UIP in rheumatic disease
| Clinical features | Radiological features | Histopathological 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.