CPAP therapy in patients with idiopathic pulmonary fibrosis and obstructive sleep apnea: does it offer a better quality of life and sleep?

BACKGROUND: The recent literature shows an increased incidence of obstructive sleep apnea (OSA) in patients with idiopathic pulmonary fibrosis (IPF). On the other hand, there are no published studies related to continuous positive airway pressure (CPAP) treatment in this patient group. Our aim was to assess the effect of CPAP on sleep and overall life quality parameters in IPF patients with OSA and to recognize and overcome possible difficulties in CPAP initiation and acceptance by these patients. METHODS: Twelve patients (ten males and two females, age 67.1 ± 7.2 years) with newly diagnosed IPF and moderate to severe OSA, confirmed by overnight attended polysomnography, were included. Therapy with CPAP was initiated after a formal in-lab CPAP titration study. The patients completed the Epworth Sleepiness Scale (ESS), the Pittsburgh Sleep Quality Index (PSQI), the Functional Outcomes in Sleep Questionnaire (FOSQ), the Fatigue Severity Scale (FSS), the SF-36 quality of life questionnaire, and the Beck Depression Inventory (BDI) at CPAP initiation and after 1, 3, and 6 months of effective CPAP therapy. RESULTS: A statistically significant improvement was observed in the FOSQ at 1, 3, and 6 months after CPAP initiation (baseline 12.9 ± 2.9 vs. 14.7 ± 2.6 vs. 15.8 ± 2.1 vs. 16.9 ± 1.9, respectively, p = 0.02). Improvement, although not statistically significant, was noted in ESS score (9.2 ± 5.6 vs. 7.6 ± 4.9 vs. 7.5 ± 5.3 vs. 7.7 ± 5.2, p = 0.84), PSQI (10.7 ± 4.4 vs. 10.1 ± 4.3 vs. 9.4 ± 4.7 vs. 8.6 ± 5.2, p = 0.66), FSS (39.5 ± 10.2 vs. 34.8 ± 8.5 vs. 33.6 ± 10.7 vs. 33.4 ± 10.9, p = 0.44), SF-36 (63.2 ± 13.9 vs. 68.9 ± 13.5 vs. 72.1 ± 12.9 vs. 74.4 ± 11.3, p = 0.27), and BDI (12.9 ± 5.5 vs. 10.7 ± 4.3 vs. 9.4 ± 4.8 vs. 9.6 ± 4.5, p = 0.40). Two patients had difficulty complying with CPAP for a variety of reasons (nocturnal cough, claustrophobia, insomnia) and stopped CPAP use after the first month, despite intense follow-up by the CPAP clinic staff. Heated humidification was added for all patients in order to improve the common complaint of disabling nocturnal cough. CONCLUSION: Effective CPAP treatment in IPF patients with OSA results in a significant improvement in daily living activities based on the FOSQ, namely an OSA-specific follow-up instrument. Improvement was also noted in other questionnaires assessing quality of life, though not to a statistically significant degree, probably because of the multifactorial influences of IPF on physical and mental health. The probability of poor CPAP compliance was high and could only be eliminated with intense follow-up by the CPAP clinic staff.

A clear cell tumor of the lung presenting as a rapidly growing coin lesion: is it really a benign tumor?

There are few reported cases of clear cell tumor of the lung, a very rare benign mesenchymal neoplasm. We describe a 41-year-old asymptomatic man who presented with a coin lesion in a routine chest roentgenogram that was absent in a roentgenogram performed a year earlier. After a thorough workup, including radionuclide scintigraphy, the diagnosis of cell tumor of the lung was established. Somatostatin receptor positivity was demonstrated, and this clear cell tumor of the lung had a rapid growth rate. Because of these features, the benign nature of such a tumor remains questionable.

Transient lung herniation through a thoracic cage defect: a case report.

We report a benign condition of transient lung herniation through a congenital structural defect of the thoracic cage, in a young, otherwise healthy, asymptomatic individual. A brief review of the existing literature on this rare entity is also presented.

