Identification Of An Inflammatory Variant Of Usual Interstitial Pneumonia ("Blue"-Interstitial Pneumonia): Implications For The Prognosis And Treatment Of Idiopathic Interstitial Pneumonia

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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Characterisation of patients with interstitial pneumonia with autoimmune features

European Respiratory Journal ◽

10.1183/13993003.01565-2015 ◽

2016 ◽

Vol 47 (6) ◽

pp. 1767-1775 ◽

Cited By ~ 113

Author(s):

Justin M. Oldham ◽

Ayodeji Adegunsoye ◽

Eleanor Valenzi ◽

Cathryn Lee ◽

Leah Witt ◽

...

Keyword(s):

Interstitial Pneumonia ◽

Usual Interstitial Pneumonia ◽

American Thoracic Society ◽

Idiopathic Interstitial Pneumonia ◽

European Respiratory Society ◽

Differential Survival ◽

Female Predominance ◽

Clinical Domain ◽

Better Than ◽

Society Research

Patients with interstitial lung disease (ILD) may have features of connective tissue disease (CTD), but lack findings diagnostic of a specific CTD. A recent European Respiratory Society/American Thoracic Society research statement proposed criteria for patients with interstitial pneumonia with autoimmune features (IPAF).We applied IPAF criteria to patients with idiopathic interstitial pneumonia and undifferentiated CTD-ILD (UCTD). We then characterised the clinical, serological and morphological features of the IPAF cohort, compared outcomes to other ILD cohorts and validated individual IPAF domains using survival as an endpoint.Of 422 patients, 144 met IPAF criteria. Mean age was 63.2 years with a slight female predominance. IPAF cohort survival was marginally better than patients with idiopathic pulmonary fibrosis, but worse than CTD-ILD. A non-usual interstitial pneumonia pattern was associated with improved survival, as was presence of the clinical domain. A modified IPAF cohort of those meeting the clinical domain and a radiographic or histological feature within the morphological domain displayed survival similar to those with CTD-ILD.IPAF is common among patients with idiopathic interstitial pneumonia and UCTD. Specific IPAF features can identify subgroups with differential survival. Further research is needed to replicate these findings and determine whether patients meeting IPAF criteria benefit from immunosuppressive therapy.

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Systemic sclerosis and idiopathic interstitial pneumonia: histomorphometric differences in lung biopsies

Jornal Brasileiro de Pneumologia ◽

10.1590/s1806-37132009000600006 ◽

2009 ◽

Vol 35 (6) ◽

pp. 529-540 ◽

Cited By ~ 9

Author(s):

Edwin Roger Parra ◽

Leandro Hideki Otani ◽

Erika Franco de Carvalho ◽

Alexandre Ab'Saber ◽

Vera Luiza Capelozzi

Keyword(s):

Extracellular Matrix ◽

Endothelial Cells ◽

Systemic Sclerosis ◽

Epithelial Cells ◽

Interstitial Pneumonia ◽

Smooth Muscle Actin ◽

Elastic Fiber ◽

Usual Interstitial Pneumonia ◽

Idiopathic Interstitial Pneumonia ◽

Elastic Fibers

OBJECTIVE: The aim of this study was to examine the parenchymal and extracellular matrix remodeling process in two histologic patterns-nonspecific interstitial pneumonia (NSIP) and usual interstitial pneumonia (UIP)-in cases of idiopathic and sclerosis/systemic sclerosis (SSc)-associated interstitial pneumonia. METHODS: We examined 15 cases of idiopathic NSIP, 10 cases of idiopathic UIP, 5 cases of SSc-UIP and 9 cases of SSc-NSIP. In the lung parenchyma, epithelial cells, endothelial cells and myofibroblasts were evaluated by immunohistochemical staining, whereas histochemical staining was used in order to evaluate collagen/elastic fibers in the extracellular matrix. RESULTS: The percentage of surfactant protein A-positive epithelial cells was significantly greater in idiopathic NSIP than in SSc-NSIP, as well as being greater in idiopathic UIP than in SSc-UIP. Idiopathic NSIP and idiopathic UIP presented significantly higher immunoexpression of alpha smooth muscle actin in myofibroblasts than did SSc-NSIP and SSc-UIP. The percentage of CD34 endothelial cells in the pulmonary microvasculature was significant lower in idiopathic UIP than in SSc-UIP. The density of collagen fibers was significantly greater in idiopathic NSIP and idiopathic UIP than in SSc-NSIP and UIP. In contrast, the elastic fiber density was significantly lower in idiopathic UIP than in SSc-UIP. CONCLUSIONS: Increased collagen synthesis, destruction of elastic fibers, high myofibroblast proliferation and poor microvascularization might represent a remodeling process found in idiopathic interstitial pneumonia, whereas the reverse might represent a repair process in SSc-associated interstitial pneumonia.

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Cumulative Incidences of Lung Cancer in Various Interstitial Lung Diseases

10.21203/rs.3.rs-493370/v1 ◽

2021 ◽

Author(s):

Takafumi Suzuki ◽

Hiroyuki Sakashita ◽

Masako Akiyama ◽

Takayuki Honda ◽

Masaru Ejima ◽

...

Keyword(s):

Lung Cancer ◽

Connective Tissue ◽

Interstitial Pneumonia ◽

Connective Tissue Disease ◽

Hypersensitivity Pneumonitis ◽

Usual Interstitial Pneumonia ◽

Idiopathic Interstitial Pneumonia ◽

Cancer Development ◽

Idiopathic Interstitial Pneumonias ◽

Chronic Hypersensitivity Pneumonitis

Abstract Background Interstitial lung disease (ILD) patients often develop lung cancer. However, previous studies on the incidences of lung cancer in ILD patients focused on specific aetiologies, such as idiopathic pulmonary fibrosis (IPF). The lung cancer incidences in these patients have not been investigated, and thus, we aimed to evaluate them here. Methods ILD patients at our hospital were retrospectively reviewed. The cumulative incidences of lung cancer in patients with various ILDs were estimated with Kaplan-Meier curves and compared between ILD groups using log-rank tests. The association between several variables at initial diagnosis and lung cancer development was assessed with Cox proportional hazards regression analysis to identify predictors. Results In all, 606 ILD patients, including 161 with IPF, 133 with non-IPF idiopathic interstitial pneumonias, 160 with chronic hypersensitivity pneumonitis, 87 with connective tissue disease-related ILDs, 19 with pulmonary sarcoidosis, and 46 with other ILDs, were included. Twenty-eight patients developed lung cancer. The cumulative incidences of lung cancer at 1, 3, and 5 years were: 1.9, 5.7, and 12.3% with IPF, respectively; 0.8, 0.8, and 4.0% in non-IPF idiopathic interstitial pneumonias; 2.0, 4.6, and 11.0% in chronic hypersensitivity pneumonitis; and 1.1, 1.1, and 2.9% in connective tissue disease-related ILDs. IPF patients had a higher incidence of lung cancer than non-IPF idiopathic interstitial pneumonia patients (p = 0.036). A radiological usual interstitial pneumonia pattern, forced vital capacity value, and pack-years were associated with lung cancer development (hazard ratios 2.959, 1.031, 1.011; 95% confidence intervals 1.257–6.963, 1.006–1.057, 1.002–1.020, p = 0.013, 0.017, 0.020, respectively). Conclusions The lung cancer incidence is higher in IPF patients than in non-IPF idiopathic interstitial pneumonia patients and is equally high in patients with chronic hypersensitivity pneumonitis and IPF.

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