Re-evaluation of diagnostic parameters is crucial for obtaining accurate data on idiopathic pulmonary fibrosis.

BACKGROUND: The FinnishIPF registry is a prospective, longitudinal national registry study on the epidemiology of idiopathic pulmonary fibrosis (IPF). It was designed to describe the characteristics, management and prognosis of prevalent and incident IPF patients. The study was initiated in 2012. METHODS: We present here results limited to five university hospitals. Patients with IPF were screened from hospital registries using ICD-10 diagnosis codes J84.1 and J84.9. All patients who gave informed consent were included and evaluated using novel diagnostic criteria. Point prevalence on the 31(st) of December in 2012 was calculated using the reported population in each university hospital city as the denominator. RESULTS: Patients with ICD-10 codes J84.1 and J84.9 yielded a heterogeneous group - on the basis of patient records assessed by pulmonologists only 20-30 % of the cases were IPF. After clinical, radiological and histological re-evaluation 111 of 123 (90 %) of patients fulfilled the clinical criteria of IPF. The estimated prevalence of IPF was 8.6 cases/100 000. 60.4 % were men. Forty four percent of the patients were never-smokers. At diagnosis, the patients' mean age was 73.5 years and mean FVC was 80.4 % and DLCO 57.3 % of predicted. CONCLUSIONS: Our results suggest that hospital registries are inaccurate for epidemiological studies unless patients are carefully re-evaluated. IPF is diagnosed in Finland at a stage when lung function is still quite well preserved. Smoking in patients with IPF was less common than in previous reports.

Large bowel obstruction in a young woman simulating a malignant neoplasm: a case report of actinomyces infection.

Pelvic and intra-abdominal Actinomycosis can be difficult to diagnose preoperatively and it may also mimic many other diseases, including malignancies. We present a patient with pelvic Actinomycosis probably caused by a long-standing intrauterine device (IUD). We emphasize the challenges in diagnostic process and stress that though a rare disease, intra-abdominal Actinomycosis should be suspected in cases with intra-abdominal mass of uncertain etiology. The early recognition may spare the patient from extensive surgical operation.

Apoptotic activity is increased in the newly formed fibromyxoid connective tissue in bronchiolitis obliterans organizing pneumonia.

Bronchiolitis obliterans organizing pneumonia (BOOP) and usual interstitial pneumonia (UIP) are clinically and histologically distinguishable interstitial lung diseases both sharing the presence of the newly formed fibromyxoid connective tissue as a histologic feature. In BOOP the newly formed connective tissue is susceptible to even complete reversal, but in UIP it participates in the remodeling of the pulmonary tissue. In this study we have tested the hypothesis that the apoptotic activity in the fibromyxoid lesions is higher in BOOP than in UIP. Apoptotic activity in cells of the newly formed connective tissue in BOOP and UIP was visualized by TUNEL. Apoptosis-regulating proteins bcl-2, mcl-1, and bax were visualized by immunohistochemistry. The number of the apoptotic events was given as an apoptotic index giving the percentage of the apoptotic cells and bodies of the total cell number. Our results show that the apoptotic activity is clearly higher in the fibromyxoid lesions of BOOP (mean, 0.7; S.D., +/-0.51) compared with those of UIP (mean, 0. 13; S.D., +/-0.14, p < 0.003). The results thus suggest that apoptosis has an important role in the resolution process of the newly formed connective tissue in BOOP. The low level of apoptosis in UIP could, on the other hand, suggest that a decreased apoptosis in cells of the fibromyxoid stroma might pathogenetically relate to a decreased resolution of these lesions in UIP. No significant difference was found in the expression of bcl-2, mcl-1, and bax in BOOP and in UIP, suggesting that regulation of apoptosis might partly bypass the influence of these proteins.

Intraluminal fibromyxoid lesions in bronchiolitis obliterans organizing pneumonia are highly capillarized.

Bronchiolitis obliterans organizing pneumonia (BOOP) and usual interstitial pneumonia (UIP; ie, cryptogenic fibrosing alveolitis of mural type, CFA) are clinically and histologically distinguishable interstitial lung diseases. Both contain intraluminal lesions of newly formed fibromyxoid connective tissue. In BOOP, the fibromyxoid lesions are susceptible to complete reversal, but in UIP they are supposed to participate in the remodeling of the interstitium. Our hypothesis was that capillarization of the intraluminal fibromyxoid lesions is more frequent in BOOP compared with UIP. In this study, we stained diagnostic thoracoscopic or open lung biopsy specimens of patients with BOOP (n = 9) and UIP (n = 10) with antibodies against human laminin, von Willebrand factor, and CD34 to reveal the microvasculature of intraluminal fibromyxoid lesions. Our results show that in BOOP there is abundant capillarization in the newly formed intraluminal fibromyxoid lesions often reminiscent of granulation tissue. The mean number of capillaries per area unit (mm2) was 107 +/- SD 74 in samples stained for laminin, 103 +/- SD 46 for von Willebrand factor, and 63 +/- SD 36 for CD34. In marked contrast, in UIP, the corresponding accounts were significantly lower, being 14 +/- SD 15 for laminin (P < .003), 11 +/- SD 14 for von Willebrand factor (P < .001) and 6 +/- SD 6 for CD34 (P < .001). The intraobserver (P < .001) and interobserver correlations (P < .002) were highly significant, showing that our results are reproducible. We conclude that the content and nature of the newly formed intraluminal connective tissue, for example, in the form of vascular growth factors, are different in BOOP and in UIP, and this partly leads to the different clinical course of these diseases.