Nocardia and Lungs in COPD: Beyond Immuno-deficiencies.

Nocardia is an opportunistic pathogen and Pulmonary Nocardiosis (PN) occurred in more than half of the cases in subjects with immuno-suppressed status. COPD is one of the most common comorbidity observed in immuno-competent patients with PN. In this perspective study, we report the clinical patterns, the outcomes and the comorbidities of all cases of PN admitted in our Unit in the years 1999-2012. Among 6545 patients admitted in our Unit during the study time, we identified PN in 4 patients. COPD was coexistent in 3 out of 4 cases. A delayed time for the diagnosis was observed. Clinical-radiological improvement was detected in all cases after one month of specific anti-PN therapy. According to our experience, PN is a rare disease that should be suspected also in immuno-competent patients. COPD is confirmed to be a risk factor for the development of PN, probably due to reduced respiratory defenses and prolonged steroid therapy.

Human chitotriosidase: a sensitive biomarker of sarcoidosis.

BACKGROUND: Sarcoidosis is a multisystem granulomatous disease of unknown etiology. No suitable biomarkers are available to evaluate the evolution of this disease, which still has an unpredictable clinical course. Some years ago our research group proposed chitotriosidase as a potential biomarker with prognostic value, that however needed to be validated. AIMS AND METHODS: The aims of this study were to evaluate the sensitivity and specificity of chitotriosidase in a population of 232 sarcoidosis patients under the observation of our Sarcoidosis Regional Referral Centre in Siena and to analyse enzyme concentrations in different disease phenotypes (as defined by the recently published COS classification) to define its prognostic value. RESULTS: Serum chitotriosidase concentrations were significantly higher in patients than in healthy controls (p<0.0001) and were directly correlated with ACE levels (r=0.25, p<0.0001). ROC curve analysis revealed 88.6 % sensitivity and 92.8 % specificity. Enzyme concentrations were significantly higher in stage 3 sarcoidosis than in stage 0 (p=0.02). The lowest concentrations of chitotriosidase were found in untreated patients in remission (COS-1), while the highest enzyme concentrations were found in symptomatic patients with persistent disease on steroids and with functional deterioration in the last year (COS-9). In COS-9 subgroup, chitotriosidase decreased significantly after the increasing of steroid dose or the introduction of a new immunosuppressant therapy (p<0.01). CONCLUSION: Chitotriosidase proved to be a biomarker with good sensitivity and specificity that is easily detected in serum. It can be proposed in clinical practice to identify progressive patients requiring close follow-up, to detect relapses and to evaluate the effects of therapy.

Assessment of cardiac involvement in sarcoidosis by echocardiography.

The main objective of this study is to determine the prevalence of left ventricular systolic and diastolic dysfunction in patients with chronic sarcoidosis without clinical evidence of heart disease. The study includes 69 chronic sarcoidosis patients, 30 diagnosed by organ biopsy and 39 by clinical history, chest X-ray, high resolution computerized tomography (HRCT) and bronchoalveolar lavage (BAL), without suspected cardiac involvement. The control group consisted of 26 subjects selected from a population of hospital workers. The examination includes 12-lead ECG and echocardiographic examination. The results show that there were no differences in atrial size, left ventricular diameters, wall thickness, left ventricular ejection fraction or endocardial fractional shortening between the sarcoid group and controls. Signs of diastolic dysfunction were found in 33 (55%) patients, however, this group was significantly older than the others and had marginally higher blood pressure. Sarcoid patients had lower midwall fractional shortening (mFS) than controls; patients with diastolic dysfunction also had lower mFS but the difference was not significant. In conclusion, the results demonstrated an absence of left ventricular systolic dysfunction, evaluated by traditional echocardiographic methods, in our chronic sarcoidosis patients and an apparent absence of any relation between left ventricular diastolic dysfunction and sarcoidosis. Lower mFS was found among patients, particularly those with a long history of sarcoidosis. Further analysis is required to evaluate the significance of this index as a potential marker of heart involvement in chronic sarcoidosis.

Human chitotriosidase: a potential new marker of sarcoidosis severity.

Sarcoidosis is a multisystemic granulomatous disorder of unknown etiology. The unpredictable clinical course of the disease has prompted research into biomarkers useful for predicting outcome. Among the potential markers of sarcoidosis, a recently proposed indicator was chitotriosidase, a chitinase produced by activated macrophages. Chitotriosidase is involved in the defense against pathogens containing chitin. Increased concentrations of chitotriosidase have been observed in a number of lysosomal storage diseases including Gaucher disease and more recently also in sarcoidosis. In 2004, significantly higher serum chitotriosidase activity was reported for the first time in sarcoidosis patients with respect to controls (p < 0.01); a similar increase was subsequently observed in bronchoalveolar lavage of these patients. In 2007, an increase in enzyme activity was described in juvenile sarcoidosis. Chitotriosidase activity was found to be correlated with angiotensin-converting enzyme levels in serum, radiological stages and quantitative high-resonance CT score for sarcoidosis, suggesting that this enzyme could be a potential marker of disease severity worthy of further study. To evaluate the sensitivity and specificity of this marker, further analysis was done in other granulomatous and diffuse lung diseases. Here, we review the principal literature and the recent evidence of chitotriosidase as a possible marker of sarcoidosis.