A Narrative Review of Self-Reported Scales to Evaluate Depression and Anxiety Symptoms in Adult Obstructive Sleep Apnea Patients.

Background and Objectives: Obstructive sleep apnea (OSA) is a prevalent chronic condition that has been associated with mental disorders like depression and anxiety. This study intends to provide a practical overview of the most relevant self-reported and self-rating scales that assess depression and anxiety in OSA patients. Materials and Methods: A search for articles was performed using PubMed, Google Scholar, and Semantic Scholar using a combination of words for obstructive sleep apnea, depression, anxiety, and scales. The tools were ordered by type (screening and rating) and arranged chronologically according to the year of publication. Results: Three scales were identified for assessing depression, which were the Center for Epidemiologic Studies Depression Scale (CES-D), the Hospital Anxiety and Depression Scale (HADS-D), and the Patient Health Questionnaire-9 (PHQ-9). For rating depression, two scales were discussed: the Zung Self-Rating Depression Scale (SDS) and the Beck Depression Inventory (BDI), which has three versions (the BDI, the BDI-II, and the Fast Screen (BDI-FS)). For assessing anxiety, the Generalized Anxiety Disorder-7 (GAD-7) scale was identified. Two scales were reviewed for rating anxiety: the State-Trait Anxiety Inventory (STAI) and the Beck Anxiety Inventory (BAI). Each scale is accompanied by a brief description of its practicality and psychometric qualities and an analysis of its strengths and limitations. Conclusions: The findings of this review will contribute to the understanding of the importance of assessing mental health comorbidities in the context of OSA, ultimately guiding clinical practice and future research in this area.

Post-Infection Oscillometry and Pulmonary Metrics in SARS-CoV-2 Patients: A 40-Day Follow-Up Study.

The COVID-19 pandemic, caused by the SARS-CoV-2 virus, has had significant impacts on pulmonary function. This study aimed to comprehensively evaluate pulmonary function and structure in patients 40 days post-SARS-CoV-2 infection, employing an array of testing methodologies including spirometry, plethysmography, forced oscillometry, and CT scanning. It also sought to establish potential correlations between these metrics and evaluate if forced oscillometry could provide additional value in post-infective lung function assessment. A 40-day post-infection follow-up observational study was conducted involving 66 patients with confirmed SARS-CoV-2 infection. The results revealed decreases in FVC and FEF25-75 with the increasing severity of COVID-19. Specifically, patients with severe symptoms exhibited statistically significant decreases in FVC (mean = 86.8) compared with those with mild symptoms (mean = 106.0; p = 0.018). The FEF25-75 showed a similar trend, with severe patients exhibiting a mean of 77.7 compared with 82.9 in the mild group (p = 0.017). Furthermore, resonant frequency (RF) increased with disease severity, with the severe group exhibiting a statistically significant increase (mean = 17.4) compared with the mild group (mean = 14.3; p = 0.042). CT scans showed an increase in ground-glass opacities with disease severity, with 81.8% of severe patients demonstrating this finding (p = 0.037). Multiple regression analysis revealed that Reactance at 4 Hz (X4), Forced Expiratory Flow 25-75% (FEF25-75), and Resonant Frequency (RF) were significantly related to COVID-19 severity. Specifically, for each unit increase in these factors, the risk of the event was estimated to increase by a factor of 3.16, 2.09, and 1.90, respectively. Conversely, Resistance at 4 Hz (R4) and Airway Resistance (RAW) were found to significantly decrease the event hazard, highlighting their potential protective role. Spirometry, plethysmography, and forced oscillometry are effective in assessing these changes. Forced oscillometry may be particularly beneficial in identifying subtle changes in lung function post-COVID-19. Further studies are warranted to validate these findings and develop strategies to manage post-infective pulmonary changes in SARS-CoV-2 patients.

Longitudinal Analysis of Pulmonary Function Impairment One Year Post-COVID-19: A Single-Center Study.

