A MultiCenter Study of Combined PEX, Rituximab, and Steroids in Acute Idiopathic Pulmonary Fibrosis Exacerbations

INCLUSION CRITERIA: 1. A diagnosis of idiopathic pulmonary fibrosis that fulfills American Thoracic Society Consensus Criteria.1 2. Unexplained worsening or development of dyspnea or hypoxemia within the preceding 30 days. 3. Radiographic imaging showing ground-glass abnormality and/or consolidation superimposed on a reticular or honeycomb pattern consistent with UIP. EXCLUSION CRITERIA 1. Diagnosis of documented infection, thromboembolic disease, an additional etiology for acute lung injury/adult respiratory distress syndrome, congestive heart failure. 2. Presence of active hepatitis B infection. 3. Coagulopathy defined as an INR \> 1.8, PTT \> 2 x control, and platelet count \< 50K. 4. Hyperosmolar state or diabetic ketoacidosis to suggest uncontrolled diabetes mellitus or uncontrolled hypertension. 5. Hemodynamic instability. 6. History of reaction to blood products, murine-derived products, or prior exposures to human-murine chimeric antibodies, 7. History of malignancy. 8. Unwillingness to accept a blood transfusion. 9. Unwillingness to agree to full supportive medical care (e.g., intubation) for up to 2 weeks after enrollment. 10. Inability or unwillingness to complete post-treatment surveillance for 60 days. 11. Diagnosis of major comorbidities expected to interfere with subjects study participation for 28 days. 12. Treatment for \>5 days within the preceding month with \>20 mg prednisone (or equivalent dose corticosteroid) or any treatment during the preceding month with a potent cellular immunosuppressant (e.g., cyclophosphamide, methotrexate, mycophenolate, azathiaprine, calcineurin inhibitors, etc.) unless the patient has a BAL negative for opportunistic pathogens (e.g, Pneumocystis, viruses, intracellular organisms, mycobacteria, etc.). 13. Current treatment with an angiotensin converting enzyme inhibitor that cannot be discontinued and/or substituted with another antihypertensive agent (to minimize potential hemodynamic complications during PEX).