Abstract
Idiopathic inflammatory myopathies are rare autoimmune disorders characterized by proximal muscle weakness, elevation of muscle enzymes, abnormal electromyogram and imaging studies revealing areas of edema and inflammation. Initial approach to inflammatory myopathies includes steroids and immunosuppressive agents, with most individuals responding satisfactorily to therapy. However, treatment-refractory myopathies prompts clinicians to use second line agents to achieve remission. In this case series, we describe three patients with refractory idiopathic inflammatory myopathies who were treated with tacrolimus (TAC) added to mycophenolate mofetil (MMF) and steroid therapy, who achieved clinical and biochemical remission.
Highlights
Dermatomyositis (DM), inclusion-body myositis (IBM), polymyositis (PM) and necrotizing autoimmune myositis are classified among the idiopathic inflammatory myopathies
Immune cell infiltrates affecting the endomysium is seen with PM, infiltration of the perivascular, perimysial and perifascicular regions is characteristic of DM, basophilic granular inclusion bodies near rimmed vacuoles in IBM and scattered necrotic fiber with macrophages in necrotizing autoimmune myositis [3,4,5,6]
The management of recalcitrant myositis cases includes the use of ACTH, biologics such as rituximab, intravenous immunoglobulin (IVIG) or drug combination of TAC added to mycophenolate mofetil (MMF)
Summary
Dermatomyositis (DM), inclusion-body myositis (IBM), polymyositis (PM) and necrotizing autoimmune myositis are classified among the idiopathic inflammatory myopathies. Eight months after TAC was initiated, weakness improved markedly and labs normalized (CK 117 U/L, LDH 251 U/L). Three months after disease onset, the patient reported improvement of skin lesions on his hands but continued to have significant proximal muscle weakness evidenced by difficulty standing from a seated position and inability to lift his arms above his head. CK (1495 U/L), LDH (495 U/L), and ESR (42 mm/hr) remained elevated; TAC 2 mg/day was added to the existing regimen and PDN was slowly tapered. Eleven months after TAC initiated, muscle weakness had significantly improved and labs normalized (CK 102 U/L and LDH 183 U/L). Patient 3 51-year-old woman with a history of hypertension, hyperlipidemia, and stroke presented with a two-month history of muscle weakness; she reported a remote exposure to statins. Rituximab was added to the regimen of MMF (3 g/day) and prednisone
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