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PubMed2001

Erythropoietic response to hypoxaemia in diffuse idiopathic pulmonary fibrosis, as opposed to chronic obstructive pulmonary disease.

The aim of this study was to assess the erythropoietic response to hypoxaemia in patients with diffuse idiopathic pulmonary fibrosis (DIPF), and to speculate on the underlying mechanisms. Patients on an established chronic respiratory failure due to DIPF or chronic obstructive pulmonary disease (COPD) were studied. The erythropoietic response to hypoxaemia in both conditions was assessed. We studied 18 patients with DIPF and 29 patients with COPD in respiratory failure in a stable stage, free from acute infection and congestive heart failure. Blood gases, erythrocytic parameters, as well the serum levels of iron, ferritin and erythropoietin were determined. All the DIPF patients studied, apart from two, had normal or subnormal haematocrit values. The patients with COPD had an inconsistant response to hypoxaemia; 12 had normal or subnormal haematocrit values and the remaining 17 were erythraemic. The mean value of erythropoietin (EPO) in both DIPF and COPD patients was significantly higher than normal. In conclusion, patients with DIPF exhibit a lack of erythropoietic response to hypoxaemia, despite the augmented erythropoietin levels. This may reflect a defective bone marrow erythropoietic response in DIPF patients. It is suggested that the pathophysiology of DIPF underlies this mechanism.

Adult↗
PubMed2004

The transcription factor NRF2 protects against pulmonary fibrosis.

The molecular mechanisms of pulmonary fibrosis are poorly understood, although reactive oxygen species are thought to have an important role. NRF2 is a transcription factor that protects cells and tissues from oxidative stress by activating protective antioxidant and detoxifying enzymes. We hypothesized that NRF2 protects lungs from injury and fibrosis induced by bleomycin, an anti-neoplastic agent that causes pulmonary fibrosis in susceptible patients. To test this hypothesis, mice with targeted deletion of Nrf2 (Nrf2-/-) and wild-type (Nrf2+/+) mice were treated with bleomycin or vehicle, and pulmonary injury and fibrotic responses were compared. Bleomycin-induced increases in lung weight, epithelial cell death, and inflammation were significantly greater in Nrf2-/- mice than in Nrf2+/+ mice. Indices of lung fibrosis (hydroxyproline content, collagen accumulation, fibrotic score, cell proliferation) were significantly greater in bleomycin-treated Nrf2-/- mice, compared with Nrf2+/+ mice. NRF2 expression and activity were elevated in Nrf2+/+ mice by bleomycin. Bleomycin caused greater up-regulation of several NRF2-inducible antioxidant enzyme genes and protein products in Nrf2+/+ mice compared with Nrf2-/- mice. Further, bleomycin-induced transcripts and protein levels of lung injury and fibrosis markers were significantly attenuated in Nrf2+/+ mice compared with Nrf2-/- mice. Results demonstrated that NRF2 has a critical role in protection against pulmonary fibrosis, presumably through enhancement of cellular antioxidant capacity. This study has important implications for the development of intervention strategies against fibrosis.

Animals↗
PubMed1995

Aberrant appearance of lung surfactant protein A in sera of patients with idiopathic pulmonary fibrosis and its clinical significance.

Pulmonary surfactant protein A (SP-A) is known to be a major phospholipid-associated glycoprotein in pulmonary surfactant, which is specific to the lung. In this study, the SP-A concentrations in sera of patients with various lung diseases were determined using an enzyme-linked immunosorbent assay. Patients with idiopathic pulmonary fibrosis (IPF) and pulmonary alveolar proteinosis (PAP) exhibited prominently high concentrations of serum SP-A compared to those of other lung diseases and healthy volunteers, although there were significant increases in serum SP-A concentrations in patients with pulmonary tuberculosis, chronic pulmonary emphysema, diffuse panbronchiolitis and bacterial pneumonia compared to those of healthy volunteers. Successive measurement in 2 patients with IPF showed that serum SP-A levels reflect the disease activity of IPF. In patients with IPF, serum SP-A concentrations were significantly correlated with those of serum lactate dehydrogenase, whereas there were no significant correlations of serum SP-A concentrations with erythrocyte sedimentation rate, arterial oxygen saturation, vital capacity and carbon monoxide diffusing capacity. Determination of serum SP-A will contribute to diagnosing IPF and PAP, and may reflect the disease activity of IPF.

Biomarkers↗
PubMed2003

Genetics of pulmonary fibrosis.

