Editorial: A New Classification of Adult Autoimmune Myositis

Senécal, Jean‐Luc; Raynauld, Jean‐Pierre; Troyanov, Yves

doi:10.1002/art.40063

Cited by 83 publications

(72 citation statements)

References 39 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Achieving sustained remission with normal CK levels, normal strength, and no corticosteroids is indeed a goal [4], and remission may be present without full recovery of strength. In a large cohort of treated anti-HMGCR myopathy, strength recovery was often seen with persistent serum CK elevation > 500 UI/L, a sign of ongoing activity [11].…”

Section: Discussionmentioning

confidence: 99%

“…Statin-induced immune-mediated necrotizing myopathy (IMNM) was initially described in patients on statin therapy who, despite statin discontinuation, developed a persistent myopathy, responsive only to immunosuppression [1]. It was later found that autoantibodies to 3-hydroxy-3-methyl-glutaryl-coenzyme A reductase (HMGCR) define that myopathy [2][3][4], that very high serum levels of creatine kinase (CK) and widespread damage on magnetic resonance imaging (MRI) are common [5], that sarcolemmal and capillary membrane attack complex (MAC) deposition are present on muscle biopsy [2,[6][7][8], and that intense immunosuppressive treatment is often needed [2,[7][8][9][10][11][12]. Recently, the pathogenicity of anti-HMGCR was demonstrated [13][14][15], and during the 224th European Neuromuscular Centre (ENMC) International Workshop [16], in the presence of proximal weakness and elevated CK levels, anti-HMGCR myopathy was defined.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Statin-induced anti-HMGCR myopathy: successful therapeutic strategies for corticosteroid-free remission in 55 patients

et al. 2020

Self Cite

View full text Add to dashboard Cite

Objective: To describe successful therapeutic strategies in statin-induced anti-HMGCR myopathy. Methods: Retrospective data from a cohort of 55 patients with statin-induced anti-HMGCR myopathy, sequentially stratified by the presence of proximal weakness, early remission, and corticosteroid and IVIG use at treatment induction, were analyzed for optimal successful induction and maintenance of remission strategies. Results: A total of 14 patients achieved remission with a corticosteroid-free induction strategy (25%). In 41 patients treated with corticosteroids, only 4 patients (10%) failed an initial triple steroid/IVIG/steroid-sparing immunosuppressant (SSI) induction strategy. Delay in treatment initiation was independently associated with lower odds of successful maintenance with immunosuppressant monotherapy (OR 0.92, 95% CI 0.85 to 0.97, P = 0.015). While 22 patients (40%) presented with normal strength, only 9 had normal strength at initiation of treatment.Conclusion: While corticosteroid-free treatment of anti-HMGCR myopathy is now a safe option in selected cases, initial triple steroid/IVIG/SSI was very efficacious in induction. Delays in treatment initiation and, as a corollary, delays in achieving remission decrease the odds of achieving successful maintenance with an SSI alone. Avoiding such delays, most notably in patients with normal strength, may reset the natural history of anti-HMGCR myopathy from a refractory entity to a treatable disease.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Statin-induced anti-HMGCR myopathy: successful therapeutic strategies for corticosteroid-free remission in 55 patients

et al. 2020

Self Cite

View full text Add to dashboard Cite

show abstract

“…However, in the last 15 years, it has been acknowledged that muscle biopsies from some patients with myositis have significant myofiber necrosis and minimal, if any, lymphocytic infiltrates. These patients are now widely recognized to have immune-mediated necrotizing myopathy (IMNM), sometimes referred to as necrotizing autoimmune myopathy (NAM), a distinct type of myositis included in most recent classification schemes for the inflammatory myopathies [2, 3••, 4]. …”

