Diffuse Lung Disease |

Early Use of Rituximab for Rapidly Progressive Interstital Lung Disease Associated With an Anti-PL-12 Antisynthetase Syndrome FREE TO VIEW

Usman Nazir, MD; Mark Hamblin, MD
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University of Kansas Medical Center, Kansas City, KS

Chest. 2014;146(4_MeetingAbstracts):381A. doi:10.1378/chest.1994393
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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: The Anti-Synthetase Syndrome is a subgroup of the idiopathic inflammatory myopathies. The clinical features of myositis, Raynaud’s phenomenon, and mechanic hands are variably present making diagnosis challenging. The ILD is the major determinant of morbidity and mortality, and an acute presentation is associated with a poor prognosis

CASE PRESENTATION: Three months prior to admission, a 64 year-old male developed cyanosis of his left index finger and thumb raising concern for tissue ischemia. He subsequently developed cough symptoms treated with Ciprofloxacin, but developed a rash successfully treated with a prednisone taper. Over the next 3 weeks cough recurred with progressive dyspnea leading to a hospitalization for acute hypoxemic respiratory failure. Infection was excluded, and he was urgently transferred due to rapid deterioration. Upon presentation, he was requiring 6 liters nasal cannula (NC) to maintain oxygenation. His exam was notable for bibasilar rales. There was no evidence of a skin rash, hyperkeratosis, or muscle weakness Chest CT demonstrated basilar predominant interstitial changes. ANA, Anti-Jo1, and cpk were negative, but his aldolase was elevated with a positive SS-A antibody. He had no features of myositis with negative EMG studies and a normal MRI of his legs; however, an Anti-synthetase syndrome was suspected. He was initiated on high-dose IV methylprednisolone and mycophenolate. He responded to treatment, and was discharged home on 2 L NC and 60 mg prednisone. Within 24 hours his oxygen requirement increased to 7 L at rest and he was re-admitted. He was resumed on IV methylprednisolone, but had no improvement after 5 days. At that time his myositis antibody panel resulted with anti-PL-12 antibodies. Given his refractory clinical picture, he was dosed with IV Rituximab. Within 24 hours of his first infusion his oxygen requirement decreased to 4 L and he clinically improved. He was discharged home with a second infusion of rituximab 2 weeks later. At 6 month follow-up he was off oxygen at rest, with 2 L required on exertion

DISCUSSION: This case demonstrates the subtle presentation of this disease, as well as the successful application of early anti-B cell therapy

CONCLUSIONS: This case displays a rapidly progressive disease treated aggressively with early use of rituximab resulting in a favorable outcome

Reference #1: La Corte R, et al; Antisynthetase syndrome the occurrence of anti-Ro/SSA antibodies causes a more severe interstitial lung disease. Autoimmune 2006;39:249-53

DISCLOSURE: The following authors have nothing to disclose: Usman Nazir, Mark Hamblin

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