Identifying genetic determinants of pulmonary fibrosis is at an early stage of evolution. It is now well recognized that "pulmonary fibrosis" covers a broad range of lung diseases including most topically the idiopathic interstitial pneumonias that have been classified recently. Additionally, it is recognized that the diffuse lung diseases of children that may progress to fibrosis are quite different from those of adults. Defining clinical phenotype is an absolute prerequisite to precise identification of genetic determinants, and this is at least part of the reason why we understand relatively little of these genetic determinants to date. In children, a number of mutations have been identified, particularly with regard to surfactant protein. In adults, families with idiopathic pulmonary fibrosis are being collected but there are no genetic data on these at this point. In sporadic disease, associations have been reported in early phase genes with disease predisposition and also, importantly, with disease severity: future studies will inevitably incorporate issues of severity of disease in analyses.

Journal Article↗
PubMed2003

Operation for lung cancer in patients with idiopathic pulmonary fibrosis: surgical contraindication?

BACKGROUND: Patients with idiopathic pulmonary fibrosis have an increased incidence of lung cancer. The purpose of this study was to determine the outcome of surgical treatment of lung cancer with idiopathic pulmonary fibrosis. METHODS: From January 1992 through December 2001, 64 patients who had simultaneous lung cancer and idiopathic pulmonary fibrosis were treated. Twenty-one (33%) of them underwent surgical resection of lung cancer, and their data were reviewed. RESULTS: There were 56 men and 8 women with an average age of 69 years (range, 43 to 85 years). In the surgical group, there were no early postoperative deaths, and nonfatal complications occurred in 2 patients (10%). Among the 14 patients with stage I cancer, a second primary lung cancer developed in 5 (36%). The causes of death in the surgical group were cancer related in 7 patients, exacerbation of idiopathic pulmonary fibrosis in 7, and other in 2. Five of the 7 patients who died of a cancer-related cause had development of a second primary lung cancer. The actuarial 2-year survival rate of the surgical group was 52% overall, 58% for patients with N0 or N1 disease and 25% for those with N2 disease (p = 0.05). CONCLUSIONS: The long-term results in one surgical group were poor partly because of the high incidence of a second primary lung cancer and partly because of the poor natural history of idiopathic pulmonary fibrosis. These patients require intensive surveillance even after curative resection of lung cancer.

Adult↗
PubMed2004

[Perioperative lung injury: acute exacerbation of idiopathic pulmonary fibrosis and acute interstitial pneumonia after pulmonary resection].

The mortality rate after surgical resection for lung cancer has been reported to range between 1% and 3%, with 30% caused by acute exacerbation of idiopathic pulmonary fibrosis (IPF) or acute interstitial pneumonia (AIP). Approximately 20% of patients with IPF have lung cancer, while 2% to 4% of lung cancer patients have IPF. The incidence of postoperative acute exacerbation of IPF is about 20%. Some investigations in Japan revealed that 10% to 17% of lung cancer patients undergoing lung resection, who have not been diagnosed with IPF preoperatively, have localized-usual interstitial pneumonia (Lo-UIP) lesions. Approximately 20% of patients with Lo-UIP show postoperative acute exacerbation, while about 0.5% of those without Lo-UIP develop AIP after surgery. There is no confirmed treatment or prophylaxis. Most patients who develop postoperative acute exacerbation or AIP are treated with methylpredonisolone (1,000 mg/day x 3 days), but the mortality rate is 50% or greater. We emphasize that more efforts should be made to develop strategies to prevent postoperative acute exacerbation of IPF and AIP.

Acute Disease↗
PubMed1998

Heterogeneous point mutations of the p53 gene in pulmonary fibrosis.

Lung cancer is a frequent complication in pulmonary fibrosis. Overexpression of p53 proteins has been demonstrated by immunostaining in bronchoepithelial cells in patients with idiopathic pulmonary fibrosis. However, it is still unclear whether this overexpressed p53 protein is wild-type or mutant. It was hypothesized that pulmonary fibrosis may be a precancerous lesion with deoxyribonucleic acid point mutations in bronchoepithelial cells. Mutations of the p53 gene were tested for by fluorescence-based single-strand conformation polymorphism (FSSCP), cloning-sequencing and immunostaining techniques. Out of 10 tissue samples that demonstrated overexpression of p53 protein by immunostaining, nine (90%) exhibited point mutations and eight (80%) exhibited heterogeneous point mutations of the p53 gene. The mutations found in pulmonary fibrosis were scattered throughout the central part of the p53 gene, and both guanine (G):cytosine (C) to adenine (A):thymine (T) and A:T to G:C transitions were frequently observed. In conclusion, frequent heterogeneous point mutations of the p53 gene were detected in pulmonary fibrosis. These mutations may have resulted from several types of deoxyribonucleic acid damage that occurred in bronchoepithelial cells and this may explain previous findings of a very high incidence of lung cancer complicating pulmonary fibrosis.

