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.