Bronchial Involvement Research Articles

Sarcoidosis - a multisystem disease.

Sarcoidosis is a systemic inflammatory disease, characterised by granuloma formation upon an unknown trigger in genetically predisposed individuals. The inflammation is characterised by an activation of both the innate immune system, with macrophages differentiating into epitheloid cells and dendritic cells, and the adaptive immune system, particularly T helper (Th) 1 and Th17 cells. Since all organs can be affected to varying extents, clinical presentation is often diverse. Most commonly, the lungs, lymph nodes, skin and eyes are involved, whereas cardiac, renal and neurological manifestations are less common but associated with higher morbidity. Depending on the clinical symptoms, a detailed evaluation including thorough clinical examination, imaging and laboratory tests should explore all possible organ involvements. In some patients, fatigue manifests as a para-sarcoidosis symptom impacting quality of life, even if sarcoidosis is in remission. Some acute syndromic presentations, such as Löfgren's syndrome, have a good prognosis and are commonly self-limiting. If possible, a topical treatment, for example for cutaneous sarcoidosis or bronchial involvement, should be applied. Treatment of severe cases with persisting disease activity necessitates long-term immunosuppressive drugs, with glucocorticoids as the first-line option. Steroid-sparing and second-line drugs include methotrexate, azathioprine, mycophenolate mofetil and immunomodulators such hydroxychloroquine, with the latter being first-line therapy in cutaneous sarcoidosis. Tumour necrosis factor-alpha inhibitors (particularly adalimumab and infliximab) are used as third-line agents but are administered earlier in cases of persistent disease activity, severe organ-involvement or intolerance to conventional drugs. Treatment decisions should be based on a multidisciplinary approach, depending on organ involvement and treatment tolerability. Para-sarcoidosis manifestations, particularly fatigue, should also be carefully addressed, where the patient could also be enrolled in multidimensional rehabilitation programmes. With various organ involvement and different phenotypes, larger studies including real-world data from registries are necessary to evaluate different sarcoidosis endotypes and preferential treatment pathways.

Sarcoidosis - a multisystem disease.

Sarcoidosis is a systemic inflammatory disease, characterised by granuloma formation upon an unknown trigger in genetically predisposed individuals. The inflammation is characterised by an activation of both the innate immune system, with macrophages differentiating into epitheloid cells and dendritic cells, and the adaptive immune system, particularly T helper (Th) 1 and Th17 cells. Since all organs can be affected to varying extents, clinical presentation is often diverse. Most commonly, the lungs, lymph nodes, skin and eyes are involved, whereas cardiac, renal and neurological manifestations are less common but associated with higher morbidity. Depending on the clinical symptoms, a detailed evaluation including thorough clinical examination, imaging and laboratory tests should explore all possible organ involvements. In some patients, fatigue manifests as a para-sarcoidosis symptom impacting quality of life, even if sarcoidosis is in remission. Some acute syndromic presentations, such as Löfgren's syndrome, have a good prognosis and are commonly self-limiting. If possible, a topical treatment, for example for cutaneous sarcoidosis or bronchial involvement, should be applied. Treatment of severe cases with persisting disease activity necessitates long-term immunosuppressive drugs, with glucocorticoids as the first-line option. Steroid-sparing and second-line drugs include methotrexate, azathioprine, mycophenolate mofetil and immunomodulators such hydroxychloroquine, with the latter being first-line therapy in cutaneous sarcoidosis. Tumour necrosis factor-alpha inhibitors (particularly adalimumab and infliximab) are used as third-line agents but are administered earlier in cases of persistent disease activity, severe organ-involvement or intolerance to conventional drugs. Treatment decisions should be based on a multidisciplinary approach, depending on organ involvement and treatment tolerability. Para-sarcoidosis manifestations, particularly fatigue, should also be carefully addressed, where the patient could also be enrolled in multidimensional rehabilitation programmes. With various organ involvement and different phenotypes, larger studies including real-world data from registries are necessary to evaluate different sarcoidosis endotypes and preferential treatment pathways.

