Mycophenolate mofetil treatment with or without a calcineurin inhibitor in resistant inflammatory myopathy

Abstract

To evaluate the efficacy and tolerability of mycophenolate mofetil (MMF) with or without calcineurin inhibitors (CNIs) in patients with inflammatory myopathy taking prednisolone, but refractory to conventional immunosuppressive therapy. The records of patients with inflammatory myopathy who had previously failed treatment with at least one immunosuppressant were retrospectively evaluated. We selected patients treated with MMF and divided them into two groups depending on whether or not there was concomitant use of CNIs. We investigated the efficacy by changes in creatine kinase (CK) levels, forced vital capacity (%FVC), prednisolone dose, and high-resolution computed tomography (HRCT) findings. Interstitial lung disease (ILD) progression was defined by more than 10% decline of %FVC from baseline. We identified 19 patients on MMF treatment. There were seven (36.8%) patients on MMF and CNIs, including five on cyclosporine and two on tacrolimus. At baseline, no significant difference was seen in the prevalence of ILD between patients taking or not taking CNIs (85.7% vs. 75.0%, P = 0.68). Improvement in CK was seen in patients treated with CNIs (P = 0.04) but not in those without (P = 0.39). No significant improvement in %FVC and HRCT findings were found in patients with ILD in either group, and there were no differences in death or ILD progression. The combination of CNIs and MMF might be more effective for decreasing CK levels than MMF alone. Neither treatment arm had a beneficial effect on ILD over a variable observation period.

Macrophagic myofasciitis: an atypical presentation for a rare disease with a challenging approach

“…The decision to use mycophenolate mofetil and Tac in this patient was based on evidence supporting their efficacy in other muscle inflammatory diseases and strengthens the potential role of these drugs in this condition. The concurrent use of Tac with mycophenolate has been shown to increase plasma levels of active mycophenolic acid, allowing a reduction in the mycophenolate dose, while achieving efficacy, and ultimately a reduction in adverse effects [34,35].…”

Novel Therapeutic Approaches in Connective Tissue Disease-Associated Interstitial Lung Disease

“…Data for both mycophenolate and azathioprine in IIM-ILD come from case series and retrospective studies. [74][75][76][77] One of the larger retrospective studies included 110 patients treated with azathioprine (n ¼ 66) or mycophenolate (n ¼ 44) at a single center in the United States. The baseline FVC% and DLCO% predicted were lower in the azathioprine group.…”

Hydroxychloroquine-induced Retinal Toxicity

“…In SLE, hydroxychloroquine monotherapy is often highly effective (Hanaoka et al, 2019), and given that the drug is systemically well tolerated, patients often continue the drug for many years (Wang et al, 1999). Hydroxychloroquine has proven efficacious in reducing the number of disease flares, preventing endorgan damage, permitting a reduction in systemic corticosteroid dose, and exerting an anti-thrombotic effect through a reduction in serum anti-phospholipid antibodies (Alarcon et al, 2007;Pons-Estel et al, 2009;Willis et al, 2012;Mok et al, 2016;Ponticelli and Moroni, 2017;Hsu et al, 2018).…”