Idiopathic pulmonary fibrosis in Sweden: report from the first year of activity of the Swedish IPF-Registry.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is an emerging problem in the western world, being related to increasing age and implying significant costs for the diagnosis and management of affected patients. The epidemiology of IPF is not well understood. METHODS: To allow estimates of the problem and eventually to evaluate quality of the care of IPF patients in Sweden, a national IPF Registry was started in the autumn of 2014. Data on criteria used to diagnose IPF, demographics, lung function, and quality of life (measured with the King's Brief Interstitial Lung Disease Questionnaire, K-BILD) were reported directly to the registry, based at the coordinating centre (Karolinska University Hospital, Stockholm, Sweden) via a web-based platform. RESULTS: During the first year, the registry was implemented in 11 (33%) of the 33 respiratory units in the country. Seventy-one patients were registered between October 2014 and October 2015, 50 (70.4%) males and 21 (29.6%) females. Median age was 70 (range 47-86). The mean K-BILD score at the first inclusion in the registry was 54.3+9.5. CONCLUSIONS: The main features of IPF patients in this first Swedish cohort were consistent with data published in the literature in main multinational randomized controlled trials. The K-BILD questionnaire showed that quality of life of patients with IPF and their perception of the disease are quite poor at the time of inclusion in the registry.

Aberrant nonfibrotic parenchyma in idiopathic pulmonary fibrosis is correlated with decreased ß-catenin inhibition and increased Wnt5a/b interaction.

Idiopathic pulmonary fibrosis (IPF), an insidious disease with grave prognosis, is characterized by heterogeneous fibrosis with densely fibrotic areas surrounded by nonfibrotic normal-looking tissue, believed to reflect a temporal development. The etiology is incompletely elucidated, but aberrant wound healing is believed to be involved. Embryonic signaling pathways, including Wnt signaling, are reactivated in wound healing, and we therefore aimed to investigate Wnt signaling, and hypothesized that Wnt signaling would correspond to degree of fibrosis. Material from 10 patients with IPF were included (four diagnostic biopsies and six donated lungs) and compared to healthy controls (n = 7). We investigated markers of Wnt signaling (ß-catenin, Wnt3a, ICAT, Wnt5a/b, DAAM1 and NLK) histologically in lung parenchyma with variable degree of fibrosis. Our results suggest that Wnt signaling is significantly altered (P < 0.05) already in normal-looking parenchyma. The expression of Wnt3a and ICAT decreased (both P < 0.01) in IPF compared to healthy lungs, whereas ß-catenin, Wnt5a/b, DAAM1 and NLK increased (P < 0.05 for all). ICAT is further decreased in dense fibrosis compared to normal-looking parenchyma in IPF (P < 0.001). On the basis of our results, we conclude that from a Wnt perspective, there is no normal parenchyma in IPF, and Wnt signaling corresponds to degree of fibrosis. In addition, ß-catenin and Wnt5a appears coupled, and decreased inhibition of ß-catenin may be involved. We suggest that the interaction between ß-catenin, ICAT, and Wnt5a/b may represent an important research area and potential target for therapeutic intervention.

Middle lobe syndrome: a nationwide study on clinicopathological features and surgical treatment.

INTRODUCTION: Middle lobe syndrome (MLS) is a relatively uncommon lung disease that is characterized by damage to the middle lobe and often needs surgical intervention. OBJECTIVE: To study clinical, radiological and histological features of all patients who underwent surgical resection for MLS in Iceland over a 13-year period, including evaluation of surgical outcome. METHODS: Information on patients who underwent surgery of the right middle lobe in Iceland from 1984 to 2006 was obtained from a centralized diagnosis and pathology registry. Clinical data were collected retrospectively from clinical records from hospitals and from private offices. All pathology specimens were reviewed. RESULTS: We studied 18 patients, 3 males and 15 females between the ages 2 and 86 years (mean 55). The most common clinical features were recurrent infection (n = 15), chronic cough with productive sputum (n = 9), chest pain (n = 8) or dyspnea (n = 7). The most common findings on chest radiographs and on computerized tomography of the chest were atelectasis, consolidation and bronchiectasis. One patient had a foreign body. The most common major histological finding was bronchiectasis in nine patients, and two had foreign body reaction. Minor findings included bronchiolitis, organizing pneumonia and peribronchial inflammation. All patients survived surgery with minor peri- and postoperative complications. CONCLUSION: MLS is more common in females, and recurrent infections, chronic productive cough and dyspnea were the most common symptoms. Bronchiectasis is the most common histological finding. MLS can be treated effectively with lobectomy with low mortality and rate of complications.

[Closed needle pleural biopsy in Iceland 1990-1999.].

OBJECTIVE: Closed pleural biopsy is done to determine causes of pleural effusion after fluid analysis has been nondiagnostic. The aim of the study was to evaluate results of closed pleural biopsy in Iceland. MATERIAL AND METHODS: All pathology reports of closed pleural biopsy from 1990-1999 at the Department of Pathology were reviewed. Hospital records for these patients were screened. RESULTS: There were 130 samples from 120 individuals. There were 74 males and 46 females. Deceased are 75/120. The most common diagnosis was pleuritis or fibrosis in 85/120, cancer in 15 and three had tuberculosis but 17/120 had other results. The sensitivity of a closed pleural biopsy for diagnosing cancer was 27%. Adding cytology increased the sensitivity to 56%. Of those that had pleuritis on initial biopsy the cause was found to be cancer in 33/85, pneumonia in 11, trauma in 5 and in 8 there were other causes. In 25/85 no etiology could be found. By doing further studies it was demonstrated that the cause for the fluid was malignancy in 55/120. Of those most had lung cancer or 24/55, and the second most common cause was breast cancer in 7. Despite further studies no cause was found in 32/85. CONCLUSIONS: Closed pleural biopsy has fairly low sensitivity for diagnosis of cancer but it can be increased by adding cytologic evaluation. It is necessary to do further investigations and follow-up in patients that have inflammation in pleural biopsy.