Centrilobular Ground-glass Opacities Research Articles

Regional anesthesia in a patient with primary ciliary dyskinesia: A case report

BACKGROUND Primary ciliary dyskinesia (PCD) is an inherited autosomal-recessive disorder of impaired mucociliary clearance characterized by chronic respiratory diseases, otolaryngological diseases, central nervous system abnormalities, reproductive system abnormalities, and cardiac function abnormalities. General anesthesia in these patients is associated with a higher incidence of respiratory complications than in patients without the disease. CASE SUMMARY A 16-year-old male patient was referred to the emergency room complaining of right ankle pain due to distal tibiofibular fracture. Three years prior, he had been diagnosed with PCD. At that time, he had experienced several episodes of pneumonia, sinusitis, and chronic middle ear infections, for which he underwent surgical interventions. At the current admission, he presented with cough and sputum but no other respiratory symptoms. A chest computed tomography scan revealed centrilobular ground-glass opacities in both lower lobes and a calcified nodule in the left lower lobe. For the surgical procedure and postoperative pain management, combined spinal-epidural anesthesia was employed. The patient’s postoperative pain score was measured by the numerical rating scale (NRS). On the day of surgery, his NRS was 5 points. By the second postoperative day, the NRS score had decreased to 2–3 points. The epidural catheter was removed on the fourth day following the operation. The patient was subsequently discharged no respiratory complications. CONCLUSION We performed combined spinal-epidural anesthesia in a patient with PCD. The patient experienced no additional respiratory complications and was discharged with a low NRS score for pain.

Pulmonary veno-occlusive disease with vanished pulmonary consolidation.

Pulmonary veno-occlusive disease (PVOD) is an extremely rare cause of pulmonary hypertension. Previously reported computed tomography (CT) findings of PVOD included centrilobular ground-glass opacities, a mosaic pattern, and septal lines; however, chest CT revealing pulmonary consolidation disappearance with repositioning has not been reported.

The HRCT Imaging Findings of the Peripheral Pulmonary Vessels in Patients with Pulmonary Hypertension (PH)

The prominence of the pulmonary vasculature and/or tortuosity (n = 16) and dilatation of the central PA (n = 39) with RV hypertrophy were frequently observed. The interlobular septal thickening (n = 18, 45%), and centrilobular (perivascular) ground glass opacities (n = 6, 15%) were clearly depicted on gapless HRCT. PA aneurysmal dilatation and irregular dilatation with arterio-venous fistula were observed in 2 (5%) and 3 cases (7.5%), respectively. Bronchial artery dilatation was observed in 5 cases.

Pulmonary capillary hemangiomatosis: an unusual cause of primary pulmonary hypertension in pediatric age group

Pulmonary capillary hemangiomatosis (PCH) is a rare and controversial entity that is known to be a cause of pulmonary hypertension and is microscopically characterized by proliferation of dilated capillary-sized channels along and in the alveolar walls. Clinically, it is mostly seen in adults. PCH’s main clinical presentations are progressive dyspnea, fatigue, hemoptysis, palpitations and later irreversible pulmonary hypertension and right-sid­ed heart failure. It is characterized by severe hypoxemia, centrilobular ground-glass opacities on computed tomography and pulmonary congestion triggered by pulmonary vasodilating therapy. The imaging features include diffuse centrilobular ground‑glass opacities with features of pulmonary hypertension. We present a case of PCH in a 4‑year‑old boy who was diagnosed with post-partum hemorrhage (PPH) in echocardiography and computed tomography of the thorax.

Imaging Features in BMPR2 Mutation-associated Pulmonary Arterial Hypertension.

Background Germline mutation in the BMPR2 gene is common in patients with pulmonary arterial hypertension (PAH). However, its association with imaging findings in these patients is, to the knowledge of the authors, unknown. Purpose To characterize distinctive pulmonary vascular abnormalities at CT and pulmonary artery angiography in patients with and without BMPR2 mutation. Materials and Methods In this retrospective study, chest CT scans, pulmonary artery angiograms, and genetic test data were acquired for patients diagnosed with idiopathic PAH (IPAH) or heritable PAH (HPAH) between January 2010 and December 2021. Perivascular halo, neovascularity, centrilobular ground-glass opacity (GGO), and panlobular GGO were evaluated at CT and graded on a four-point severity scale by four independent readers. Clinical characteristics and imaging features between patients with BMPR2 mutation and noncarriers were analyzed using the Kendall rank-order coefficient and the Kruskal-Wallis test. Results This study included 82 patients with BMPR2 mutation (mean age, 38 years ± 15 [SD]; 34 men; 72 patients with IPAH and 10 patients with HPAH) and 193 patients without the mutation, all with IPAH (mean age, 41 years ± 15; 53 men). A total of 115 patients (42%; 115 of 275) had neovascularity, and 56 patients (20%; 56 of 275) had perivascular halo at CT, and so-called frost crystals were observed on pulmonary artery angiograms in 14 of 53 (26%) patients. Compared with patients without BMPR2 mutation, patients with BMPR2 mutation more frequently showed two distinctive radiographic manifestations, perivascular halo and neovascularity (38% [31 of 82] vs 13% [25 of 193] in perivascular halo [P < .001] and 60% [49 of 82] vs 34% [66 of 193] in neovascularity [P < .001], respectively). "Frost crystals" were more frequent in patients with BMPR2 mutation compared with noncarriers (53% [10 of 19] vs 12% [four of 34]; P < .01). Severe perivascular halo frequently coexisted with severe neovascularity in patients with BMPR2 mutation. Conclusion Patients with PAH with BMPR2 mutation showed distinctive features at CT, specifically perivascular halo and neovascularity. This suggested a link between the genetic, pulmonary, and systemic manifestations that underly the pathogenesis of PAH. © RSNA, 2023 Supplemental material is available for this article.

Peribronchiolar Metaplasia: A Marker of Cigarette Smoke-Induced Small Airway Injury in a Rural Cohort.

Peribronchiolar metaplasia (PBM) is considered a reaction to injury characterized by the proliferation of bronchiolar epithelium into immediately adjacent alveolar walls. While an association of PBM with diffuse interstitial lung diseases has been recognized, the clinical significance of PBM remains uncertain. A cohort (n = 352) undergoing surgical resection of a lung nodule/mass in a rural area was retrospectively reviewed. Multivariate logistic regression analysis was performed to determine the association of PBM with clinical, physiological, radiographic, and histologic endpoints. In the total study cohort, 9.1% were observed to have PBM as a histologic finding in resected lung tissue (n = 32). All but one of these patients with PBM were ever-smokers with a median of 42 pack years. Clinical COPD was diagnosed in two-thirds of patients with PBM. Comorbid gastroesophageal reflux disease (GERD) was significantly associated with PBM. All patients with PBM demonstrated radiologic and histologic evidence of emphysema. Measures of pulmonary function were not impacted by PBM. Mortality was not associated with the histologic observation of PBM. In a logistic regression model, centrilobular-ground glass opacity interstitial lung abnormality and traction bronchiectasis on the CT scan of the chest and histologic evidence of fibrosis, desquamative interstitial pneumonia and anthracosis all strongly predicted PBM in the cohort. A constellation of radiologic and histologic smoking-related abnormalities predicted PBM in study cohort. This confirms a co-existence of lung tissue responses to smoking including PBM, emphysema, and fibrosis. Acknowledging the physiologically "silent" nature of small airway dysfunction on pulmonary function testing, our findings support PBM as a histologic marker of small-airway injury associated with cigarette smoking.

PULMONARY CAPILLARY HEMANGIOMATOSIS: A CONUNDRUM FOR EXPERTS

PULMONARY CAPILLARY HEMANGIOMATOSIS: A CONUNDRUM FOR EXPERTS

An Overview of Human T-Lymphotropic Virus Type 1 Lung Injury.

Previous studies have demonstrated the development of pulmonary impairment in individuals infected with human T-lymphotropic virus type 1 (HTLV-1). Complications, such as alveolitis and bronchiectasis, were found in individuals who developed tropical spastic paraparesis/HTLV-1-associated myelopathy (TSP-HAM) due to chronic inflammation. These patients exhibited increased levels of lymphocytes (CD4+ and CD25+), cytokines (IL-2, IL-12, and IFN-γ), inflammatory chemokines (MIP-1α and IP-10), and cell adhesion molecules (ICAM-1) in the bronchoalveolar lavage fluid, with the result of chronic inflammation and lung injury. The main lesions observed at Chest high-resolution computed tomography were centrilobular nodules, parenchymal bands, lung cysts, bronchiectasis, ground-glass opacity, mosaic attenuation, and pleural thickening. It can lead to progressive changes in pulmonary function with the development of restrictive and obstructive diseases. Recent studies suggest a causal relationship between HTLV-1 and pulmonary diseases, with intensification of lesions and progressive decrease in pulmonary function. This summary updates a previous publication and addresses the general lack of knowledge regarding the relationship between TSP-HAM and pulmonary disease, providing direction for future work and the management of these individuals.

Interstitial lung abnormalities and interstitial lung diseases associated with cigarette smoking in a rural cohort undergoing surgical resection

BackgroundCigarette smoking is a risk factor for interstitial lung abnormalities (ILAs) and interstitial lung diseases (ILDs). Investigation defining the relationships between ILAs/ILDs and clinical, radiographic, and pathologic findings in smokers have been incomplete. Employing a cohort undergoing surgical resection for lung nodules/masses, we (1) define the prevalence of ILAs/ILDs, (2) delineate their clinical, radiographic and pathologic predictors, and (3) determine their associations with mortality.MethodsPatients undergoing resection of lung nodules/masses between 2017 and 2020 at a rural Appalachian, tertiary medical center were retrospectively investigated. Predictors for ILAs/ILDs and mortality were assessed using multivariate logistic regression analysis.ResultsIn the total study cohort of 352 patients, radiographic ILAs and ILDs were observed in 35.2% and 17.6%, respectively. Among ILA patterns, subpleural reticular changes (14.8%), non-emphysematous cysts, centrilobular (CL) ground glass opacities (GGOs) (8% each), and mixed CL-GGO and subpleural reticular changes (7.4%) were common. ILD patterns included combined pulmonary fibrosis emphysema (CPFE) (3.1%), respiratory bronchiolitis (RB)-ILD (3.1%), organizing pneumonitis (2.8%) and unclassifiable (4.8%). The group with radiographic ILAs/ILDs had a significantly higher proportion of ever smokers (49% vs. 39.9%), pack years of smoking (44.57 ± 36.21 vs. 34.96 ± 26.22), clinical comorbidities of COPD (35% vs. 26.5%) and mildly reduced diffusion capacity (% predicated 66.29 ± 20.55 vs. 71.84 ± 23). Radiographic centrilobular and paraseptal emphysema (40% vs. 22.2% and 17.6% vs. 9.6%, respectively) and isolated traction bronchiectasis (10.2% vs. 4.2%) were associated with ILAs/ILDs. Pathological variables of emphysema (34.9% vs. 18.5%), any fibrosis (15.9% vs. 4.6%), peribronchiolar metaplasia (PBM, 8% vs. 1.1%), RB (10.3% vs. 2.5%), and anthracosis (21.6% vs. 14.5%) were associated with ILAs/ILDs. Histologic emphysema showed positive correlations with any fibrosis, RB, anthracosis and ≥ 30 pack year of smoking. The group with ILAs/ILDs had significantly higher mortality (9.1% vs. 2.2%, OR 4.13, [95% CI of 1.84–9.25]).ConclusionsIn a rural cohort undergoing surgical resection, radiographic subclinical ILAs/ILDs patterns were highly prevalent and associated with ever smoking and intensity of smoking. The presence of radiographic ILA/ILD patterns and isolated honeycomb changes were associated with increased mortality. Subclinical ILAs/ILDs and histologic fibrosis correlated with clinical COPD as well as radiographic and pathologic emphysema emphasizing the co-existence of these pulmonary injuries in a heavily smoking population.

