Diffuse Lung Disease |

B-Cell Depletion Salvage Therapy in Rapidly Progressive Dermatomyositis Related Interstitial Lung Disease FREE TO VIEW

Khaled Eissa, MBBCh; Jaime Palomino, MD
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Tulane University, New Orleans, LA

Chest. 2014;146(4_MeetingAbstracts):385A. doi:10.1378/chest.1994028
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SESSION TITLE: Interstitial Lung Disease Case Report Posters

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Interstitial lung disease is a major cause of morbidity and mortality in patients with inflammatory myopathies. Glucocorticoids are the initial standard treatment. However, many patients fail to respond and continue to progress despite treatment with high dose glucocorticoids. Limited data exists on the use of Rituximab in the treatment of inflammatory mypathies related ILD1,2,3. We report a case of rapidly progressive dermatomyositis associated ILD treated successfully with B-cell depletion therapy.

CASE PRESENTATION: A 66 year old man was diagnosed with dermatomyositis and ILD 6 years ago. His respiratory and muscular condition worsened despite high dose steroids. A trial of Azathioprine and then Mycophenolate was conducted with mild clinical improvement but discontinued secondary to side effects. As his condition progressed rapidly after the discontinuation of Mycophenolate, he received rescue therapy with intravenous Rituximab (1000 mg) weekly for 2 weeks. Three weeks after infusion, patient demonstrated remarkable clinical improvement. Computed tomography chest scan demonstrated interval improvement of interstitial findings (Figure 1). His pulmonary function testing showed remarkable improvement of lung volumes (Table 1). His creatine kinase improved from 2500 IU/L to 300 IU/L with corresponding improvement in his muscle power and functional status.

DISCUSSION: Different immunosuppressive drugs have been used as add on to glucocorticoids for the treatment of dermatomyositis related ILD, especially for patients who fail to respond appropriately. Our case demonstrated success of Rituximab with clinical, radiological and laboratory improvement. Previous case report demonstrated possible relapse that corresponded to the B-cells reinstitution which required repeat treatment with Rituximab1.

CONCLUSIONS: B-cell depletion therapy with Rituximab has a great potential in managing rapidly progressive cases.

Reference #1: L Brulhart, J‐M Waldburger, and C Gabay. Rituximab in the treatment of antisynthetase syndrome. Ann Rheum Dis. Jul 2006; 65(7): 974-975.

Reference #2: Sem M, Molberg O, Lund MB, et al. Rituximab treatment of the anti-synthetase syndrome: a retrospective case series. Rheumatology (Oxford). 2009 Aug;48(8):968-71.

Reference #3: Néel A, Colman N, Germaud P, et al. Salvage B-cell depletion therapy in rapidly progressive dermatomyositis-associated interstitial lung disease. Joint Bone Spine. 2013 Sep 20. pii: S1297-319X(13)00201-7.

DISCLOSURE: The following authors have nothing to disclose: Khaled Eissa, Jaime Palomino

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