Evaluation of patients with fibrotic interstitial lung disease: Preliminary results from the Turk-UIP study

OBJECTIVE: Differential diagnosis of idiopathic pulmonary fibrosis (IPF) is important among fibrotic interstitial lung diseases (ILD). This study aimed to evaluate the rate of IPF in patients with fibrotic ILD and to determine the clinical-laboratory features of patients with and without IPF that would provide the differential diagnosis of IPF. MATERIAL AND METHODS: The study included the patients with the usual interstitial pneumonia (UIP) pattern or possible UIP pattern on thorax high-resolution computed tomography, and/or UIP pattern, probable UIP or possible UIP pattern at lung biopsy according to the 2011 ATS/ERSARS/ALAT guidelines. Demographics and clinical and radiological data of the patients were recorded. All data recorded by researchers was evaluated by radiology and the clinical decision board. RESULTS: A total of 336 patients (253 men, 83 women, age 65.8 +/- 9.0 years) were evaluated. Of the patients with sufficient data for diag-nosis (n=300), the diagnosis was IPF in 121 (40.3%), unclassified idiopathic interstitial pneumonia in 50 (16.7%), combined pulmonary fibrosis and emphysema (CPFE) in 40 (13.3%), and lung involvement of connective tissue disease (CTD) in 16 (5.3%). When 29 patients with definite IPF features were added to the patients with CPFE, the total number of IPF patients reached 150 (50%). Rate of male sex (p<0.001), smoking history (p<0.001), and the presence of clubbing (p=0.001) were significantly high in patients with IPE None of the women <50 years and none of the men <50 years of age without a smoking history were diagnosed with IPE Presence of at least 1 of the symptoms suggestive of CTD, erythrocyte sedimentation rate (ESR), and antinuclear antibody (FANA) positivity rates were significantly higher in the non-IPF group (p<0.001, p=0.029, p=0.009, respectively). CONCLUSION: The rate of IPF among patients with fibrotic ILD was 50%. In the differential diagnosis of IPF, sex, smoking habits, and the presence of clubbing are important. The presence of symptoms related to CTD, ESR elevation, and EANA positivity reduce the likelihood of IPF

The Predictors of Obesity Hypoventilation Syndrome in Obstructive Sleep Apnea

Background: As obesity increases, the frequency of obstructive sleep apnea and obesity hypoventilation syndrome increases also. However, obesity hypoventilation syndrome frequency is not known, as capnography and arterial blood gas analysis are not routinely performed in sleep laboratories. Aims: To investigate the frequency and predictors of obesity hypoventilation syndrome in obese subjects. Study Design: Retrospective clinical study Methods: Obese subjects who had arterial blood gas analysis admitted to the sleep laboratory and polysomnography were retrospectively analyzed. Subjects with restrictive (except obesity) and obstructive pulmonary pathologies were excluded. Demographics, Epworth-Sleepiness-Scale scores, polysomnographic data, arterial blood gas analysis, and spirometric measurements were recorded. Results: Of the 419 subjects, 45.1% had obesity hypoventilation syndrome. Apnea hypopnea index (p<0.001), oxygen desaturation index (p<0.001) and sleep time with SpO2<90% (p<0.001) were statistically higher in subjects with obesity hypoventilation syndrome compared to subjects with eucapnic obstructive sleep apnea. The nocturnal mean SpO2 (p<0.001) and lowest SpO2 (p<0.001) were also statistically lower in subjects with obesity hypoventilation syndrome. Logistic regression analysis showed that the lowest SpO2, oxygen desaturation index, apnea hypopnea index and sleep time with SpO2 <90% were related factors for obesity hypoventilation syndrome. Conclusion: Obesity hypoventilation syndrome should be considered when oxygen desaturation index, apnea hypopnea index and sleep time with SpO2 <90% are hig

ADULT ONSET STILL'S DISEASE WITH PLEUROPERICARDITIS

We present a case of adult onset Still's disease (AOSD) revealed by pleuropericarditis. A 33 yr old man was admitted with fever, fatigue, chest and back pain. Radiological and laboratory investigations showed pleuropericarditis with leucocytosis, anemia, high erthyrocyte sedimentation rate and serum C-reactive protein level. Pleural fluid consisted predominantly of lymphocytes. Cytological and microbiological analysis were inconclusive and empiric antituberculous therapy with methylprednisolone was started. The symptoms improved. When steroid doses were reduced, clinical and laboratory findings worsened. Thoracoscopic pleural biopsy was obtained and it showed chronic fibrinous pleuropericarditis. Hepatosplenomegaly and history of arthralgia, high ferritin levels, negative ANA, RF, were consistent with AOSD. Antituberculosis therapy was stopped. Methotrexate was started. Since the diagnosis of AOSD requires the exclusion of specific etiologies of pleuropericarditis, it may be defined with some delay in time

Echocardiographic Findings in Obesity Hypoventilation Syndrome

Objective: In Obesity Hypoventilation syndrome (OHS), pulmonary hypertension (PH) is an important morbidity and mortality reason compared to pure Obstructive Sleep Apnea syndrome (OSAS). However, few studies are available in the literature about this subject. For this reason, we evaluated OHS and pure OSAS cases in our study from the point of right heart-related echocardiographic parameters and PH. Materials and Methods: Obese [body mass index (BMI) >30 kg/m2] cases diagnosed by polysomnography were included in the study. The subjects were divided to two groups as OHS and pure OSAS. OHS criteria were defined as obesity (BMI >30 kg/m2) and for another reason unexplained (neuromuscular, chest wall, restrictive or obstructive pulmonary diseases) daytime hypercapnia (PaCO2 >45 mmHg). Patients with severe hypothyroidism, renal and heart insufficiency, cardiac drug anamnesis were not included in the study. Transthoracic echocardiography was performed to all cases and those with left-heart pathology were excluded from the study. Systolic pulmonary artery pressure (PABs) >35 mmHg was accepted as PH. Results: Of the 115 cases studied (mean age: 49.3±10.6/year, female/male: 53/62, BMI: 40.5±6.1 kg/m2), 70 were pure OSAS and 45 were OHS. In the OHS group, PABs, right ventricular diameter and pulmonary velocities were significantly higher than the pure OSAS group (p=0.002, p=0.015, p=0.012, respectively). The frequency of PH in OHS was significantly higher than group with pure OSAS (48.8% vs. 17.1%, p<0.001). In the overall group, there was a positive correlation between PABs value and apnea-hypopnea index, OSAS phase, oxygen desaturation index, SpO2 <90% elapsed time, and OHS presence (r=0.307, p=0.001; r=0.259, p=0.005; r=0.405, p<0.001; r=0.162, p<0.001; r=0.305, p=0.001, respectively). There was negative correlation between PABs and mean and minimum SpO2 (r=-0.404, p<0.001; r=-0.344, p<0.001, respectively). Conclusion: In OHS cases; PAPs, right ventricular diameter and pulmonary velocities, and PH frequency are higher than pure OSAS. Echocardiographic evaluation should be routinely performed for OHS cases