Pulmonary Alveolar Proteinosis After Lung Transplantation

<h3>Introduction</h3> Secondary pulmonary alveolar proteinosis (PAP) is a minor subtype of the disease entity with poorer prognosis than common autoimmune PAP. It has been reported to complicate hematologic disorders, immunodeficiency, and infections. However, PAP associated with lung transplantation is not well recognized. Here, we report two cases of PAP after lung transplantation. <h3>Case Report</h3> Case 1: A four-year-old boy underwent a double-lung transplantation. He was maintained standard immunosuppressive regimen. The immediate post-operative course was uneventful. On post-operative day (POD) 23, chest computer tomography (CT) scan showed ground-glass opacities (GGOs) with interlobular septal thickening, along with worsening oxygenation. An acute rejection was clinically suspected, and the patient underwent steroid pulse therapy. It improved temporarily, but worsened again within a week. Despite the repeated steroid pulse, administration of anti-thymocyte globulin and inhibitors of the mammalian target of rapamycin, the situation gradually deteriorated and eventually complicated by infectious diseases. The patient died from respiratory failure on POD 248. At autopsy, diffuse intra-alveolar eosinophilic materials stained by surfactant apoprotein A immunohistochemistry were found and led to a diagnosis of PAP. Case 2: A 52-year-old man underwent double-lung transplantation. He was maintained standard immunosuppressive regimen. The post-operative course was uneventful. On POD 99, routine chest CT scan showed bilateral centrilobular ground-glass opacities mimicking rejection. A diagnostic trial of steroid increase was ineffective. Bronchoalveolar lavage fluid analysis and transbronchial biopsy demonstrated intra-alveolar eosinophilic materials which stained by periodic acid-Schiff. He was diagnosed with PAP and followed up with tapering immunosuppression in the standard manner, resulting in a clinical and radiological improvement. <h3>Summary</h3> PAP is a rare cause of graft dysfunction after lung transplantation. In case 1, the patient was misdiagnosed as acute rejection, and immunosuppression was intensified, which led to fatal consequences. PAP after lung transplantation is potentially a transient or slowly progressive pathology as seen in case 2. Transplant physicians should be aware of this rare complication.

Computed tomography with 6-year follow-up demonstrates the evolution of HTLV-1 related lung injuries: A cohort study.

Previous observational studies have demonstrated the development of pulmonary impairments in human T-lymphotropic virus type 1 (HTLV-1) infected individuals. The main observed lesions due to chronic inflammation of viral infection in situ are bronchiectasis and lung-scarring injuries. This lung inflammation may be the causal agent of restrictive and obstructive lung diseases, primarily in tropical spastic paraparesis/HTLV-1-associated myelopathy (TSP-HAM) patients. We conducted a prospective cohort study to compare spirometry and high-resolution computed tomography (HRCT) findings among 28 HTLV-1-carrier patients over the course of 6 years (2014-2019) (male/female: 7/21; mean age: 54.7 ± 9.5, range: 41-68 years). Chest HRCT exams revealed the development and evolution of lung lesions related to TSP-HAM: including centrilobular nodules, parenchymal bands, lung cysts, bronchiectasis, ground-glass opacity, mosaic attenuation, and pleural thickening. Spirometry exams showed maintenance of respiratory function, with few alterations in parameters suggestive of obstructive and restrictive disorders primarily in individuals with lung lesions and TSP-HAM. The findings of the present study indicate that pulmonary disease related to HTLV-1 is a progressive disease, with development of new lung lesions, mainly in individuals with TSP-HAM. To improve clinical management of these individuals, we recommend that individuals diagnosed with PET-MAH undergo pulmonary evaluation.