Adult↗
PubMed1995

Can CT distinguish hypersensitivity pneumonitis from idiopathic pulmonary fibrosis?

OBJECTIVE: The clinical management of patients with idiopathic pulmonary fibrosis differs markedly from that of patients with hypersensitivity pneumonitis. However, the two diseases often cannot be differentiated on clinical grounds. The purpose of this study was to establish whether CT can be used to make the distinction. MATERIALS AND METHODS: Thirty-six patients with idiopathic pulmonary fibrosis and 27 patients with hypersensitivity pneumonitis were studied. All diagnoses were confirmed or supported by open lung biopsy. Three of the patients with idiopathic pulmonary fibrosis had desquamative interstitial pneumonia, and the remainder had usual interstitial pneumonia. In 19 of the 27 patients with hypersensitivity pneumonitis, the disease was chronic (symptoms lasting more than 1 year), while eight had acute or subacute symptoms. Two radiologists, who had not previously seen any of the cases and were blinded to the diagnosis, reviewed the CT images by consensus. The extent and distribution of CT features (including ground-glass attenuation, honeycombing, and micronodules) were recorded. In each case, a CT diagnosis was made, and the level of diagnostic confidence was recorded. RESULTS: A CT diagnosis was made with a high level of confidence in 39 (62%) of 63 patients. In these patients, the CT diagnosis was correct in 35 cases (90%): 23 of 26 patients with a CT diagnosis of idiopathic pulmonary fibrosis and 12 of 13 patients with a CT diagnosis of hypersensitivity pneumonitis. In the three patients with desquamative interstitial pneumonia, the CT diagnoses were probable hypersensitivity pneumonitis in two cases and definite hypersensitivity pneumonitis in one case. Of the 19 patients with chronic hypersensitivity pneumonitis, only seven had a definite diagnosis of hypersensitivity pneumonitis based on CT findings; three had a definite diagnosis of idiopathic pulmonary fibrosis. The patients with idiopathic pulmonary fibrosis and usual interstitial pneumonia were more likely to have honeycombing and peripheral or lower lung zone predominance of disease, and less likely to have micronodules, than were patients with chronic hypersensitivity pneumonitis. The patients with idiopathic pulmonary fibrosis and desquamative interstitial pneumonia had widespread ground-glass opacity indistinguishable from some cases of acute or subacute hypersensitivity pneumonitis. CONCLUSION: Our results show that CT can be used to distinguish idiopathic pulmonary fibrosis from hypersensitivity pneumonitis in most but not all cases. Desquamative interstitial pneumonia cannot reliably be distinguished from acute or subacute hypersensitivity pneumonitis. Chronic hypersensitivity pneumonitis may have findings identical to those of usual interstitial pneumonia. Lung biopsy should still be considered the gold standard for diagnosis of interstitial lung disease.

Alveolitis, Extrinsic Allergic↗
PubMed1998

Measurement of hepatocyte growth factor in serum and bronchoalveolar lavage fluid in patients with pulmonary fibrosis.

The present study evaluated the clinical significance of hepatocyte growth factor (HGF) in patients with pulmonary fibrosis. Twenty-one patients with a diagnosis of pulmonary fibrosis [14 with idiopathic pulmonary fibrosis (IPF) and seven with pulmonary fibrosis associated with a collagen vascular disorder (PF-CVD]) and 21 normal subjects as control were studied. HGF levels in sera of patients with pulmonary fibrosis (0.34 +/- 0.02 ng ml-1) were elevated significantly as compared with normal subjects (0.21 +/- 0.01 ng ml-1) (P < 0.0001). HGF/albumin levels in broncho-alveolar lavage fluid (BALF) of patients with pulmonary fibrosis (72 +/- 17 ng g-1 albumin) were also significantly elevated as compared with normal subjects (under the detection limit) (P < 0.01). HGF levels in sera correlated significantly with elastase levels in sera and C-reactive protein, and correlated negatively with PaO2. HGF levels in sera were significantly higher in smokers with pulmonary fibrosis (0.42 +/- 0.03 ng ml-1) as compared with non-smokers with pulmonary fibrosis (0.29 +/- 0.03 ng ml-1) (P < 0.005). HGF/albumin levels in BALF correlated significantly with elastase/albumin levels in BALF, lactate dehydrogenase/albumin in BALF, Immunoglobulin A/albumin in BALF, total cell count/albumin in BALF, total number of alveolar macrophage/albumin in BALF, total number of neutrophil/albumin in BALF, CEA/albumin in BALF, CA19-9/albumin in BALF, and SCC/albumin in BALF. These results suggest that following lung injury, HGF may be a mediator involved in the repair which leads to pulmonary fibrosis.

