Classification of idiopathic interstitial pneumonias: making sense of the alphabet soup

Abstract

Since Liebow and Carrington's original classification of idiopathic interstitial pneumonias, there have been controversies over which histological patterns should be included and how they relate to clinicopathological diseases such as cryptogenic fibrosing alveolitis/idiopathic pulmonary fibrosis (CFA/IPF). Because of these differences and the wealth of overlapping terminology, a consensus classification system has been proposed, devised by a group of clinicians, radiologists and pathologists. Seven histological patterns are recognized: usual interstitial pneumonia (UIP), non-specific interstitial pneumonia (NSIP), diffuse alveolar damage (DAD), organizing pneumonia (OP), desquamative interstitial pneumonia (DIP), respiratory bronchiolitis (RB) and lymphocytic interstitial pneumonia (LIP), each with a clinicopathological counterpart, the most well defined being UIP and CFA/IPF. The system is applicable both in terms of the pathologist identifying histological patterns in isolation and in terms of the pathologist's role in contributing to the final clinicopathological diagnosis. It will probably provide greater consistency in diagnosis, early studies suggesting that the system is reproducible, and also identify purer cohorts for studies investigating causation. It also highlights the fact that the 'gold standard for diagnosis' is no longer a surgical lung biopsy in isolation but more the clinicopathological conference, when clinical, imaging and histological data are jointly discussed to produce the final clinicopathological diagnosis.

Interstitial Lung Disease in West Highland White Terriers

“…12 Controls were determined to have no underlying lung disease. The distribution of the ECM in the alveolar septa was analyzed subjectively and categorized as either ''concentric'' (surrounding the capillary endothelium and widening the distance between the capillary and alveolar epithelium) or ''eccentric'' (widening the interstitium but displacing the capillary so that it remains closely apposed to one side of the alveolar epithelium).…”

Pathologic reparation of the stem cell niche zones of respiratory acini in idiopathic pulmonary fibrosis

“…ИЛФ / ОИП является наиболее частой формой ИИП и по данным ряда авторов составляет от 47 до 62 % всех случаев ИИП [3,4]. ИЛФ отличается от других ИИП быстро прогрессирующим течени ем, развитием "сотового" легкого, резистентностью к проводимой терапии и очень плохим прогнозом.…”

Remodelamento das pequenas vias aéreas nas pneumonias intersticiais idiopáticas

“…Atualmente, as (Kaminski et al, 2003). (Demedts e Costabel, 2002;Nicholson, 2002;Travis et al, 2002b;Kaminski et al, 2003;Flaherty et al, 2004).…”

“…Devido ao maior impacto clínico e epidemiológico e às controvérsias existentes, UIP e NSIP foram intensamente discutidas naquele fórum e serão abordadas de modo mais aprofundado a seguir (Demedts e Costabel, 2002;Nicholson, 2002;Travis et al, 2002b;Kaminski et al, 2003;Flaherty et al, 2004). …”