Survey of Physician's Knowledge, Attitudes, and Practices Regarding Idiopathic Pulmonary Fibrosis in Turkey.

OBJECTIVES: The aim of this study is to evaluate the approaches of Turkish pulmonologists to the diagnosis and treatment of idiopathic pulmonary fibrosis (IPF) in daily clinical practice. MATERIALS AND METHODS: A questionnaire containing 38 questions about the IPF diagnosis and treatment was given to pulmonologists between January 22 and 29, 2018, and the data of 158 physicians who responded to the questionnaire were evaluated. RESULTS: This survey showed that the mean number of patients that physicians followed up and managed annually was 8.3 and 5, respectively. The mean symptom duration before the diagnosis was 9-12 months. Patients were seen on average by three physicians prior to confirmed diagnosis. Almost 80% of the physicians have an opportunity to access a pathologist and radiologist specialized in IPF. However, only 26% of them have an opportunity to access regular multidisciplinary meetings. Although antifibrotics were the most commonly prescribed drugs, approximately 10% of patients were prescribed steroids, N-acetylcysteine, and immunosuppressants. Most of the physicians (81%) were aware of international guidelines; however, the Turkish Thoracic Society IPF Diagnosis and Treatment Consensus Report was read by only 41% of them. CONCLUSION: This survey may lead to the IPF awareness in Turkey, and it may help to close the gaps regarding the diagnosis and treatment.

Do higher cut-off values for tuberculin skin test increase the specificity and diagnostic agreement with interferon gamma release assays in immunocompromised Bacillus Calmette-Guérin vaccinated patients?

PURPOSE: Immunocompromised patients with latent tuberculosis infection (LTBI) are at high risk of progression to active tuberculosis. Detection and treatment of LTBI in this group of patients are very important to control active tuberculosis. Tuberculin skin test (TST) and interferon gamma release assays (IGRAs) are two methods for detection of LTBI. Diagnostic agreement between two tests are poor especially in Bacillus Calmette-Guérin (BCG) vaccinated immunocompromised patients. In this study, we tried to figure out if the use of a higher cut-off for TST increases diagnostic agreement with IGRAs and TST specificity and or not. MATERIALS/METHODS: In this retrospective study, BCG vaccinated solid organ transplantation (SOT) candidates and patients scheduled for anti-tumor necrosis factor-alpha (anti- TNFα) treatment patients who underwent both TST and IGRAs between 2011 and 2017 were enrolled in the study. Diagnostic agreement between the two tests was assessed for 5, 10, 15mm cut-off values for all participants, SOT candidates and anti- TNFα treatment subgroups separately. RESULTS: Fifty female and 55 male total 105 patients were included. In the anti- TNFα treatment group 92.8% of the patients were receiving at least one immunosuppressive drug. For all participants kappa (κ) values were 0.303, 0.370, 0.321 respectively for 5, 10 and 15mm cut-offs. For SOT candidates κ values were 0.488, 0.422, 0.288 respectively. For anti- TNFα treatment group κ values were 0.235, 0.332, 0.275 respectively. CONCLUSIONS: In BCG vaccinated immunocompromised patients, the agreement between TST and QFT-GIT was poor regardless of cut-off value. And increasing the cut-off does not improve agreement.

Seven patients with cryptogenic organizing pneumonia succesfully treated with clarithromycin.

Cryptogenic organizing pneumonia is a type of idiopathic interstitial pneumonia with a subacute presentation characterized by variable degrees of cough and dyspnea. As a consequence of the upregulation of inflammatory mediators in cryptogenic organizing pneumonia, it typically responds to anti-inflammatory therapy. Although the majority of patients recover completely with long term oral corticosteroids, relapse is common and their long term use is associated with many adverse effects. Macrolides antiinflammatory agents that appear effective and safe for the treatment of cryptogenic organizing pneumonia. Herein, we analyzed the clinical properties of seven cryptogenic organizing pneumonia patients without severe functional loss who were treated with clarithromycin. (Sarcoidosis Vasc Diffuse Lung Dis 2018; 35: 165-170).

Association of serum KL-6 levels with interstitial lung disease in patients with connective tissue disease: a cross-sectional study.

