Patient case (clinical description): A 47-year-old man comes to the office for routine checkup. He is complaining of chronic cough and occasional but progressive dyspnea.  Other medical conditions include hypertension and osteoarthritis.  The patient smokes a pack of cigarettes daily and does not use alcohol or illicit drugs.  He used to be a construction worker. On examination, there are decreased breath sounds and percussive dullness at the base of both lungs.  Chest CT scan reveals a mild bilateral pleural effusion and diffuse thickening of the pleura. The patient's documents show chronic exposure to asbestosis. The specimen of the lungs reveled  pulmonary fibrosis that is most predominant in the lower lobes, characterized  by the presence of asbestos bodies (golden-brown beaded rods with translucent centers).

Patient's own account: I'm a 47-year-old former construction worker. I'm a man and I smoke a pack of cigs a day but I don't drink or do drugs. I went to my routine checkup because I've been coughing so much lately. I also had shortness of breath and it got worse over time. I already suffer from hypertension and osteoarthritis. My doctor found that I have decreased breath sounds. I did a chest scan and it seems that I have fluid around my lungs, and the lining of my lungs has become thicker in many areas. I have been exposed a lot to asbestos fibers. The other lung exam showed that I have pulmonary fibrosis, especially in the lower part of my lungs and they found some asbestos there.

Clinical trial: Therapeutic Plasma Exchange, Rituximab and IV Ig for Severe Acute Exacerbation of IPF Admitted in ICU

Eligibility criteria:
Inclusion Criteria:

          1. Patient ≥ 18 years of age

          2. Admitted to ICU in the last 72 h

          3. Definite or probable IPF diagnosis defined on 2018 ATS/ERS/JRS/ALAT guidelines or a
             possible usual interstitial pneumonia pattern on HRCT without etiology.

          4. Definite AE-IPF according to the 2018 revised criteria :

               1. Previous or concurrent diagnosis of idiopathic pulmonary fibrosis (if the
                  diagnosis of IPF is not previously established, this criterion can be met by the
                  presence of radiologic and or histopathologic changes consistent with usual
                  interstitial pneumonia (UIP) pattern on the current evaluation);

               2. Acute worsening or development of dyspnea typically of less than one-month
                  duration;

               3. Computed tomography with new bilateral ground-glass opacity and/or consolidation
                  superimposed on a background pattern consistent with a UIP pattern (if no
                  previous computed tomography is available, the qualifier "new" can be dropped);

               4. Deterioration not fully explained by cardiac failure or fluid overload.

          5. PaO2/FiO2 ratio < 200 measured on FiO2 1

        Exclusion Criteria:

          1. Known hypersensitivity intravenous immunoglobulins or rituximab

          2. Severe heart failure

          3. Active and uncontrolled bacterial fungal or parasitic infection ruled out by at least
             one of these two conditions

               1. Procalcitonin value at inclusion < 0.25 ng/mL OR

               2. Adapted antimicrobial therapy for at least 48 hours at inclusion

          4. Positive multiplex PCR for Influenzae A and B, or VRS

          5. Deep Veinous Thrombosis or Pulmonary embolism in the last six months

          6. Prior exposures to human-murine chimeric antibodies

          7. Ongoing treatment with a cellular immunosuppressant (e.g., cyclophosphamide,
             methotrexate, mycophenolate, azathioprine, calcineurin inhibitors, etc.)

          8. Subject treated with more than 2 boluses of methylprednisolone (total dose > 500mg of
             methylprednisolone) or one dose > 10mg/kg in the last 72 hours

          9. Uncorrectable coagulopathies or thrombocytopenia < 30000/mm3

         10. Active cancer (other than basal cell carcinoma of the skin)

         11. Other source of immunosuppression (i.e. HIV infection, solid organ transplant,
             lymphoma or leukemia)

         12. Pregnancy

         13. Patient listed for lung transplantation

         14. Patient on ECMO

         15. Patient with a do-not-intubate order at inclusion

         16. Concurrent participation in other experimental trials

         17. Not Affiliation to the French social security

         18. Not Written informed consent from the patient or a legal representative if appropriate

         19. Hypersensitivity to corticosteroids, cotrimoxazole / atovaquone

         20. Patients with severe renal insufficiency (creatine clearance <15ml / min)
No condition on gender to be admitted to the trial.
No healthy subjects accepted to join the trial.
Subject must be at least 18 Years old.


Eligibility (NLI label): Entailment