Case report: Small cell lung cancer presenting as the “sunray sign” in the chest radiograph and recurrent hemoptysis

Lung cancer prognosis has not changed in the last few decades due to diagnosis at an advanced stage. The majority of cases with early disease are asymptomatic, and whenever clinical presentations with cough, dyspnea, hemoptysis, or chest pain occur, when these cases have progressed to an advanced stage. Lung cancer tops the list of all causes of cancer-related deaths globally and is ahead of digestive tract malignancies. Small-cell lung cancer (SCLC) has the worst outcome, with survival rates in the range of weeks to months from diagnosis. SCLC is usually manifested as an extensive disease due to its delayed presentation, early metastasis, systemic nature, and poor response to available treatment options. “sunray sign” in chest radiographs is first described in literature and constitutes a hilar mass or radiopacity with inhomogeneous linear opacities spreading toward the periphery like sunrays, which is a marker of interstitial lymphatic involvement due to malignant spread of disease. The “sunray sign” is an indicator of underlying lung malignancy with central airway or main stem bronchus involvement and lymphatic dissemination in linear opacities. In this case report, we have reported a 51-year-old male who presented with cough and hemoptysis with progressive worsening of shortness of breath. Chest X-rays documented round opacities occupying the right hilum with linear opacities emerging toward the periphery in lung parenchyma, showing the typical “sunray sign.” Bronchoscopy was done after clinical stabilization and showed endobronchial polypoidal growth in the right main stem bronchus, causing partial occlusion of the bronchial lumen. Endobronchial needle aspiration (EBNA) cytology and forceps-guided (FB) histopathology are suggestive of “small cell histological type” lung malignancy for the “sunray sign” in our case. A high index of suspicion is a must to rule out underlying malignancy, and bronchoscopy is the “gold standard” test in cases with the sunray sign to confirm the diagnosis.

Endobronchial Tuberculosis: Diagnosis and Treatment Approach

Endobronchial tuberculosis (EBTB) is defined as tuberculosis (TB)infection of the tracheobronchial tract. Tracheal and bronchial involvement from TB infection was first reported by Morten in 1698. Endobronchial tuberculosis is a form of TB that is difficult to diagnose because the lesions are frequently undetectable by sputum examination and chest X-ray alone. Endobronchial tuberculosis is present in 10-40% of active TB patients and more than 90% of cases are accompanied by bronchial stenosis of various degrees. The main goal of therapy in active EBTB is to eradicate Mycobacterium tuberculosis in the tracheobronchial tract and further prevent stenosis. Complications of EBTB apart from airway stenosis are atelectasis, haemoptysis and shortness of breath accompanied by wheezing even after administration of anti-tuberculosis drugs (ATD).

The role of PMCT for the assessment of the cause of death in natural disaster (landslide and flood): a Sicilian experience

In this report, the authors provide a contribution of PMCT in assessing the cause of death due to natural disasters. Here, the PMCT findings of 43 subjects who died during both landslide and flood were described. The post-mortem imaging revealed, clearly, traumatic injuries and/or the presence of foreign material in airways allowing to assess the cause of death of each subject, together with external inspection and the collected circumstantial data. Particularly, the PMCT has been helpful for characterization and localization of the clogging substance in airways providing findings on bronchial branches involvement. Moreover, the investigation offered detailed data on skeletal injuries in all anatomic districts and put in evidence both the precise fracturing site and the characteristics of fracture stubs for each bone fracture. This report supports the recommendation of the virtual autopsy in a case with several victims, as in natural disasters, and its role as an alternative diagnostic investigation when the standard autopsy is not feasible.

Granulomatosis with polyangiitis with obstructive pneumonia progressing to hypertrophic pachymeningitis

Rationale:Bronchial involvement alone is a rare initial manifestation of granulomatosis with polyangiitis (GPA). Herein, we report a case of refractory GPA with obstructive pneumonia caused by bronchial involvement.Patient concerns:A 65-year-old man complained of a 2-week cough and fever.Diagnoses:Considering the presence of opacities and multiple consolidations in both lungs due to obstruction or stenosis on the bronchus, which did not respond to antibiotics, and proteinase-3-antineutrophil cytoplasmic autoantibody positivity, he was diagnosed with GPA. Positron emission tomography- computed tomography scan revealed no abnormal findings in the upper respiratory tract.Interventions:He was treated with prednisolone (PSL, 50 mg/d) and intravenous cyclophosphamide.Outcomes:His general and respiratory symptoms improved. However, 8 weeks after PSL treatment at 20 mg/d, he developed a relapse of vasculitis along with sinusitis and hypertrophic pachymeningitis. Hence, PSL treatment was resumed to 50 mg/d, and weekly administration of rituximab was initiated. Consequently, the symptoms gradually mitigated.Lessons:GPA with bronchial involvement is often intractable and requires careful follow-up, which should include upper respiratory tract and hypertrophic pachymeningitis assessment.

Long-term Outcomes of Juvenile Onset Recurrent Respiratory Papillomatosis with Pulmonary Involvement.

To investigate the clinical characteristics and long-term outcomes of juvenile onset recurrent respiratory papillomatosis (JORRP) with or without pulmonary involvement. A group of patients with JORRP who had clinical course over an extended period of time (at least 5 years) in the Department of Otolaryngology Head and Neck Surgery, Beijing Tongren Hospital were included in this retrospective study. Lung/bronchus involvement was revealed by lung imaging. Data on mortality rate, frequency of surgical interventions, and age of disease onset were collected and analyzed. The 192 patients (107 male and 85 female) included had a median [quartiles] age of JORRP onset of 2 [1, 4] years, and median follow-up duration of 10 [7, 13] years; 17 patients (8.9%) had papilloma with bronchial and pulmonary involvement 7.0 [4.0, 12.5] years after the onset of the disease. Compared to patients without lung involvement, patients with lung involvement had a younger age of disease onset (P= .001), higher frequency of surgical interventions (P< .001), higher mortality rate (OR=94.909), and an increased risk of tracheotomy that could not be decannulated (P< .001). They also had a younger age of disease onset, and a higher frequency of surgical interventions and mortality compared to patients with tracheotomy but free from lung involvement (P< .001). Children with JORRP and with pulmonary involvement have a higher average number of operations per year than those without pulmonary involvement, and pulmonary involvement indicates a higher incidence of tracheotomy that cannot be decannulated. 4 Laryngoscope, 131:E2277-E2283, 2021.

Fetal ultrasound and magnetic resonance imaging: a primer on how to interpret prenatal lung lesions.

Fetal lung lesions include common lesions such as congenital pulmonary airway malformation (CPAM), bronchopulmonary sequestration (BPS) and combined CPAM-BPS hybrid lesions, as well as less common entities including congenital lobar emphysema/obstruction, bronchial atresia, bronchogenic cysts and rare malignant pulmonary lesions such as pleuropulmonary blastoma. Fetal lung lesions occur in approximately 1 in 15,000 live births and are thought to arise from a spectrum of abnormalities related to airway obstruction and malformation, with the lesion type depending on the timing of insult, level of bronchial tree involvement, and severity of obstruction. Lesions vary from small and asymptomatic to large and symptomatic with significant mass effect on surrounding structures. Accurate diagnosis and characterization of these anomalies is crucial for guiding patient counseling as well as perinatal and postnatal management. The goal of this review is to provide an overview of normal fetal lung appearance and imaging features of common and uncommon lesions on both ultrasound and MR imaging, and to discuss key aspects in reporting and evaluating the severity of these lesions.

Does the carinal involvement have the same surgical outcome as the main bronchus involvement in patients with non-small cell lung cancer?

Carinal and main bronchus involvement were compared in terms of the survival of patients with N0-1 non-small cell lung cancer (NSCLC). Sixty-six NSCLC patients who underwent complete surgical carinal resection/reconstruction (Carina group) and complete resection because of main bronchus involvement (Main Bronchus group) between 2006 and 2016 were retrospectively analyzed. The Carina group included 30 patients and the Main Bronchus group included 36. In the Carina group, conditions other than carinal involvement that rendered patients pathological (p) T4, and in the Main Bronchus group, conditions that would upstage the pT status from pT2 were excluded. Patients with mediastinal lymph node metastases were excluded. Thus, an isolated main bronchial invasion and isolated carinal invasion patient population was tried to be obtained. The overall 5-year survival rate was 49.4% (median 61.5 ± 19.9months). The 5-year survival rates of patients in the Carina group was 49.2% (median 63.3months), and that of patients in the Main Bronchus group was 46.4% (median 55.9months). The difference between survival rates was not statistically significant (p = 0.761). The survival rates of pN0 and pN1 patients also did not differ significantly (63.2% vs. 45.5%, p = 0.207). Recurrence was significantly more common in the Main Bronchus group than the Carina group (28.1% vs. 7.1%; p = 0.04). Isolated carinal invasion had a comparable outcome to isolated main bronchus invasion in pN0-1 patients with NSCLC who are undergoing anatomical surgical resection.

