SESSION TITLE: ILD Case Report Posters II
SESSION TYPE: Case Report Poster
PRESENTED ON: Sunday, March 23, 2014 at 01:15 PM - 02:15 PM
INTRODUCTION: Non Specific Interstitial Pneumonia (NSIP) is a type of diffuse parenchymal lung disease with distinct clinico-pathologic features. It typically presents with progressive dyspnea and cough that develop sub-acutely. There are few reports of patients presenting with an acute exacerbation (AE) of NSIP at the time of initial diagnosis.
CASE PRESENTATION: A 49 year old female smoker presented with two weeks of progressively worsening dyspnea and non-productive cough. Examination revealed tachypnea with a room air saturation of 67%, bilateral rales and absence of fever. Initial laboratory studies demonstrated leukocytosis at 14.6, elevated inflammatory markers with an ESR and CRP of greater than 120 and 340 respectively, and a BNP of 210. A CT angiography of the chest was obtained which excluded pulmonary embolism but revealed bilateral, extensive ground glass opacities. The patient was treated with empiric anti-biotics as well as intravenous methylprednisone 125 mg every 6 hours. A decision was made to proceed with surgical lung biopsy. On day two of hospitalization the patient was intubated and underwent wedge resection of the right upper and lower lobes. Histo-pathologic analysis revealed extensive interstitial lymphocytic infiltration consistent with mixed cellular-fibrotic NSIP and associated changes approaching the level of diffuse alveolar damage. A trichrome stain revealed interstitial fibrosis and special stains were negative for microorganisms. Granulomas or giant cells were notably absent. The patient’s hypoxia quickly improved and intravenous steroids were transitioned to oral prednisone. Successful extubation occurred on hospital day 3 with discharge home on room air by hospital day 6. Follow-up imaging at six weeks showed near complete resolution of all GGO’s.
DISCUSSION: It has been well described that patients with Idiopathic Pulmonary Fibrosis may experience AE’s and recently AE’s have been described among patients with NSIP.1
CONCLUSIONS: We believe our case represents an AE as the initial presentation of NSIP, which to our knowledge has not been previously described in the absence of prior imaging consistent with possible NSIP. Additionally our patient’s accelerated clinical and radiographic improvement is unique.
Reference #1: Park N, Kim S, et al. Acute Exacerbation of Interstitial Pneumonia Other Than IPF. Chest 2007: 132; 214-220
DISCLOSURE: The following authors have nothing to disclose: Michael Colancecco, Andres Sosa
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