Adult↗
PubMed2006

Inhibitory effects of Hu-qi-yin on the bleomycin-induced pulmonary fibrosis in rats.

The currently accepted approaches to treatment of pulmonary fibrosis are based on the treatment of alveolitis and pulmonary fibrosis, however clinically available anti-inflammatory and antifibrotic agents are not often beneficial. Hu-qi-yin, one of the traditional Chinese herbal formulas, has been used for clinical therapy of pulmonary fibrosis in China. The aim of the present study is to evaluate the preventive effects of Hu-qi-yin on the bleomycin (BLM)-induced pulmonary fibrosis in rats. The degree of fibrosis was evaluated with hydroxyproline contents in serum and lung tissue 28 days post-BLM instillation. The semi-quantitative analyses of lung sections were conducted to evaluate the intensity of alveolitis and fibrosis. Furthermore, the expression of transforming growth factor-beta(1) (TGF-beta(1)) was quantitatively studied at the protein and mRNA levels by immunohistochemistry and in situ hybridization, respectively. Oral treatment with Hu-qi-yin improved the body weight loss of rats in doses of 3.8, 7.6g/kg compared with BLM-treated control group, and significantly inhibited the alveolitis and pulmonary fibrosis in a dose-dependent manner as reflected by decreases of the hydroxyproline contents of serum and lung, and amelioration of alveolitis and pulmonary fibrosis fraction 28 days after BLM administration. The one of the possible mechanisms of the protective effect of Hu-qi-yin was via reduction of the overexpression of TGF-beta(1) protein and mRNA. These results suggested a great potential that Hu-qi-yin might be effective in the treatment of pulmonary fibrosis.

Administration, Oral↗
PubMed1995

Suppressive effect of anti-alpha 1-antichymotrypsin serum on pulmonary fibrosis induced by phorbol myristate acetate in vivo.

BACKGROUND: PMA induces pulmonary fibrosis in the rabbit (1). Pulmonary fibrosis induced by PMA occurs in the alveolar wall and has the same pattern as idiopathic pulmonary fibrosis (IPF)(2), so this system can be used as an animal model for IPF. PMA also increases the content of alpha-1-antichymotrypsin (ACT) in cultured alveolar macrophages of bronchoalveolar lavages (BAL), and dexamethasone inhibits this PMA-induced increase (3). Here we investigated the role of ACT in pulmonary fibrosis induced by PMA. EXPERIMENTAL DESIGN: Rabbits were treated intratracheally for 6 days with saline, dimethyl sulphoxide (DMSO) used as a solvent of PMA, PMA dissolved in DMSO or PMA plus anti-ACT rabbit serum. BAL samples were obtained. ACT in cell pellet and cell-free fluid of BAL were assayed by radioimmunoassay. Sections of the lung were examined histologically by a point count method. The ratio of fibrosis to elastosis (fibrotic ratio) was evaluated for each rabbit by the ratio of total points of collagen stained by the Azan-Mallory method to those of elastic fiber stained by the Elastica van Gieson method. Hydroxyproline (HP) was assayed biochemically, and the amount of HP in the alveolar wall for each rabbit was calculated using the assayed values of HP and the ratio of histologic collagen points in the alveolar wall to those in the lung tissue by a point count method. RESULTS: The fibrotic ratio of the PMA group increased fourfold compared with that of the saline group. The ratio of the PMA plus anti-ACT group decreased and was similar to that of the saline group. The ratio of the DMSO group was about two times as much as that of the saline or the PMA plus anti-ACT groups. The calculated amount of hydroxyproline in the alveolar wall of the PMA group increased and was approximately 1.5-fold compared with that of the saline group. The amount of HP of the PMA plus anti-ACT group decreased and was similar to that of the saline group. In the BAL, the amount and the percentage of ACT in cell pellet per macrophage of the PMA group increased more than those of the saline and DMSO groups. The amount and percentage of the PMA plus anti-ACT group were significantly less than those of the PMA group. Those of the DMSO group were similar to those of the saline group. CONCLUSIONS: These findings suggest that anti-ACT has a suppressive effect on pulmonary fibrosis induced by PMA and that ACT is important in the PMA model of pulmonary fibrosis.