Persistent pulmonary impairment post-COVID-19 has been reported, albeit variably. This single-center observational study aims to longitudinally evaluate pulmonary function in 140 COVID-19 survivors one year after recovery, assessing associations with disease severity and pre-existing lung conditions. Participants aged 18 and older, with confirmed SARS-CoV-2 infection, were evaluated using spirometry and Diffusion Capacity of Lungs for Carbon Monoxide (DLCO) tests. Pulmonary function parameters like Forced Expiratory Volume at 1 s (FEV1), Forced Vital Capacity (FVC), and Total Lung Capacity (TLC) were measured. Participants were stratified by age, gender, body mass index, smoking status, and lung damage severity via computed tomography (CT). The cohort consisted of mostly males (58.6%), with a mean age of 53.8 years and body mass index of 24.9 kg/m2. Post-COVID fibrosis was seen in 22.7%, 27.3%, and 51.9% of mild, moderate, and severe disease patients, respectively (p = 0.003). FVC significantly reduced with disease severity (p < 0.001), while FEV1, FEF25-75, and DLCO showed a non-significant downward trend. FEV1/FVC ratio increased with disease severity (p = 0.033), and TLC and RV significantly declined (p = 0.023 and p = 0.003, respectively). A one-year follow-up indicated a non-significant change in FVC, FEV1, FEV1/FVC ratio, FEF25-75, and RV compared with the 40-day measurement, but it revealed significant improvements in DLCO and TLC (p = 0.010). There were significant mean increases in FVC, FEV1, DLCO, TLC, and RV across all disease severities over one year. They were most pronounced in the patients with a history of severe COVID-19, who had a better recovery over one year, compared with the mild and moderate COVID-19 patients whose lung function almost normalized. One year after the SARS-CoV-2 infection, we observed a significant association between disease severity and post-COVID fibrotic changes. Though some lung function parameters remained stable over the year, significant improvements were noted in DLCO and TLC. Particularly, individuals with severe disease showed substantial recovery in lung function, indicating the potential reversibility of COVID-19-related pulmonary damage.

Impact of CPAP Therapy Adherence on Global Cognition in Patients with Moderate to Severe Obstructive Sleep Apnea: A One-Year Follow-Up.

Background: Obstructive sleep apnea increases (OSA) cognitive impairment risk. The objective of this study was to determine the impact of continuous positive airway pressure (CPAP) adherence on global cognition using the Montreal Cognitive Assessment questionnaire (MoCA). Materials and Methods: Thirty-four new patients diagnosed with moderate or severe OSA (apnea-hypopnea index AHI ≥ 15 events/h) from the CPAP group were compared with thirty-one moderate to severe OSA patients from the no-CPAP group. In addition, all patients completed the MoCA test, a patient health questionnaire (PHQ-9) for depressive symptoms, and a generalized anxiety questionnaire (GAD-7) for anxiety symptoms, at baseline, after 6 months, and after 1 year. Results: At baseline, there were no significant differences between the two groups regarding total MoCA scores, 20.9 ± 3.5 CPAP group to 19.7 ± 2.9 no-CPAP group (p = 0.159), PHQ-9 (p = 0.651), and GAD-7 (p = 0.691). After one year, improvement was observed for a total MoCA score of 22.7 ± 3.5 (p < 0.001) for the CPAP group, and significant variance of score between groups was more accentuated for delayed recall and attention (p < 0.001) sub-topic. Moreover, PHQ-9, GAD-7 scores, and the Epworth Sleepiness Scale (ESS) decreased significantly (p < 0.001) after CPAP therapy. The MoCA score was significantly correlated with years of education (r = 0.74, p < 0.001) and had a negative correlation with body mass index (BMI) (r = -0.34), ESS (r = -0.30) and PHQ-9 (r = -0.34). Conclusions: One year of CPAP adherence improved global cognition associated with OSA.

