Neck mass and bilateral pleural effusions in a 53-year-old female.

Chylothorax indicates the accumulation of chyle in the pleural cavity. It is a rare cause of pleural effusion, especially bilaterally. In clinical practice, the presence of milky fluid in the pleural cavity raises the suspicion of chylothorax. The most common cause is trauma, iatrogenic or non, owing to thoracic duct injury, which transports chyle from the lymphatic system into the bloodstream. The case we describe is of a 53-year-old female who was referred to our hospital with bilateral pleural effusions and a left supraclavicular mass. From the diagnostic studies, the nontraumatic causes of chylothorax were excluded. The potential diagnosis was traumatic chylothorax, a diagnosis of exclusion, as it appeared after muscle stretch and receded with a fat-free diet and repose without any relapse.

Genotype-Phenotype Relationships in Inheritable Idiopathic Pulmonary Fibrosis: A Greek National Cohort Study.

BACKGROUND: Monogenic and polygenic inheritances are evidenced for idiopathic pulmonary fibrosis (IPF). Pathogenic variations in surfactant protein-related genes, telomere-related genes (TRGs), and a single-nucleotide polymorphism in the promoter of MUC5B gene encoding mucin 5B (rs35705950 T risk allele) are reported. This French-Greek collaborative study, Gen-Phen-Re-GreekS in inheritable IPF (iIPF), aimed to investigate genetic components and patients' characteristics in the Greek national IPF cohort with suspected heritability. PATIENTS AND METHODS: 150 patients with familial PF, personal-family extrapulmonary disease suggesting short telomere syndrome, and/or young age IPF were analyzed. RESULTS: MUC5B rs35705950 T risk allele was detected in 103 patients (90 heterozygous, 13 homozygous, allelic frequency of 39%), monoallelic TRG pathogenic variations in 19 patients (8 TERT, 5 TERC, 2 RTEL1, 2 PARN, 1 NOP10, and 1 NHP2), and biallelic ABCA3 pathogenic variations in 3. Overlapping MUC5B rs35705950 T risk allele and TRG pathogenic variations were shown in 11 patients (5 TERT, 3 TERC, 1 PARN, 1 NOP10, and 1 NHP2), MUC5B rs35705950 T risk allele, and biallelic ABCA3 pathogenic variations in 2. In 38 patients, neither MUC5B rs35705950 T risk allele nor TRG pathogenic variations were detectable. Kaplan-Meier curves showed differences in time-to-death (p = 0.025) where patients with MUC5B rs35705950 T risk allele alone or in combination with TRG pathogenic variations presented better prognosis. CONCLUSION: The Gen-Phen-Re-GreekS in iIPF identified multiple and overlapping genetic components including the rarest, underlying disease's genetic "richesse," complexity and heterogeneity. Time-to-death differences may relate to diverse IPF pathogenetic mechanisms implicating "personalized" medical care driven by genotypes in the near future.

Bilateral Deep Vein Thrombosis and Pulmonary Embolism Due to Right Common Iliac Artery Aneurysm with a Contained Rupture.

Venous thromboembolism (comprising deep venous thrombosis and/or pulmonary embolism) is a common disease, often of multifactorial cause. Focal iliac artery aneurysms are relatively rare, and only a few reports exist in the literature describing patients with venous thromboembolism resulting from venous floe disruption due to iliac artery aneurysm. Thus, we report a case of a 65-year-old male presenting with pulmonary embolism and bilateral deep vein thrombosis associated with a contained rupture of the right common iliac artery aneurysm.

Increased monocyte count and red cell distribution width as prognostic biomarkers in patients with Idiopathic Pulmonary Fibrosis.

