This Phase IIb trial will compare effectiveness and safety of a multi-component autoantibody reduction therapy (AART), consisting of therapeutic plasma exchange (TPE), rituximab, and intravenous immunoglobulin (IVIg) for treatment of patients with progressive idiopathic pulmonary fibrosis (IPF).
Age between 40-85 years old.
A diagnosis of IPF that fulfills latest ATS/ERS Consensus Criteria.
A diagnosis of Progressive IPF (P-IPF), defined as a relative decrease of FVC%p >10% during the preceding twelve months, and substantiated by replicate values at least three weeks apart.
Ability and willingness to give informed consent (no surrogates) and adhere to requirements.
Diagnoses of current infection by clinical or microbial assessments.
Diagnoses of an additional or alternative etiology for lung dysfunction based upon clinical assessment, including sepsis, congestive heart failure, thromboembolism, worsened pulmonary artery hypertension, etc. using routine clinical evaluations under direction of the attending physician.
History or serologic evidence of hepatitis B or C infection. Positive serology for Hepatitis C will not exclude patients if their circulating HC virus RNA test is negative.
Coagulopathy, defined as an INR >1.6, PTT >2x control, fibrinogen <100 mg/dL, or platelet count <50,000 unless these abnormalities can be reversed.