Animals↗
PubMed1985

[Respiratory function and alveolar biological changes under the effect of CDP-choline in pulmonary interstitial pathology: pulmonary fibrosis and sarcoidosis].

Various anomalies of pulmonary surfactant have been described in relation to acute respiratory distress syndromes, hypersensitivity lung disease and pulmonary sarcoidosis. Phosphatidylcholine (PC) is the essential phospholipid component of pulmonary surfactant. Cytidine diphosphocholine (CDP-choline) is an essential intermediary in the biosynthesis of PC. The authors studied two groups of patients: one group consisted of diffuse interstitial pulmonary fibrosis and the other consisted of pulmonary sarcoidosis with parenchymal involvement. They observed quantitative and qualitative abnormalities of the phospholipid fractions of surfactant and more particularly of PC. The finding of a marked decrease in this phospholipid, especially in the cases of pulmonary fibrosis, justified the study of the therapeutic effects of CDP-choline. After one month of treatment with this substance, at a dose of 1 g I.M. per day, the PC fraction had returned to normal and, at the same time, there was an improvement in the PaO2 at rest and after exercise. Long term administration of CDP-choline appears to be valuable in the maintenance of the phospholipid equilibrium of pulmonary surfactant and in the improvement of the quality of alveolar gas exchange.

Adult↗
PubMed2001

Idiopathic pulmonary fibrosis and malignancy.

The reported frequency of lung cancer in the setting of diffuse pulmonary fibrosis varies greatly, depending on the country of origin and the type of study. Most recent reports regarding diffuse pulmonary fibrosis in general and idiopathic pulmonary fibrosis in particular and lung cancers come from Japan; only a few clinical studies of this issue are available from other countries of the world, including the United States. The reported frequency ranges from 4.8% in the United States to 48.2% in Japan. The most frequent type of cancer is adenocarcinoma. Risk factors may include cigarette smoking, exposure to metal dusts, onset of idiopathic pulmonary fibrosis at an older age, and male predominance. Possible pathologic mechanisms are summarized. Given the very poor prognosis of idiopathic pulmonary fibrosis itself, with a mean survival of only 2.8 years, and that different diagnostic criteria were used in each study, it is likely that many of these studies are flawed because they evaluate lesions other than idiopathic pulmonary fibrosis. Thus, the frequency of lung cancer in idiopathic pulmonary fibrosis is still uncertain, and clearly requires follow-up of cohorts of clinically well-characterized patients, using standard diagnostic criteria for idiopathic pulmonary fibrosis. Finally, if the association between idiopathic pulmonary fibrosis and lung cancer is reconfirmed in these studies, the molecular and genetic mechanisms governing the development of lung cancer in this setting require additional study.

Biopsy↗
PubMed1989

Dietary fish oil inhibits bleomycin-induced pulmonary fibrosis in the rat.

Intratracheal bleomycin induces pulmonary fibrosis in experimental animals, but the mechanisms involved are poorly understood. Since altered levels of fatty acid metabolites are associated with bleomycin-induced lung injury, we examined the effects of a change in dietary fat on bleomycin-induced fibrosis. Previously we have shown that an essential fatty acid-deficient diet can reduce the severity of bleomycin-induced pulmonary fibrosis. The present study examined the effect of replacement of usual dietary fat with menhaden oil, rich in eicosapentaenoic acid, on the development of pulmonary fibrosis. Weanling rats were raised on a standard laboratory diet or a diet consisting of a fat-free powder to which was added 25% (w/w) of menhaden oil. After 8 weeks of feeding, the animals received either 1.5 units of bleomycin or an equivalent volume of saline intratracheally. In animals receiving the laboratory diet, bleomycin treatment produced a 44% increase in total lung protein content when compared to saline-treated controls (p less than 0.001) and a 77% increase in total lung hydroxyproline content (p less than 0.01). In contrast, bleomycin-treated animals receiving the menhaden oil diet had only small increases, which did not reach statistical significance, in protein and hydroxyproline content in the lung. Bronchoalveolar lavage cellularity did not differ among the treatment groups, but the percentage of lavage macrophages was slightly diminished in bleomycin-treated animals receiving the laboratory diet. Cellular differentials of lavage fluid did not differ significantly between bleomycin- and saline-treated animals receiving the menhaden oil diet. Bleomycin-induced histologic changes, quantitated by morphometric analysis, were significantly reduced with the menhaden oil diet. We conclude that a diet rich in eicosapentaenoic acid can significantly ameliorate bleomycin-induced pulmonary fibrosis, possibly via alterations in eicosanoid metabolism.

