SESSION TITLE: Interstitial Lung Disease Cases I
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Sunday, October 26, 2014 at 03:15 PM - 04:15 PM
INTRODUCTION: Interstitial lung disease (ILD) complicating systemic sclerosis remains a leading cause of morbidity and mortality, with limited therapeutic options. Treatment with anti-CD20 antibody, rituximab, may be helpful.
CASE PRESENTATION: A 65-year-old woman, former smoker, presented with one year of progressive exertional dyspnea (World Health Organization functional class II-III). Comorbidities included chronic lymphocytic leukemia (without B-symptoms), gastroesophageal reflux disease, and Raynaud’s. Physical exam was notable for bibasilar crackles, sclerodactyly, and telangiectasias. Her pulmonary function test showed mild restriction (forced vital capacity, 76%) with moderate decrease in diffusion capacity (44%). Laboratory studies revealed a positive anti-Scl-70, normal renal indices, and mild thrombocytosis. An echocardiogram suggested pulmonary hypertension with a preserved right ventricle, but a right heart catheterization was normal. A chest computed-tomography revealed bibasilar ground glass opacities consistent with pathologic confirmation of non-specific interstitial pneumonia -fibrotic subtype. She worsened despite azathioprine and prednisone, and declined cyclophosphamide. Rituximab at a weekly dose of 375mg/m2 for 4-cycles was administered resulting in improvements in skin tightness and dyspnea. Her lung function and 6-minute-walk test showed marked reversal of the prior worsening (see Figure 1).
DISCUSSION: The treatment of scleroderma-associated-ILD remains limited. The largest study using cyclophosphamide showed small but unsustained improvement in lung function. Azathioprine and mycophenolate have been considered, but controlled studies on these agents remain limited. Rituximab has found an increasing role in autoimmune disorders and may also help in systemic sclerosis. We add to the limited series of case reports supporting the potential use of rituximab for scleroderma-associated-ILD.
CONCLUSIONS: The role of Rituximab for the treatment of ILD complicating systemic sclerosis should be further investigated in controlled studies.
Reference #1: Daoussis D, et al. Effect of long-term treatment with rituximab on pulmonary function and skin fibrosis in patients with diffuse systemic sclerosis. Clin Exp Rheumatol 2012;30:S17-22.
Reference #2: Smith V, et al. Two-year results of an open pilot study of a 2-treatment course with rituximab in patients with early systemic sclerosis with diffuse skin involvement. J Rheumatol 2013;40:52-7.
DISCLOSURE: The following authors have nothing to disclose: Andres Borja Alvarez, Augustine Lee
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