Section: Introductionmentioning

confidence: 99%

Immune-Mediated Necrotizing Myopathy

2018

View full text Add to dashboard Cite

Purpose of Review Immune-mediated necrotizing myopathy (IMNM) is a type of autoimmune myopathy characterized by relatively severe proximal weakness, myofiber necrosis with minimal inflammatory cell infiltrate on muscle biopsy, and infrequent extra-muscular involvement. Here, we will review the characteristics of patients with IMNM. Recent Findings Anti-signal recognition particle (SRP) and anti-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) autoantibodies are closely associated with IMNM and define unique subtypes of patients. Importantly, the new European Neuromuscular Centre criteria recognize anti-SRP myopathy, anti-HMGCR myopathy, and autoantibody-negative IMNM as three distinct subtypes of IMNM. Anti-SRP myopathy patients have more severe muscle involvement, have more common extra-muscular features, and may respond best to immunosuppressive regimens that include rituximab. In contrast, anti-HMGCR myopathy is often associated with statin exposure and intravenous immunoglobulin treatment may be an effective treatment, even as monotherapy. Both anti-SRP and anti-HMGCR myopathy tend to be most severe in younger patients. Furthermore, children with these forms of IMNM may present with dystrophy-like features which are potentially reversible with immunosuppressant treatment. IMNM patients with either autoantibody may experience fatty replacement of muscle soon after disease onset, suggesting that intense and early immunosuppressant therapy may provide the best chance to avoid long-term disability. Summary IMNM is composed of anti-SRP myopathy, anti-HMGCR myopathy, and autoantibody-negative IMNM. Both anti-SRP and anti-HMGCR myopathy can cause severe weakness, especially in younger patients. Anti-SRP myopathy patients tend to have the most severe weakness and most prevalent extra-muscular features. Autoantibody-negative IMNM remains poorly described.

show abstract

“…Major advances in knowledge regarding inflammatory myositis have recognized the limitations of such criteria. Developments of more recent classifications have emerged; one of which emphasized clinicoserological profiles, extra muscular organ involvement and autoantibodies [4]. These newer classifications highlight the heterogeneity of these myositis syndromes and how this has implications for prognosis and response to therapy.…”

Section: Discussionmentioning

confidence: 99%

An unusual presentation of inflammatory myopathy in a patient on natalizumab treatment for multiple sclerosis: A case report and review of myopathological classification of inflammatory myopathy

Zulfiqar¹,

Chua²,

Hassan³

2018

Int. J. Clin. Rheumatol

View full text Add to dashboard Cite

Acquired immune and inflammatory myopathies (IIMs) are traditionally subdivided into dermatomyositis, polymyositis, inclusion body myositis (IBM) and necrotizing autoimmune myopathy (NAM). Our patient is a 38-year old female who had history of Multiple Sclerosis and was on Natalizumab.Almost a year later, she presented with acute proximal bilateral upper extremity weakness with elevated CPK. She was initially labeled as having 'viral myositis'. Her subsequent two admissions demonstrated worsening of muscle weakness with dysphagia which was initially unresponsive to high dose of steroids. She had an EMG which showed irritative myopathy and a muscle biopsy which showed IMPP. She eventually responded to pulse dose of steroids and IVIG and Natalizumab was held. The temporal relationship between the development of inflammatory myopathy in our patient and subsequent improvement in her course after discontinuation of the drug suggests that natalizumab may have played a role in our patient's disease and warrants further investigation and vigilance. In addition to the case report, we hope to elaborate on the myopathological descriptions increasingly being used to describe the IIMs and to explore the potential link between natalizumab and the onset of IIM in our patient.

show abstract

Editorial: A New Classification of Adult Autoimmune Myositis

Cited by 83 publications

References 39 publications

Statin-induced anti-HMGCR myopathy: successful therapeutic strategies for corticosteroid-free remission in 55 patients

Statin-induced anti-HMGCR myopathy: successful therapeutic strategies for corticosteroid-free remission in 55 patients

Immune-Mediated Necrotizing Myopathy

An unusual presentation of inflammatory myopathy in a patient on natalizumab treatment for multiple sclerosis: A case report and review of myopathological classification of inflammatory myopathy

Contact Info

Product

Resources

About