Animals↗
PubMed2003

The role of gastroesophageal reflux in idiopathic pulmonary fibrosis.

Fibroblast foci are indicative of idiopathic pulmonary fibrosis and appear to be a cellular attempt to repair the damaged alveolus. Although this progressive, often fatal, clinical syndrome is thought to be dependent on alveolar injury of unknown origin, significant clinical and preclinical evidence points to gastric acid as a causative harmful agent. Graded instillation of various forms of acid in several animal models resulted in aspiration-induced lung injury, including pulmonary fibrosis in pigs. Moreover, compelling clinical data suggest that a high percentage of patients with idiopathic pulmonary fibrosis also experience abnormal esophageal acid exposure, without necessarily experiencing the typical symptoms of gastroesophageal reflux disease (GERD). Aggressive, long-term therapeutic trials of patients with GERD and evaluation of the therapeutic effects on pulmonary disease will allow determination of the real influences of abnormal esophageal acid exposure in the development of idiopathic pulmonary fibrosis.

Algorithms↗
PubMed2001

Outcome of mechanical ventilation for acute respiratory failure in patients with pulmonary fibrosis.

OBJECTIVE: During the course of idiopathic pulmonary fibrosis patients may need invasive mechanical ventilation because of acute respiratory failure. We reviewed the charts of all patients with idiopathic pulmonary fibrosis admitted to our ICU for mechanical ventilation to describe their ICU course and prognosis. DESIGN AND SETTING: Retrospective, observational case series, from December 1996 to March 2001, in an 18-bed medical ICU in a tertiary university hospital. PATIENTS: Fourteen consecutive patients with idiopathic ( n=11) or secondary ( n=3) pulmonary fibrosis admitted to the medical ICU for mechanical ventilation. MEASUREMENTS AND RESULTS: Relevant factors of history and hospital course such as diagnostic and therapeutic interventions were retrieved as well as laboratory and radiological results. All patients were admitted for severe acute hypoxemic respiratory failure (PaO(2)/FIO(2) 111+/-64 mmHg), with a high clinical suspicion of lower respiratory tract infection. Despite ventilatory support and adjunctive therapies (antibiotics, steroids, or immunosuppressive drugs), all patients gradually worsened and eventually died in the ICU after a mean stay of 7.6+/-4.6 days. CONCLUSIONS: In this study mechanical ventilation for acute respiratory failure in pulmonary fibrosis patients was associated with a 100% mortality, despite aggressive therapeutic and diagnostic procedures.

Acute Disease↗
PubMed2000

Sarcoidosis with pulmonary fibrosis: CT patterns and correlation with pulmonary function.

OBJECTIVE: The purpose of our study was to identify CT patterns of pulmonary fibrosis in patients with sarcoidosis and to correlate these patterns with pulmonary function tests. MATERIALS AND METHODS: We conducted a retrospective review of CT scans of 80 patients with proven sarcoidosis and evidence of fibrotic changes on chest radiographs. RESULTS: Three main CT patterns were identified: bronchial distortion (n = 38, 47%), mainly central; honeycombing (n = 23, 29%), mainly peripheral; and linear (n = 19, 24%), mainly diffuse. In most cases, a pattern was clearly identified as shown by the good agreement between observers (kappa = 0.87). Nodules were significantly associated with the linear (87%) and distorted (71%) patterns, but not with the honeycomb pattern (35%). The honeycomb pattern was most often associated with restriction and decreased lung diffusing capacity for carbon monoxide. Patients with bronchial distortion had lower expiratory airflow rates. The linear pattern was generally associated with the least functional impairment. CONCLUSION: CT may be a useful tool for defining subgroups of patients with fibrotic pulmonary sarcoidosis. CT reveals three main patterns that may reflect different distributions of fibrotic lesions in the lung with different functional pulmonary impairments. The persistence of active pulmonary lesions suggested by the presence of nodular lesions was often associated with linear and distorted patterns.

Adolescent↗