It was aimed to evaluate KL-6 glycoprotein levels to determine if it may be a diagnostic marker for the connective tissue diseases (CTDs) predicting CTD-related interstitial lung diseases (ILDs) (CTD-ILD) development and to examine if there was a difference between patients and healthy controls. The study included 113 patients with CTD (45 CTD without lung involvement, 68 CTD-ILD) and 45 healthy control subjects. KL-6 glycoprotein levels were analyzed with ELISA in patients and the control group. The relationship between KL-6 glycoprotein levels and CTD-ILD was assessed. In the comparison of all the groups in the study, significantly higher levels of KL-6 were determined in the CTD-ILD group than in either the CTD without pulmonary involvement group or the healthy control group (p < 0.008 and p < 0.001, respectively). There was no statistically significant difference between the KL-6 levels in the healthy control group and the CTD without pulmonary involvement group (p = 0.289). The KL-6 levels did not differ significantly according to the connective tissue diseases in the diagnostic groups (systemic lupus erythematosus, Sjögren's syndrome, rheumatoid arthritis, mixed connective tissue disease, scleroderma, polymyositis/ dermatomyositis). In the healthy control group, there was a statistically significant difference between KL-6 levels in smokers and non-smokers. Smokers had significantly higher serum KL-6 levels compared with non-smokers (p < 0.05). There was no statistically significant difference between smoking status (pack-year) and serum KL-6 levels. There was no statistically significant correlation between serum KL-6 levels and time since diagnosis of CTD and CTD-ILD. The level of KL-6 as a predictive factor could be used to identify the clinical development of ILD before it is detected on imaging modality. Further prospective clinical studies are needed to define whether levels of KL-6 might have prognostic value or might predict progressive ILD.

Long term effects of tear gases on respiratory system: analysis of 93 cases.

AIM: This study aimed to assess the long-term respiratory effects of tear gases among the subjects with history of frequent exposure. MATERIALS AND METHODS: A questionnaire by NIOSH and pulmonary function tests was performed in 93 males exposed to the tear gases frequently and 55 nonexposed subjects. RESULTS: The mean numbers of total exposure and last 2 years exposure were 8.4 ± 6.4 times, 5.6 ± 5.8 times, respectively. Tear gas exposed subjects were presented with a higher rate for cough and phlegm more than 3 months (24.7% versus 11.3%, P > 0.05). Mean FEV1/FVC and % predicted MMFR in smoker exposed subjects are significantly lower than those in smoker controls (81.7% versus 84.1%, P = 0.046 and 89.9% versus 109.6%, P = 0.0004, resp.). % predicted MMFR in nonsmoker exposed subjects is significantly lower than that in nonsmoker controls (99.4% versus 113.1%, P = 0.05). Odds ratios for chest tightness, exercise dyspnea, dyspnea on level ground, winter morning cough, phlegm, and daily phlegm were increased almost 2 to 2.5 folds among tear gas exposed subjects. CONCLUSION: The rates for respiratory complaints were high in the case of the exposure to the tear gases previously. Tears gas exposed subjects were found to be under the risk for chronic bronchitis.

Extrapulmonary involvement in patients with sarcoidosis in Turkey.

BACKGROUND AND OBJECTIVE: Extrapulmonary sarcoidosis is common, and is almost always associated with concomitant thoracic involvement. Extrapulmonary manifestations vary on the basis of gender, age at presentation and ethnicity. The aim of this study was to investigate extrapulmonary involvement in patients with sarcoidosis in Turkey. METHODS: This study was conducted by Turkish Thoracic Society Clinical Problems Study Group. New cases of sarcoidosis between 1 June 2004 and 31 May 2006 were recorded on electronic case record forms sent to all potential investigators and information about extrapulmonary involvement was collected. RESULTS: One hundred and nineteen of 293 patients (83 female, 36 male, mean age = 45 ± 12 years) had extrapulmonary involvement in this study (40.6%). The median time to diagnosis was 6 months and this was longer than patients with just thoracic sarcoidosis (P = 0.001). Extrapulmonary symptoms were present in 181 (61.8%) patients, and skin lesions, arthralgia and back pain were the commonest (33.4%, 20.8% and 16.4%, respectively). Incidence of organ involvement was independent of age with the exception of ocular involvement, which was higher in those under the age of 40 years (P = 0.007). CONCLUSIONS: Skin and peripheral lymph node involvement were the most common sites of extrapulmonary involvement and ocular involvement was more common in those under the age of 40 years in patients with sarcoidosis in a Turkish population.

