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Background: The present survey coordinated by the French expert centres for rare pulmonary diseases investigated French pulmonologists' current diagnostic and therapeutic practice for idiopathic pulmonary fibrosis (IPF)., Methods: From December 7, 2011 to February 18, 2012, all French pulmonologists (n = 2608) were contacted. Those who reported following up at least one IPF patient (n = 509) were administered a 26-item questionnaire by phone or e-mail., Results: 509 pulmonologists (41% of responders, 20% of French pulmonologists) were involved in the management of IPF patients. Of those, 36% discussed the cases with radiologists and pathologists. Out of 406 community pulmonologists practicing outside of reference or competence (e.g. expert) centres, 141 (35%) indicated referring patients to those centres. The 2011 international guidelines for IPF were known by 67% of pulmonologists involved in IPF, 84% of whom considered them appropriate for practice. About 58% of patients were diagnosed with mild to moderate IPF as defined by percentage predicted forced vital capacity ≥50% and percentage predicted diffusing capacity of the lung for carbon monoxide ≥35%. Management resulted from multidisciplinary discussion in 36% of the cases. By the end of December 2011, 49% of patients with mild to moderately severe IPF were treated with oral corticosteroids, and 27% received no treatment., Conclusions: Despite correct awareness of international IPF guidelines, modalities of multidisciplinary discussion and of early diagnosis and management need to be improved through the network of expert centres., (Copyright © 2013. Published by Elsevier Ltd.)

Introduction: Urinary stress incontinence affects 10% to 30% of the female population and may have a major impact on psychosocial health. In interstitial lung disease, chronic cough may lead to development of urinary incontinence, but the prevalence and impact of this symptom are unknown., Objectives: To determine the rate and impact of urinary stress incontinence among women with chronic cough due to interstitial lung disease., Methods: 28 female patients with chronic cough secondary to interstitial lung disease and 15 controls were evaluated by questionnaires to determine the prevalence of cough-related urinary incontinence, its severity, and its impact on quality of life., Results: Cough-related urinary incontinence was present in 14/28 patients with interstitial lung disease and chronic cough (50%), but in only 1/15 controls (7%, p=0.005). On a 5-points quality of life scale, the median impact of urinary incontinence was 3 (minimum=1, maximal=5), and the median impact of chronic cough was 3.5. The majority of patients (64%) believed that incontinence was a natural phenomenon due to ageing, all were ashamed by this symptom and 79% were unable to mention it to their caring physician. Only one physician had previously addressed this issue., Conclusion: Cough-related urinary incontinence is common in patients with interstitial lung disease and is largely overlooked. It may significantly alter quality of life. A systematic questioning by the physician would allow to promptly refer these patients for appropriate therapeutic interventions, such as perineal training.

The real need for extensive staging at the time of diagnosis is discussed in regard to small cell lung carcinoma. We performed a decisional retrospective analysis on a series of 182 patients, based on three staging steps: the first step included physical examination and routine biologic tests. The second step consisted of liver ultrasonography and needle aspiration of any clinically detectable tumor mass, and the third step included bone marrow examination, radionuclide bone scan, thoracic, abdominal, and brain CT scan. A stepwise multivariate logistic regression performed on 11 variables considered in the first step shows that a four-parameter model can predict the spread of the disease (limited or extensive): weight loss, performance status, and elevated LDH or alkaline phosphatase levels. Limited disease can be predicted in two ways: (1) elevated LDH with normal alkaline phosphatases, no weight loss, and good performance status, or (2) normal LDH and alkaline phosphatases. In this series, 28 percent of patients can be predicted as having extensive disease and can be treated with chemotherapy alone without chest irradiation. After the second step, the probability of disease being extensive is only 25 percent, and only 84 (46.15 percent) patients would need to undergo the third step of staging procedures (brain CT scan, bone marrow aspiration and biopsy, radionuclide bone scan) with this method. We conclude that a multistep approach represents a simple staging method and offers the advantage of harmlessness and lower costs for patients not to be evaluated in prospective clinical trials.

In 40 to 60% of bone marrow grafts there are pulmonary complications of which the most frequent is the occurrence of an interstitial pneumonia. We report 7 cases here of a more rare complication, that of bronchiolitis obliterans (BO). Between December 1979 and November 1989, 7 patients (3.4% of our cases of GMO) have developed over several months a chronic obstructive respiratory failure (a mean VEMS of 43% of the theoretical value) in the year following the transplantation (mean delay 190 days). 6 patients presented with cutaneous, digestive or hepatic signs of chronic graft v host illness (GVH) whereas the prevalence of this complication in the population studied was 17%. Treatment combining bronchodilators and immunosuppressants was only successful in 2 cases and the outcome was fatal in the 5 other cases as a result of respiratory failure (mean delay 208 days between the appearance of respiratory symptoms and death). The pathogenesis of BO after GMO remains poorly understood. It may rest on an immune process during the course of which the BO would be the result of a chronic pulmonary GVH. Another hypothesis is that the state of the immunosuppression in these patients would favour the appearance of a bronchiolitis of an infectious origin, particularly viral. The prognosis of BO after GMO is very poor and in the absence of specific effective treatment the therapeutic strategy remains essentially that of prevention by the early detection of respiratory anomalies.

A cyclic and sequential combination of adriamycin, vincristine (or VM 26) and cyclophosphamide was used, with a follow up of 4 to 33 months in 38 patients with a "micro-cellular" carcinoma of the lung. Radiotherapy was used in association in a variable manner. Chemotherapy alone resulted in marked regression of the lesions (regression is greater than 50%) in approximately 65 per cent of cases and a level of complete regression which varied from 31 to 45 per cent according to the degree of diffusion of the lesions at the time of admission. In this study, survival in apparently localised forms was 70 per cent after one year and 55 per cent at eighteen months, whilst it was 34 per cent at one year and nil at eighteen months in patients with evidence of metastatic disease at the time of entry. These encouraging results indicate a hope for improvement, in certain patients reacting favourably to this protocol, in the still grave prognosis of "small cell" carcinoma of the lung.