Juvenile dermatomyositis: treatment with intravenous gammaglobulin

Collet, K.; Dalac, S.; Maerens, B.; Courtois, Justine; Izac, M; Lambert, D.

doi:10.1111/j.1365-2133.1994.tb02906.x

Cited by 47 publications

(15 citation statements)

References 6 publications

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“…IVIG has been used in the treatment of JDM since the 1980s as reported in case reports and small case series. The majority of reported patients experienced improvement in skin disease and muscle strength, and the use of IVIG also reduced the cumulative glucocorticoid dose in most patients [85][86][87][88]. In 2011 a large retrospective study including 78 JDM patients confirmed the efficacy of IVIG in controlling JDM disease activity, particularly for steroid resistant patients [89].…”

Section: Intravenous Immunoglobulins (Ivig)mentioning

confidence: 99%

Treatment of Juvenile Dermatomyositis: An Update

Papadopoulou

Wedderburn

2017

Pediatr Drugs

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Abstract:The Idiopathic Inflammatory Myopathies of childhood consist a heterogeneous group of autoimmune diseases characterised by proximal muscle weakness and pathognomonic skin rashes. The overall prognosis of juvenile myositis has improved significantly over recent years, but the long-term outcome differs substantially from patient to patient suggestive of distinct clinical phenotypes with variable responses to treatment. High doses of corticosteroids remain the cornerstone of therapy along with other immunosuppressant therapies depending on disease severity and response. The advent of biological drugs has revolutionised the management of various paediatric rheumatologic diseases, including inflammatory myopathies. There are few data from randomised controlled trials to guide management decisions, thus several algorithms for the treatment of juvenile myositis have been developed using international expert opinion. The general treatment goals now include elimination of active disease and normalization of physical function, so as to preserve normal growth and development, and to prevent long-term damage and deformities. This review summarizes the newer and possible future therapies of juvenile inflammatory myopathies, including evidence supporting their efficacy and safety.

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Section: Intravenous Immunoglobulins (Ivig)mentioning

confidence: 99%

Treatment of Juvenile Dermatomyositis: An Update

Papadopoulou

Wedderburn

2017

Pediatr Drugs

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“…[13][14][15][16][17][18][19][20][21][22][23][24] In 1993, Dalakas et al 25 reported the efficacy of IVIG in a doubleblind, placebo-controlled study for DM. We conducted a prospective study to assess the efficacy of IVIG for steroidresistant PM/DM in Japan.…”

Section: Introductionmentioning

confidence: 99%

Prospective study of high-dose intravenous immunoglobulin for the treatment of steroid-resistant polymyositis and dermatomyositis

Hara

Kinoshita

Saito

et al. 2003

Modern Rheumatology

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High-dose intravenous immunoglobulin (IVIG) therapy has been effective in treating many autoimmune and systemic inflammatory diseases. In the present prospective study, we evaluated the efficacy of IVIG for patients with polymyositis (PM) and dermatomyositis (DM) refractory to treatment with high-dose corticosteroids. PM/DM was defined as steroid-resistant when the muscle strength of a patient did not improve despite the administration of more than 50 mg prednisolone per day for more than 4 weeks. A total of 12 patients with biopsy-proven, steroidresistant PM/DM received one infusion of polyethylene glycol-treated human IgG at a dose of 0.4 g per kg per day for five successive days. Three of the patients received a second infusion. All patients were followed for up to 3 months after the infusion. Finally, 8 patients (6 PM and 2 DM; 5 men and 3 women) aged 29-67 years (mean 48 years) were analyzed. Their clinical response was assessed by

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“…Gastrointestinal disturbance, anorexia, fatigue, stomatitis, and alopecia are the most commonly reported adverse effects. [57][58][59][60] Additionally, a case of adult-onset amyopathic DM refractory to corticosteroid therapy responded to low-dose (10% of the standard dose of 1 mg kg day) IVIG. Renal impairment and pulmonary toxicity are also reported.…”

Section: Other Systemic Therapiesmentioning

confidence: 99%

Juvenile-Onset Clinically Amyopathic Dermatomyositis

Walling¹,

Gerami

Sontheimer

2010

Pediatric Drugs

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Juvenile-onset amyopathic dermatomyositis is an uncommon variant of juvenile-onset dermatomyositis (JDM), characterized by the hallmark cutaneous features of dermatomyositis for at least 6 months without clinical or laboratory evidence of muscle disease. Cutaneous calcinosis, vasculopathy, and interstitial lung disease frequently complicate the course of classic JDM (typical JDM with myositis) but are infrequent in amyopathic JDM. Recent literature suggests that approximately 75% of amyopathic JDM patients will remain free from muscle disease after years of follow-up, while approximately 25% of patients will evolve to having classic JDM. No clinical, laboratory, or ancillary parameters have been found to be predictive for this transition to muscle disease. Treatment of the cutaneous disease of amyopathic JDM centers on photoprotection and topical therapies directed against inflammation. Oral antimalarials are effective for cutaneous disease not adequately controlled with topical care. Systemic corticosteroids, while central to the treatment of classic JDM, are controversial in the treatment of amyopathic JDM. Randomized controlled trials are not available to guide the management of this disease. Proponents for early aggressive systemic corticosteroid therapy for amyopathic JDM advocate that this intervention may decrease the likelihood of progression to classic JDM, and/or prevent disease-specific complications of JDM such as calcinosis. Opponents of early intervention with systemic corticosteroids favor expectant management directed toward controlling skin disease, citing the predictable adverse effects of systemic corticosteroids in the face of uncertain benefit. Other therapeutic options for severe and recalcitrant cutaneous disease, including methotrexate, intravenous immunoglobulin, and rituximab, are reviewed, as are treatment options for calcinosis cutis. In weighing the available evidence, the authors conclude that early aggressive treatment of amyopathic JDM with systemic immunosuppressant agents should be avoided in most cases as the risk of these medications will outweigh the measurable benefit. The reported literature suggests a good prognosis for amyopathic JDM. Ongoing clinical follow-up is recommended in all cases to allow early detection of subtle signs of muscle disease.

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Juvenile dermatomyositis: treatment with intravenous gammaglobulin

Cited by 47 publications

References 6 publications

Treatment of Juvenile Dermatomyositis: An Update

Treatment of Juvenile Dermatomyositis: An Update

Prospective study of high-dose intravenous immunoglobulin for the treatment of steroid-resistant polymyositis and dermatomyositis

Juvenile-Onset Clinically Amyopathic Dermatomyositis

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