BACKGROUND Lung cancer (LC) is frequently associated with idiopathic pulmonary fibrosis (IPF). Despite this well-known association, the outcome of LC in patients with IPF is unclear. The objective of ...this study was to evaluate the impact of LC on survival of patients with associated IPF. METHODS A total of 260 patients with IPF were reviewed, and 186 IPF cases had complete clinical and follow-up data. Among these, five cases were excluded because LC was radiologically suspected but not histologically proven. The remaining 181 cases were categorized in two groups: 23 patients with biopsy-proven LC and IPF (LC-IPF) and 158 patients with IPF only (IPF). Survival and clinical characteristics of the two groups were compared. RESULTS Prevalence of histologically proven LC was 13%, and among those with LC-IPF cumulative incidence at 1 and 3 years was 41% and 82%. Patients with LC were more frequently smokers (91.3% vs 71.6%, P = .001), with combined pulmonary fibrosis and emphysema (52% vs 32%, P = .052). Survival in patients with LC-IPF was significantly worse than in patients with IPF without LC (median survival, 38.7 months vs 63.9 months; hazard ratio = 5.0; 95% CI, 2.91-8.57; P < .001). Causes of death in the study group were respiratory failure in 43% of patients, LC progression in 13%, and LC treatment-related complications in 17%. CONCLUSIONS In patients with IPF, LC has a significant adverse impact on survival. Diagnosis and treatment of LC in IPF are burdened by an increased incidence of severe complicating events, apparently as lethal as the cancer itself.
Background Anaphylaxis after Hymenoptera stings has been reported in subjects with mastocytosis, but few data exist regarding disease prevalence in populations allergic to these insects. Objective ...The incidence of clonal mast cell (MC) disorders in subjects with both systemic reactions to Hymenoptera stings and increased serum baseline tryptase (sBT) levels was assessed by using bone marrow (BM) aspirates and biopsy specimens. Methods Subjects with a history of a systemic reaction caused by a Hymenoptera sting underwent the standard diagnostic work-up for Hymenoptera allergy, and sBT levels were measured. Subjects with an increased sBT level had BM evaluation that included histology/cytology, flow cytometry, and detection of KIT mutations. Results Forty-four (11.6%) of 379 subjects with systemic reactions had increased sBT levels (>11.4 ng/mL), and 31 (70.5%) of these had a history of anaphylaxis. Thirty-four subjects with increased sBT levels underwent a BM analysis. Histology detected diagnostic or subdiagnostic MC infiltrates in 22 (65%) of 34 patients. Abnormal MCs were identified by means of flow cytometry and cytology in 26 (78.8%) of 33 and 20 (58.8%) of 34 subjects, respectively. A KIT mutation was detected in 17 (54.8%) of 31 subjects. The diagnosis was indolent systemic mastocytosis in 21 (61.7%) of 34 subjects and monoclonal MC activation syndrome in 9 (26.5%) of 34 subjects. All subjects with anaphylaxis had one of those 2 disorders. Conclusion The concomitant presence of systemic reactions (especially anaphylaxis) after Hymenoptera stings and increased sBT levels strongly suggests that a BM examination is indicated for the diagnosis of clonal MC disease.
On the basis of these considerations and previous clinical observations,6 we vaccinated several patients with SM without observing important side effects. ...almost all of them achieved a relevant ...protection and decreased their grade of reaction from IV to I. The 2 patients who maintained a grade IV reaction on maintenance SIT could achieve a benefit by increasing the maintenance dose.8 We are aware that the population evaluated in our study is small, but if we take into account the rarity of SM and the low frequency of severe HVA, this study includes the largest cohorts of patients with HVA and SM treated with SIT. An accessory observation is that SM was indeed diagnosed in 16 of 121 patients with severe HVA. ...it can be supposed that severe HVA could be a clinical manifestation of underlying SM, as reported with idiopathic anaphylaxis.9 This would support the usefulness of tryptase assay screening in patients with Hymenoptera-induced anaphylaxis.
IgG4 -related disease was first described in adults with autoimmune pancreatitis but is now known to affect multiple organs. Lung involvement has never been described in children to our knowledge. ...Here, we report an adolescent presenting with recurrent dry cough and hemoptysis who was found to have venous ectasia in the left upper lobe, and diffuse bronchiectasis. Sustained high levels of IgG4 (1,090 mg/dL) were found, and the endobronchial biopsy revealed a marked infiltration of plasma cells producing IgG4 (ratio of IgG4 plasma cells to IgG plasma cells >50%). This unique case highlights the occurrence of IgG4 -related disease in a child and underscores the importance of careful scrutiny of all investigations in complex pediatric respiratory cases.
Summary Digital pathology allows networks of “remote” specialist pathologists to report the findings of preimplantation kidney biopsies. We sought to validate the assessment of preimplantation kidney ...transplant biopsies for diagnostic purposes using whole-slide images according to the recommendations of the College of American Pathologists. Sixty-two consecutive, previously reported, preimplantation kidney biopsies were scanned using the ScanScope Digital Slide Scanner at 0.5 μ m/pixel (20× objective). The slides were assessed for percent glomerulosclerosis, tubular atrophy, interstitial fibrosis and vascular narrowing using the Remuzzi criteria by two pathologists, one using glass slides and the other using the whole-slide images viewed on a widescreen computer monitor. After a 2-week washout period, all of the slides were re-assessed by the same pathologists using the opposite mode of reporting to that used in the first evaluation. Very high glass-digital intraobserver concordance was achieved for the overall score and for individual grades by both pathologists ( κ range, 0.841-0.973). The overall scores obtained by both pathologists and using both methods were identical. The times needed to assess the biopsies were 14 minutes when using a light microscope and 18 minutes, including scanning time, which averaged 2 minutes 20 seconds per slide, when using digital microscopy. Digital microscopy is a reliable, fast, and safe method for the assessment of preimplantation kidney biopsies.