Large Lung Consolidation: A Rare Presentation of Pulmonary Sarcoidosis.

Sarcoidosis is a complex inflammatory disease of uncertain origin, characterized by non-necrotizing epithelioid cell granulomas (NNEGs) affecting multiple organ systems. Although many different clinical and pathological phenotypes can be present, with different organs involved, the lung is the most common site described. In this case report, we (a) present and discuss the broad differential diagnosis of a patient presenting with a solitary lung mass with clinical and imaging features of lung cancer that ultimately was confirmed with a rare manifestation of stage II pulmonary sarcoidosis, and (b) analyze and compare similar cases from the literature.

CPAP Therapy on Depressive and Anxiety Symptoms in Patients with Moderate to Severe Obstructive Sleep Apnea Syndrome.

Backgrounds and Objectives: There is a link between sleep apnea syndrome (OSA) and depressive and anxiety symptoms, but the underlying mechanisms are not fully understood. The study aimed to determine the prevalence of these symptoms in patients with OSA and to evaluate the impact of continuous positive airway pressure (CPAP) on depression and anxiety scores. Materials and Methods: Ninety-nine consecutive new patients diagnosed with moderate or severe (apnea-hypopnea index AHI ≥ 15 events/h) OSA were accepted for the CPAP therapy. Patients completed a patient health questionnaire (PHQ-9) for depressive symptoms and a generalized anxiety questionnaire (GAD-7) for anxiety symptoms before the CPAP treatment, after 6 months, and after 1 year of CPAP therapy with compliance. For both scores (PHQ-9 and GAD-7), a cut point ≥10 was used to indicate the presence of clinically depressive and anxiety symptoms. Results: Forty-eight individuals (48.48%) had PHQ-9 scores above the cut-off point ≥ 10, and twenty-seven participants (27.27%) had GAD-7 scores above the cut-off point ≥10. A significant correlation has been shown between PHQ-9 score and BMI (p < 0.0001), AHI (p < 0.0001), ODI (p < 0.001), ESS (p < 0.001), GAD-7 score (p < 0.0001), and [t90] (p < 0.0001), while GAD-7 was correlated with AHI (p < 0.030), ODI (p < 0.006), and [t90] (p < 0.001). The PHQ-9 and GAD-7 scores decreased significantly after 6 months of CPAP therapy in all groups and after 1 year of CPAP use. Conclusions: OSA patients should be screened for depression and anxiety. Furthermore, CPAP should be the first choice of treatment before starting other treatments for depression and anxiety symptoms.

The Effects of COVID-19 on Skeletal Muscles, Muscle Fatigue and Rehabilitation Programs Outcomes.

Background and Objectives: Consequences due to infection with SARS-CoV-2 virus can have a direct impact on skeletal muscle, due to the fact that both cardiac and skeletal muscle tissue show robust ACE2(angiotensin-converting enzyme 2) expression, suggesting a potential susceptibility to SARS-CoV-2 infection in both types of tissues. From the articles analyzed we concluded that the musculoskeletal damage is firstly produced by the inflammatory effects, cytokine storm and muscle catabolism. However, myopathy, polyneuropathy and therapies such as corticoids were also considered important factors in muscle fatigue and functional incapacity. Pulmonary rehabilitation programs and early mobilization had a highly contribution during the acute phase and post-illness recovery process and helped patients to reduce dyspnea, increase the capacity of physical effort, overcome psychological disorders and improved the quality of their life. Materials and Methods: We have included in this review 33 articles that contain data on muscle damage following SARS-CoV-2 infection. We used the following keywords to search for articles: SARS-CoV-2, COVID-19, muscle weakness, muscle disease, muscle fatigue, neurological disorders. As a search strategy we used PubMed, Cochrane Database of Systematic Reviews; Database of Abstracts of Reviews of Effects and Health Technology Assessment Database to collect the information. We also have chosen the most recent articles published in the last 5 years. Conclusions: Muscular damage, as well as the decrease in the quality of life, are often a consequence of severe SARS-CoV-2 infection through: systemic inflammation, corticotherapy, prolonged bed rest and other unknown factors. Pulmonary rehabilitation programs and early mobilization had a highly contribution during the acute phase and post-illness recovery process and helped patients to reduce dyspnea, increase the capacity of physical effort, overcome psychological disorders and improve the quality of their life.