A massive hemorrhagic pleural effusion does not exclude the diagnosis of tuberculosis: a case report.

We report a case of an immunocompetent 18-year-old man with a massive hemorrhagic, exudative, lymphocytic pleural effusion. Blind transthoracic pleural biopsy showed granuloma formation, while the pleural fluid culture was positive for Mycobacterium tuberculosis, confirming the diagnosis of primary tuberculous pleuritis. A massive hemorrhagic pleural effusion is extremely rare in tuberculosis, but tuberculosis is a very protean disease and should always be included in the differential diagnosis of pleural effusions.

Sodium valproate as a cause of recurrent transudative pleural effusion: a case report.

INTRODUCTION: There are few reported cases of neutrophilic pleural effusions associated with valproic acid therapy. Most of them are of eosinophilic exudates with or without blood eosinophilia. CASE PRESENTATION: This case study describes a 70-year-old man with recurrent episodes of eosinophilic transudative pleural effusions associated with sodium valproate treatment. The recurrence of effusion after re-administration of the drug is strongly suggestive of an association between them. To the best of our knowledge, this is the first reported case with a pleural effusion with these characteristics caused by sodium valproate. CONCLUSION: This is the first report in the literature, with a full understanding of the etiology but with an unknown drug mechanism. This case report is of interest to different medical specialists (such as pulmonologists, neurologists, cardiologists) and pharmacologists.

Congenital bronchial atresia presenting as a cavitary lesion on chest radiography: a case report.

BACKGROUND: Congenital bronchial atresia is a rare anomaly, which usually presents in adulthood as an incidental finding on routine examinations. CASE PRESENTATION: In this report we present a patient with a cavitary lesion at his right upper lobe, found by chance on chest radiography. Computed tomography of the chest revealed the characteristic findings of a mucocele with distal oligemia and hyperlucency of the affected lung parenchyma. Further examination including bronchoscopy virtually excluded other possible disorders and the diagnosis of congenital bronchial atresia was established. CONCLUSION: The radiological presentation of congenital bronchial atresia may occasionally mimic serious lung diseases. The procedure of choice for the diagnosis is the computed tomography of the chest. Bronchoscopy is not diagnostic but is valuable in doubtful cases to exclude different disorders.

Unilateral lymphocytic pleuritis as a manifestation of familial Mediterranean fever.

Familial Mediterranean fever (FMF) is an autosomal recessive disease affecting predominantly populations surrounding the Mediterranean basin. It is the most prevalent hereditary periodic fever syndrome characterized mainly by recurrent and short attacks of fever and serositis (pleuritis, arthritis, peritonitis). Unilateral polymorphonuclear exudative pleuritis associated with fever has been reported as the solitary manifestation of the first FMF attack, in < 10% of patients. This case study describes a 30-year-old Greek man with recurrent episodes of lymphocytic exudative pleuritis associated with fever. After a thorough workup (clinical criteria and molecular genetic testing identifying homozygosity polymorphisms of the FMF gene), the diagnosis of FMF was established. Treatment with colchicine, 2 mg/d, eliminated FMF attacks. To our knowledge, this is the first well-documented case report of a patient with FMF presenting with a lymphocytic exudative pleural effusion.

Right-sided Bochdalek Hernia Presenting as a Solitary Pulmonary Nodule.

Bochdalek hernia is a well-known disorder, which represents visceral herniation through a posterior diaphragmatic defect. In this report we present an adult man with a right-sided Bochdalek hernia mimicking a solitary pulmonary nodule. Furthermore, we perform a brief review of the literature, with emphasis on novel imaging techniques that establish the diagnosis. In conclusion, Bochdalek hernia should be considered in the differential diagnosis of a posterior diaphragmatic or paraspinal opacity. Correct diagnosis is of paramount importance and can be easily established by non-invasive imaging techniques.

