Familial Idiopathic Interstitial Pneumonia Histopathology and Survival in 30 Patients
ABSTRACT Context.-Familial idiopathic interstitial pneumonia (F-IIP) describes the unexplained occurrence of diffuse parenchymal lung disease in related individuals. Prevailing wisdom suggests that the histopathology of F-IIP is indistinguishable from that of idiopathic pulmonary fibrosis, namely, usual interstitial pneumonia (UIP). Objective.-To define the histopathology of F-IIP in lung tissue samples. Design.-Tissue sections from 30 patients with F-IIP, enrolled in a national research program, were evaluated by 3 pulmonary pathologists using 15 predefined histopathologic features. Each feature was recorded independently before a final diagnosis was chosen from a limited list dichotomized between UIP or "not UIP." These 2 groups were then compared to survival. Results.-The consensus diagnosis for the F-IIP cohort was an unclassifiable parenchymal fibrosis (60%), with a high incidence of histopathologic honeycombing, fibroblast foci, and smooth muscle in fibrosis. Usual interstitial pneumonia, strictly defined, was identified in less than half of the F-IIP cases (range, 23%-50%). Interobserver agreement was fair (κ = 0.37) for 2 observers for the overall diagnosis of UIP. Findings unexpected in UIP were prevalent. The survival for the entire F-IIP cohort was poor, with an estimated mortality of 93% and a median age at death of 60.9 years. Subjects with UIP had a shorter survival and younger age at death. Conclusions.-Pulmonary fibrosis was the dominant histopathology identified in our patients, but diagnostic features of UIP were seen in less than 50% of the samples. Overall survival was poor, with mortality accelerated apparently by the presence of a UIP pattern of disease.
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ABSTRACT: A new international statement defines usual interstitial pneumonia (UIP) which is a histological and radiological form of idiopathic pulmonary fibrosis (IPF) more precisely than previously. In the diagnosis of IPF, either in high resolution computed tomography (HRCT) a UIP pattern must be present or alternatively specific combinations of HRCT and surgical lung biopsy findings can be accepted. In about two third of the cases IPF can be diagnosed by clinical and radiological criteria. Thus surgical lung biopsy is needed in about one third of cases to achieve the ultimate diagnosis, which requires multidisciplinary cooperation. In large clinical trials conducted during the last decade, lung biopsy was performed in about 30--60% of the cases. The most serious complication of lung biopsy is mortality within 30 days after the procedure, with a frequency of about 3--4% reported in most studies. Because of the histological variability, surgical lung biopsy should be taken from a minimum of two lobes. The number of fibroblast foci in surgical lung biopsy has been shown to correlate with survival in several studies.Respiratory research 04/2013; 14(1):43. DOI:10.1186/1465-9921-14-43 · 3.38 Impact Factor
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ABSTRACT: Idiopathic pulmonary fibrosis (IPF) is the most common type of idiopathic interstitial pneumonia and has a dismal prognosis. Median age at IPF onset is 60-70 years and it is mainly related to cigarette smoke exposure. Its clinical profile is heterogeneous and different clinical phenotypes are now better defined: familial IPF, slow and rapid progressors, combined pulmonary fibrosis and emphysema, anti-neutrophil cytoplasmic antibodies/microscopic polyangiitis and IPF, and IPF associated with lung cancer. Acute exacerbation associated with rapid functional decline is an event that does not happen infrequently and affects survival. Diagnosis requires a typical usual interstitial pneumonia (UIP) pattern on computed tomography in the appropriate clinical setting or morphological confirmation of the UIP pattern when imaging findings are not characteristic enough. Surgical lung biopsy is the gold standard to obtain valuable information for histological analysis. However, less invasive procedures (transbronchial lung biopsy or even improved transbronchial lung biopsy by cryoprobes) are now under consideration. Prognostic indicators are mainly derived by pulmonary function tests. Recently, staging systems have been proposed. © 2013 S. Karger AG, Basel.Respiration 06/2013; 86(1):5-12. DOI:10.1159/000353580 · 2.92 Impact Factor
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ABSTRACT: The purpose of this review is to provide an update on the diagnosis and classification of interstitial lung disease (ILD), with a specific focus on newly described ILD subtypes and phenotypes. In addition, the strengths and limitations of the current approach to ILD diagnosis and management are discussed. Idiopathic pleuroparenchymal fibroelastosis and acute fibrinous and organizing pneumonia are new entities that have been described in small case series. Undifferentiated connective tissue disease-associated ILD, smoking-related interstitial fibrosis, familial ILD, unclassifiable ILD, and subclinical ILD have also been better characterized in recent publications. New data regarding these conditions are summarized in this review. The multidisciplinary approach to ILD is reviewed, and complementary classification schemes are described that may help direct the management and improve prognostication of some ILDs. ILDs are a large and heterogeneous group of diseases with several newly characterized subtypes and phenotypes. The current approach to ILD classification has limitations in some patients that can be minimized by considering complementary classification schemes.Current opinion in pulmonary medicine 07/2013; DOI:10.1097/MCP.0b013e328363f48d · 2.96 Impact Factor