Table 1.
Clinical characteristics
| Sex | Age (yrs) at PPFE diagnosis | Presenting symptoms | Pneumothorax | Smoking history | Allergies | ILD family history | Transplantation (age at transplantation) | Diagnosis before transplantation | PPFE time delay (yrs) after transplantation | CLAD/GVHD | Acute infections | Chemotherapy | Survival after the diagnosis of PPFE/death cause | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Lung transplant | ||||||||||||||
| Case 1 | M | 60 | Dyspnea | No | Ex-smoker (40 packs/yr) | No | No | Left lung transplant (56 yrs) | IPF | 4 | CLAD mixed pattern (obstructive prevalence) | CMV recurrent infections | Mycophenolate, azathioprina, cyclosporine, tacrolimus | Dead/2 yrs/lung squamous cell carcinoma and maxillary sinus carcinoma |
| Case 2 | M | 56 | Dyspnea | No | Ex-smoker (40 packs/yr) | No | No | Left lung transplant (54 yrs) | IPF | 2 | RAS | CMV acute infection | Mycophenolate, azathioprina, cyclosporine | Dead/1 yr/respiratory failure |
| Case 3 | M | 52 | Dyspnea and dry cough | No | No | No | No | Double-lung transplant (48 yrs) | Idiopathic pulmonary hypertension | 4 | CLAD mixed pattern | CMV recurrent infections | Mycophenolate, cyclosporine, tacrolimus, rituximab | Dead/2 yrs/respiratory failure |
| Case 4 | M | 43 | None | No | No | Grass pollen allergy | No | Double-lung transplant (39 yrs) | Postembolic pulmonary hypertension | 4 | CLAD mixed pattern | Acute respiratory infection | Mycophenolate, tacrolimus | Alive/6 yrs |
| HSCT | ||||||||||||||
| Case 5 | M | 33 | Dyspnea | No | No | No | No | Allogeneic HSCT (20 yrs) | AML | 13 | Acute and chronic GVHD | No | ICE, cyclophosphamide | Dead/8 yrs/respiratory failure |
| Case 6 | F | 61 | Dyspnea, dry cough, and weight loss | Yes | No | No | No | Autologous HSCT (56 yrs) | AML | 5 | - | Recurrent respiratory infection (CMV once) | ICE, cyclophosphamide, busulfan | Alive/15 yrs |
PPFE, pleuroparenchymal fibroelastosis; ILD, interstitial lung disease; CLAD, chronic lung allograft dysfunction; GVHD, acute or chronic graft versus host disease; M, male; IPF, idiopathic pulmonary fibrosis; CMV, cytomegalovirus; RAS, restrictive allograft syndrome; HSCT, hematopoietic stem cell transplantation; AML, acute myeloid leukemia; ICE, ifosfamide-carboplatin-etopside; F, female; -, no data.