Table 1

Clinical and haemodynamic characteristics of each patient at PH diagnosis

CharacteristicsPatient 1Patient 2Patient 3Patient 4Patient 5Patient 6Patient 7Patient 8Patient 9
Clinical characteristicsPMPMPMPM overlap SLEDMDMPMPMPM
 Raynaud’s phenomenon+++++
 Arthritis (history)+++++++
 Disease duration (years)
 WHO functional class232443233
WHO PH group331213331
HRCT scan
 Lung fibrosis on HRCT+++NA++++
 Wells score232NA02313
 Lung involvement (%)707010NA090309010
Extent of ILDExtensiveExtensiveLimitedExtensiveLimitedExtensiveExtensiveExtensiveLimited
Pulmonary function test
 TLC (% of predicted)6680645751876550105
 DLCO (% of predicted)665368NA3967696242
6 Min walking test (m)NA143420NANANA308235307
 Mean PAP (mm Hg)454066416032253352
 PCWP (mm Hg)125735114111414
 Cardiac index (L/min/m2)NANA2.
 Cardiac output (L/min)NA3.
 PVR (dynes/s/cm5)NA7439281551749315242461109
  • ANA, antinuclear antibody; DLCO, diffusing capacity for carbon monoxide; DM, dermatomyositis; ILD, interstitial lung disease; PAP, pulmonary arterial pressure; PCWP, pulmonary capillary wedge pressure; PH, pulmonary hypertension; PM, polymyositis; PVR, pulmonary vascular resistance; RHC, right heart catheterisation; SLE, systemic lupus erythematosus; TLC, total lung capacity; NA, not available.