Intravenous immunoglobulin in juvenile dermatomyositis--four year review of nine cases.

Sansome, Alison; Dubowitz, Victor

doi:10.1136/adc.72.1.25

Cited by 109 publications

(58 citation statements)

References 20 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: 97%

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: 97%

Treatment of Juvenile Dermatomyositis: An Update

Papadopoulou

Wedderburn

2017

Pediatr Drugs

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“…Other reduction in ICAM-1 expression in capillaries, and a return of endomysial capillary numbers to normal studies have involved smaller numbers of patients. 12,49,61,73,91,93,105 Our own experience with IVIg is in 5 patients who had repeat muscle biopsies after completion of the course of therapy. The authors summarized in Table 4.…”

Section: Steroid-resistant Casesmentioning

confidence: 97%

“…There are occasional reports of improvement immunosuppression with azathioprine, methotrexate, 79 cyclosporin, or cyclophosphamide, or intravein the myositis following plasmapheresis or removal of the primary malignancy. 67 nous immunoglobulin 93 therapy, all of which have been found to be effective in small uncontrolled tri-…”

Section: Steroid-resistant Casesmentioning

confidence: 99%

Treatment of inflammatory myopathies

Mastaglia¹,

Phillips²,

Zilko³

1997

Muscle Nerve

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The treatment of the immune‐mediated inflammatory myopathies remains largely empirical. Corticosteroids are usually effective in polymyositis and dermatomyositis but may need to be combined with methotrexate or azathioprine in some patients. Intravenous immunoglobulin (IVIg) is effective as add‐on therapy in some patients not adequately controlled with steroids or immunosuppressive agents, but further controlled trials of IVIg are necessary to define the indications and optimal dose regimens. Cyclophosphamide, cyclosporin, or chlorambucil may be effective in patients with refractory polymyositis or dermatomyositis. Low‐dose whole body or lymphoid irradiation is a last option in severely disabled patients resistant to all other treatments. As a small proportion of patients with inclusion body myositis respond to corticosteroid or immunosuppressive therapy, a 3–6‐month trial of such therapy is justified in this condition. More specific immunotherapy for these disorders awaits identification of the target antigens and further clarification of the immunopathogenetic mechanisms. © 1997 John Wiley & Sons, Inc. Muscle Nerve 20:651–664, 1997.

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“…Det finnes også en retrospektiv undersøkelse (24) og kasusrapporter (25,26) som tyder på at slik behandling, sammen med prednisolon, har effekt.…”

Section: Inflammatoriske Myopatierunclassified