The prognostic significance of the histologic pattern of interstitial pneumonia in patients presenting with the clinical entity of cryptogenic fibrosing alveolitis.

:Lone cryptogenic fibrosing alveolitis (CFA) is a progressive interstitial lung disease, with a median survival of 3 to 6 yr from the onset of dyspnea. CFA can be subdivided into prognostically significant histopathologic patterns, including nonspecific interstitial pneumonia (NSIP). We reviewed 78 patients with a clinicopathologic diagnosis of CFA, biopsied between 1978 and 1989, to evaluate the prevalence and prognostic significance of these histopathologic patterns, in particular NSIP. Biopsy appearances were reclassified by two pulmonary histopathologists as usual interstitial pneumonia (UIP) (47%), NSIP (36%), or desquamative interstitial pneumonia (DIP)/respiratory bronchiolitis-associated interstitial lung disease (RBILD) (17%). The kappa coefficient of agreement between pathologists was 0.49. In 67 cases, follow-up was complete to death or 10 yr after biopsy, with 50 deaths during a median follow-up of 42 mo (UIP, 89%; NSIP, 61%, DIP/RBILD, 0%). Survival was highest in DIP/RBILD and higher in NSIP than UIP, p < 0.0005. When analysis was confined to patients with UIP or NSIP, the mortality of UIP remained higher, p < 0.01, but the 5-yr survival in patients with fibrotic NSIP was only 45%, indicating that this histologic appearance is often associated with a poor outcome. A response to treatment was more frequent in DIP/RBILD than in NSIP (p < 0.01) or UIP (p < 0.0005). This study confirms the prognostic value of subclassifying patients with CFA according to histopathologic pattern. However, in patients with clinically typical CFA, a histologic diagnosis of fibrotic NSIP needs to be interpreted with caution and does not necessarily denote a good outcome.

Nicholson AG ，Colby TV ，du Bois RM ，Hansell DM ，Wells AU ... -  《AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE》

A histologic pattern of nonspecific interstitial pneumonia is associated with a better prognosis than usual interstitial pneumonia in patients with cryptogenic fibrosing alveolitis.

Daniil ZD ，Gilchrist FC ，Nicholson AG ，Hansell DM ，Harris J ，Colby TV ，du Bois RM ... -  《AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE》

Idiopathic nonspecific interstitial pneumonia: prognostic significance of cellular and fibrosing patterns: survival comparison with usual interstitial pneumonia and desquamative interstitial pneumonia.

Travis WD ，Matsui K ，Moss J ，Ferrans VJ ... -  《AMERICAN JOURNAL OF SURGICAL PATHOLOGY》

Histopathologic variability in usual and nonspecific interstitial pneumonias.

Flaherty KR ，Travis WD ，Colby TV ，Toews GB ，Kazerooni EA ，Gross BH ，Jain A ，Strawderman RL ，Flint A ，Lynch JP ，Martinez FJ ... -  《AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE》

Histopathological approach to patterns of interstitial pneumonia in patient with connective tissue disorders.

Nicholson AG ，Colby TV ，Wells AU 《SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES》