Airway Involvement Research Articles

The Diagnosis of Granulomatosis With Polyangiitis When Serology and Biopsies are Negative.

Granulomatosis with polyangiitis (GPA) is a potentially fatal condition which often manifests in the head and neck. Currently, diagnosis relies on antineutrophil cytoplasmic autoantibody (c-ANCA) serology and mucosal or renal biopsy. However, a significant proportion of patients with GPA limited to the head and neck are seronegative and biopsy negative. This study evaluates the role of clinical diagnosis of GPA in the absence of positive laboratory findings. Case series with chart review. Academic Tertiary Medical Center. This was a retrospective review of 143 patients treated in an outpatient otolaryngology clinic at a tertiary care hospital for known or suspected GPA from 1998 to 2021. Presenting symptoms, C-ANCA status at initial presentation, biopsy results, long-term serology results, and time to initiation of treatment were analyzed. Twenty-six of 143 (18.2%) patients were seronegative; only 3 of these patients (12%) had positive biopsies. Seventeen (73.9%) of these patients presented with nasal and sinus disease and 12 (52.2%) presented with airway involvement. Only 4 (17.4%) patients had renal involvement. Delay in treatment of patients with negative laboratory workup ranged from 0 months to 11 years. All patients who were seronegative and/or biopsy negative at presentation responded clinically to immunosuppressive therapy. GPA cases are often limited to the upper respiratory tract, making diagnosis difficult, particularly in seronegative patients. These results suggest that, when GPA is suspected, despite negative serology, the diagnosis of GPA should be made on clinical grounds, and empiric therapy encouraged to prevent delay in treatment.

Saddled by the Saddle Nose: A Case of Wegener's Granulomatosis

Wegener's Granulomatosis with polyangiitis (GPA) is a systemic disease of unknown etiology characterized by necrotizing granulomatous inflammation, tissue necrosis, and variable degrees of vasculitis in small- and medium-sized blood vessels. The classical clinical triad consists of involvement of the upper airways, lungs, and kidneys. Saddle-nose deformity is a well-recognized complication of GPA but more commonly occurs due to leprosy, trauma, cocaine abuse, and sarcoidosis. Since this entity is not commonly encountered in our country, we present a unique case of a middle-aged woman presenting with saddle-nose deformity and the diagnostic dilemmas faced by us. This case is being reported due to its rarity in Indian literature.

Predictors of medications-free and long-term remission in anti-neutrophil cytoplasmic antibody-associated vasculitis: Real-world evidence.

In this study, we report the outcomes of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in daily practice based on Connective Tissue Diseases Research Center-Vasculitis Registry (CTDRC-VR) data. Patients were included if they were 18 years or older, had a diagnosis of the groups of AAV based on 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis, and were followed for a period longer than 2 years or were died. Complete clinical remission was defined as granulomatosis with polyangiitis (BVAS/GPA) of 0. Sustained remission was defined as a complete clinical remission for at least six months and tapering prednisolone dose to ≤ 7.5 mg/d. Long-term remission was defined as complete clinical remission for ≥ 5 years and tapering prednisolone dose to ≤ 7.5 mg/d. Medications-free remission was defined as complete clinical remission and discontinuation of glucocorticoids, cytotoxic medications and biologics. Sixty patients with AAV were enrolled in this study. Sustained and long-term remission were developed in 91.7 and 72.1 percent of patients, respectively. Relapse was developed in 27 (45%) patients. Medications-free remission was developed in 23 (33.3%) patients. Vasculitis induced damage was developed in 40 (66.7%) patients. Patients with damage had significantly lower age and higher BVAS at the baseline. Upper airway and renal involvement, and non-adherence in patients with damage was significantly more common. Induction therapy leads to long-term and medications-free remission in 72% and 38% of patients with AAV, respectively.

The Omicron variant of COVID-19 and its association with croup in children: a single-centre study in Hong Kong.