BACKGROUND: Idiopathic Pulmonary Fibrosis (IPF) represents a chronic lung disease with unpredictable course. METHODS: We aimed to investigate prognostic performance of complete blood count parameters in IPF. Treatment-naïve patients with IPF were retrospectively enrolled from two independent cohorts (derivation and validation) and split into subgroups (high and low) based on median baseline monocyte count and red cell distribution width (RDW). RESULTS: Overall, 489 patients (derivation cohort: 300, validation cohort: 189) were analyzed. In the derivation cohort, patients with monocyte count ≥ 0.60 K/µL had significantly lower median FVC%pred [75.0, (95% CI 71.3-76.7) vs. 80.9, (95% CI 77.5-83.1), (P = 0.01)] and DLCO%pred [47.5, (95% CI 44.3-52.3) vs. 53.0, (95% CI 48.0-56.7), (P = 0.02)] than patients with monocyte count < 0.60 K/µL. Patients with RDW ≥ 14.1% had significantly lower median FVC%pred [75.5, (95% CI 71.2-79.2) vs. 78.3, (95% CI 76.0-81.0), (P = 0.04)] and DLCO%pred [45.4, (95% CI 43.3-50.5) vs. 53.0, (95% CI 50.8-56.8), (P = 0.008)] than patients with RDW < 14.1%. Cut-off thresholds from the derivation cohort were applied to the validation cohort with similar discriminatory value, as indicated by significant differences in median DLCO%pred between patients with high vs. low monocyte count [37.8, (95% CI 35.5-41.1) vs. 45.5, (95% CI 41.9-49.4), (P < 0.001)] and RDW [37.9, (95% CI 33.4-40.7) vs. 44.4, (95% CI 41.5-48.9), (P < 0.001)]. Patients with high monocyte count and RDW of the validation cohort exhibited a trend towards lower median FVC%pred (P = 0.09) and significantly lower median FVC%pred (P = 0.001), respectively. Kaplan-Meier analysis in the derivation cohort demonstrated higher all-cause mortality in patients with high (≥ 0.60 K/µL) vs. low monocyte count (< 0.60 K/µL) [HR 2.05, (95% CI 1.19-3.53), (P = 0.01)]. CONCLUSIONS: Increased monocyte count and RDW may represent negative prognostic biomarkers in patients with IPF.

Reduction in Hospitalizations for Respiratory Diseases during the First COVID-19 Wave in Greece.

INTRODUCTION: During the first COVID-19 wave, a considerable decline in hospital admissions was observed worldwide. AIM: This retrospective cohort study aimed to assess if there were any changes in the number of patients hospitalized for respiratory diseases in Greece during the first CO-VID-19 wave. METHODS: In the present study, we evaluated respiratory disease hospitalization rates across 9 tertiary hospitals in Greece during the study period (March-April 2020) and the corresponding period of the 2 previous years (2018-2019) that served as the control periods. Demographic data and discharge diagnosis were documented for every patient. RESULTS: Of the 1,307 patients who were hospitalized during the study period, 444 (35.5%) were males with a mean (±SD) age of 66.1 ± 16.6 years. There was a 47 and 46% reduction in all-cause respiratory morbidity compared to the corresponding periods of 2018 and 2019, respectively. The mean incidence rate for respiratory diseases during the study period was 21.4 admissions per day, and this rate was significantly lower than the rate during the same period in 2018 (40.8 admissions per day; incidence rate ratio [IRR], 0.525; 95% confidence interval [CI], 0.491-0.562; p < 0.001) or the rate during 2019 (39.9 admissions per day; IRR, 0.537; 95% CI, 0.502-0.574; p < 0.001). The greatest reductions (%) in the number of daily admissions in 2020 were observed for sleep apnoea (87% vs. 2018 and 84% vs. 2019) followed by admissions for asthma (76% vs. 2018 and 79% vs. 2019) and chronic obstructive pulmonary disease (60% vs. 2018 and 51% vs. 2019), while the lowest reductions were detected in hospitalizations for pulmonary embolism (6% vs. 2018 and 23% vs. 2019) followed by tuberculosis (25% vs. both 2018 and 2019). DISCUSSION/CONCLUSION: The significant reduction in respiratory admissions in 2020 raises the reasonable question of whether some patients may have avoided seeking medical attention during the COVID-19 pandemic and suggests an urgent need for transformation of healthcare systems during the pandemic to offer appropriate management of respiratory diseases other than COVID-19.

Exhaled Nitric Oxide (FeNO) in Patients Hospitalized for an Exacerbation of Bronchiectasis and/or COPD : FeNO Levels in COPD and Bronchiectasis.