Drug-induced pulmonary toxicity in a patient treated with mesalazine and azathioprine for ulcerative colitis.

We report a case of an 18-yr-old male with high-grade persistent fever, productive cough, malaise, diarrhea and associated abnormal findings on chest radiography. The patient suffered from ulcerative colitis receiving mesalazine and azathioprine. After a further and scrutinized work-up the diagnosis of drug-induced pulmonary toxicity was made. His condition was improved after discontinuing both drugs and the administration of corticosteroids. This is a rarely reported case of eosinophilic pneumonia. Although it has not been reported previously, the possibility of mesalazine and azathioprine synergism cannot be excluded. The clinical, aetiological, diagnostic and therapeutic aspects of the disease are discussed demonstrating the paramount importance of bronchoalveolar lavage in the diagnosis of this disorder. One should be aware of this entity in patients with inflammatory bowel disease.

Mefloquine-induced eosinophilic pneumonia.

Mefloquine has been widely used for prophylaxis and treatment of patients with chloroquine-resistant malaria; the drug is usually well tolerated. Rarely, adverse effects may be severe, including gastrointestinal disturbances, neuropsychiatric reactions, cardiovascular manifestations, skin lesions, musculoskeletal symptoms, and bone marrow toxicity. We describe a 67-year-old woman with fever, dyspnea on exertion, peripheral blood eosinophilia, and diffuse pulmonary infiltrates on chest radiography. She had taken mefloquine for malaria prophylaxis for an 8-week trip to South Africa. A thorough work-up led to the diagnosis of eosinophilic pneumonia caused by the mefloquine. Her condition improved after the drug was discontinued. To our knowledge, this is the first report of mefloquine-induced eosinophilic pneumonia. Clinicians should be aware of this rare, potential adverse effect of mefloquine.

Pulmonary sarcoidosis associated with psoriasis vulgaris: coincidental occurrence or causal association? Case report.

BACKGROUND: Sarcoidosis is rarely associated with a distinct disease. One disease infrequently associated with sarcoidosis is psoriasis. CASE PRESENTATION: This case study describes a 38-year-old male, who presented with chest pain, high-grade fever, arthralgias and a skin rash accompanied by bilateral hilar lymphadenopathy on his chest radiograph. Extensive investigations including fiber-optic bronchoscopy with bronchoalveolar lavage and labial and skin biopsies, demonstrated that two distinct clinical entities co-existed in the same patient: pulmonary sarcoidosis and psoriasis vulgaris. Combination therapy for both diseases was applied and the patient was greatly improved. CONCLUSION: This is the first well-documented case of sarcoidosis and psoriasis in the same patient, reported on the basis of safe and widely-used techniques that were not available until fairly recently. These disorders might share common pathogenic mechanisms that could explain their co-existence in the patient.

Delayed response to anti-tuberculosis treatment in a patient on infliximab.

The introduction of infliximab, an anti-TNF-alpha agent, in the treatment of rheumatic diseases has offered important therapeutic advances in recent years. The main adverse effect from the usage of this drug is susceptibility to infections, mainly reactivation of latent tuberculosis. We present a 23 year-old male with ankylosing spondylitis, who developed endobronchial and widespread pulmonary tuberculosis, 2 years after initiation of treatment with infliximab. The patient had already been treated for a positive PPD skin test with a 9-month prophylactic course of isoniazid. He was treated with a five drug anti-tuberculosis scheme but he showed an extremely slow therapeutic response with daily high fever, even 4 months after initiation of treatment. Seven months after beginning anti-tuberculosis therapy, bronchoscopy still revealed necrotic and inflammatory tissue at the site of the original lesions. This unusual clinical course of tuberculosis infection was attributed to immunosuppression due to the long-lasting anti-TNF-alpha action of infliximab.

8-Isoprostane, a marker of oxidative stress, is increased in the expired breath condensate of patients with pulmonary sarcoidosis.

