Intravenous Immunoglobulin in Idiopathic Inflammatory Myopathies: a Practical Guide for Clinical Use

Gandiga, Prateek C.; Ghetie, Daniela; Anderson, Elizabeth; Aggrawal, Rohit

doi:10.1007/s11926-023-01105-w

Cited by 9 publications

(2 citation statements)

References 104 publications

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“…The positive response to IVIG indicated an effective management strategy for her muscle inflammation. IVIG is a safe and effective treatment against both muscular and extra-muscular manifestations of myositis [ 4 ]. Additionally, dietary modifications and weight management were crucial for her overall health and to reduce systemic inflammation.…”

Section: Discussionmentioning

confidence: 99%

Upper Gastrointestinal (GI) Manifestations of Inflammatory Myositis: A Tale of Two Patients

Hakobyan,

Acob,

Aleksanyan

et al. 2024

Cureus

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Myositis is a group of rare autoimmune disorders characterized by chronic inflammation of skeletal muscles that leads to a hallmark triad of muscle weakness, fatigue, and myalgia. Extra-muscular manifestations are sometimes seen and involve various organ systems, including the gastrointestinal (GI) tract. In this case series, two patients with polymyositis (PM) and dermatomyositis (DM), both of whom developed dysphagia as a complication of myositis, are discussed. Case 1 was a female with a known history of biopsy-proven dermatomyositis who presented with progressive peripheral edema and weakness affecting all extremities. Concurrently, she displayed symptoms of pneumonia and dysphagia associated with frequent spontaneous or self-induced vomiting to alleviate retrosternal discomfort. Esophagogastroduodenoscopy (EGD) revealed esophageal dilatation and an absence of a contractile response, consistent with myositis. Treatment comprised intravenous immunoglobulin (IVIG), mycophenolate, and lifestyle modifications, including dietary adjustments and maintaining an upright position postprandial. The second case was a female with muscle weakness and dysphagia. Video-fluoroscopic swallow assessment was significant for pharyngeal dysfunction without a sensory response to penetrated material, and the patient was at high risk of aspiration with any oral intake. The presence of pharyngeal dysfunction and dysphagia prompted treatment with IVIG, mycophenolate, and percutaneous endoscopic gastrostomy (PEG) tube placement. These cases have highlighted the upper GI complications observed in patients with myositis, accentuating the necessity for a personalized treatment approach. Timely intervention has shown promising results in symptomatic relief and improving patient outcomes. This emphasizes the importance of a multidisciplinary approach when addressing myositis-related upper GI manifestations.

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Section: Discussionmentioning

confidence: 99%

Upper Gastrointestinal (GI) Manifestations of Inflammatory Myositis: A Tale of Two Patients

Hakobyan,

Acob,

Aleksanyan

et al. 2024

Cureus

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“…Resolution of the aberrant immunopathological parameters, including interception of complement activation products and downregulation of T cells, ICAM-I, VCAM, TGF-beta and MHC-I molecules, was also noted. 3,4 There is also evidence that IVIG is efficacious in calcinosis in case reports. [5][6][7] In 2015, a retrospective study found that 5 out of 8 DM treated with IVIG experienced improvement.…”

Section: Discussionmentioning

confidence: 99%

Subcutaneous immunoglobulin for the treatment of calcinosis associated with juvenile dermatomyositis

Rodríguez‐Lozano,

Mata‐Aguilera,

Rivas‐Larrauri

et al. 2024

Int J of Rheum Dis

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Juvenile dermatomyositis (JDM) is an autoimmune disorder primarily involving the skin and muscles. Calcinosis, a complication of JDM, presents significant treatment challenges and is associated with long-term morbidity. It involves the abnormal deposition of insoluble calcium salts in tissues, including skin, subcutaneous tissue, and muscle. 1,2 Dystrophic calcification occurs at sites of injured tissue in the setting of normal serum calcium and phosphorus levels and occur in association with the underlying disease. 1,2 Currently, there are no widely effective medical therapies for calcinosis, underscoring a significant gap in treatment options. We report on two cases of patients with refractory JDM-associated calcinosis that showed remarkable response to subcutaneous immunoglobulin (SCIG), highlighting a potential therapeutic advancement.

