Pulmonary Complications of Inflammatory Myopathy

Corticosteroids in Myositis and Scleroderma

“…Interstitial lung disease (ILD) can affect 5–46% of patients with PM and up to 70–100% of those with anti-synthetase syndrome. 9 This syndrome consists of fever, mechanic’s hands, Raynaud phenomenon, myositis, ILD and arthritis, and is frequently associated with the anti-Jo-1 antibody. 1 Finally, patients with PM have a 2 fold increase in the incidence of malignancy compared to the general population within the first five years of disease onset.…”

“…1,6,7 Initial treatment usually consists of oral prednisone (0.5–1.5mg/kg/day), with IV treatments (500–1000mg/day for 3–5 days) reserved for severe cutaneous disease or muscle weakness, dysphagia, respiratory muscle involvement, and ILD. 1,8,9 One RCT comparing monthly oral pulse dexamethasone (six cycles of 40mg/day for four consecutive days at 28-day intervals) with daily prednisolone (70 to 90mg/day) for 28 days followed by a slow taper, did not demonstrate improved efficacy with pulse dosing, but did show fewer adverse effects. 14 High dose (60–80 mg/day) CS treatment is usually continued for 2–4 weeks with slow tapers extending 3–6 months.…”

“…1,6 In patients with severe disease and ILD, or in patients with co-morbidities such as osteoporosis and diabetes combination therapy with another immunosuppressant is initiated immediately. 9 …”

“…8 Additionally, cyclosporine has been shown to be as effective as MTX for myositis, with favorable outcomes for treatment of ILD. 1,9 Mycophenolate mofetil (MMF) has also shown efficacy and tolerability, and is generally considered the CS-sparing drug of choice when PM is complicated by ILD. 1,9 A growing body of evidence supports the use of intravenous immunoglobulin (IVIG) for more severe disease presentations, including dysphagia, respiratory muscle involvement, and refractory cases.…”

“…1,9 Mycophenolate mofetil (MMF) has also shown efficacy and tolerability, and is generally considered the CS-sparing drug of choice when PM is complicated by ILD. 1,9 A growing body of evidence supports the use of intravenous immunoglobulin (IVIG) for more severe disease presentations, including dysphagia, respiratory muscle involvement, and refractory cases. 1 Cyclophosphamide (CYC) may be required for recalcitrant cases and patients with significant cardiac or pulmonary disease.…”