Sarcoidosis caused by interferon therapy.

Interferon alpha (IFN-alpha) is an immunomodulator that is used as an antiviral agent in active chronic viral hepatitis C. IFN therapy can cause an induction or exacerbation of sarcoidosis. Although several reports in the gastroenterology literature have suggested an association between IFN therapy and sarcoidosis, this association has rarely been described elsewhere. A 47-year-old woman developed sarcoidosis after cessation of treatment with IFN and ribavirin for chronic hepatitis C. Her sarcoidosis showed liver, pulmonary and skin involvement. She continues to be monitored regularly in the Department of Pulmonary Diseases without steroid therapy. Her sarcoidosis improved spontaneously. We conclude that patients should be monitored for sarcoidosis during and after IFN therapy.

Diabetes insipidus presentation before renal and pulmonary features in a patient with Wegener's granulomatosis.

We report a case of a 47-year-old woman with Wegener's granulomatosis complicated by central diabetes insipidus. The patient had initially seronegative polyarthritis which mostly responded well to methotrexate and steroid therapy. Eight months later the patient suffered from polyuria and polydipsia. There were no abnormalities of the anterior pituitary hormones. MR images showed only loss of brightness of the posterior pituitary. Extensive evaluation of the patient revealed the presence of ANCA, in c-ANCA pattern and also PR3 positivity. Three months later findings of glomerulonephritis, as suggested by an active urine sediment and gradual proteinuria, and, finally, asymptomatic pulmonary nodules completed the clinical picture of Wegener's disease within 1 year. Renal biopsy showed crescent formation in two glomeruli, consistent with ANCA-related glomerulonephritis which showed pauci-immün depositions by direct immunofluorescence. Diabetes insipidus symptoms mostly regressed; renal and pulmonary findings completely disappeared with glucocorticoid and pulse cyclophosphamide treatment. These findings show that diabetes insipidus may rarely develop early in the disease process and ANCA positivity was directly indicative of Wegener's granulomatosis before the classic clinical signs of the disease.

Multiple endobronchial mass lesions due to sarcoidosis.

Sarcoidosis is a systemic granulomatous disease that primarily affects the lung and lymphatic systems of the body. The lungs are affected in over 90% of sarcoid patients. Although paranchymal lung disease is more common, the airways may also be involved. Bronchial mucosa is often affected in sarcoidosis, but endobronchial mass lesions are very rare. We present a case with multiple endobronchial mass lesions due to sarcoidosis.

Prognostic significance of blood group antigen expression of tumor tissue in lung cancer patients.

AIMS AND BACKGROUND: Many prognostic factors have been evaluated both for SCLC and NSCLC. The prognostic significance of blood group antigen expression of tumor tissues has been studied particularly in NSCLC, yielding divergent results. The aim of the present study was to investigate the prognostic value of the tumoral expression of blood group antigens ABH in lung cancer. METHODS: The presence of blood group antigens was assessed immunohistochemically in paraffin-embedded tumor samples from 92 patients diagnosed between 1996 and 1997. Monoclonal antibodies were used to detect blood group antigens. RESULTS: The median survival was longer in NSCLC patients whose tumors were positive for blood group antigen A (P = 0.009). Since the expression of blood group antigen A in tumor cells was limited to patients with type A or AB blood, survival analysis of these patients showed survival to be longer in non-small cell lung cancer patients with blood group antigen A-positive tumors (P= 0.0019). CONCLUSIONS: Expression of blood group antigen A in tumor cells is an important, favorable prognostic factor in patients with non-small cell lung cancer, which could be useful to stratify patients with blood group A or AB according to possible outcome, and to guide therapeutic decision-making. The expression of blood group antigens ABH should be evaluated in larger series of lung cancer patients (including small and non-small cell lung cancer) with all blood types.