STUDY OBJECTIVE: 8-Isoprostane is considered an index of oxidative stress. Measurement of 8-isoprostane in the expired breath condensate, a totally noninvasive method, has not been used to explore the level of inflammation in pulmonary sarcoidosis. Therefore, the aim of our study was to measure the levels of 8-isoprostane in the expired breath condensate of patients with sarcoidosis, and to investigate the relation of 8-isoprostane level to disease activity. PATIENTS: We investigated 30 patients with pulmonary sarcoidosis (active disease, n = 14; nonactive disease, n = 16) and 12 healthy subjects as control group. METHODS: 8-Isoprostane was measured in the expired breath condensate of all subjects, and its levels were compared between the control and sarcoidosis groups as well as between the subgroups of patients with active and nonactive disease. In the group with sarcoidosis, 8-isoprostane levels were further correlated with markers that may reflect disease activity, such as serum angiotensin-converting enzyme (sACE) level, serum calcium level, and pulmonary function test results. RESULTS: The concentration of 8-isoprostane was increased in patients with sarcoidosis compared to control subjects (mean, 64.23 pg/mL; 95% confidence interval [CI], 37.00 to 91.46 pg/mL; vs mean, 20.75 pg/mL; 95% CI, 16.06 to 25.44 pg/mL; p = 0.04). The difference was primarily due to the patients with active disease, who had significantly higher levels of 8-isoprostane (mean, 111.4 pg/mL; 95% CI, 62.56 to 160.30 pg/mL; p < 0.001) compared to patients with nonactive disease (mean, 22.94 pg/mL; 95% CI, 15.89 to 29.99 pg/mL) or healthy subjects. 8-Isoprostane levels in patients with nonactive disease did not differ from those in healthy subjects (p > 0.05). In the patients with sarcoidosis, 8-isoprostane levels were positively correlated with sACE level (p < 0.0001, r = 0.69), but was not correlated with serum calcium level or pulmonary function test values. CONCLUSIONS: Our data suggest that 8-isoprostane levels are increased in the expired breath condensate of patients with sarcoidosis and might serve as an index of disease activity.

Reduced intracellular Mg concentrations in patients with acute asthma.

STUDY OBJECTIVES: To determine the intracellular and extracellular Mg concentrations in patients with acute asthma and their correlation with parameters expressing the disease severity. PATIENTS: Thirty patients with acute asthma (FEV(1), 56% predicted [SD, 14.5]), 20 patients with stable asthma (FEV(1), 97% predicted [SD, 10]), and 20 healthy subjects (FEV(1), 97% predicted [SD, 8]). METHODS: Mg concentrations in erythrocytes and plasma were measured four times: at hospital admission, after 2 days, after 5 days, and at hospital discharge. Percentage of predicted FEV(1) and peak expiratory flow rate variability were recorded simultaneously. Similar measurements were carried in all study groups. RESULTS: Mg concentrations of healthy subjects and patients with stable asthma remained unchanged in both plasma and erythrocytes. Initial Mg content in erythrocytes was significantly lower in patients with acute asthma (1.77 fmmol per cell; 95% confidence interval [CI], 1.71 to 1.83) compared to normal subjects (1.94 fmmol per cell; 95% CI, 1.82 to 2.00) and patients with stable asthma (1.92 fmmol per cell; 95% CI, 1.87 to 1.96) [p < 0.0001], and it increased significantly after the resolution of the exacerbation (from 1.77 fmmol per cell [95% CI, 1.71 to 1.83] at hospital admission to 1.90 fmmol per cell [95% CI, 1.83 to 1.98] at hospital discharge; p < 0.0001). No correlation was observed between parameters of disease severity and the initial values of Mg concentrations in erythrocytes and plasma. CONCLUSIONS: Acute asthma is associated with lower erythrocyte Mg content while plasma levels remain unchanged. This decrease in intracellular Mg content occurs regardless of the severity of the exacerbation and returns to normal values after control has been achieved.