Endothelial dysfunction: The possible link between cardiovascular comorbidities and phenomenon of inflammaging from COPD.

Aging is a risk factor for many chronic noncommunicable diseases, including chronic obstructive pulmonary disease (COPD), which is often associated with cardiovascular disease (CVD). Moreover, aging is associated with a mild form of systemic inflammation. The aim of our study was to analyze the relationship between age, systemic and vascular inflammation, and the presence of CVD comorbidities in a stable COPD population. Forty COPD patients were divided into 2 age groups (<65 and ≥65 years of age), from which we collected the following inflammatory biomarkers: C-reactive protein, tumor necrosis factor alpha (TNF-α), interleukin-6 (IL-6), and endothelin-1 (ET-1). Elderly COPD patients had more frequent exacerbation events per year (2 vs 1, P = .06), a higher prevalence of CVD (3 vs 2, P = .04), more limited exercise tolerance (6-minute walking test distance, 343 [283-403] vs 434 [384-484]; P = .02), and mild systemic inflammation (TNF-α, 9.02 [7.08-10.96] vs 6.48 [5.21-7.76]; P = .03; ET-1, 2.24 [1.76-2.71] vs 1.67 [1.36-1.98] pg/mL; P = .04). A weak correlation between age and ET-1 (r = 0.32, P = .04) was observed. Mild systemic inflammation, characterized by a slightly increased level of TNF-α, and endothelial dysfunction, marked by elevated ET-1, could be liaisons between aging, COPD, and CVD comorbidities.

Bronchoalveolar Lavage as a Diagnostic Tool in an Atypical Pulmonary Langerhans Cell Histiocytosis.

Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse cystic lung disease that occurs almost exclusively in young adult smokers. High-resolution computed tomography of the chest allows a confident diagnosis of PLCH in typical presentation, when nodules, cavitating nodules, and cysts coexist and show a predominance for the upper and middle lung. Atypical presentations require histology for diagnosis. Histologic diagnosis rests on the demonstration of increased numbers of Langerhans cells and/or specific histological changes. PLCH is one of the few diseases in which bronchoalveolar lavage (BAL) has a high diagnostic value and can in some circumstances replace lung biopsy. We present a case of PLCH in an elderly non-smoker. Chest imaging revealed the presence of advanced interstitial lung disease with a fibrocystic pattern. BAL cellular analyses disclosed a macrophage pattern with CD1a phenotype that strongly supports the PLCH diagnosis, even in the setting of atypical clinical presentation and a lack of smoking exposure. PLCH is extremely rare in non-smokers and could represent a distinct phenotype.

ANCA-associated vasculitis in idiopathic pulmonary fibrosis: A case report and brief review of the literature.