Exhaled nitric oxide (FeNO) represents an important marker of airway inflammation, yet its role in chronic obstructive pulmonary disease (COPD) and/or bronchiectasis is not well studied. We aimed to measure FeNO in patients with COPD, bronchiectasis, and combination of COPD/bronchiectasis during an acute exacerbation (AE) of the underlying disease as well as to describe the characteristics of patients with COPD/bronchiectasis overlap in patients hospitalized for an acute exacerbation (AE). Seventy-nine patients were enrolled in the study as follows: COPD n = 45, bronchiectasis n = 18, and COPD and bronchiectasis n = 16. FeNO was measured with a commercially available analyzer within 24 hours of admission and at discharge. FeNO differed significantly on admission when compared at discharge in the whole group (16.91 ± 16.14 vs 12.48 ± 10.67, p = 0.008, respectively). On admission, FeNO was 17.80 ± 18.77 ppb in COPD patients, 17.12 ± 6.59 in bronchiectasis patients, and 11.55 ± 2.42 in patients with COPD/bronchiectasis overlap. At discharge, FeNO was 12.40 ± 12.11 ppb in COPD patients, 15.50 ± 6.39 in bronchiectasis patients, and 9.00 ± 3.22 in patients with combination. FeNO differed significantly in bronchiectasis patients versus patients with COPD/bronchiectasis overlap at admission (p = 0.043) and at discharge (p = 0.020) and versus COPD patients at discharge (p = 0.043). FeNO decreased significantly during the AE in all groups (p = 0.001 for COPD, p = 0.021 for bronchiectasis, and p = 0.026 for combination). FeNO levels in patients with COPD and/or bronchiectasis exacerbation are possibly increased at admission and decrease at discharge. The differences in FeNO levels between groups may reflect different underlying inflammatory mechanisms.

Investigation of admission serum creatinine as a predictor of hospital length of stay in triple-vaccinated COVID-19 inpatients.

BACKGROUND: This study assessed the association between admission kidney function and the duration of hospitalization in triple-vaccinated coronavirus disease 2019 (COVID-19) inpatients during the omicron surge in Larissa, central Greece. METHODS: Regression analysis was used to estimate the effect of kidney function biomarkers on hospital length of stay (LoS) within a dataset from a cohort of 51 subjects. RESULTS: Sex- and age-adjusted admission serum creatinine was associated with hospital LoS (p=0.034). CONCLUSIONS: Serum creatinine concentration on admission should be further evaluated as a possible clinical predictor of hospital LoS among triple-vaccinated COVID-19 inpatients both at the country and global level.

Anti-Ro52/Anti-nuclear Matrix Protein 2 Positive Clinically Amyopathic Dermatomyositis Presented Only With Interstitial Lung Disease.

This report contemplates a unique case of clinically amyopathic dermatomyositis (CADM) that presented as interstitial lung disease. The patient was a 55-year-old woman who showed up with progressive exercise intolerance and a dry cough without muscular or dermatological clinical manifestations. Diagnostic workup and imaging revealed the presence of interstitial lung disease, and further evaluation led to a positive autoimmune panel for anti-nuclear matrix protein 2 (anti-NXP2) and anti-Ro52 antibodies, establishing the diagnosis of anti-NXP2 plus anti-Ro52 antibodies-positive amyopathic idiopathic inflammatory dermatomyositis. The patient was started on intravenous corticosteroids. She showed improvement on her chest X-ray (CXR) and was then switched to oral corticosteroids. After six months of steroid treatment, corticosteroids were stopped, and the patient was re-evaluated one month later disease relapse.

A Unique Case of Conservatively Treated Actinomyces Empyema Complicated Due to Drug Reaction With Eosinophilia and Systemic Symptoms Syndrome.

