpretation of muscle injury and helps exclude cardiac events. Together, they enhance biochemical assessment of disease activity and organ involvement in DM.

4.4. Myoglobin

Myoglobin is a heme protein responsible for intracellular oxygen storage and delivery in skeletal muscle [65]. It is released rapidly following muscle injury, making it an early but nonspecific marker of tissue damage [66]. Elevated myoglobin levels at IIM diagnosis indicate active muscle necrosis [67]. In DM, higher myoglobin concentrations are associated with more severe disease and can signal rhabdomyolysis risk [66]. A 2025 study further linked myoglobin levels to oxidative stress markers, suggesting a pathogenic role in promoting reactive oxygen species formation [68]. Although myoglobin reflects acute muscle fiber injury and may signal an increased risk of rhabdomyolysis, its clinical utility in idiopathic inflammatory myopathies remains context-dependent. In IIMs, including dermatomyositis, elevated myoglobin levels may accompany disease flares or severe muscle involvement; however, myoglobin lacks disease specificity and does not reliably discriminate between inflammatory myopathy and other causes of muscle breakdown, such as toxic or statin-associated rhabdomyolysis [66].
In seronegative IIMs or in patients receiving statins, myoglobin elevation should therefore be interpreted cautiously and in conjunction with clinical features, autoantibody profiles, creatine kinase dynamics, electromyography, and histopathological findings. Rather than serving as a diagnostic discriminator, myoglobin may be most useful as an adjunct marker of acute muscle injury severity and renal risk assessment in selected clinical scenarios.
Clinical use: Moderate evidence. Sequential measurement of serum myoglobin can aid in the early detection of acute exacerbations or renal complications secondary to rhabdomyolysis. However, its diagnostic value is limited by low disease specificity, as elevations also occur in other myopathic and traumatic conditions.

4.5. miR-1, miR-133a/b, miR-206

The muscle-specific microRNAs miR-1, miR-133a/b, and miR-206 (myomiRs) regulate muscle differentiation and regeneration [69]. Their downregulation in IIMs contributes to impaired myogenesis and chronic inflammation [70]. Cytokines such as TNF-α, IFN-α, and IL-1β suppress miR-1 and miR-133a/b expression, while reduced miR-206 levels correlate with decreased regenerative capacity and sustained inflammation [71].
Clinical use: High evidence. Circulating myomiRs represent promising noninvasive biomarkers that reflect disease activity, severity, and therapeutic response. However, their expression changes are not specific to dermatomyositis, as similar alterations occur in other myopathic and systemic inflammatory conditions [72].

4.6. MMP-1/MMP-3

Matrix metalloproteinases (MMP-1 and MMP-3) mediate extracellular matrix degradation and remodeling during tissue inflammation [73]. Their expression, induced by cytokines such as TNF-α and IL-1β, contributes to muscle fiber damage in IIMs [73]. In DM, both enzymes are upregulated near inflammatory infiltrates [74]. MMP-3 expression is regulated by the IFN-γ–STAT1 axis, and its inhibition by resveratrol suggests therapeutic potential [75,76].
Clinical use: Moderate evidence. Reflect active tissue remodeling and inflammation in dermatomyositis, though not disease-specific.

4.7. KL-6

KL-6 is a mucin-like glycoprotein secreted by type II alveolar cells following epithelial injury [77]. In IIMs, serum KL-6 is markedly elevated in patients with ILD and inversely correlates with pulmonary function parameters such as FVC and DLCO [78]. Persistently high KL-6 levels predict RP-ILD, chronic progression, and increased mortality [79,80].
Clinical use: High evidence. Reliable marker for diagnosis, prognosis, and monitoring of ILD in patients with DM.

4.8. SP-D

Surfactant protein D (SP-D), produced by type II pneumocytes and Clara cells, participates in pulmonary innate immunity and epithelial repair [81]. Its presence in serum reflects alveolar injury and ILD activity. Elevated SP-D levels in DM and PM patients correlate with disease severity and reduced lung function [82]. Persistent elevation predicts adverse outcomes, while combined SP-D and KL-6 analysis improves diagnostic precision [83,84].
Clinical use: Moderate-to-high evidence. Serves as a sensitive marker of alveolar injury and ILD activity in DM, complementing KL-6 for risk stratification and monitoring. Primarily informative for DM-associated ILD; not specific for DM overall and may be elevated in other ILDs.