The clinical characteristics of 89 cases of non-tuberculous mycobacterium pulmonary disease complicated with tracheobronchial lesions

Objective: To explore the clinical characteristics of non-tuberculous mycobacterium(NTM) pulmonary disease complicated with tracheobronchial lesions. Methods: From January 2014 to December 2018, there were 1 006 patients who were admitted to Guangzhou Chest Hospital for the first time and received examination by bronchoscopy. A total of 89 patients with complete data were selected, including 40 males and 49 females, aged 20 to 85 years, with 46 patients (52%) aged 60 years or older. The clinical symptoms and signs of the patients were analyzed with chest imaging, bronchoscopy and pathological examination results. Comparisons between groups were made by Chi-square test and t test. Results: The number of patients with NTM complicated with tracheobronchial lesions accounted for 8.9%(89/1 006) of those hospitalized with NTM and received bronchoscopy during the same period. Clinical symptoms included cough and sputum (89/89), different degrees of hemoptysis or blood sputum (52/89), and shortness of breath (50/89). Chest CT showed that 72 cases (72/89, 81%) had lung lesions involving more than 3 lung fields, and 83 cases (93%) had bronchiectasis and 63 cases (63/89, 71%) with cavities. Pulmonary atelectasis was shown in 45 cases (45/89, 51%). By bronchoscopy, 39 cases (39/89, 44%) were diagnosed as tuberculous lesions involving bilateral upper bronchi, while lesions of the right lower bronchus was found in 27 cases (27/89, 30%).The percentage of patients with multilobar bronchial involvement was 50.6%(45/89). The morphological characteristics of the bronchial lesions included scar stenosis or atresia in 63 cases (63/89, 71%), hyperemia and edema in 46 cases (46/89, 52%), and multiple types of lesions in 48 cases (48/89, 54%). Conclusions: Patients with NTM lung disease complicated with tracheobronchial inflammatory lesions did not have specific manifestations clinically. Lung lesions are extensive, often accompanied by bronchiectasis and cavity formation. Endoscopic changes were mostly manifested as multilobar tracheobronchial involvement characterized by scar stenosis, congestion and edema, mainly involving bilateral upper bronchi and the right lower bronchus.

Pasos adelante y hacia atrás en la pandemia COVID-19

Han pasado 9 meses -tiempo como el que dura una gestación-, cuando tuvimos la comunicación proveniente de Wuhan, China, sobre una enfermedad viral ocasionada por el SARS-CoV-2, el día 31 de diciembre de 2019. Desde entonces, el virus ha invadido prácticamente todo el globo terráqueo. Se conoce ahora su morfología, genoma y su asidero selectivo al receptor de la enzima convertidora de angiotensina 2 (ACE2), abundante en el endotelio de arterias y venas, pero también en el epitelio del tracto respiratorio(1). Al ingresar al cuerpo humano y multiplicarse, ocasiona un síndrome de insuficiencia respiratoria aguda, seguido por shock cardiogénico, eventos tromboembólicos, afectación de bronquios, pulmones, sistema nervioso(2), riñones(3), sistema gastrointestinal(4), la mente(5), piel(6), entre otros. El niño parece infectarse poco, frecuentemente de manera asintomática, pero su carga viral puede contagiar a los adultos(7). El menor desarrollo de COVID-19 en los niños se asociaría a una menor cantidad de receptores ACE2 en su tracto respiratorio, alguna protección dada por los resfríos comunes, menores niveles de citoquinas inflamatorias, entre otros(8).

PNEUMONIA OR LYMPHOMA? DO NOT BE FOOLED!

