# Treatment Algorithms for Inflammatory Myopathies in Adults: from Guidelines to Clinical Practice

**Authors:** Albert Selva-O’Callaghan, Ernesto Trallero-Araguás, Albert Gil-Vila, Ana Matas-Garcia, Clara Edo, Jose Milisenda, Iago Pinal-Fernández

PMC · DOI: 10.1007/s40674-025-00239-5 · Current treatment options in rheumatology · 2026-02-21

## TL;DR

This paper reviews treatment guidelines for inflammatory myopathies and combines them with clinical experience to guide real-world treatment decisions.

## Contribution

The paper offers a pragmatic, consensus-based approach to managing inflammatory myopathies by integrating guidelines and recent evidence.

## Key findings

- Existing treatment guidelines for inflammatory myopathies show significant heterogeneity.
- Combining guidelines with clinical experience helps address management challenges in real-world settings.
- Recent studies on novel therapies may influence future treatment strategies.

## Abstract

Inflammatory myopathies are a heterogeneous group of systemic autoimmune disorders with highly variable clinical presentations. The limited number of clinical trials has hindered the development of strong evidence-based management strategies. Therefore, there is a pressing need for consensus-driven treatment guidelines tailored to the various clinical and immunological phenotypes. This review combines existing published guidelines with the authors’ clinical experience to provide a comprehensive, pragmatic approach to management.

A comparative review of existing treatment guidelines was recently published, revealing significant heterogeneity in therapeutic approaches and recommendations. This observation is consistent with our own experience during a multidisciplinary meeting, primarily involving internal medicine specialists and rheumatologists. Furthermore, recent clinical trials and observational studies investigating novel therapies offer promising prospects that could influence future clinical decision-making.

Although establishing a unified therapeutic algorithm remains challenging, this review aims to translate current knowledge into clinical practice by integrating existing guidelines, our own clinical experience, and the most recent evidence published on the topic.