4.9. Overall Synthesis of Cellular and Soluble Biomarkers

Cellular and soluble biomarkers in DM reflect a spectrum from acute-phase and metabolic responses to tissue-specific injury and repair mechanisms. Acute-phase proteins such as CRP, ferritin, and calprotectin capture systemic inflammation and immune activation, while enzymes like LDH and CK-MB indicate ongoing muscle damage. Myoglobin complements these markers by reflecting both acute injury and oxidative stress, and myomiRs provide insight into impaired regenerative processes. MMPs reveal active extracellular matrix remodeling, whereas KL-6 and SP-D serve as lung-specific indicators of epithelial injury in ILD. Individually, these biomarkers provide limited specificity; however, their integrated assessment enables a more comprehensive evaluation of disease activity, organ involvement, and prognosis in DM, particularly when combining systemic inflammatory, muscular, and pulmonary markers. As illustrated in Figure 1, these cellular and soluble biomarkers interact with immunological and interferon-driven pathways, forming a dynamic network that underlies inflammation, tissue damage, and fibrotic remodeling in dermatomyositis.

5. Prognostic or Therapeutic Response Biomarkers

Several biomarkers discussed in the immunological and cellular domains also exhibit prognostic or treatment-response value; therefore, this section synthesizes cross-cutting evidence from longitudinal and outcome-oriented studies rather than introducing a distinct biomarker category.

5.1. ISGs

Elevated ISG expression correlates with worse outcomes in DM and anti-MDA5+ patients, linking high IFN-I scores to increased disease activity, elevated CRP/ferritin, and poorer short-term survival [85,86]. ISGs guide therapy selection, especially with JAK inhibitors, and monitor treatment response [86]. Most useful for early diagnosis, prognostic stratification, and guiding targeted therapies; routine use for ILD monitoring remains investigational.

5.2. CXCL9/CXCL10

Serum CXCL9 and CXCL10 levels predict prognosis in DM-ILD, with persistent elevation indicating higher mortality [22,87,88]. Reduction with JAK inhibitor therapy correlates with clinical improvement, supporting their use for therapeutic monitoring [89]. Strong candidates for prognostic and therapy-guiding biomarkers, especially in ILD, pending further validation.

5.3. Ferritin

High ferritin (>1000–1600 ng/mL) predicts RP-ILD risk and mortality in anti-MDA5+ DM [90]. Decreases correlate with treatment response, supporting use in therapeutic monitoring [91]. Validated for prognosis in ILD, but cutoff standardization and broader application are needed [89,92].

5.4. KL-6

Elevated KL-6 identifies ILD presence and severity, correlates with lung function decline, and predicts mortality and acute exacerbations [93,94]. Declines with effective therapy allow monitoring treatment response, confirming its value as a diagnostic and prognostic biomarker. Wider standardization is required for routine clinical application [95].

5.5. MHC Class I

Persistent MHC-I overexpression correlates with disease activity and severity, decreasing with immunosuppressive therapy [96,97]. Used for diagnosis, prognosis, and monitoring treatment response, especially in early DM; requires standardization for routine clinical application [98].

5.6. miR-1, miR-133a/b, miR-206

Altered myomiR levels reflect active disease and therapeutic response [99,100]. miR-206 correlates with disease progression and regeneration capacity. Clinically, myomiRs are promising biomarkers for prognosis and treatment monitoring, pending assay standardization [101,102].

5.7. C5b-9 (Membrane Attack Complex)

C5b-9 deposition is highly specific for DM diagnosis, especially in juvenile cases [103]. Levels decrease with effective immunosuppression, offering potential therapeutic monitoring, though non-invasive assays are not standardized [104,105]. Currently, primarily diagnostic, with investigational use in longitudinal monitoring.