SESSION TITLE: Medical Student/Resident Lung Pathology SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Mucosa-associated lymphoid tissue (MALT) Lymphoma, also known as Extranodal Marginal Zone B cell lymphoma (EMZL), is a rare type of non-Hodgkin’s lymphoma with an incidence rate of 18.3 cases per 1 million in the United States. The objective of this case is to describe a challenging case of EMZL presenting as persistent localized lung consolidation that was mistaken for pneumonia. CASE PRESENTATION: 77-year-old male, former smoker who presented with complains of nonproductive cough for several weeks, chest-x-ray (CXR) showed a 4.3 cm rounded hazy right parahilar mass. A follow up computed tomography (CT) of the chest revealed 4.7 x2.8 cm consolidative opacity along the interlobar fissure in the right upper lobe (RUL). Reviewing back a year ago, CT scan done for nonspecific chest pain, showed the mass size was unchanged but appeared to be less solid compared to the current scan. Patient underwent a Fluorodeoxyglucose (FDG)-positron emission tomography (PET) scan which showed elevated FDG activity SUV 3.0 associated with a consolidative pulmonary opacity in the posterior portion of the right upper lobe silhouetting the major fissure. The patient subsequently underwent a CT guided percutaneous biopsy of the right upper lobe airspace consolidation. Histologic sections showed interstitial expansion and bronchial epithelium involvement by small monocytoid- appearing lymphocytes and significant amount of plasma cells (Figure 1). Immunohistochemistry was positive for CD20 and BCL-2 and negative for BCL-6 and CD10. CD138 which highlights plasma cell component was positive. Immunostaining for kappa and lambda showed findings most suggestive of monotypic lambda expression. Fluorescence in situ hybridization (FISH) studies for MALT1 (18q21) rearrangement identified a MALT1 gene rearrangement and B cell clonality studies done by PCR were positive for a clonal rearrangement. Therefore, the final diagnosis was small B-cell non-hodgkin lymphoma consistent with extranodal MALT. In retrospect the lymphoma was likely present a year ago however mistaken as a pneumonia. Given the lack of symptoms and minimal progression of over one year, was consistent with the indolent clinical behavior of the tumor. Decision was made not to treat at this point by the physician and patient. Till date patient remain asymptomatic. DISCUSSION: EMZL has been associated with persistent inflammatory state, either infectious or autoimmune in nature. EMZL can present in various ways from an incidental finding on imaging to ambiguous symptoms such as dyspnea, chest pain, cough, and rarely hemoptysis. Prognosis in MALT-type lymphomas is generally good with mean survival of more than 10 years. CONCLUSIONS: In conclusion, EMZL of the lung is uncommon and unique therefore a collaborative effort addressing early diagnosis and less invasive diagnostic testing for these high-risk EMZL patients are needed. Reference #1: Khalil MO, Morton LM, Devesa SS, et al. Incidence of marginal zone lymphoma in the United States, 2001 to 2009 with a focus on primary anatomic stie. Br J Haematol 2014; 165:67 Reference #2: Santos IG, Marchiori E, Zanetti G, Mano CM, SarcielliLuz B, Vianna FG. Primary pulmonary mucosaasociated lymphoid tissue lymphoma computed tomography findings: A case report. Cases J. 2009;2:6329. [PMCID:PMC2740136] [PubMed: 19829788] DISCLOSURES: No relevant relationships by Hussein Hussein, source=Web Response No relevant relationships by Ahmad JABRI, source=Web Response No relevant relationships by Sonya Joshi, source=Web Response No relevant relationships by Sujith Modugula, source=Web Response No relevant relationships by Elizabeth Verghese, source=Web Response

Management of the Disrupted Airway in Children.

Our objective was to gather data that would enable us to suggest more specific guidelines for the management of children with airway disruption. Retrospective case series with data from five tertiary medical centers. Children younger than 18 years of age with a disrupted airway were enrolled in this series. Data pertaining to age, sex, etiology and location of the disruption, type of injury, previous surgery, presence of air extravasation, management, and outcome were obtained and summarized. Twenty children with a mean age of 4.4 years (range 1 day-14.75 years) were included in the study. All were evaluated by flexible endoscopy and/or microlaryngoscopy in the operating room. Twelve (60%) children had tracheal involvement; seven had bronchial involvement; and one had involvement of the cricoid cartilage. Nine children had air extravasation, and all these children required surgical repair. Of the 11 who did not have air extravasation, only one underwent surgical repair. Complete healing of the disrupted airway was seen in all cases. This series suggests that if there is no continuous air extravasation demonstrated on imaging studies or clinical examination, nonoperative management may allow for spontaneous healing without sequelae. However, surgical repair may be considered in those patients with continuous air extravasation unless a cuffed tube can be placed distal to the site of injury. For children in whom airway injury occurs in a previously operated area, the risk of extravasation is reduced. This risk is also diminished if positive pressure ventilation can be avoided or minimized. 4 Laryngoscope, 131:921-924, 2021.

