Combined PEX, Rituximab and Steroids in Acute Idiopathic Pulmonary Fibrosis Exacerbations

This is a prospective, open-label Phase II, non-randomized clinical trial to assess the feasibility and safety of combined plasma exchange (PEX), rituximab, and conventional corticosteroid administration in patients with acute IPF exacerbations. INCLUSION CRITERIA: 1. A diagnosis of idiopathic pulmonary fibrosis that fulfills American Thoracic Society Consensus Criteria. 2. Unexplained worsening or development of dyspnea or hypoxemia within 30 days leading to the current hospitalization. 3. Radiographic imaging showing ground-glass abnormality and/or consolidation superimposed on a background of reticular or honeycomb pattern consistent with UIP. 4. Intent on the part of the treating physician to use high dose steroid therapy as a therapeutic effort to treat a diagnosis of acute IPF exacerbation. EXCLUSION CRITERIA 1. Diagnosis of documented infection based upon clinical evaluation and microbial testing. 2. Diagnosis of thromboembolic disease by clinical assessment. 3. Diagnosis of an additional etiology for ALI/ARDS based upon clinical assessment to include sepsis, aspiration, trauma, inhalational injury, acute pancreatitis, drug toxicity, blood product transfusion reaction, or stem cell transplantation. 4. Diagnosis of congestive heart failure that accounts for the hypoxemia. 5. Presence of active hepatitis B infection. 6. Coagulopathy defined as an INR \> 1.8, PTT \> 2 x control, and platelet count \< 50K. 7. Hyperosmolar state or diabetic ketoacidosis to suggest uncontrolled diabetes mellitus or uncontrolled hypertension (systolic BP \> 160 mm Hg and diastolic BP \> 100 mm Hg) which would contraindicated the use of corticosteroids. 8. Hemodynamic instability defined as a vasopressor requirement which would contraindicate the use of plasmapheresis. 9. History of reaction to blood products, murine-derived products, or prior exposures to human-murine chimeric antibodies, 10. History of malignancy. 11. Inability or unwillingness to accept a blood transfusion. 12. Inability or unwillingness to complete post- treatment surveillance for 60 days. 13. Diagnosis of major comorbidities expected to interfere with subjects study participation for 60 days.