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.
This review aims to evaluate current treatment approaches for sporadic late-onset nemaline myopathy (SLONM) and explore how emerging omics data, particularly proteomics and transcriptomics, can contribute and optimize therapeutic strategies. SLONM presents with clinical and pathological variability and is frequently associated with monoclonal gammopathy of undetermined significance (MGUS), which can influence both disease progression and response to treatment. Standard therapies include immunosuppressive agents, intravenous immunoglobulin (IVIG), chemotherapy, and autologous stem cell transplantation (ASCT), with ASCT showing particularly favorable outcomes in MGUS-associated cases. Recent proteomic studies have identified distinct molecular patterns in SLONM, including altered expression of sarcomeric and immune-related proteins, as well as deposits of immunoglobulin light chains. Complementary transcriptomic data highlight immune dysregulation and metabolic stress as key contributors to disease pathology. Together, these findings support the development of omics-informed precision therapies that target plasma cell clones and immune pathways, offering improved patient stratification and more tailored treatment approaches. Integrating omics data with clinical features deepens our understanding of SLONM pathogenesis and helps explain the variability in treatment responses. Targeted therapies—especially plasma cell-directed approaches in MGUS-positive patients and immunomodulatory strategies in MGUS-negative cases—are showing encouraging results. Future research should focus on validating omics-based biomarkers to refine diagnosis and support the development of personalized treatment protocols, ultimately improving outcomes in this rare but treatable muscle disorder.
Patients with autoimmune rheumatic diseases (ARDs) and active malignancy pose a unique therapeutic dilemma: controlling autoimmune disease while preserving tumor immune surveillance. This review synthesizes existing evidence, identifies knowledge gaps, and proposes practical considerations for co-management. Emerging studies suggest conventional synthetic and biologic DMARDs, particularly TNF inhibitors, may be safer than previously assumed in patients with cancer, with limited evidence of increased recurrence risk. Some therapies, including abatacept and JAK inhibitors, warrant caution due to potential malignancy signals. Immune checkpoint inhibitors (ICIs) frequently trigger autoimmune flares; however, most are manageable with corticosteroids or DMARDs, and ICI therapy can often continue. Data on concurrent chemotherapy or radiation remain sparse, and treatment decisions require individualized assessment of cancer type, prognosis, and autoimmune disease activity. Overall, shared decision-making integrating rheumatology and oncology perspectives is essential. While TNF inhibitors remain the preferred biologics when needed, therapy should be tailored to cancer stage, autoimmune disease severity, and patient values. Prospective studies are urgently needed to guide immunomodulatory therapy use in this complex, high-risk population.
In the last ten years, many clinical trials evaluating the efficacy of targeted therapies in polymyalgia rheumatica (PMR) have been published. Knowledge in this field is evolving very rapidly and a review of the current evidence available will help physician to follow these many innovations. The interleulin-6 receptor inhibitors – tocilizumab and sarilumab – have been evaluated in several randomized controlled trials. The results are consistent and demonstrate an efficacy of these drugs in early PMR and in relapsing or in glucocorticoid-dependent PMR. Among the therapies targeting lymphocytes, rituximab was efficient in decreasing the PMR-AS and preventing the use of long-term glucocorticoids. On the other hand, abatacept was not proven efficient in early PMR. The other promising drugs are baricitinib and secukinumab. Baricitinib was superior to placebo in a small randomized controlled trial. Secukinumab was Mainly studied in giant cell arteritis, but an international phase 3 trial is ongoing in PMR. In the last national recommendations for the management of PMR published (in France and in Norway), the use of targeted therapies (tocilizumab and sarilumab) has been incorporated in early disease or in relapsing disease.
There is a strong need for tools to monitor disease activity and identify prognostic markers to ensure optimal treatment efficacy in giant cell arteritis (GCA). Vascular ultrasonography (US) is widely implemented in clinical practice to establish diagnosis, and ongoing research continues to investigate its broader utility in GCA. In this review, we explore the role of US in disease activity monitoring and as a prognostic marker. US scoring systems reflecting the extent and severity of vascular inflammation, such as the OMERACT Ultrasonography GCA Score (OGUS), halo count and Southend Halo Score have shown sensitive to change. Positive US findings and worsening in these scores have been reported at relapse. Recent studies indicate that OGUS can predict relapse and the need for glucocorticoid sparing treatment. Sensitivity to change of US is well-supported by the literature but the discriminative and prognostic value of US is not well established. Further studies are needed to define an US relapse, including the degree of vessel wall thickening, the rate at which it develops in parallel to clinical activity, and whether US provides added value in clinical practice. Additional prospective studies are warranted to confirm and evaluate recent prognostic findings of US.
