Seminars in respiratory and critical care medicine
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Usual interstitial pneumonia (UIP) is a distinct histological lesion observed in idiopathic pulmonary fibrosis (IPF) but can be found in other etiologies. The diagnosis of UIP can be established by surgical lung biopsy or by high-resolution thin-section computed tomographic (CT) scans (provided the radiographic features are classical). Historically, patients labeled as "IPF" encompassed a group of disorders, including UIP as well as other idiopathic interstitial pneumonias that differ from UIP in prognosis and responsiveness to therapy. ⋯ Corticosteroids or immunosuppressive agents have been most often used, but data affirming benefit are lacking. Lung transplantation is a viable option for patients failing medical therapy. This review discusses diagnostic criteria for UIP (both histopathological and radiographic), natural history and clinical course, and therapeutic approaches (both current and future).
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Semin Respir Crit Care Med · Dec 2006
ReviewHigh-resolution computed tomography of interstitial pulmonary fibrosis.
Idiopathic pulmonary fibrosis (IPF), also referred to as cryptogenic fibrosing alveolitis (CFA), is the clinical-radiological-pathological syndrome associated with the most common form of fibrosing interstitial pneumonia, usual interstitial pneumonia (UIP). Unfortunately, the diagnosis of IPF carries with it a relatively poor prognosis. The characteristic features on high-resolution computed tomography (HRCT), consisting of reticular abnormality and honeycombing with basal and peripheral predominance, are virtually diagnostic within the correct clinical context. ⋯ When ground-glass attenuation is seen in patients with IPF, it commonly progresses to fibrosis and honeycombing. Complications of IPF include accelerated progression and deterioration, lung cancer, spontaneous air leak, and secondary infection. This article discusses the key imaging features as correlated with histopathology, differential diagnosis, complications, and follow-up evaluation of idiopathic pulmonary fibrosis.
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Semin Respir Crit Care Med · Dec 2006
ReviewAcute interstitial pneumonia and acute exacerbations of idiopathic pulmonary fibrosis.
Acute interstitial pneumonia (AIP) and acute exacerbations of idiopathic pulmonary fibrosis (AEIPF) are similar respiratory disorders characterized by the rapid development of progressive dyspnea and cough. Both frequently lead to respiratory failure and death. Pathologically, each is characterized by the presence of a diffuse alveolar damage (DAD) pattern; in AIP, DAD is the sole pattern, whereas in AEIPF DAD is superimposed upon a background usual interstitial pneumonia. ⋯ Despite minimal evidence, glucocorticoids are frequently begun once microbiological evaluation confirms the absence of infection. Despite therapy, the case fatality rate ranges up to 70% for both, with most patients dying in the first 2 weeks. Survivors of the acute event can recover to their previous baseline; however, most AIP survivors will stabilize with some functional impairment, whereas in those with AEIPF, progressive fibrosis with functional deterioration is the rule.