Corticosteroid Monotherapy Is Usually Insufficient Treatment for Idiopathic Inflammatory Myopathy

Mathur, Tanisha; Manadan, Augustine; Thiagarajan, Saravanan; Hota, Bala; Block, Joel A.

doi:10.1097/mjt.0b013e3182987983

Cited by 10 publications

(4 citation statements)

References 16 publications

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“…Nine patients were classified as amyopathic DM. The details of the PM and DM patients have already been described [10]. Four patients were classified as IBM and represent the primary cohort.…”

Section: Resultsmentioning

confidence: 99%

The Low Prevalence of Inclusion Body Myositis in an Outpatient Rheumatology Myositis Cohort

Edigin

Hassan²,

Mathur

et al. 2020

Cureus

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Introduction Sporadic inclusion body myositis (IBM) is a rare type of myopathy of unknown etiology typified by the presence of distinctive muscle fiber inclusions. IBM belongs to a larger family of conditions called the idiopathic inflammatory myopathies (IIM). This study seeks to compare the prevalence, clinical manifestations, and disease course of IBM patients to a cohort of polymyositis (PM) and dermatomyositis (DM) patients in an outpatient rheumatology myositis cohort. Methods We conducted a retrospective chart review of all adult patients attending rheumatology clinics at the Cook County Hospital from 2006 to 2011 with the International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9) diagnoses of idiopathic inflammatory myopathies (IIM). Data collected included patient demographics, serial muscle strength testing, serial creatine kinase (CK), muscle biopsies, and immunosuppressive therapies received. Results We identified 112 patients with IIM with the following breakdown: 66-dermatomyositis, 42polymyositis, and four-IBM. These four patients represent the primary cohort. They had a mean age of 67 years (range 62-74), the mean follow-up period of 15 months (range 3-47), mean initial muscle strength in the weakest group of 3.75 (range 2-5, standard deviation [SD]=1.3), mean initial CK of 1,968 U/L (range 313-4,795, SD=1,970), mean final muscle strength in the weakest group of 3.75 (range 3-4, SD=0.5), mean final CK of 1,326 U/L (range 213-2,943, SD=1,200). Conclusions Our IBM patients had many features similar to other described IBM cohorts, including older age, male predominance, slow progression (stable CK and muscle strength), and lack of response to immunosuppressive therapy. Interestingly, we found a low prevalence of IBM in our outpatient rheumatology IIM cohort.

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Section: Resultsmentioning

confidence: 99%

The Low Prevalence of Inclusion Body Myositis in an Outpatient Rheumatology Myositis Cohort

Edigin

Hassan²,

Mathur

et al. 2020

Cureus

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“…Although it is considered a first-line drug, studies have shown that more than half of the patients fail to obtain a complete clinical response to CG in monotherapy and adding other immunosuppressive drugs in often necessary (B) [95,96].…”

Section: What Initial Dose Of Gc Should Be Used and For How Long In Pmentioning

confidence: 99%

Guidelines of the Brazilian Society of Rheumatology for the treatment of systemic autoimmune myopathies

et al. 2019

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Background: Recommendations of the Myopathy Committee of the Brazilian Society of Rheumatology for the management and therapy of systemic autoimmune myopathies (SAM). Main body: The review of the literature was done in the search for the Medline (PubMed), Embase and Cochrane databases including studies published until June 2018. The Prisma was used for the systematic review and the articles were evaluated according to the levels of Oxford evidence. Ten recommendations were developed addressing the management and therapy of systemic autoimmune myopathies. Conclusions: Robust data to guide the therapeutic process are scarce. Although not proven effective in controlled clinical trials, glucocorticoid represents first-line drugs in the treatment of SAM. Intravenous immunoglobulin is considered in induction for refractory cases of SAM or when immunosuppressive drugs are contra-indicated. Consideration should be given to the early introduction of immunosuppressive drugs. There is no specific period determined for the suspension of glucocorticoid and immunosuppressive drugs when individually evaluating patients with SAM. A key component for treatment in an early rehabilitation program is the inclusion of strengthbuilding and aerobic exercises, in addition to a rigorous evaluation of these activities for remission of disease and the education of the patient and his/her caregivers.

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“…In general, patients respond well to standard immunosuppressive therapies, such as glucocorticosteroids and intravenous immunoglobulins ( 5 – 7 ). However, a large number of patients are resistant to current therapies, and treatment of idiopathic inflammatory myopathies still remains a formidable challenge ( 8 ).…”

Section: Introductionmentioning

confidence: 99%

Effect of butylphthalide intervention on experimental autoimmune myositis in guinea pigs

et al. 2017

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Idiopathic inflammatory myopathies are a group of rare muscular diseases that are characterized by acute, subacute or chronic proximal and symmetric muscle weakness, muscle fiber necrosis and infiltration of inflammatory cells, particularly activated CD8+ cytotoxic T cells and phagocytes. 3-n-butylphthalide (NBP) protects mitochondria and reduces the inflammatory response in multiple disease models. In myositis, it has remained elusive whether NBP can protect muscle cells from muscle fiber injury. Experimental autoimmune myositis (EAM) was induced in a total of 40 guinea pigs by myosin immunization. After 4 weeks, low- or high-dose NBP solution was intraperitoneally injected. Saline solution was used as a negative control. After 10 days, the clinical manifestations were assessed by determining rodent grasping power, histopathological changes, Ca2+-adenosinetriphosphatase (ATPase) activity by an ATPase kit, and mRNA expression of interferon (IFN)-γ, retinoic acid receptor-related orphan nuclear receptor (ROR)γt and forkhead box (Fox) p3 in muscle tissue by reverse-transcription quantitative polymerase chain reaction analysis. It was demonstrated that NBP improved the myodynamia of guinea pigs with EAM and reduced the pathological inflammatory cell infiltration in a dose-dependent manner. NBP improved the Ca2+-ATPase activity of the muscle mitochondrial membrane and muscle plasma membrane in animals with EAM. It also reduced the mRNA expression of IFN-γ and RORγt, and significantly increased the mRNA expression of Foxp3 in muscle tissue. These results provided a basis for the consideration of NBP as a novel agent for the treatment of myositis and other muscular diseases associated with autoimmunity and inflammation.

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Corticosteroid Monotherapy Is Usually Insufficient Treatment for Idiopathic Inflammatory Myopathy

Cited by 10 publications

References 16 publications

The Low Prevalence of Inclusion Body Myositis in an Outpatient Rheumatology Myositis Cohort

The Low Prevalence of Inclusion Body Myositis in an Outpatient Rheumatology Myositis Cohort

Guidelines of the Brazilian Society of Rheumatology for the treatment of systemic autoimmune myopathies

Effect of butylphthalide intervention on experimental autoimmune myositis in guinea pigs

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