Table 1– Clinical characteristics of patients with or without pulmonary fibrosis at time of diagnosis
FibrosisNo fibrosisp-value
Subjects n1320
Age yrs5760ns
Female4 (31)13 (65)ns
Male9 (69)7 (35)ns
Renal manifestations13 (100)20 (100)ns
Fever8 (61)19 (95)0.02
Fatigue4 (31)8 (40)ns
Weight loss1 (8)2 (10)ns
Arthralgias4 (31)4 (20)ns
Myalgias6 (46)1 (5)0.008
Skin involvement1 (8)3 (15)ns
GI involvement1 (8)0 (0)ns
Peripheral nervous system1 (8)3 (15)ns
Haemoptysis5 (38)4 (20)ns
Dyspnoea4 (31)0 (0)0.01
Chronic cough3 (23)0 (0)ns
Laboratory
P-ANCA11 (85)20 (100)ns
P-ANCA and C-ANCA1 (8)
Radiological CT
Ground-glass opacities3 (23)8 (40)ns
Reticular pattern10 (77)2 (10)0.002
Bronchiectasis5 (38)2 (10)ns
Interlobular septal thickening9 (69)0 (0)0.0001
Honeycombing6 (46)0 (0)0.001
Airspace consolidation0 (0)2 (10)ns
Thin-walled parenchymal cysts2 (15)0 (0)ns
Pleural thickening3 (23)0 (0)ns
CT pattern of fibrosis
UIP7 (54)
NSIP4 (31)
  • Data are presented as n absolute patients (% of group analysed), unless otherwise stated. GI: gastrointestinal; P-ANCA: perinuclear anti-neutrophilic cytoplasmic antibody; C-ANCA: cytoplamsic anti-neutrophilic cytoplasmic antibody CT: computerised tomography; UIP: usual interstitial pneumonia; NSIP: nonspecific interstitial pneumonia; ns: nonsignificant.