RATIONALE: Idiopathic pulmonary fibrosis (IPF) is a progressive disease with poor prognosis. Patients with IPF represent a heterogeneous population with several described clinical phenotypes. More recently, the development of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis in IPF patients, with an incidence higher than that in the general population, has drawn attention. PATIENT CONCERNS: A 64-year-old woman previously diagnosed with IPF presented to the emergency department with hemoptysis and hypoxemic respiratory failure. DIAGNOSES: High-resolution chest computed tomography revealed bilateral ground-glass opacities associated with areas of consolidation superimposed on the patient's fibrotic background pattern. Diffuse alveolar hemorrhage was confirmed by the presence of hemorrhagic bronchoalveolar lavage fluid. Hematological and biochemical investigations revealed an inflammatory syndrome, moderate anemia, and rapidly progressive glomerulonephritis. Serological analysis revealed perinuclear antineutrophil cytoplasmic antibody positivity and high levels of antimyeloperoxidase antibodies antibodies. The patient underwent kidney biopsy, which revealed necrotizing glomerulonephritis. Clinical and laboratory findings were diagnostic of microscopic polyangiitis with lung and renal involvement. INTERVENTIONS: Cyclophosphamide in combination with methylprednisolone was administered as remission induction therapy. The maintenance therapy consisted of mycophenolate mofetil and prednisone. OUTCOMES: The patient achieved clinical, radiological, and serological remission within six weeks of treatment. LESSONS: The association between IPF and ANCA-associated vasculitis may represent a distinct clinical phenotype. Autoimmune testing for ANCAs should be considered part of the diagnostic work-up and follow-up of patients with IPF because of the clinical and therapeutic implications of developing vasculitis in an already vulnerable patient.

Physical Activity and Respiratory Muscle Strength in Patients with Sarcoidosis: An Observational Study.

BACKGROUND: The literature results related to the physical assessment in patients with sarcoidosis are contradictory and limited. The objective of this study was to compare the physical activity and respiratory muscle strength of patients at different stages of sarcoidosis. METHODS: Since 2003, we monitored 386 patients with ILDs, from which 107 patients were with sarcoidosis. Of the 107 patients with sarcoidosis, 57 were included in this study. A matched control group of 50 healthy patients were also included in the study. Kings Sarcoidosis Questionnaire (KSQ), maximal inspiratory/expiratory pressure and 6-minute walking test were recorded. RESULTS: We observed significant differences between the exercise capacity of the four sarcoidosis stages, 561.00 (531.00-586.00) vs 557.00 (510.00-599.00) vs 469.00 (430.00-515.00) vs 457.00 (397.00-496.00), independent-samples Kruskal-Wallis H-test H (4107) = 34.757, p < 0.001. Also, we observed significant differences between the exercise capacity of patients with sarcoidosis and healthy controls, 496.00 (437.00-550.00) vs 561.00 (531.00-586.00), Mann-Whitney U-test, p < 0.001. In addition, we observed that patients with sarcoidosis had significantly lower MIP and MEP than healthy controls, 84.00 (79.00-91.00) vs 90.00 (83.00-97.00); p = 0.001, and 85.00 (79.00-89.00) vs 89.00 (85.00-92.00), p < 0.001, respectively. There were significant associations between exercise capacity and KSQ scores, eg, general health (r = 0.206, p = 0.027), lung health (r = 0.280, p = 0.003). CONCLUSION: Patients with different stages of sarcoidosis showed reduced quality of life, exercise capacity and respiratory muscle strength both among themselves and compared to healthy controls.

Ultrasound mapping of lung changes in idiopathic pulmonary fibrosis.

INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is the most common and severe form of idiopathic interstitial pneumonia, accounted for 20% of cases of interstitial lung disease (ILD). In this study, we sought to compare the lung changes of IPF using a lung ultrasound (LUS) protocol of 12 zones with "current standard" high resolution computed tomography (HRCT) diagnostic method and overlap it with the functional pulmonary test as a complete clinical and imaging evaluation. METHODS: Thirty-one patients were included in the study and performed HRCT and pulmonary functional tests (PFT). A 12-lung zones' LUS protocol was used and compared with HRCT and PFT. RESULTS: The HRCT total fibrotic score had a correlation coefficient of 0.454 (P < 0.005) with predicted FVC and 0.713 with predicted DLCO (P < 0.001). Both the median of the number of B-lines and the average of the thickness of the pleural line obtained in the LUS assessment had a positively and statistically significant correlation with the HRCT fibrotic score P < 0.001. The pleural thickness of 2.4 mm is the cut-off value of the mild form of fibrosis with a sensitivity of 0.958 and a specificity of 0.994. CONCLUSION: B-lines and the average thickness of the pleural line as LUS markers of the fibrotic interstitial syndrome are highly and positively correlated with HRCT score, FVC and DLCO. LUS as a complementary method in the clinical management of IPF could be used more often by skilled clinicians to assess patients in terms of possible diagnosis and monitoring of IPF.

