Long-term effects of early pulse methylprednisolone and intravenous immunoglobulin in patients with dermatomyositis and polymyositis

Abstract

Objective To evaluate the long-term effects of pulse intravenous methylprednisolone (IVMP) or intravenous immunoglobulin (IVIG) administered during the first year of diagnosis in dermatomyositis (DM) and polymyositis (PM) patients. Methods This is a retrospective single-center cohort study of patients with DM/PM followed for up to 4 years from 2001 to 2017. We used Cox regression models to estimate hazard ratios (HRs) and assess the effects of early pulse IVMP or IVIG on 3 outcomes: complete clinical response, corticosteroid discontinuation, and survival. Analysis was adjusted for clinical, laboratory, and treatment covariates. Results A total of 204 patients were included and categorized into 4 initial treatment groups: pulse IVMP (n = 46), pulse IVMP+IVIG (n = 55), IVIG (n = 10), and without IVMP or IVIG (n = 93). The groups of early pulse IVMP and pulse IVMP+IVIG had a higher HR for complete clinical response in the multivariate models (HR = 1.56, 95% CI = 1.05–2.33, p = 0.029; and HR = 1.58, 95% CI = 1.02–2.45, p = 0.041, respectively). Only the group of pulse IVMP+IVIG had a significant association with corticosteroid discontinuation in the multivariate analysis (HR = 1.65, 95% CI = 1.02–2.68, p = 0.043). Early pulse IVMP or IVIG had no impact on mortality. Conclusion Despite having a more severe disease profile, patients with DM/PM submitted to pulse IVMP or pulse IVMP+IVIG during the first year of diagnosis had a higher HR for complete clinical response, whereas the combination of pulse IVMP+IVIG had an association with corticosteroid discontinuation. Prospective long-term studies are warranted to confirm these benefits of early pulse IVMP and IVIG on patients with DM/PM.

Clinical manifestations, outcomes, and antibody profile of Brazilian adult patients with dermatomyositis: a single-center longitudinal study

“…Previous studies have shown an increase in patient survival or higher rates of complete clinical response when glucocorticoids were administered earlier and at high doses [32,33]. In addition, an early approach to targeted treatment with IVMP and/or IVIg was associated with a potential reduction in long-term muscle disability and better outcomes (complete clinical response and discontinuation of corticosteroids) [34,35]. It is important to emphasize that adjuvant therapies with immunomodulators and immunosuppressants, such as leflunomide, seem to be safe and effective for cases of refractory DM with primary cutaneous activity [36].…”

Diagnosis and treatment of interstitial lung disease related to systemic autoimmune myopathies: a narrative review

“…This scheme can be repeated every 21-28 days until a significant improvement is achieved. Despite the limited evidence, it is possible to associate IVIg 2 g/kg divided over three to five days to reduce the risk of infections related to IVMP, achieve faster recovery, decrease the necessity of glucocorticoids, and avoid long-term sequelae of SAMs (52,53). Cyclophosphamide is one of the most studied medications in SAM-ILD, considered as an option in rapidly progressive forms with respiratory failure, although it is usually reserved for ILD refractory to other immunosuppressive drugs due to concerns of serious adverse effects, such as infections and bladder toxicity (26).…”

COVID-19 severity and vaccine breakthrough infections in idiopathic inflammatory myopathies, other systemic autoimmune and inflammatory diseases, and healthy controls: a multicenter cross-sectional study from the COVID-19 Vaccination in Autoimmune Diseases (COVAD) survey

“…The coronavirus disease 2019 (COVID-19) pandemic and the attendant risk of infection have been a serious cause for concern among patients with idiopathic inflammatory myopathies (IIMs) and other systemic autoimmune and inflammatory diseases (SAIDs) [ 1 ]. Patients with IIMs represent a unique vulnerable subgroup, as they typically require long-term treatment with multiple immunosuppressive and immunomodulatory (IS/IM) therapy [ 2 , 3 ], which is associated with impaired host response to infections [ 3 , 4 ]. Patients with IIMs may also have multiple sequelae of their disease (e.g., impaired strength, lung fibrosis) and frequent comorbidities (e.g., cardiovascular disease, obesity, and diabetes) [ 5 ].…”