6. Discussion

Biomarkers have become essential tools in the evolving landscape of idiopathic inflammatory myopathies (IIMs), complementing clinical evaluation, improving diagnostic precision, and informing therapeutic decision-making, particularly in cases with atypical manifestations or overlapping autoimmune syndromes. Across the IIM spectrum, the combined assessment of systemic inflammation markers such as CRP, ESR, and ferritin; muscle damage indicators including LDH, CK-MB, and myoglobin; and immune dysregulation mediators such as ISGs, TNF-α, IL-6, CXCL9, and CXCL10 has enabled a more dynamic and biologically grounded characterization of disease activity. The incorporation of muscle-specific microRNAs and tissue remodeling proteins has further expanded the diagnostic armamentarium, opening new avenues for detecting subclinical inflammation, monitoring persistent disease, and assessing residual tissue injury.
Although many of these biomarkers reflect overlapping aspects of inflammation, tissue damage, prognosis, and treatment response, their organization into distinct domains aims to facilitate clinical interpretation and translational applicability. This conceptual framework is intended as a pragmatic clinical lens rather than a strict biological categorization, allowing biomarkers to be integrated across different disease stages and clinical scenarios.
Among the IIMs, dermatomyositis (DM) stands out as the most extensively studied subtype in terms of biomarker research, due to its relatively higher prevalence, distinct immunopathological profile, and strong association with systemic complications such as interstitial lung disease (ILD), severe vasculopathy, and paraneoplastic syndromes. These features have concentrated research efforts, particularly in anti-MDA5-positive cohorts. In this context, robust molecular candidates have emerged, including interferon-stimulated genes (ISGs), KL-6, SP-D, ferritin, CXCL10, and myomiRs such as miR-206, which have demonstrated diagnostic, prognostic, and even therapeutic monitoring potential. These findings highlight the capacity of biomarker-driven approaches to improve early identification of high-risk patients and support the implementation of personalized therapeutic strategies. By integrating data from proteomic, transcriptomic, and microRNA-based platforms, this field illustrates how molecular signatures can serve as a foundation for precision medicine in inflammatory myopathies, paving the way for a shift from generalized disease management to stratified and biologically informed care.

6.1. Validated Biomarkers for Clinical Use

A limited number of biomarkers currently meet the criteria for translational application. Among them, KL-6 and SP-D, both markers of alveolar epithelial injury, are the most reliable indicators of DM-associated interstitial lung disease (DM-ILD). Persistently elevated levels predict chronic progression, acute exacerbations, and increased mortality. Ferritin, reflecting macrophage activation and systemic inflammation, is another validated biomarker, particularly in anti-MDA5-positive DM, where levels above 1000–1600 ng/mL correlate with rapidly progressive ILD (RP-ILD) and poor outcomes. Chemokines such as CXCL10 (IP-10) represent an additional high-value candidate, serving as a dynamic indicator of interferon-driven inflammation and treatment response, especially under JAK inhibitor therapy. Similarly, ISG signatures (including ISG15 and MX1) correlate with disease activity and short-term survival, offering both diagnostic and pharmacodynamic utility. Together, these molecules form the backbone of clinically actionable biomarkers that connect inflammatory, fibrotic, and interferon-related pathways with prognosis.

6.2. Experimental and Emerging Biomarkers

Several biomarkers remain at an exploratory stage but show potential for future clinical translation. Circulating muscle-specific microRNAs (miR-1, miR-133a/b, miR-206) serve as non-invasive indicators of muscle injury and regeneration, correlating with disease activity and treatment response. S100A8/A9 (calprotectin) and neopterin, reflecting monocyte and neutrophil activation, demonstrate associations with systemic inflammation and pulmonary involvement, although their disease specificity remains limited. Enzymatic markers such as LDH and CK-MB continue to be valuable adjuncts in detecting subclinical muscle activity, while MMP-1/3 and TGF-β1 reflect ongoing fibrotic remodeling. Despite their biological relevance, heterogeneity across studies and the lack of standardized assays prevent their integration into clinical algorithms. Nonetheless, these biomarkers offer promising insights into disease pathogenesis and may complement validated molecules in future multimodal panels. Importantly, the limited clinical translation of these low-evidence biomarkers is driven not only by biological complexity, but also by methodological constraints, including heterogeneous patient cohorts, lack of assay standardization, and scarce multicenter validation, underscoring key research gaps that must be addressed before routine implementation.

