Table 2

UIP in rheumatic disease

Clinical featuresRadiological featuresHistopathological features
▸ Age less than 60 years common
▸ Men more frequently affected than women (despite RD prevalence in women)
▸ Systemic manifestations common (but not always)
▸ Laboratory evidence of collagen vascular disease common (not always)
▸ Sometimes only non-specific serum markers (erythrocyte sedimentation rate, C-reactive protein)
▸ Reticular opacities with lobular distortion
▸ Honeycomb cysts uncommon and fewer than UIP in IPF
▸ Traction bronchioloectasis
▸ Airway-associated abnormalities
▸ Pleural effusion, sometimes
▸ Fibrosis more haphazard and more airway-centred
▸ Nodular inflammatory (lymphoid) infiltration, often with germinal centres
▸ NSIP-like alveolar septal fibrosis common
▸ Follicular bronchiolitis common
▸ Bronchiolar remodelling common (peribronchiolar metaplasia)
▸ Pleural inflammation and fibrosis common
▸ Occasional fibroblast foci (always fewer than UIP in IPF)
  • IPF, idiopathic pulmonary fibrosis; RD, rheumatic diseases; UIP, usual interstitial pneumonia.