Sandra Chartrand1, Jeffrey J Swigris1, Lina Peykova1, Jonathan Chung1, Aryeh Fischer2. 1. From the Department of Medicine, Hôpital Maisonneuve-Rosemont, Université de Montréal, Montréal, Quebec, Canada; Department of Medicine, National Jewish Health, Denver; University of Colorado School of Medicine, Aurora, Colorado; University of Chicago School of Medicine, Chicago, Illinois, USA.S. Chartrand, MD, FRCPC, Department of Medicine, Hôpital Maisonneuve-Rosemont, Université de Montréal; J.J. Swigris, DO, MS, Department of Medicine, National Jewish Health, and the University of Colorado School of Medicine; L. Peykova, MS, Department of Medicine, National Jewish Health; J. Chung, MD, University of Chicago School of Medicine; A. Fischer, MD, University of Colorado School of Medicine. 2. From the Department of Medicine, Hôpital Maisonneuve-Rosemont, Université de Montréal, Montréal, Quebec, Canada; Department of Medicine, National Jewish Health, Denver; University of Colorado School of Medicine, Aurora, Colorado; University of Chicago School of Medicine, Chicago, Illinois, USA.S. Chartrand, MD, FRCPC, Department of Medicine, Hôpital Maisonneuve-Rosemont, Université de Montréal; J.J. Swigris, DO, MS, Department of Medicine, National Jewish Health, and the University of Colorado School of Medicine; L. Peykova, MS, Department of Medicine, National Jewish Health; J. Chung, MD, University of Chicago School of Medicine; A. Fischer, MD, University of Colorado School of Medicine. aryeh.fischer@ucdenver.edu.
Abstract
INTRODUCTION: Interstitial lung disease (ILD) is 1 possible manifestation of the idiopathic inflammatory myopathies (IIM). Occasionally, patients presenting with ILD are mistakenly diagnosed with idiopathic interstitial pneumonia (IIP), but after multidisciplinary evaluation, their ILD is determined to be because of antisynthetase syndrome (SynS) or myositis spectrum of disease. METHODS: We used retrospective analytic methods to identify patients with ILD evaluated at the National Jewish Health between February 2008 and August 2014 and believed initially to have IIP but ultimately diagnosed with SynS or myositis spectrum of disease. RESULTS: The cohort included 33 patients; most were white women with a mean age at presentation of 55 years. Their pulmonary physiologic impairment was moderate. In 31 cases, the ILD pattern by thoracic high-resolution computed tomography scan was nonspecific interstitial pneumonia (NSIP), organizing pneumonia (OP), or a combination of the 2. Surgical lung biopsy was performed in 21 patients; NSIP was the most common pattern. Less than one-third of the cohort had positive antinuclear antibodies. Two-thirds had positive SSA. All patients had either myositis-specific or myositis-associated autoantibody. Most had subtle extrathoracic symptoms or signs of SynS; 12 had an elevated serum creatine phosphokinase, but none had clinical evidence of myositis. None met the Peter and Bohan classification criteria for polymyositis/dermatomyositis. CONCLUSION: Among patients who present with presumed IIP, a multidisciplinary evaluation that includes the integration of clinical evaluations by rheumatologists and pulmonologists, morphologic (both histopathologic and radiographic) data, and serologic features is helpful in the detection of occult SynS or the myositis spectrum of disease.
INTRODUCTION:Interstitial lung disease (ILD) is 1 possible manifestation of the idiopathic inflammatory myopathies (IIM). Occasionally, patients presenting with ILD are mistakenly diagnosed with idiopathic interstitial pneumonia (IIP), but after multidisciplinary evaluation, their ILD is determined to be because of antisynthetase syndrome (SynS) or myositis spectrum of disease. METHODS: We used retrospective analytic methods to identify patients with ILD evaluated at the National Jewish Health between February 2008 and August 2014 and believed initially to have IIP but ultimately diagnosed with SynS or myositis spectrum of disease. RESULTS: The cohort included 33 patients; most were white women with a mean age at presentation of 55 years. Their pulmonary physiologic impairment was moderate. In 31 cases, the ILD pattern by thoracic high-resolution computed tomography scan was nonspecific interstitial pneumonia (NSIP), organizing pneumonia (OP), or a combination of the 2. Surgical lung biopsy was performed in 21 patients; NSIP was the most common pattern. Less than one-third of the cohort had positive antinuclear antibodies. Two-thirds had positive SSA. All patients had either myositis-specific or myositis-associated autoantibody. Most had subtle extrathoracic symptoms or signs of SynS; 12 had an elevated serum creatine phosphokinase, but none had clinical evidence of myositis. None met the Peter and Bohan classification criteria for polymyositis/dermatomyositis. CONCLUSION: Among patients who present with presumed IIP, a multidisciplinary evaluation that includes the integration of clinical evaluations by rheumatologists and pulmonologists, morphologic (both histopathologic and radiographic) data, and serologic features is helpful in the detection of occult SynS or the myositis spectrum of disease.
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