This case contemplates the unusual presentation, challenging diagnostic workup and conservative therapeutic process of a patient with Actinomyces empyema complicated along the way due to drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome. The patient was a 40-year-old male, who presented with pleurodynia and fever. Laboratory exams showed elevated inflammatory markers and imaging revealed two biconvex fluid pockets located in the right lower lobe, from which the fluid was positive for Actinomyces meyeri. The initial conservative process with intravenous antibiotics and successful drainage with intrapleural fibrinolysis improved our patient. However, after a few days, the patient's fevers relapsed, and as regress of the empyema was discussed as a complication, he developed a maculopapular symmetrical rash of the trunk and legs accompanied by enlarged lymph nodes, eosinophilia, thrombocytopenia, and atypical lymphocytes. The diagnosis of DRESS syndrome due to antibiotic therapy for actinomyces empyema was established and a balance between bactericidal and immunosuppression medication had to be found. Fortunately, the patient withstood prolonged antibiotic therapy and got fully treated without any relapses.

Intrahepatic Gallbladder Rupture and Biloma Mimicking Pulmonary Embolism Following Orthopedic Surgery.

In this case report, a 75-year-old male with a history of coronary artery disease, type 2 diabetes, hypertension, and benign prostate hypertrophy developed postoperative fever and chest pain following left knee arthroplasty. Upon admission to the emergency department, pulmonary embolism was considered highly probable, and the patient was treated with anticoagulation and antibiotics due to diagnostic uncertainty. However, further investigations revealed a complex condition involving an intraparenchymal gallbladder rupture resulting in a biloma secondary to choledocholithiasis. The patient's history of receiving spinal anesthesia with intrathecal morphine was identified as a potential causative factor to the sphincter of Oddi constriction, leading to increased biliary pressure and gallbladder rupture. This case highlights the importance of having a broad differential diagnosis in postoperative patients, especially when the clinical presentation is atypical. With the diagnosis being confirmed, the patient underwent successful treatment, including biliary stenting, drainage of the biloma, and ultimately cholecystectomy. This case underlines the need for vigilance and a multidisciplinary approach in managing complex postoperative complications, emphasizing that clinical presentations may sometimes deviate significantly from the expected, requiring further investigation and individualized treatment.

Familial Mediterranean Fever in a 28-Year-Old Male Presented as a Painless Massive Pleural Effusion.

This case describes the first patient with familial Mediterranean fever (FMF) with massive left pleural effusion as the first clinical manifestation, to whom a video-assisted thoracoscopic surgery was performed to support the diagnosis. The patient was a 28-year-old male, who presented with dry cough and dyspnea but no fever. The lab findings showed hypoxemia (partial pressure of oxygen = 65 mm Hg) accompanied by elevated inflammatory markers, including C-reactive protein at 7 mg/dl (<0.5 mg/dl), erythrocyte sedimentation rate of 46 mm/h (<20 mm/h), and serum amyloid at 56.7 mg/L (<10 mg/L). X-ray indicated the left pleural effusion was part of a bilateral recurrent painless pleuritis, as the right pleural thickening implied. Numerous biopsies were taken during the thoracoscopy, and the histopathology examination reported non-specific fibrous pleurisy. Colchicine administration, at first empirically for upcoming pericarditis, at the end was a significant clue for the diagnosis. Positive molecular testing for mutations in the familial Mediterranean fever (MEFV) gene contributed to the diagnosis of FMF, which was based on the Tel-Hashomer clinical criteria. The purpose of this article is hopefully to raise further awareness about patients with FMF presented with unusual manifestations of the disease.

CSF2RB mutation-related hereditary pulmonary alveolar proteinosis: the "long and winding road" into adulthood.

Genetic analysis pre-lung transplantation diagnosed a case of hereditary pulmonary alveolar proteinosis (PAP) complicated by fibrosis in adulthood. The need for genetic testing in GM-CSF autoantibody negative and unclassifiable PAP is highlighted. https://bit.ly/3QcsYwM.

Recurrent Pleuritic Chest Pain, Lobar Consolidation, and Pleural Effusion in a 50-Year-Old Woman.