Impact of moderate to severe obstructive sleep apnea on the cognition in idiopathic pulmonary fibrosis.

INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is a relentlessly progressive lung disease with a fatal prognosis to whose rapid evolution multiple comorbidities may contribute, one of the most common being obstructive sleep apnea (OSA). There are several potential factors and conditions for the emergence of a cognitive deficit in relation to IPF or associated morbidities. OBJECTIVES: The goals of this study were to assess cognition in patients with IPF in stable phase and to identify clinical cognition modifiers. METHODS: In a cross-sectional study, 23 patients with IPF were evaluated using Montreal Cognitive Assessment (MoCA), an instrument for detecting mild cognitive impairments and were screened for OSA through overnight cardiorespiratory polygraphy and for anxiety and depression with three specific scale (Generalized Anxiety Disorder 7-item scale: GAD-7; the Patient Health Questionnaire: PHQ-9; Hospital Anxiety and Depression Scale: HADS). RESULTS: MoCA score was lower in patients with IPF when compared to controls subjects (24 [21,26] vs. 27 [26,28], p = 0.003) but not as significantly as in COPD patients (21 [18.8,23.3], p<0.0001). OSA was diagnosed in 19 (82.6%) IPF patients, 12 patients showed the presence of moderate-severe forms (63.15%). IPF patients with cognitive impairment (MoCA<23) exhibit a higher severity of OSA (apneea hypopnea index-AHI: 33.0±19.1 vs. 12.44±8.2, p = 0.018), and a higher Epworth score (7.1±3.3 vs. 4.3±1.8, p = 0.013). Anxiety and depression scores were not correlated with MoCA results. CONCLUSIONS: Impaired cognition in patients with IPF is mild and affect the areas of visuospatial abilities, language and working memory. OSA could be a possible predictor of IPF cognition deficit. Given the high prevalence of multiple types of sleep disorders in IPF patients, these should be investigated at least by cardiorespiratory polygraphy.

The reliability of lung ultrasound in assessment of idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is the severest form of idiopathic interstitial pneumonia, with a median survival time estimated at 2-5 years from the time of diagnosis. It occurs mainly in elderly adults, suggesting a strong link between the fibrosis process and aging. Although chest high-resolution computed tomography (HRCT) is currently the method of choice in IPF assessment, diagnostic imaging with typical usual interstitial pneumonia (UIP) provides definitive results in only 55%, requiring an invasive surgical procedure such as lung biopsy or cryobiopsy for the final diagnostic analysis. Lung ultrasound (LUS) as a noninvasive, non-radiating examination is very sensitive to detect subtle changes in the subpleural space. The evidence of diffuse, multiple B-lines defined as vertical, hyperechoic artifacts is the hallmark of interstitial syndrome. A thick, irregular, fragmented pleura line is associated with subpleural fibrotic scars. The total numbers of B-lines are correlated with the extension of pulmonary fibrosis on HRCT, being an LUS marker of severity. The average distance between two adjacent B-lines is an indicator of a particular pattern on HRCT. It is used to appreciate a pure reticular fibrotic pattern as in IPF compared with a predominant ground glass pattern seen in fibrotic nonspecific interstitial pattern. The distribution of the LUS artifacts has a diagnostic value. An upper predominance of multiple B-lines associated with the thickening of pleura line is an LUS feature of an inconsistent UIP pattern, excluding the IPF diagnosis. LUS is a repeatable, totally radiation-free procedure, well tolerated by patients, very sensitive in detecting early changes of fibrotic lung, and therefore a useful imaging technique in monitoring disease progression in the natural course or after initiation of treatment.