The fifth wave of the coronavirus disease 2019 (COVID-19) pandemic in Hong Kong was dominated by the Omicron variant, which may cause more upper airway involvement in children. This study was performed to identify any associations between the Omicron variant of COVID-19 and croup in children. This retrospective study reviewed the electronic medical records of patients admitted to Tuen Mun Hospital in Hong Kong from 1 January 2018 to 31 March 2022 under the diagnostic code for croup (J05.0 in the International Classification of Diseases 10th Edition). Patients were categorised into three groups according to their admission periods, namely, non-COVID-19, COVID-19-pre-Omicron, and COVID-19-Omicron groups. Disease associations and severity were compared according to incidence, Westley Croup Score, length of hospital stay, medication use, respiratory support, and intensive care unit admissions. The COVID-19 incidence among patients with croup was significantly higher in the COVID-19-Omicron group than in the COVID-19-pre-Omicron group (90.0% vs 2.0%; P<0.001). Compared with patients in the COVID-19-pre-Omicron and non-COVID-19 groups, patients in the COVID-19-Omicron group also had a higher Westley score (moderate and severe disease in the COVID-19-Omicron group: 56.7%; COVID-19-pre-Omicron group: 22.0%, P=0.004; non-COVID-19 group: 24.8%, P<0.001), longer median hospital stay (COVID-19-Omicron group: 3.00 days; COVID-19-pre-Omicron group: 2.00 days, P<0.001; non-COVID-19 group: 2.00 days, P=0.034), and higher mean dexamethasone requirement (COVID-19-Omicron group: 0.78 mg/kg; COVID-19-pre-Omicron group: 0.49 mg/kg, P<0.001; non-COVID-19 group: 0.58 mg/kg, P=0.001). The Omicron variant of COVID-19 is associated with croup and can cause more severe disease in Hong Kong children.

Angiœdème bradykinique héréditaire : à propos d’un cas homozygote décrit au Niger et revue de la littérature

Hereditary angioedema (HAE) is a rare, potentially serious disease. It is a pathology characterized by the occurrence of recurrent, localized subcutaneous or submucosal edemas, without associated urticaria, lasting between twenty-four (24) and seventy-two (72) hours. It disappears without leaving behind and can reoffend. It is an autosomal dominant disease that results from a hereditary deficiency of C1 inhibitory function. The gene encoding C1-inh (SERPING 1) was located on chromosome 11 (11q12-q13.1). Our work is a clinical case study with review of the literature about a case of hereditary bradykinic angioedema registered in the Dermato-allergology department of the Amirou Boubacar Diallo National Hospital. The case report is a 37-year-old women with a history of chronic lupus erythematosus referred from the ORL department for an allergic assessment of facial edema. Her clinical history reports several episodes of angioedema of the face, extremities of the members occurring sporadically and which last on average 48 to 72hours sometimes accompanied by digestive disorders and Quincke's edema. His blood and genetic tests reported a quantitative and qualitative deficit in C1 inhibitor, a lowered level of C4 complement and a homozygous mutation in the SERPING1 gene that led to the diagnosis of hereditary bradykinic angioedema type I. In conclusion, hereditary bradykinic angioedema is a rare disease that may be underestimated because of its lack of knowledge by many practitioners. The clinical picture is often misleading, morbimortality is linked to the involvement of the upper airways and abdominal attacks. The diagnosis is based on the exploration of the complement system.

Lung Involvement in Patients with Ulcerative Colitis: Relationship between Exhaled Nitric Oxide and Lung Function.

Ulcerative colitis (UC) is characterized by immune system dysregulation with frequent extraintestinal manifestations, including airway involvement. A reduction in CO diffusing capacity and functional alterations in small airways have been described. An extended analysis of fractional exhaled nitric oxide (FeNO) may distinguish the sites of production, and the presence of small airway inflammation may be a useful, non-invasive marker for patient follow-up. The aim of our study was to compare the PFTs as well as FeNO and CANO values of UC patients with different clinical disease activities and healthy subjects to reveal lung function abnormalities and the presence of subclinical airway inflammation. We enrolled 42 adult outpatients at different clinical activity stages of UC (39 ± 13 years) and a healthy control group of 41 subjects (29 ± 3 years). C-reactive protein (CRP) and FeNO values at different flows (50,100, and 200 mL/s) were collected. All patients performed pulmonary function tests (PFTs) with static volumes and diffusing capacity (DLCO). FeNO and CANO values were significantly increased in UC patients when compared with controls (p = 0.0008 and p < 0.0001, respectively) and were proportional to disease activity (FeNO class 3: 28.1 ppb vs. classes 1-2: 7.7 ppb; CANO values class 3: 8.6 ppb vs. classes 1-2: 2.7 ppb (p < 0.0001)). TLC and DLCO were significantly reduced in severe (Mayo 3) UC patients (p = 0.010 and p = 0.003, respectively). The results of this study show significant lung functional abnormalities in UC patients and suggest the presence of airway inflammation directly correlated with disease activity, suggesting the need for an integrated approach in routine assessment.

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome following Ticagrelor administration leading to serious complications.

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is rarely reported with administration of Ticagrelor. We report a case, where such complication developed after 7 days of Ticagrelor administration, leading to acute severe complications requiring hospitalization. There was airway and renal involvement with elevated inflammatory markers. On withdrawing Ticagrelor, there was rapid resolution of symptoms.

Anaesthesia and Airway Challenges in Patients with Corrosive Pharyngo-oesophageal Stricture: Experience of a Tertiary Care Centre

Background: Ingestion of corrosive substances causes necrosis of the tissues of the upper airway and upper gastrointestinal tract resulting not only in acute airway injuries but also long-term sequelae like strictures. Medical and surgical management of these strictures has been discussed extensively; however, there is little literature available on the anaesthetic management of pharyngo-oesophageal strictures. We hereby discuss our experience in managing these patients undergoing surgical correction. Methods: Out of a total of 57 cases of corrosive ingestion injuries between 2008 and 2016, medical records of 15 patients with complex pharyngeal or high oesophageal strictures undergoing surgical intervention were reviewed. The preoperative findings, the extent of airway involvement and any airway intervention done were noted. Based on the surgical intervention, patients were divided into two groups; Group A included patients undergoing repeated string dilatation and Group B included those undergoing coloplasty. Type of anaesthesia given, details of airway management, intraoperative and postoperative course and complications were noted. Results: Group A consisted of 10 patients who underwent endless string dilatation, and Group B consisted of five patients who underwent coloplasty. In Group A, general anaesthesia with endotracheal intubation was given to all patients except for one patient who was tracheostomised. Two patients had significant glottic narrowing and required a smaller size tube. In Group B, four out of five patients were tracheostomised, while one had a normal glottic opening. General anaesthesia with epidural analgesia was given to all patients. Severe haemodynamic changes were seen during tunnelling through the posterior mediastinum for colonic interposition. All patients were extubated on the table. None of the patients required postoperative ventilatory or inotropic support. Conclusion: Management of corrosive pharyngo-oesophageal strictures is challenging and requires a multidisciplinary approach. Careful assessment and management of the airway, vigilant intraoperative haemodynamic monitoring and good pain management are the key contributions of anaesthesia care.

A 58-year-old man with progressive dyspnea suffering from Munier-Kuhn syndrome

A 58-year-old man who was a former smoker and had a 20-pack-year smoking history presented to the hospital with worsening shortness of breath. Pulmonary function tests revealed mixed obstructive-restrictive physiology. Forced vital capacity (FVC) was predicted at 68%, forced expiratory volume in 1 second (FEV1) at 60% was predicted, FEV1/FVC at 67% was predicted, and Carbon monoxide (DLCO) diffusion capacity is expected to be 70%. Chest images show increased interstitial density around the hilum. There is massive tracheobronchiomegaly with a tortuous appearance in the airways. There is also a small degree of parenchymal scarring and fibrosis-like reticular perihilar interstitial opacity. CT images show traction bronchiectasis is a finding in the setting of fibrotic lung disease. When lung tissue becomes inelastic due to the formation of dense fibrosis, the airways appear blocked and dilated. Although this patient has dilated airways, the bronchiectasis is greater than the small amount of parenchymal fibrosis seen. Therefore, this process is not caused by parenchymal fibrosis, but rather by inherent pathology of the airway wall. The most frequently reported symptoms of Mounier-Kuhn syndrome are severe cough, difficulty breathing, and recurrent respiratory infections. Patients may complain of chest pain and hemoptysis, but systemic symptoms are rare and require immediate investigation for other diseases. Pulmonary function tests often reveal obstructive pulmonary disease, but in advanced stages there may be limited parenchymal changes, resulting in a mixed appearance, as in a patient's case. Because airway changes are considered irreversible, treatment is aimed at managing symptoms and reducing long-term complications. Recommended lifestyle modifications include quitting smoking and avoiding irritants/particulates. Concurrent COPD or asthma is addressed to limit further morbidity. The infection is treated aggressively, usually requiring broad-spectrum antibiotics to treat atypical infections. The use of expectorants and chest physiotherapy is considered helpful in preventing recurrent infections. Tracheal stenting may be considered if there are specific airways prone to collapse, but the spread of airway involvement limits its potential utility.

Treatment of infantile hemangioma with topical β-blockers in pediatric practice: a review of the literature

The purpose of this review is to acquaint the audience with the benefits of infantile hemangioma (IH) treatment with local β-blockers, which are safe for the child, allow for the complete cure of hemangiomas without surgery, complications, scars, and cosmetic defects, and have practically no systemic side effects. We aimed to analyze the effectiveness and limitations of the use of local β-blockers, to show the possibility and expediency of IH management in pediatric practice, and to demonstrate that the goal of therapy is not the treatment of complications, but the prevention of their occurrence and complete resolution of the hemangioma without surgical intervention. The use of topical β-blockers, such as timolol gel or solution, provides a non-surgical, non-invasive treatment option for IH, minimizing the systemic exposure and possible side effects associated with oral β-blockers. Local treatment is best started before the age of 2 months - at an early stage, when IHs are small in size, potentially preventing the need for more invasive treatment methods. Topical use is associated with a lower risk of systemic side effects, such as hypotension or bradycardia, that can occur with oral β-blockers. Topical treatment can be applied at home, which can be more convenient for parents and caregivers, and also increases the opportunity for treatment and follow-up by pediatricians. In case of natural disasters or military operations, treatment can be done remotely using teledermatology. The effectiveness of local β-blockers mostly depends on the age at which treatment was started: the earlier it was started, the higher the effectiveness. Treatment of complicated IH with deep soft tissue, mucosal, or airway involvement usually requires a combination of systemic propranolol and topical β-blockers or other interventions such as laser therapy. The choice of treatment should be selected individually according to the degree of risk of IH, its size, localization, child's age, weight, and other parameters in each clinical case. β-adrenoblockers are the most modern, effective, non-surgical, and safe method of treating IH, they can be used both for systemic and local application. IH can be successfully treated by pediatricians and dermatologists. No conflict of interests was declared by the authors.

Facial burn in children: a review

Burns are a common cause of injury among children. Children with facial burns often experience pain, regardless of the cause, size, or depth of the burn injury. Burns in the facial area may lead to fatal consequences because of the involvement of the upper airway and cosmetic deformity of the face. Major burn injury in the facial area remains a significant cause of morbidity and mortality in children. Burn scars on the face in children may disturb the self-image and psychosocial processes. Multi-disciplinary approaches and advances in burn care are often helpful to manage the burn injury in children and increase the survival of the patients. Pain has adverse emotional and physiological impacts, and adequate pain control is an important factor in improving outcomes. The goal of the treatment of facial burns in children is to achieve a good aesthetic outcome and also normal head movement with neck mobilization.

Histaminic Angioedema in Children: A Rare Entity Not to be ignored

Angioedema is a common symptom to different etiologies, the diagnostic orientation is based on the associated signs. The most common are histamine, non IgE-mediated, benign and often associated with urticaria. Angioedema by anaphylaxis and bradykinin angioedemas are rare but potentially severe by upper airway involvement. We report the case of a 14 years old patient with a history of bilateral orbital swelling associated with deep urticaria, and a brother followed for angioedema. Presenting bilateral palpebral and jugal edema, associated with uticarian tasks in the trunk and back. The diagnosis of histaminic angioedema was made and confirmed by the response to corticosteroids and antihistamines, and a biologic workup ruling out differential diagnoses. Treatment was based on the management of the crisis and the implementation of in-depth treatment in the event of recurrent and disabling crises. Measures associated with this drug treatment are also essential, mainly therapeutic education.

LM-DNN: pre-trained DNN with LSTM and cross Fold validation for detecting viral pneumonia from chest CT

ABSTRACT Some of the viruses may cause lung parenchyma and airway involvement. Usually, viral pneumonia causes ground-glass opacities, bilateral peripheral distribution, consolidation, vascular thickening, and reticular opacity. These features are common in COVID-19 rather than Non-Covid-19 viral pneumonia. However, in advanced cases, COVID-19 viral pneumonia may cause organising pneumonia and fibrosis of the lung. Atypical findings of Non-Covid-19 pneumonia have included central peripheral distribution, pleural effusion, lymphadenopathy, nodules, tree-in-bud opacities, and pneumothorax. Therefore, differentiating Non-Covid-19 pneumonia from COVID-19 pneumonia at chest computed tomography (CT) is necessary. In that case, CT scans of the thorax are one of the essential tools for early identification and future prognosis of viral pneumonia. We have proposed a Computer-Aided Diagnostic (CAD) system that can detect features of chest CT using a Deep Neural Network (DNN) with Long Short-Term Memory (LSTM). Transfer learning using pre-trained DNN models (ResNet50, VGG19, InceptionV3, Xception, DenseNet121, and VGG16) is applied to retain both high-level and low-level features effectively. The deep features are passed to the LSTM layer. The LSTM is utilised as a classifier and detects long short-term dependencies. The proposed method employs a hybrid DNN-LSTM network for automatic detection to take advantage of the uniqueness of the two models. The proposed models are trained with common and different features present in the chest CT of COVID-19 and Non-Covid-19 viral pneumonia. The 5-fold cross-validation (CV) method validated and tested the proposed model. The proposed DNN model’s performance is quite improved with LSTM and CV. As a result, the proposed LM-DNN (VGG16+LSTM+CV) model has achieved the classification test accuracy of 91.58% and specificity of 93.86%, which offers superior performance with state-of-the-art. Also, the DenseNet121+LSTM+CV model has reached the classification test accuracy of 90.1% and sensitivity of 92%.

HRCT imaging of pulmonary involvement in granulomatosis with polyangiitis and microscopic polyangiitis at disease onset and during follow-up

BackgroundThe pulmonary involvement in patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) is well known at disease onset but data during follow-up (after the induction regimen and when the first relapse occurs) are limited. Our goal was to analyze chest high-resolution computed tomography (HRCT) findings of (ANCA)-associated vasculitis patients. MethodAll consecutive unselected AAV patients over eighteen with positive ANCA status and with HRCT chest performed at the diagnosis were retrospectively enrolled between 2004 and 2019 at the Toulouse University Hospital (France). Two experienced pulmonologists and one expert respiratory radiologist reviewed independently HRCT chest scans. ResultsA total of 157 AAV patients were included in the study. Two-thirds of AAV patients had pulmonary involvement at diagnosis. Diffuse alveolar hemorrhage (DAH) was observed in 31.2 % of cases, nodules and masses in 18.5 %, bronchial airway involvement in 13.4 %, and interstitial involvement in 12.7 %. Following the induction regimen, chest HRCT scans over a two-year period demonstrated significant improvement in DAH and nodular manifestations, whereas bronchial airway involvement exhibited variability and half of cases of interstitial lung disease (ILD) had progressive course. Outcomes and survival rates are better for nodular and bronchial involvement. DAH was the most frequent cause of deaths. Progressive fibrotic changes in ILD over time could impact prognosis despite AAV remission. ConclusionEmploying a pattern-based approach with HRCT chest scans to assess lung involvement could be valuable in predicting treatment response, relapse, mortality, and could improved the management of AAV patients.