CASE PRESENTATION: A 50-year-old woman with a history of permanent atrial fibrillation (AF) treated with radiofrequency catheter ablation (RFCA) 6 months ago was admitted to the respiratory department of a tertiary hospital because of recurrent episodes of pleuritic chest pain in the preceding 5 months. The patient reported multiple visits to a regional hospital, where she was treated with broad-spectrum antibiotics after discovery of a left alveolar consolidation on chest radiograph (Fig 1), subsequently imaged with CT scan (Fig 2). On treatment failure and appearance of a left-sided pleural effusion during outpatient follow-up, the patient was re-admitted. Pleural fluid was obtained via thoracocentesis characterized by exudative features and lymphocytic predominance. Abdomen CT scan, with IV and per os contrast agent, was devoid of findings consistent with malignancy, and serum autoantibody levels were below positivity cut off values (antinuclear, cyclic citrullinated peptide antibody, rheumatoid factor, and anti-neutrophil cytoplasmic antibodies). The patient underwent flexible bronchoscopy without endobronchial pathology on visual inspection. Microbiologic studies and cytological examination of samples obtained by bronchial washing/aspiration yielded no clinically relevant information. Lung perfusion/ventilation scintigraphy was ordered to exclude chronic thromboembolic pulmonary hypertension; however, a deficit in vascularization for the left inferior lobe was found, prompting further investigation (Fig 3). Progression of left inferior lobe consolidation and the presence of a small pericardial effusion became evident on reimaging after a 2-month interval. The patient was empirically started on corticosteroids. After emergence of left hilar lymphadenopathy (< 1 cm), a PET-CT scan was performed. The left lower inferior lobe consolidation, whose metabolic activity pattern was consistent with that of inflammation (standardized uptake value equal to 4.4) (Fig 4), as well as the left sided-pleural effusion were markedly improved compared with previous imaging 20 days after corticosteroid initiation (Fig 2). On the grounds of recalcitrant pleuritic pain and pleural effusion recurrence during corticosteroid tapering, the patient was referred to the respiratory department of our university hospital to have her condition diagnosed.

Predictors of SARS-CoV-2 IgG Spike Antibody Responses on Admission and Clinical Outcomes of COVID-19 Disease in Fully Vaccinated Inpatients: The CoVax Study.

BACKGROUND: SARS-CoV-2 vaccines have shown high efficacy in protecting against COVID-19, although the determinants of vaccine effectiveness and breakthrough rates are yet to be determined. We aimed at investigating several factors affecting the SARS-CoV-2 IgG Spike (S) antibody responses on admission and clinical outcomes of COVID-19 disease in fully vaccinated, hospitalized patients. METHODS: 102 subjects were enrolled in the study. Blood serum samples were collected from each patient upon admission for the semiquantitative determination of the SARS-CoV-2 IgG S levels with lateral flow assays. Factors influencing vaccine responses were documented. RESULTS: 27 subjects had a negative antibody test upon hospital admission. Out of the 102 patients admitted to the hospital, 88 were discharged and 14 died. Both the absence of anti-S SARS-CoV-2 antibodies and poor clinical outcomes of COVID-19 disease were associated with older age, lower Ct values, and a shorter period between symptom onset and hospital admission. Ct values and time between symptom onset and hospitalization were independently associated with SARS-CoV-2 IgG S responses upon admission. The PaO2/FiO2 ratio was identified as an independent predictor of in-hospital mortality. CONCLUSIONS: Host- and disease-associated factors can predict SARS-CoV-2 IgG S responses and mortality in hospitalized patients with breakthrough SARS-CoV-2 Infection.

Clinical relevance of circulating autoantibodies in idiopathic pulmonary fibrosis; A NAt hard to break.

Patients with idiopathic pulmonary fibrosis are screened for circulating autoantibodies as part of the initial interstitial lung disease workup. Management of seropositive idiopathic pulmonary fibrosis is currently considered no different than that of lone idiopathic pulmonary fibrosis. Emerging data however suggest that the former may possess distinct characteristics in terms of pathophysiology, histopathology, prognosis and amenability to immunomodulation. In that context, the aim of our study was to evaluate the influence of autoantibody status on: (i) the decline of forced vital capacity; (ii) the decline of diffusing capacity of lung for carbon monoxide; and (iii) 3-year survival; in a cohort of 102 idiopathic pulmonary fibrosis patients. In a pilot sub-study, we also sought to evaluate whether changes in antibody status during disease course affect the aforementioned parameters by potentially reflecting activity of the autoimmunity component of the pro-fibrotic mechanism.