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Benefits of Early Versus Late Initiation of Intravenous Immunoglobulin in the Treatment of Patients With Anti–3‐Hydroxy‐3‐Methylglutaryl‐Coenzyme A Reductase Immune‐Mediated Necrotizing Myopathy

Sharf,

Do,

Ghetie

et al. 2024

Arthritis Care & Research

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Background/ObjectiveNo clinical trials have been conducted to establish optimal and effective treatment in Immune‐Mediated Necrotizing Myopathy (IMNM), which can have a refractory course with increased morbidity from permanent muscle damage especially in cases with delay in diagnosis and treatment. A subset of autoimmune necrotizing myopathy is associated with antibodies against 3‐hydroxy‐3‐methylglutaryl‐coenzyme A reductase (HMGCR). Treatment involves withdrawing statins and using a combination of immunosuppressant and immunomodulatory treatment. Our study aims to provide longitudinally collected data on outcomes of early versus late initiation of intravenous immunoglobulin (IVIG) using our myositis center cohort of patients with Anti‐HMGCR IMNM.MethodsWe conducted a retrospective chart review of 31 adult patients of the Oregon Health & Science University (OHSU) Myositis Center who were diagnosed with anti HMGCR‐IMNM from September 2016 through October 2022 and reviewed physical exam, serologic lab data and their treatment including prednisone use as well as treatment response at the 0‐months (the evaluation immediately prior to IVIG initiation), 3‐months, 6‐months and 12‐months on treatment. We divided this cohort into those who received IVIG at or prior to 6 months after receiving the diagnosis of Anti‐HMGCR IMNM and refer this as the “Non‐Delayed” cohort, and those who received IVIG after 6 months following their diagnosis, which we referred to as “Delayed” cohort. Diagnosis of Anti‐HMGCR IMNM was defined as per the 2016 ENMC criteria as having all 3 of: elevated serum CK, proximal muscle weakness, and anti‐HMGCR antibodies. We evaluated the response to treatment by using a limited total improvement score (TIS) as per 2016 ACR/EULAR myositis response criteria.ResultsAmongst the 31 total patients, 19 were included within the Non‐Delayed cohort, and 12 within the Delayed cohort. The two cohorts had a comparable amount of time between onset of symptoms and diagnosis; however, the Delayed cohort had a significantly longer time between diagnosis and IVIG treatment (p‐value <0.001). At disease onset, cohorts had a comparable serum CK (p‐value >0.999), but Delayed patients had an expected lower serum CK (p‐value 0.016) at the 0‐month time point. At the 0‐month time point, 9 (47%) of the Non‐Delayed patients required the use of a walker or wheelchair, while 8 (66%) of the Delayed cohort did. Non‐Delayed patients demonstrated significant improvement in MMT8 at the 12‐months intervals (p‐value <0.001). Average serum CK values of all patients measured at the 3‐month, 6‐month, and 12‐month did not significantly differ between the Non‐Delayed and Delayed group. TIS improved more in the Non‐Delayed group than in the Delayed group (p‐value 0.002 at 3‐months, 0.019 at 6‐months and 0.001 at 12‐months). Seven patients in the delayed group had permanent residual muscle weakness requiring walker or wheelchair use at 12‐months while none of the patients in the Non‐Delayed group did.ConclusionThough our results have limitations, they contribute to a growing body of evidence which suggests that IVIG may prove to be a valuable addition to an early and aggressive induction regimen in patients afflicted by anti HMGCR IMNM. Delayed in IVIG treatment may lead to the development of permanent residual weakness and long‐term disability.

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Intravenous Immunoglobulin in Idiopathic Inflammatory Myopathies: a Practical Guide for Clinical Use

Cited by 9 publications

References 104 publications

Upper Gastrointestinal (GI) Manifestations of Inflammatory Myositis: A Tale of Two Patients

Upper Gastrointestinal (GI) Manifestations of Inflammatory Myositis: A Tale of Two Patients

Subcutaneous immunoglobulin for the treatment of calcinosis associated with juvenile dermatomyositis

Benefits of Early Versus Late Initiation of Intravenous Immunoglobulin in the Treatment of Patients With Anti–3‐Hydroxy‐3‐Methylglutaryl‐Coenzyme A Reductase Immune‐Mediated Necrotizing Myopathy

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