Cyclophosphamide in Severe Polymyositis

Bombardieri, Stefano; Hughes, G. R. V.; Neri, R; Bravo, Pilar; Bono, Luisa

doi:10.1016/s0140-6736(89)92416-1

Cited by 45 publications

(13 citation statements)

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“…Cyclophosphamide has been ineffective in our hands 1691 in spite of occasional promising results reported by others [70]. The drug may be helpful, however, in a subset of patients who have interstitial lung disease.…”

Section: Prednisone Faiiures and Nonsteroidd Immunosuppressive Therapiesmentioning

confidence: 80%

Immunopathogenesis of inflammatory myopathies

Dalakas

1995

Ann Neurol.

137

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Immune-mediated mechanisms appear to play a primary role in the pathogenesis of polymyositis (PM) and dermatomyositis (DM). The serum of patients with active DM has high levels of circulating complement fragments C3b, C4b, and C5b-9 membranolytic attack complex (MAC) and demonstrates a very high C3 uptake in an vitro assay system. The MAC and the immune complex-specific C3bNEO fragment are deposited on the endomysial capillaries early in the disease and lead sequentially to loss of capillaries, muscle ischemia, muscle fiber necrosis, and perifascicular atrophy. In contrast, in PM the muscle fiber injury is initiated by sensitized CD8+ cytotoxic T cells that recognize heretofore unknown and probably endogenous muscle antigens in the context of major histocompatibility complex (MHC) class I expression. A restricted (oligoclonal) pattern of T-cell receptor with prominence of Va1, Vb6, and Vb15 genes is noted within the endomysial infiltrates suggesting that the T-cell response is antigen driven. In both PM and DM, intercellular adhesion molecule (ICAM)-1 and vascular cell adhesion molecule (VCAM)-1 are upregulated in the endomysial endothelial cells and function as ligands for the leukocyte integrins leukocyte function-associated antigen (LFA)-1 and very late activating antigen (VLA)-4, allowing activated lymphocytes to adhere to the endothelial cells and migrate to the muscle fibers. Among viruses, only the retroviruses human immunodeficiency virus (HIV) and human T-cell lymphotropic virus (HTLV)-1 have been convincingly shown to trigger PM, which is mediated by nonviral-specific, cytotoxic CD8+ cells. The treatment of inflammatory myopathies remains empirical. Many patients respond to steroids to some degree and for some period of time. Azathioprine, methotrexate, cyclosporine, cyclophosphamide, and plasmapheresis can be of mild to moderate benefit. High-dose intravenous immunoglobulin (IVIg) is a promising therapeutic modality for some patients resistant to therapies. In a controlled study, IVIg was effective in DM not only in improving the clinical symptoms but also in reversing the underlying immunopathology. The role of IVIg in PM and IBM is under study in control trials.

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Section: Prednisone Faiiures and Nonsteroidd Immunosuppressive Therapiesmentioning

confidence: 80%

Immunopathogenesis of inflammatory myopathies

Dalakas

1995

Ann Neurol.

137

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“…Cyclophosphamide may be useful in treating myositis 16. However, because of its severe side effects, particularly bone marrow suppression, infection and induction of malignancy, we considered that its utility would be limited for patients with myositis who do not have interstitial pneumonia or vasculitis.…”

Section: Discussionmentioning

confidence: 99%

Successful treatment using rituximab in a patient with refractory polymyositis complicated by scleroderma renal crisis

et al. 2017

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Corticosteroids are the first-line treatment for patients with inflammatory myopathies. Myositis can be a clinical feature of scleroderma (polymyositis-scleroderma overlap syndrome), and treatment of this syndrome is a challenge for clinicians because moderate to high doses of corticosteroids are considered a risk factor for development of acute kidney injury in affected patients. We report here the case of a 56-year-old woman with scleroderma who developed polymyositis and was successfully treated with rituximab. Initial treatment of the polymyositis with prednisolone 40 mg/day was rapidly tapered to 2.5 mg/day due to development of scleroderma renal crisis, for which four weekly infusions of rituximab (500 mg; off-label) were given. She responded well to rituximab in addition to prednisolone 2.5 mg/day. Rituximab may improve inflammatory myopathies, even in cases where high-dose corticosteroids should be avoided due to complications. Rituximab should be considered as a treatment option in cases of refractory polymyositis.

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“…After the introduction of IVCY therapy, muscle strength was significantly improved, and thereafter low-dose methotrexate normalized the serum CKlevel. Based on successful outcome after IVCYtherapy in patients with lupus nephritis (20), IVCY therapy has also been challenged in patients with polymyositis (16,(21)(22)(23)(24)(25); a summary of published reports is presented in relatively large-scale controlled study should be undertaken. Recently, low-dose methotrexate has almost becomean established treatment in patients with active rheumatoid arthritis (5-10).…”

Section: Discussionmentioning

confidence: 99%

Successful Treatment of Refractory Polymyositis with Pulse Intravenous Cyclophosphamide and Low-dose Weekly Oral Methotrexate Therapy.

et al. 1993

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A 36-year-old womangradually developed dysphagia and muscle weakness of the lower extremities. Diagnosis of polymyositis was given from elevation of serumcreatine kinase and pathological findings of a muscle biopsy. Despite oral prediiisolone and intravenous pulse methylprednisolone therapy, her muscle weakness persisted, and then pulse intravenous cyclophosphamide (IVCY) therapy was initiated and repeated five times in total, which resulted in significant improvement in muscle strength. Thereafter, weekly administration of methotrexate at low dosage further normalized the serum creatine kinase level. Wemay conclude that IVCYand low-dose weekly methotrexate together could be an alternative in refractory polymyositis. (Internal Medicine 32: 749-752 1993)

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Cyclophosphamide in Severe Polymyositis

Cited by 45 publications

References 0 publications

Immunopathogenesis of inflammatory myopathies

Immunopathogenesis of inflammatory myopathies

Successful treatment using rituximab in a patient with refractory polymyositis complicated by scleroderma renal crisis

Successful Treatment of Refractory Polymyositis with Pulse Intravenous Cyclophosphamide and Low-dose Weekly Oral Methotrexate Therapy.

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