Reduced immunogenicity of the mRNA vaccine BNT162b2 in patients with idiopathic pulmonary fibrosis.

Patients with #IPF do not mount appreciable anti-spike antibody responses to two doses of #SARSCoV2 mRNA vaccine compared to the general population. National authorities should prioritise patients with IPF for booster doses. https://bit.ly/3K2KXQ0.

Eight Weeks Unsupervised Pulmonary Rehabilitation in Previously Hospitalized of SARS-CoV-2 Infection.

The aim of our study was to determine the impact of unsupervised Pulmonary Rehabilitation (uns-PR) on patients recovering from COVID-19, and determine its anthropometric, biological, demographic and fitness correlates. All patients (n = 20, age: 64.1 ± 9.9 years, 75% male) participated in unsupervised Pulmonary Rehabilitation program for eight weeks. We recorded anthropometric characteristics, pulmonary function parameters, while we performed 6 min walk test (6 MWT) and blood sampling for oxidative stress measurement before and after uns-PR. We observed differences before and after uns-PR during 6 MWT in hemodynamic parameters [systolic blood pressure in resting (138.7 ± 16.3 vs. 128.8 ± 8.6 mmHg, p = 0.005) and end of test (159.8 ± 13.5 vs. 152.0 ± 12.2 mmHg, p = 0.025), heart rate (5th min: 111.6 ± 16.9 vs. 105.4 ± 15.9 bpm, p = 0.049 and 6th min: 112.5 ± 18.3 vs. 106.9 ± 17.9 bpm, p = 0.039)], in oxygen saturation (4th min: 94.6 ± 2.9 vs. 95.8 ± 3.2%, p = 0.013 and 1st min of recovery: 97.8 ± 0.9 vs. 97.3 ± 0.9%), in dyspnea at the end of 6 MWT (1.3 ± 1.5 vs. 0.6 ± 0.9 score, p = 0.005), in distance (433.8 ± 102.2 vs. 519.2 ± 95.4 m, p < 0.001), in estimated O2 uptake (14.9 ± 2.4 vs. 16.9 ± 2.2 mL/min/kg, p < 0.001) in 30 s sit to stand (11.4 ± 3.2 vs. 14.1 ± 2.7 repetitions, p < 0.001)] Moreover, in plasma antioxidant capacity (2528.3 ± 303.2 vs. 2864.7 ± 574.8 U.cor., p = 0.027), in body composition parameters [body fat (32.2 ± 9.4 vs. 29.5 ± 8.2%, p = 0.003), visceral fat (14.0 ± 4.4 vs. 13.3 ± 4.2 score, p = 0.021), neck circumference (39.9 ± 3.4 vs. 37.8 ± 4.2 cm, p = 0.006) and muscle mass (30.1 ± 4.6 vs. 34.6 ± 7.4 kg, p = 0.030)] and sleep quality (6.7 ± 3.9 vs. 5.6 ± 3.3 score, p = 0.036) we observed differences before and after uns-PR. Our findings support the implementation of unsupervised pulmonary rehabilitation programs in patients following COVID-19 recovery, targeting the improvement of many aspects of long COVID-19 syndrome.

Chronic Progressive Dyspnea in a 71-Year-Old Man: A Diagnostic Ithaca After 8 Years of Consultation.

CASE PRESENTATION: A 71-year-old ex-bus driver (ex-smoker, 20 pack-years) was admitted for the first time to the respiratory department because of chronic dry cough and progressive exertional dyspnea with insidious onset 8 years ago. The patient also reported weight loss of about 20 kg in 3 years and proximal muscle weakness. A decade ago, he was diagnosed with gastric adenocarcinoma and subjected to partial gastrectomy and splenectomy, followed by an unspecified chemotherapy regimen. Additionally, the patient has coronary disease and underwent coronary bypass graft surgery 7 years ago. In the course of his disease, many diagnostic procedures have been performed including Mantoux tests, five CT scans, a CT-guided biopsy, two bronchoscopies, and an 18F-fluorodeoxyglucose PET scan with inconclusive results. The patient was referred to the hospital to have his long-lasting condition diagnosed and treated.