Aetiology | Sex | Age yrs | P̄pa mmHg | Specific medication | Circulating fibrocytes Col1+CD45+ | Observations | |||
Lumen | Vessel wall | Perivascular area | Blood vessels analysed | ||||||
iPAH | F | 15 | ND | Prostacyclin, bosentan | 2 | 4 | 16 | 40 | Pulmonary arteries severely remodelledPresence of CD45+ cellsOccurrence of plexiform lesions |
iPAH | F | 45 | ND | ND | 0 | 1 | 1 | 35 | Pulmonary arteries severely remodelledPresence of CD45+ cellsOccurrence of plexiform lesions |
iPAH | M | 29 | ND | Prostacyclin, treprostinil | 0 | 0 | 1 | 35 | Pulmonary arteries severely modelledPresence of CD45+ cellsNo plexiform lesions |
iPAH | M | 52 | 60 | Prostacyclin | 0 | 4 | 2 | 35 | Pulmonary arteries severely remodelledPresence of plexiform lesions with collagen depositions, CD45+ cells and few CD45+/collagen1+ fibrocytes |
iPAH | M | 44 | 83 | Flolan, iloprost | 0 | 0 | 2 | 40 | Pulmonary arteries severely remodelledNo plexiform lesions |
iPAH | F | 39 | 70 | Treprostinil | 0 | 0 | 1 | 30 | CD45 positive cellsNo plexiform lesions |
iPAH | F | 28 | ND | Prostacyclin | 6 | 24 | 13 | 30 | Frequent occurrence of plexiform lesions with collagen depositions and CD45 cells with few circulating fibrocytes |
iPAH | M | 4 | 80 | Prostacyclin, bosentan, | 1 | 4 | 2 | 30 | Pulmonary arteries severely remodelled Presence of CD45+ cellsNo plexiform lesions |
iPAH | F | 27 | 82 | Bosentan, prostacyclin | 1 | 0 | 0 | 40 | CD45 positive cells in the lumen of the vesselNo plexiform lesions |
iPAH | M | 45 | ND | Sildenafil | 1 | 1 | 0 | 35 | Rare occurrence of plexiform lesions with collagen depositions and CD45 cells Remodelled vessels |
Col1: collagen 1; P̄pa: mean pulmonary arterial pressure; iPAH: idiopathic pulmonary arterial hypertension; F: female; M: male; ND: not determined.