Small airway dysfunction and impulse oscillometry in adult patients with asthma: recent findings

Asthma is a respiratory disease affecting more than 300 million people around the world. Airflow obstruction and inflammation due to asthma usually involve large airways, but recently small airway involvement (internal diameter &lt; 2 mm) has been shown to represent one of the main determinants of asthma and asthma control. In fact, compared to large airway involvement, small airway dysfunction (SAD) has been demonstrated across all the asthma severity in the majority of patients, as assessed with Global Initiative for Asthma (GINA) steps. Clinically, SAD is associated with, among other features, exercise-induced bronchoconstriction, asthma-related night awakenings, obesity/overweight, more severe airway hyperresponsiveness, worse asthma control, and more severe exacerbations. Impulse oscillometry (IOS), a forced oscillation technique (FOT) requiring less effort than spirometry from the patients, demonstrated to accurately measure SAD in children and adults. The fall in resistance from 5 Hz to 20 Hz (R5–R20), which is the most used index for the resistance of peripheral airways, is how SAD is usually identified by IOS. Other crucial parameters measured by IOS are the reactance at 5 Hz (X5), reflecting elastic recoil of the peripheral airways, the resonant frequency (Fres), which is the frequency at which the inertial properties of the airway and the capacitance of the lung periphery are equal, and the reactance area (AX), reflecting the elastic properties of the lung periphery. In this mini review, the latest findings on the utility of IOS to identify SAD and the associations between SAD and clinical features in adult asthmatic patients were addressed.

Airway Involvement and Intervention in Non-ACE-Inhibitor-Induced Angioedema.

Characterize the presentation of patients with non-angiotensin-converting enzyme inhibitor (ACEI)-induced angioedema and determine risk factors associated with patient disposition and possible need for airway intervention. The medical records of adult patients in the Emergency Department (ED) and diagnosed with non-ACEI-induced angioedema over 4.5 years were included. Demographics, vital signs, etiology, timeline, presenting symptoms, physical exam including flexible laryngoscopy, medical management, and disposition were examined. Statistical analyses were conducted using SPSS V 23.0 software calculating and comparing means, standard deviations, medians, and correlation of categorical and ordinate variables. A total of 181 patients with non-ACEI-induced angioedema were evaluated with flexible laryngoscopy by otolaryngology. Notably, 11 patients (6.1%) required airway intervention and were successfully intubated. Statistically significant factors (p ≤ 0.05) associated with airway intervention included the diastolic blood pressure (DBP) and mean arterial pressure (MAP) (p = 0.006 and 0.01 respectively), symptoms of dysphonia (p = 0.018), the presence of oropharyngeal, supraglottic, and hypopharyngeal edema (p ≤ 0.001 for each site), and the number of edematous anatomic subsites documented on physical exam (p < 0.001). Other patient demographics, prior history of angioedema, heart rate, systolic blood pressure, symptom onset, number of symptoms at presentation, and medication administered in the ED did not correlate with airway intervention. Dysphonia, DBP, MAP, anatomic location of edema and edema in multiple sites are associated with airway intervention and a higher level of care in non-ACEI-induced angioedema and can be useful in risk assessment in patient management. 4 Laryngoscope, 134:2282-2287, 2024.

Single-system pulmonary langerhans cell histiocytosis with only tracheobronchial involvement: a case report

BackgroundPulmonary Langerhans cell histiocytosis (PLCH) only with airway involvement manifested as diffuse thickening of the tracheobronchial walls is rare.Case report A 26-year-old male was admitted to the hospital with progressive wheezing, cough, and a source of blood in sputum after activity. He had no history of smoking. Chest computed tomography showed airway stenosis of different degrees with tracheobronchial wall thickening, and fiberoptic bronchoscopy demonstrated multiple nodular neoplasms in tracheobronchial, while the pulmonary parenchyma was normal. The patient’s condition partially improved after excision of partial lesions by fiberoptic bronchoscope. Histopathological results showed that CD1a and S-100 immunohistochemical staining was positive, and the molecular pathological results suggested that the BRAF V600E mutation, thus confirming the diagnosis of PLCH. The treatment of partial resection and systemic chemotherapy is effective. ConclusionsThe possibility of PLCH needs to be considered when diffuse tracheobronchial lesions without lung parenchyma involvement are encountered, which provides experience for early clinical diagnosis and adequate treatment.

Pulmonary Function in Pulmonary Sarcoidosis.

The pulmonary function test (PFT) has been widely used in sarcoidosis. It may vary due to the severity, extent, and the presence of complications of the disease. Although the PFT of most sarcoidosis patients is normal, there are still 10-30% of cases who may experience a decrease in the PFT, with a progressive involvement of lungs. Restrictive ventilatory impairment due to parenchymal involvement has been commonly reported, and an obstructive pattern can also be present related to airway involvement. The PFT may influence treatment decisions. A diffusing capacity for carbon monoxide (DLCO) < 60% as well as a forced vital capacity (FVC) < 70% portends clinically significant pulmonary sarcoidosis pathology and warrants treatment. During follow-up, a 5% decline in FVC from baseline or a 10% decline in DLCO has been considered significant and reflects the disease progression. FVC has been recommended as the favored objective endpoint for monitoring the response to therapy, and an improvement in predicted FVC percentage of more than 5% is considered effective.

Clinical features and outcomes of small airway disease in ANCA-associated vasculitis.

To clarify the prevalence, features and outcomes of small airway disease (SAD) in a Chinese cohort with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) related pulmonary involvement. SAD was recorded when the manifestations of either centrilobular nodules or air trapping were observed according to CT scans, except for infection or other airway-related comorbidities. Baseline and follow-up data were collected retrospectively. Of the 359 newly diagnosed AAV patients with pulmonary involvement, 92 (25.6%) had SAD, including 79 (85.9%) cases of anti-MPO-ANCA positive, 9 (9.8%) cases of anti-PR3-ANCA positive and 2 (2.2%) cases of double positive. Patients with SAD were more likely to be younger, female, non-smokers, have more ear-nose-throat (ENT) involvement, and have higher baseline Birmingham Vasculitis Activity Score (BVAS) compared to patients without SAD. Several AAV-related SAD patients have improved lung function and CT scans after immunosuppressive therapy. Patients with SAD had a better prognosis compared to those without SAD. When dividing all patients into three groups: isolated SAD (only small airway involvements), SAD with other lower airway involvements, and non-SAD, patients in the SAD with other lower airway involvements group had the highest risk of infection, while patients in the non-SAD group had the worst long-term outcomes. Similar results were observed in anti-MPO-ANCA positive patients when performing subgroup analyses. SAD is a unique manifestation of AAV-related lung involvement and exhibits distinct clinical features. It is vital to focus on SAD because of its association with prognosis and infection in AAV patients, especially in anti-MPO-ANCA positive patients. Moreover, SAD might represent a better response to immunosuppressors.

Imaging features and clinical value of 18F-FDG PET/CT for predicting airway involvement in patients with relapsing polychondritis

BackgroundThe clinical value of 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) in assessing relapsing polychondritis (RP) with airway involvement remains controversial. This study aimed to investigate PET/CT features of RP with airway involvement and explore its clinical value in predicting disease pattern, severity and prognosis.MethodsRP patients with airway involvement who underwent PET/CT from January 2010 to July 2022 were retrospectively reviewed. PET/CT features were analyzed both visually and semiquantitatively with the maximum standardized uptake value (SUVmax) and total lesion glycolysis (TLG). Patterns of airway involvement on PET were summarized. Correlations of SUVmax and TLG of the airway were made with spirometric indicators and serological inflammatory markers (CRP and ESR). In addition, long-term follow-up was conducted through questionnaires in regard to symptom control, subjective feeling, pulmonary function, and quality of life.ResultsFifty-two cases were finally included. 18F-FDG PET showed FDG-avid lesions with increased FDG uptake in the airway among 94.2% of the patients. Three patterns (focal, multifocal and diffuse patterns) were identified. TLG of the whole airway was lower in patients with previous therapy (p = 0.046). Bronchoscopy was more sensitive in detecting tracheal abnormalities (90.7% vs.53.5%, p = 0.039) but less sensitive for peripheral airway lesions (65.1% vs. 79.1%, p = 0.046) compared with PET. SUVmax and TLG of the airway positively correlated with spirometry indicators (FEV1%pred, FEV1/FVC, MEF 50%pred, etc.) and serological inflammatory markers. Five patients died during the follow-up, with two deaths related to airway problems. Higher FDG uptake predicted worse subjective feeling, but not with symptom control or pulmonary function.ConclusionPET/CT is a valuable tool for RP with airway involvement, particularly in assessing peripheral airway lesions, and PET/CT related parameters are significantly associated with disease patterns, severity, and long-term outcomes.