6.3. Future Directions and Implementation Barriers

Despite significant progress, several obstacles hinder the routine clinical implementation of biomarkers in DM. Many studies rely on small or monocentric cohorts, employ heterogeneous inclusion criteria, or lack standardized platforms and cut-off values, limiting reproducibility and comparability. Moreover, several promising markers, such as neopterin, S100A8/A9, and myomiRs, lack sufficient replication across diverse populations. Even well-characterized biomarkers like KL-6 and SP-D are primarily validated in anti-MDA5-positive patients, restricting their generalizability.
From a feasibility standpoint, high-throughput techniques such as transcriptomic or microRNA profiling remain largely inaccessible in non-specialized or resource-limited settings. Therefore, ferritin and LDH remain the most practical and cost-effective options for disease monitoring in such contexts. Broader implementation will require multicenter collaborations, standardized assays, and longitudinal studies to define biomarker trajectories over time and validate their predictive value for treatment response, disease flares, and organ-specific complications such as ILD.
While these barriers currently limit widespread clinical adoption, ongoing technological advances are expected to progressively complement conventional biomarker strategies rather than replace them. Beyond single-biomarker validation, emerging technological approaches are likely to redefine biomarker discovery and implementation in dermatomyositis. Multi-omics integration, combining transcriptomics, proteomics, microRNA profiling, and clinical data, offers the potential to capture disease heterogeneity at an unprecedented resolution. In this context, single-cell RNA sequencing and spatial transcriptomics may further delineate cell-specific immune and fibrotic pathways, particularly within muscle and lung tissue.
In parallel, circulating extracellular vesicles and exosomal biomarkers have emerged as promising non-invasive platforms, as they reflect tissue-specific molecular signatures and enable longitudinal monitoring of immune activation, fibrosis, and treatment response. Exosome-derived microRNAs and proteins may overcome some limitations of bulk serum measurements by providing higher specificity and stability.
Additionally, artificial intelligence–assisted analysis and machine learning algorithms are increasingly applied to integrate complex biomarker datasets, imaging features, and digital pathology. These approaches may enable the development of predictive models for disease progression, treatment response, and ILD risk stratification, moving from descriptive biomarkers toward actionable clinical decision-support tools, particularly when integrated with clinically accessible biomarkers in stepwise translational frameworks.

6.4. Proposed Composite Model

Integrative approaches combining inflammatory, fibrotic, and interferon-driven biomarkers are likely to provide superior predictive accuracy compared with isolated measurements. A practical example could be a composite panel where ferritin > 1000 ng/mL, KL-6 > 1000 U/mL, and elevated CXCL10 jointly identify patients at high risk for rapidly progressive ILD, prompting early initiation of intensive immunosuppressive or JAK inhibitor therapy. The inclusion of dynamic biomarkers such as serum myomiRs may further enhance monitoring precision and treatment personalization.

6.5. Projections and Limitations

While this review focuses on DM due to the robustness of available evidence, biomarker data remain limited or inconsistent for other IIM subtypes, including polymyositis, necrotizing autoimmune myopathy, and inclusion body myositis. Addressing these gaps requires multicenter cooperation, standardized sample handling, and harmonized analytical methodologies. Moreover, transitioning from descriptive studies to integrative, multi-omic approaches, leveraging artificial intelligence, single-cell technologies, and digital pathology, will be essential to develop composite diagnostic algorithms with superior sensitivity and specificity. It is important to acknowledge that this work is a narrative review, not a systematic one, and therefore carries inherent selection bias; however, efforts were made to ensure transparency and rigor in the literature synthesis. Future research should move beyond isolated biomarker discovery toward translational validation and equitable access to advanced diagnostics. Achieving this vision will depend on multidisciplinary collaboration between clinicians, immunologists, and computational scientists capable of integrating complex biological datasets into actionable clinical tools.

7. Conclusions

Future biomarker integration in dermatomyositis should prioritize clinically validated markers, notably ferritin, KL-6, CXCL10, and ISG15, while incorporating emerging candidates such as myomiRs and calprotectin to refine disease monitoring. A composite algorithm encompassing inflammatory, fibrotic, and interferon-driven signatures could significantly improve early risk stratification and guide timely therapeutic escalation. In Latin American and resource-limited settings, ferritin and LDH remain the most feasible biomarkers for practical implementation. Ultimately, the convergence of molecular, clinical, and computational approaches will pave the way for precision medicine in dermatomyositis, transforming biomarker research from theoretical promise into clinical reality.

Author Contributions

Conceptualization: F.J., K.W.K.J.H., C.K. and L.H.; methodology: F.J., K.W.K.J.H., C.K. and L.H.; validation: K.W.K.J.H., C.K. and L.H.; investigation: F.J. and C.K.; data curation: F.J.; writing—original draft preparation: F.J.; writing—review and editing: F.J., K.W.K.J.H., C.K. and L.H.; supervision: K.W.K.J.H., C.K. and L.H.; project administration: K.W.K.J.H. and C.K. All authors have read and agreed to the published version of the manuscript.

Funding

This research received no external funding.

Institutional Review Board Statement

Not applicable.

Informed Consent Statement

Not applicable.

Data Availability Statement

All data generated or analyzed during this study are included in this published article.

Conflicts of Interest

The authors declare no conflicts of interest.

