SESSION TITLE: Diffuse Lung Disease Student/Resident Case Report Posters
SESSION TYPE: Student/Resident Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: Anti-synthetase syndrome (ASS) is an autoimmune condition characterized by inflammatory myositis and interstitial lung disease. Our case of refractory ASS suggests that administering pulse steroids may enhance standard treatment.
CASE PRESENTATION: A 43 year-old woman presented with cough, arthralgias and dyspnea progressive over six months. No fever, rash or muscle weakness. Physical exam revealed hypoxia, bibasilar crackles, and mechanic’s hands. Autoimmune serologies including ANA, anti-dsDNA, anti-smith, rheumatoid factor, anti-SSA, anti-SSB, anti-SCl-70, and anti-centromere were negative. Anti-Jo1 antibody was positive (6.7 index). Creatinine kinase (200 U/L) and aldolase (11.9 U/L) were elevated. Chest CT revealed fibrotic changes, consolidation without honeycombing in lower lobes and ground glass opacities (GGO) in upper lobes. Pulmonary function testing demonstrated moderate restriction and severely impaired diffusion (DLCO) at 39% predicted. The diagnosis was antisynthetase syndrome. The patient received 60 mg of prednisone daily with rapid improvement in symptoms and radiographic findings. However, tapering steroids failed and 3 grams of daily mycophenolate was added to her regimen. Six weeks later, she deteriorated further with worsening hypoxia, arthralgias, mechanic’s hands, DLCO (32% predicted) and progressive GGO and lower lobe infiltration. We administered solumedrol 1 gram daily for three days with rapid clinical improvement and resumed her regimen of daily prednisone and mycophenolate. Within three weeks, the CT chest showed marked clearing and DLCO improved to 39% predicted. At four months post-pulse steroids, she remains on mycophenolate and prednisone (tapered to 20 mg/day) with resolved hypoxia, minimal CT findings and DLCO improved to 46% predicted.
DISCUSSION: Prednisone 1mg/kg/day is first line monotherapy for ASS but is often combined with other immunosuppressants. Up to 25% of cases may be refractory, with reported 45% mortality at 20 months. Rituximab is the common therapy for refractory ASS but may present a high infection-related mortality risk. We suggest that in patients with rapidly deteriorating or refractory ASS, pulse steroids may enhance response to standard therapy as a ‘booster’ treatment. The rapid response is advantageous since uncontrolled inflammation may lead to irreversible fibrotic changes.
CONCLUSIONS: Refractory ASS often has a poor outcome. Current treatments are based on expert recommendations or case series without strong level of evidence. Pulse steroids may offer an effective adjunct to therapy with acute deterioration of ASS.
Reference #1: Marie I et al. Interstitial lung disease in patients with antisynthetase syndrome. Arthritis Care & Research. 2013 May; 65 (5): 800-808
Reference #2: Anderson H et al. Long-term experience with rituximab in ASS-related ILD. Rheumatology (2015) doi; 10.1093/rheumatology/kev004; March 2015
DISCLOSURE: The following authors have nothing to disclose: Marie Rosalette Mortel, Pius Ochieng, Mary Salvatore, Mary O Sullivan
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