Organic vs. Inorganic Tracheobronchial Airway Foreign Body Aspiration: Does Type/Duration Matter?

We sought to determine the time course of clinical and histologic differences between aspirated inorganic and organic foreign bodies. In-vivo. Twenty Sinclair miniature swine (Sus scrofa domesticus) were divided into two groups-inorganic or organic foreign bodies. Either an organic (peanut) or an inorganic (Lego) foreign body was placed within a bronchus and left for 3, 5, 7, 14 or 21 days. The airway was reassessed at the predetermined endpoint at which time endoscopic, gross, and histopathological findings were documented. Specimens were scored with a pathologic scoring system to assess injury severity from the foreign body. Foreign bodies were successfully placed in all 20 swine. Two animals required early euthanasia due to respiratory compromise. The foreign body was identified grossly in eight (40%) animals. An additional three (15%) had microscopic evidence suggestive of a previous foreign body of an undetermined duration. There was no difference in injury severity between organic and inorganic foreign bodies. The 3-day group had injuries limited to the bronchial lining, whereas the longer duration groups had bronchial and adjacent lung parenchymal involvement. There was no difference in injury severity between days 5 and 21. Airway foreign bodies initially cause bronchial damage. After 5 days, the foreign body causes lung parenchymal changes. There was no difference in airway lesion severity between organic and inorganic foreign bodies. N/A Laryngoscope, 131:490-495, 2021.

Integrated intErventional bronchoscopy in the treatment of locally adVanced non-small lung cancER with central Malignant airway Obstructions: a multicentric REtrospective study (EVERMORE)

ObjectivesDespite new therapeutic perspectives, the presence of central airways occlusion (CAO) in patients with locally advanced non-small cell lung cancer (NSCLC) is associated with poor survival. There is no clear evidence on the clinical impact of interventional bronchoscopy as a part of an integrated treatment to cure these patients. Materials and methodsThis retrospective cohort study was conducted in two teaching hospitals over a 10 years period (January 2010-January 2020) comparing patients with NSCLC at stage IIIB and CAO at disease onset treated with chemotherapy/radiotherapy (standard therapy-ST) with those receiving interventional bronchoscopy plus ST (integrated treatment-IT). Primary outcome was 1-year survival. The onset of respiratory events, symptoms-free interval, hospitalization, need for palliation, and overall mortality served as secondary outcomes. ResultsA total of 100 patients were included, 60 in the IT and 40 in the ST group. Unadjusted Kaplan-Meier estimates showed greater effect of IT compared to ST on 1-year survival (HR = 2.1 95%CI[1.1-4.8], p = 0.003). IT showed a significantly higher survival gain over ST in those patients showing KRAS mutation (7.6 VS 0.8 months,<0.0001), a lumen occlusion >65% (6.6 VS 2.9 months,<0.001), and lacking the involvement of left bronchus (7 VS 2.3 months,<0.0001). Compared to ST, IT also showed a favorable difference in terms of new hospitalizations (p = 0.03), symptom-free interval (p = 0.02), and onset of atelectasis (p = 0.01). ConclusionsIn patients with NSCLC stage IIIB and CAO, additional interventional bronchoscopy might impact on 1-year survival. Genetic and anatomic phenotyping might allow identifying those patients who may gain life expectancy from the endoscopic intervention.

Mixed Airway and Pulmonary Parenchymal Disease in Patients With Primary Sjögren Syndrome: A 6-year Follow-up.

To assess pulmonary function and chronic obstructive pulmonary disease (COPD) development over time in patients with primary Sjögren syndrome (pSS), as well as the association between pulmonary function, radiographic findings, respiratory symptoms, and clinical features of pSS, taking cigarette consumption into account. Forty patients with pSS (mean age 66 yrs; range 42-81 yrs; 39 women), previously participating in a cross-sectional study on pulmonary involvement in pSS, were reassessed by pulmonary function tests after a mean follow-up time of 6 years. At follow-up, patients were also assessed by high-resolution computed tomography of the chest, as well as for pSS disease activity, respiratory symptoms, and cigarette consumption. Patients with pSS showed significantly decreased percentages of predicted total lung capacity (TLC), residual volume (RV), RV/TLC ratio, and diffusing capacity of the lungs for carbon monoxide, as well as an increase in predicted forced expiratory volume in 1 second/vital capacity (FEV1/VC) ratio from baseline to follow-up. The proportion of COPD in patients with pSS did not change significantly from baseline to follow-up (38% vs 40%, respectively). Radiographic signs of bronchial involvement and interstitial lung disease were each found in 38% of the patients. Both airway and pulmonary parenchymal disease were commonly found in patients with pSS, with a coexistence of both an obstructive and restrictive pulmonary function pattern, where the latter tended to deteriorate over time. COPD was a common finding. Airway and pulmonary involvement may be underdiagnosed in pSS, which is why special attention to clinical assessment of pulmonary involvement in patients with pSS is mandated.