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Figure 1. Conceptual representation of inflammatory, fibrotic, and interferon-driven pathways in dermatomyositis.
Figure 1. Conceptual representation of inflammatory, fibrotic, and interferon-driven pathways in dermatomyositis.
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Table 1. Biomarkers studied in inflammatory myopathies such as Dermatomyositis. The analyzed biomarkers are divided by their type, biological function, their clinical application, level of evidence and specificity for DM.
Table 1. Biomarkers studied in inflammatory myopathies such as Dermatomyositis. The analyzed biomarkers are divided by their type, biological function, their clinical application, level of evidence and specificity for DM.
BiomarkerTypeBiological FunctionClinical ApplicationLevel of EvidenceSpecificity for DMClinical Implementation
IL-6CytokineProinflammatory mediatorMarker of systemic inflammation and disease activityModerateLowNo
TNF-αCytokineRegulates inflammation, apoptosis, and lymphocyte differentiationInvolved in pathophysiology; potential therapeutic targetModerateLowNo
IFN-α/βCytokineAntiviral response and immunomodulationDiagnostic and prognostic marker; associated with lung involvementHighModerateNo
CXCL9/CXCL10ChemokineT cell recruitmentAssociated with disease activity and treatment responseHighHighYes
CCL2 (MCP-1)ChemokineMonocyte recruitmentInflammation marker and mononuclear infiltrateModerateLowNo
ISGsGene signatureIFN-induced gene activationAssociated with DM and ILD; useful for disease monitoringHighHighYes
NeopterinMetaboliteIndicator of immune activationMarker of immune activity and therapeutic responseLowLowNo
TGF-β1CytokineRegulation of fibrosis and tissue repairInvolved in muscle fibrosis and interstitial lung disease (ILD)ModerateLowNo
MHC class I HLA protein Antigen presentationOverexpressed in muscle biopsies; early diagnostic markerHighHighYes
C5b-9 (MAC)Complement complexFormation of membrane attack complexDiagnostic marker in active DMModerateHighYes
CRP/ESRInflammatory proteinAcute inflammation responseNon-specific markers of systemic inflammationHighLowNo
FerritinProteinIron storage, inflammation markerAssociated with severe anti-MDA5+ DM and progressive ILDHighModerate Yes
S100A8/A9 (calprotectin)ProteinInflammatory and chemotactic mediatorIndicates active muscle inflammationModerateModerate No
LDHEnzymeCellular energy metabolismNon-specific indicator of muscle damageHighLowNo
CK-MBEnzymeCardiac creatine kinase isoenzymeDifferentiates cardiac vs. skeletal muscle damageModerateLowNo
MyoglobinProteinOxygen storage and transport in muscleMarker of acute muscle injuryModerateLowNo
miR-1, miR-133a/b, miR-206Muscle micro RNAsPost-transcriptional regulation in muscleUseful for diagnosis, prognosis, and monitoringHighModerateYes
MMP-1/
MMP-3		MetalloproteinaseExtracellular matrix degradationMarkers of tissue remodeling and disease activityModerateLowNo
KL-6GlycoproteinMarker of pulmonary epithelial damageDiagnosis and monitoring of ILDHighHighYes
SP-DSurfactant proteinPulmonary immune functionAssociated with ILD and lung damageHighModerateYes
Note: Evidence level (High = ≥3 independent cohorts; Moderate = 1–2 cohorts; Low = exploratory).
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Jaederlund, F.; Joo Hu, K.W.K.; Karsulovic, C.; Hojman, L. Current and Emerging Biomarkers in Dermatomyositis: Clinical Utility and Future Directions. Int. J. Transl. Med. 2026, 6, 4. https://doi.org/10.3390/ijtm6010004

AMA Style

Jaederlund F, Joo Hu KWK, Karsulovic C, Hojman L. Current and Emerging Biomarkers in Dermatomyositis: Clinical Utility and Future Directions. International Journal of Translational Medicine. 2026; 6(1):4. https://doi.org/10.3390/ijtm6010004

Chicago/Turabian Style

Jaederlund, Fiona, Ka Wei Katty Joo Hu, Claudio Karsulovic, and Lia Hojman. 2026. "Current and Emerging Biomarkers in Dermatomyositis: Clinical Utility and Future Directions" International Journal of Translational Medicine 6, no. 1: 4. https://doi.org/10.3390/ijtm6010004

APA Style

Jaederlund, F., Joo Hu, K. W. K., Karsulovic, C., & Hojman, L. (2026). Current and Emerging Biomarkers in Dermatomyositis: Clinical Utility and Future Directions. International Journal of Translational Medicine, 6(1), 4. https://doi.org/10.3390/ijtm6010004

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