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Microscopic Polyangiitis (MPA) - An Uncommon Presentation of an Acute Exacerbation of Idiopathic Pulmonary Fibrosis (IPF) FREE TO VIEW

Jesse Sherratt*, DO; David Bell, MD; Douglas Frenia, MD; Sherry Werner, MD
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San Antonio Military Medical Center, San Antonio, TX

Chest. 2012;142(4_MeetingAbstracts):454A. doi:10.1378/chest.1387538
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PRESENTED ON: Tuesday, October 23, 2012 at 11:15 AM - 12:30 PM

INTRODUCTION: Pulmonary fibrosis is an uncommon manifestation of small vessel vasculitides and may precede other features of these diseases. This case describes a patient who was diagnosed with MPA 3 years after being diagnosed with IPF.

CASE PRESENTATION: An 81-year-old male with a history of IPF presented with progressively worsening fatigue, dyspnea on exertion, cough, lower extremity weakness, and weight loss with development of a new LUL ground glass opacity (GGO). His diagnosis of IPF was made in 2009 without a surgical lung biopsy in accordance with the 2002 ATS consensus on the classification of the idiopathic interstitial pneumonias. An evaluation for an etiology of his acute exacerbation of IPF revealed an elevated myeloperoxidase antibody (MPO) as well as red blood cells in his urine. He was found to have urine sediment with dysmorphic red blood cells and red blood cell casts and, despite a normal creatinine clearance, subsequent renal biopsy was consistent with focal segmental necrotizing glomerulonephritis. The patient was placed on high dose steroids as well as rituximab with gradual improvement in weight, energy level, strength and stabilization of his dyspnea.

DISCUSSION: Pulmonary fibrosis is an uncommon manifestation of MPA. Our patient presented with symptoms typical of those of an acute exacerbation of IPF to include flu like illness, cough, leukocytosis, worsening hypoxia, and worsening superimposed GGO on CT scan. These findings are often presumed to be due to infection and can delay the diagnosis of other potential etiologies to an IPF exacerbation. In a review by Amir, et al, 22 of 58 cases were noted where the diagnosis of pulmonary fibrosis preceded the diagnosis of vasculitis. In contrast to most cases in the literature, our particular patient did not present with evidence of acute renal failure at the time vasculitis was diagnosed.

CONCLUSIONS: Pulmonary fibrosis is an underappreciated finding in patients with MPA and often precedes the diagnosis of vasculitis. Inclusion of MPA in the differential when faced with an acute exacerbation of IPF may increase detection of a potentially treatable etiology.

1) American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classfication of the Idiopathic Interstitial Pneumonias. Am J Respir Crit Care Med. Am J Respir Crit Care Med 2002; 165:277-304

2) Amir B, Karim M. Pulmonary Fibrosis - An Uncommon Manifestation of Anti-Myeloperoxidase Positive Systemic Vasculitis? NDT Plus 2010; 3:351-353

3) Eschun G, Mink S, Sharma S. Pulmonary Interstitial Fibrosis as a Presenting Manifestation in Perinuclear Antineutrophilic Cytoplasmic Antibody Microscopic Polyangitis. Chest. 2003; 123:297-301

DISCLOSURE: The following authors have nothing to disclose: Jesse Sherratt, David Bell, Douglas Frenia, Sherry Werner

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San Antonio Military Medical Center, San Antonio, TX




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