## Full-text entities

- **Genes:** IL12B (interleukin 12B) [NCBI Gene 3593] {aka CLMF, CLMF2, IL-12B, IMD28, IMD29, NKSF}, mucin [NCBI Gene 100508689], TPMT (thiopurine S-methyltransferase) [NCBI Gene 7172] {aka TPMTD}, IFNB1 (interferon beta 1) [NCBI Gene 3456] {aka IFB, IFF, IFN-beta, IFNB}, IFNG (interferon gamma) [NCBI Gene 3458] {aka IFG, IFI, IMD69}, TNF (tumor necrosis factor) [NCBI Gene 7124] {aka DIF, IMD127, TNF-alpha, TNFA, TNFSF2, TNLG1F}, IFIH1 (interferon induced with helicase C domain 1) [NCBI Gene 64135] {aka AGS7, Hlcd, IDDM19, IMD95, MDA-5, MDA5}, KRT20 (keratin 20) [NCBI Gene 54474] {aka CD20, CK-20, CK20, K20, KRT21}, TNFSF13B (TNF superfamily member 13b) [NCBI Gene 10673] {aka BAFF, BLYS, CD257, TALL-1, TALL1, THANK}, CTLA4 (cytotoxic T-lymphocyte associated protein 4) [NCBI Gene 1493] {aka ALPS5, CD, CD152, CELIAC3, CTLA-4, GRD4}, IL1A (interleukin 1 alpha) [NCBI Gene 3552] {aka IL-1 alpha, IL-1A, IL1, IL1-ALPHA, IL1F1}, TRIM21 (tripartite motif containing 21) [NCBI Gene 6737] {aka RNF81, RO52, Ro/SSA, SSA, SSA1, TRIM21/Ro52}, RNPC3 (RNA binding region (RNP1, RRM) containing 3) [NCBI Gene 55599] {aka CPHD7, IGHD5, RBM40, RNP, SNRNP65}, IL23A (interleukin 23 subunit alpha) [NCBI Gene 51561] {aka IL-23, IL-23A, IL23P19, P19, SGRF}, HMGCR (3-hydroxy-3-methylglutaryl-CoA reductase) [NCBI Gene 3156] {aka LDLCQ3, LGMDR28, MYPLG}, CABIN1 (calcineurin binding protein 1) [NCBI Gene 23523] {aka CAIN, KB-318B8.7, PPP3IN}, TRIM33 (tripartite motif containing 33) [NCBI Gene 51592] {aka DDH4, ECTO, PTC7, RFG7, TF1G, TIF1G}
- **Diseases:** hypercapnia (MESH:D006935), embryopathy (MESH:D005315), IMNM (MESH:C567355), cardiac involvement (MESH:D006331), allergic reactions (MESH:D004342), pneumonitis (MESH:D011014), pulmonary fibrosis (MESH:D011658), Myocarditis (MESH:D009205), myopathy (MESH:D009135), IIM-ILD (MESH:D017563), myocardial injury (MESH:D009202), Inflammatory Myopathy (MESH:D009220), Gottron's papules and macules (MESH:C538187), thromboembolic (MESH:D013923), IBM (MESH:D018979), ASS (MESH:D020159), aspiration pneumonia (MESH:D011015), Organizing pneumonia (MESH:D000092124), OS (MESH:D000080445), heart failure (MESH:D006333), autoimmune disorders (MESH:D001327), arrhythmias (MESH:D001145), Rash (MESH:D005076), respiratory failure (MESH:D012131), Sharp syndrome (MESH:D008947), Cutaneous Dermatomyositis Disease (MESH:D003882), CDASI (MESH:D045169), systemic sclerosis (MESH:D012595), amyopathic DM (MESH:C538250), lupus (MESH:D008180), celiac disease (MESH:D002446), systemic disorders (MESH:D009422), herpes zoster (MESH:D006562), SARD (MESH:C537236), skin disease (MESH:D012871), lipoatrophy (MESH:C535905), Panniculitis (MESH:D015434), viral myocarditis (MESH:D014777), malnutrition (MESH:D044342), influenza (MESH:D007251), hypertension (MESH:D006973), muscle involvement (MESH:C566343), hyperglycemia (MESH:D006943), Dysphagia (MESH:D003680), RA (MESH:D001172), Inflammation (MESH:D007249), Arthritis (MESH:D001168), vasculitis (MESH:D014657), cutaneous disease (MESH:D004194), atrophy (MESH:D001284), Autoimmune Rheumatic Diseases (MESH:D012216), Cutaneous Ulcers (MESH:D014456), diaphragmatic dysfunction (MESH:D056989), hepatitis B (MESH:D006509), latent tuberculosis (MESH:D055985), CAM (MESH:D020786), compartment syndrome (MESH:D003161), toxicity (MESH:D064420), joint disease (MESH:D007592), myocardial involvement (MESH:C564676)
- **Chemicals:** cotrimoxazole (MESH:D015662), pimecrolimus (MESH:C117268), MTX (MESH:D008727), carbon dioxide (MESH:D002245), methylprednisolone (MESH:D008775), lovastatin (MESH:D008148), Cyclophosphamide (MESH:D003520), bisphosphonates (MESH:D004164), nintedanib (MESH:C530716), cyclic citrullinated peptide (MESH:C487763), GC (MESH:C057580), anifrolumab (MESH:C582345), Ustekinumab (MESH:D000069549), Tocilizumab (MESH:C502936), CsA (MESH:D016572), sildenafil (MESH:D000068677), TACRO (MESH:D016559), Belimumab (MESH:C511911), apremilast (MESH:C505730), bosentan (MESH:D000077300), canakinumab (MESH:C541220), 18Fluorodeoxyglucose (-), MMF (MESH:D009173), sodium thiosulfate (MESH:C017717), pravastatin (MESH:D017035), leflunomide (MESH:D000077339), creatine (MESH:D003401), tofacitinib (MESH:C479163), MFM (MESH:C027993), AZA (MESH:D001379), 18FDG (MESH:D019788), RTX (MESH:D000069283), pirfenidone (MESH:C093844)
- **Species:** PX clade (clade) [taxon 569578], Severe acute respiratory syndrome coronavirus 2 (no rank) [taxon 2697049], Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

11 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12922664/full.md

## References

80 references — full list in the complete paper: https://tomesphere.com/paper/PMC12922664/full.md

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Source: https://tomesphere.com/paper/PMC12922664