Correlation of asthma severity, IgE level, and spirometry results with HRCT findings in allergic bronchopulmonary aspergillosis.

Context:Few studies have been done for correlating asthma severity, IgE level, and spirometry results with high-resolution computed tomographic (HRCT) findings in allergic bronchopulmonary aspergillosis (ABPA). Aims:This prospective observational study was conducted to correlate asthma severity, IgE level, and spirometry results with HRCT findings in ABPA. Settings and Design:Prospective observational. Subjects and Methods:Fifty consecutive adult patients with asthma and positive specific IgE (>0.35 kUA/L) to Aspergillus fumigatus were recruited from October 2015 to July 2017. Asthma severity, IgE levels, and spirometry results were correlated with HRCT score, bronchiectasis score, air trapping segments, and low-attenuation lung volume on inspiratory CT and expiratory CT. Statistical Analysis Used:One way ANOVA, Spearman's correlation coefficients. Results:Asthma severity showed a significant positive correlation with HRCT score and bronchiectasis score. MEF pre and postbronchodilator values showed a significant negative correlation with HRCT score, bronchiectasis score, and percentage expiratory volumes -851 to -950 HU voxels. FEV1 prebronchodilator value showed a significant negative correlation with percentage expiratory volume -851 to -950 HU voxels and percentage expiratory volume -851 to -1024 HU voxels. Specific IgE antibody level showed a significant positive correlation with bronchiectasis score. Conclusions:Asthma severity, specific IgE level, and MEF values showed a good correlation with HRCT findings. The restrictive pattern is common on spirometry in patients of ABPA. In addition to central bronchiectasis, peripheral bronchial and small airway involvement was an important finding in ABPA. Expiratory HRCT may reveal air trapping in patients having no abnormality on inspiratory CT.

A case of toxic epidermal necrolysis with refractory acute respiratory distress syndrome

Toxic epidermal necrolysis (TEN) is a life-threatening cutaneous reaction with several complications. Although acute respiratory distress syndrome (ARDS) is rare, it can cause rapid and potentially fatal pulmonary dysfunction. Here, we present a case of TEN with ARDS attributed to acetaminophen. Toxic epidermal necrolysis (TEN) is a life-threatening cutaneous reaction with several complications. Although acute respiratory distress syndrome (ARDS) is rare, it can cause rapid and potentially fatal pulmonary dysfunction. Here, we present a case of TEN with ARDS attributed to acetaminophen. A 49-year-old woman with cerebral palsy was admitted to our hospital with a high fever and erythema of the face and trunk that developed 1 day earlier. She took acetaminophen twice at 4 and 1 day before admission. The erosion covered approximately 30% of the body surface area, and hemorrhagic erosions were observed on the lips (Figure 1A). TEN was diagnosed, with a severity-of-illness score (SCORTEN) of 3. Chest computed tomography (CT) revealed a bilateral infiltrative shadow in the lower lung fields (Figure 1B). Aspiration pneumonia was diagnosed. All medications were discontinued, and methylprednisolone pulse therapy (steroid pulse; 1000 mg/d for 3 days) was administered with antibiotics. On Day 2, she developed acute-onset dyspnea (SpO2: 70%-80%). Chest CT revealed spreading of the bilateral diffuse infiltrative shadow (Figure 1C). ARDS induced by TEN was diagnosed, and mechanical ventilation was initiated. Intravenous immunoglobulin therapy (IVIg; 400 mg/kg/d for 5 days) was administered after steroid pulse therapy, leading to rapid improvements in the skin and mucosal involvement. The prednisolone dosage was tapered to 30 mg/d, and re-epithelialization was completed on Day 19. Nevertheless, the ARDS responded poorly, and steroid pulse therapy was repeated on Days 19-21. Thereafter, the chest shadow and dyspnea gradually improved, and prednisolone was slowly tapered to 5 mg/d on Day 100. However, she developed an ARDS relapse a few days later and received steroid pulse therapy. The chest CT findings improved, although she could not be weaned from mechanical ventilation upon discharge at Day 150. A drug-induced lymphocyte stimulation test was positive for acetaminophen (stimulation index: 803%), and a patch test was negative. Reports describe an approximate respiratory complication rate of 40% during the acute phase of SJS/TEN.1 Prost et al reported that approximately 25% of SJS/TEN patients required mechanical ventilation (MV); of these cases, 57% had a fatal outcome.2 Lebargy et al reported that dyspnea, hypoxia, increased bronchial secretions, and normal chest X-rays within the first 3 days of illness indicated early bronchial involvement.3 To date, however, few case reports have described the features of SJS/TEN with ARDS. Three of 138 patients with SJS/TEN who were treated at our hospitals from 2000 to 2019 developed ARDS, of whom two (including the present case) survived. The third and fatal case did not begin treatment until more than 1 week postonset. Evidence regarding the effective treatment of pulmonary complications with SJS/TEN is lacking. Although antibiotics and steroids are commonly used,1 the effectiveness of systemic steroid therapy for ARDS remains controversial; some studies reported effectiveness,4, 5 while others reported no survival benefit.6, 7 Moreover, steroid-induced infections tend to worsen prognosis. We conclude that steroid initiation promptly after onset, followed by IVIg, may have contributed to our patient's survival. Additional case studies are needed to support future treatment development. The authors declare no conflict of interest.