Clinical profile of unclassifiable interstitial lung disease: Comparison with chronic fibrosing idiopathic interstitial pneumonias.

Objective Unclassifiable interstitial lung disease (ILD) is a common problem in clinical practice. These patients pose a distinct challenge with regard to appropriate evaluation and management. We investigated the clinical features and prognosis of unclassifiable ILD and compared its clinical profile with that of idiopathic pulmonary fibrosis (IPF) and idiopathic nonspecific interstitial pneumonia (NSIP). Methods Patients with IPF (n = 40), NSIP (n = 14), and unclassifiable ILD (n = 27) were selected from an ongoing database. Baseline clinical features, pulmonary function, and the extent of fibrosis on high-resolution computed tomography (HRCT) were evaluated. Mortality was estimated based on the ILD-Gender, Age, Physiology (ILD-GAP) index and composite physiologic index (CPI). Results IPF was associated with the worst survival (hazard ratio [HR] = 4.361 compared with NSIP), followed by unclassifiable cases (HR = 1.251 compared with NSIP). Increasing mortality was significantly impacted by age (HR = 1.04 per 1-year increase), lower carbon monoxide diffusing capacity of the lung (HR = 0.97), HRCT interstitial score (HR = 1.119 per 1-point increase), ILD-GAP score (HR = 1.570 per 1-point increase), and CPI (HR = 1.039 per 1-point increase). Conclusions Patients with unclassifiable ILD had an intermediate prognosis between that of IPF and NSIP. Patients at high risk of mortality can be identified using baseline clinical, physiological, and radiological features.

Interstitial lung disease as first clinical manifestation within the antisynthetase syndrome--dermatomyositis.

Diffuse interstitial pneumopathies within the diseases of the connective tissue are often a diagnosis challenge, sometimes being the initial or dominant manifestation of the underlying autoimmune disease. The case of a young man shall be presented with relatively quick dyspnoea which has appeared under the conditions of an efficient physical status and without any notable pathological history. The cause of the dyspnoea is found to be a interstitial lung disease with radiological and histopathological pattern of non-specific interstitial pneumonia. The disease screening system discovers Ac anti Ro-52 which commits research in the direction of an autoimmune disease, confirmed by highlighting Ac anti PL-7 as being an antisynthetase syndrome. Subgroup of idiopathic inflammatory myopathies, the antisynthetase syndrome is a rare chronic autoimmune disease, which is characterised by the presence of antibodies directed against aminoacyl-t-RNA-synthetases (family of intracytoplasmic enzymes with a vital role in the protein synthesis). Shortly after specifying the relevant immunological status, the consistent clinical expression with dermatomyositis is also configured. The treatment is commenced with systemic corticoid and azathioprine; despite the treatment we are witnessing a slowly progressive clinical and functional deterioration.

Short telomeres in pulmonary fibrosis: from genetics to clinical significance.

Pulmonary fibrosis has been linked molecularly and pathophysiologically by abnormal telomere maintenance. Short telomere lengths are commonly found in both the familial and sporadic forms, telomerase mutations being the most common identifiable genetic cause of the disease. Telomeres are repeated nucleotide sequences that cap the ends of chromosomes and protect them from damage. Telomeres are eroded with cell division and shorten with age. Telomere integrity is mediated by the telomerase complex, a specialized polymerase that adds sequences to the ends of chromosomes. Mutations in the genes encoding telomerase (TERT and TERC) cause pulmonary fibrosis through low telomerase activity, accelerated telomere shortening and exhaustion of lung stem cells. Mutations in TERTor TERC account for only 19% of familial pulmonary fibrosis cases, and it is likely that additional environmental, genetic and epigenetic factors contribute to telomere erosion and to disease phenotype. Identification of short telomeres has potential clinical implications in pulmonary fibrosis: it may be a marker for an increased predisposition toward the development of the disease, it might affect risk stratification as it has been associated with lower survival rates and post-transplant complications that reflect the syndromic nature of this molecular defect.