SPIROMETRIC PARAMETERS IN PATIENT WITH ALLERGIC RHINITIS

Abstract Background : the link between allergic rhinitis(AR) and asthma has been confirmed. AR is risk factor for the onset of asthma . Spirometry is noninvasive method to evaluate lower respiratory system function in AR patients. The aim of the study: to evaluate spirometric lung function and response to post- bronchodilatation test in patient with AR. Methods: This prospective cross sectional study carried out on 100 patients with persistent AR. Spirometry ,post- bronchdilatation test, serum total IgE and eosinophils in blood and sputum were performed for all patients. Results: Our results reconfirming the concept of a single air way with single disease. In our study the patients with persistent AR were have impaired spirometric parameters. Conclusion: Our study confirming the predictive value of forced expiratory flow at 25% and 75% (FEF 25-75) as marker of early bronchial involvement of small air way disease (SAD) in AR and also confirm the link between upper and lower respiratory system.

Is it possible to discriminate pulmonary carcinoids from hamartomas based on CT features?

PurposeThe purpose of this study was to determine whether the computed tomography (CT) features might be used in distinguishing pulmonary carcinoids from hamartomas. Materials and methodsNinety solid pulmonary nodules (43 carcinoids and 47 hamartomas) in 90 patients were evaluated. The following CT scan features were evaluated: size, location (peripheral/central), contour (lobulated/nodular), number of lobulation, attenuation, calcification, endobronchial status, bronchial extension and involvement, parenchymal abnormalities distal to the lesion e.g. hyperlucency, atelectasis, and nodularity. The final pathologic diagnosis of the lesions and bronchial extension were confirmed by review of histopathological specimens. ResultsOut of 43 carcinoids, 37 (86%) were typical. Twenty-three carcinoids and four hamartomas were central (p < 0.001). Ten carcinoids and one hamartoma were endobronchial. The majority of tumors had lobulated contours (65% of carcinoids, 44% of hamartomas) and carcinoids tended to have more lobulations (p = 0.052). Distal nodularity (p = 0.001), distal hyperlucency (p < 0.001), and atelectasis (p = 0.005) were significantly more common in carcinoids. Carcinoids had significantly more bronchial extension and involvement (p < 0.001; respectively). In addition, a new sign that we call “bronchial triangle sign” differentiated carcinoids with a sensitivity and specificity of 84.9% (95% CI: 69,1%–93.4%) and 91% (95% CI: 79.7%–96.6%). ConclusionTo the best of our knowledge this is the first study on discrimination of carcinoids and hamartomas. A new CT sign called “bronchial triangle sign” might be used to differentiate carcinoids from hamartomas. Distal parenchymal abnormalities are more common in carcinoids than in hamartomas.