400 Refractory pulmonary hypertension in a young woman

Abstract Aims Clinical case—Twenty-four years old Moroccan woman. Family history: parents and three siblings in good health. Methods and results Past medical history—In 2016, when she was 19 years old, she developed worsening exercise-induced dyspnoea. A right heart catheterization (RHC) was performed with evidence of increased median pulmonary artery pressure (mPAP 60 mmHg, wedge pressure 8 mmHg), cardiac index 2.27 l/min/m2. She was diagnosed with idiopathic pulmonary hypertension and combination therapy with sildenafil and macitentan was started with partial improvement. In August 2019, she became pregnant and vasodilatory therapy was suspended. The pregnancy was complicated by premature labor with foetal death. Specific therapy with sildenafil and macitentan was then restarted. Due to further clinical and haemodynamic impairment, triple combination therapy with selexipag was initiated. However, symptomatic deterioration progressed, and she was referred to a Pulmonary Hypertension Referral Center where HRTC of the chest showed centrilobular ground-glass opacities and interlobular septal thickening. Based on the imaging that was highly suspicious pulmonary veno-occlusive disease (PVOD), and the severe haemodynamic impairment (with pulmonary vascular resistance at RHC &amp;gt; 20 WU), assessment for lung transplantation was started. Recent medical history—she was transferred to our Center with Transplant Unit for lung transplant assessment. Pulmonary function testing demonstrated a restrictive disorder with severe reduction of DLCO (14%). At 6-min walking test, she could walk 100 m with desaturation up to 90% on O2 therapy. Evaluated by our multidisciplinary team, indications for lung transplantation were confirmed and she entered the transplant waiting list. At the end of February 2021: further clinical and haemodynamic deterioration with respiratory distress, reduction in urinary output and signs and symptoms of right-side heart failure; she was hospitalized in intensive care unit and required extra-corporeal membrane oxygenation (ECMO) circulatory support. Selexipag was suspended. On echocardiography: severe right ventricle hypertrophy and dilatation, tricuspid regurgitation velocity &amp;gt;4 m/s. Few days later, she underwent bilateral lung transplantation; anatomo-pathological evaluation of explanted organs confirmed PVOD. However, during post-operative monitoring, she suffered from two episodes of cardiac arrest on VT/VF which required multiple DC-shocks. Since no triggering causes were identified, an ICD was implanted for secondary prevention. Conclusions PVOD is a rare disease with clinical presentation and haemodynamic profile often similar to pulmonary arterial hypertension (and, therefore, the diagnosis is challenging) but the clinical course is more severe. The diagnosis requires clinical history and physical examination, together with multimodality imaging and functional testing. Bronchoalveolar lavage might be necessary. Patients do not usually respond satisfactorily to vasodilatory therapy but rather they are at high risk of drug-induced pulmonary oedema. Some case reports and case series have reported a slight benefit and/or clinical stabilization with vasodilatory therapy in selected patients; therefore, specific therapy can be used but cautiously and in experienced referral centres. Definitive therapy is lung transplantation (or heart-lung transplantation) and a multidisciplinary team is necessary for the appropriate management of these complicated patients. Ventricular arrhythmias are rare in patients with Group 1 pulmonary hypertension. In our specific clinical case, we suspect that ventricular arrhythmias might be related to the severely hypertrophic, and potentially fibrotic, right ventricle.

Abstract 10367: An Unusual Vasodilator Response: Recognizing a Rare Subtype of Pulmonary Arterial Hypertension

Introduction: Pulmonary veno-occlusive disease (PVOD) is a rare subtype of pulmonary arterial hypertension (PAH) caused by intimal fibrosis of the pulmonary venules and septal veins. While the hemodynamic profile is suggestive of pre-capillary pulmonary hypertension, vascular obstruction distal to the pulmonary capillary beds distinguishes PVOD from other forms of PAH. Case: A 29-year-old woman presented with progressive dyspnea and fatigue. She was tachycardic, hypotensive, and volume overloaded on exam. ECG was notable for right-axis deviation. TTE showed a dilated, hypokinetic RV and small LV with mild global hypokinesis and septal flattening throughout the cardiac cycle. A CTA chest excluded pulmonary embolism but showed diffuse centrilobular ground-glass opacities and lymphadenopathy which raised suspicion for PVOD. She underwent right heart catheterization with the following measurements (mmHg): RA 14, PA 99/56 (mean 71), PCWP 12, and CI 1.1 L/min/m 2 with PVR 28.1 WU. Following initiation of an epoprostenol infusion, she became increasingly hypoxemic with repeat CT chest demonstrating dramatic worsening of ground-glass opacities and vascular congestion. This response to a vasodilator challenge led to a clinical diagnosis of PVOD. She was urgently listed for and underwent bilateral lung transplantation. Explanted lung tissue subsequently confirmed the diagnosis. Conclusion: In most forms of PAH, vasodilators mitigate arteriolar narrowing and improve pulmonary blood flow, while in PVOD they exacerbate pulmonary capillary congestion due to increased arteriolar blood flow relative to the venous outflow due to fixed narrowing of the pulmonary venule network. Because lung biopsy is contraindicated in severe pulmonary hypertension, the clinician must recognize that worsening pulmonary edema in response to vasodilator challenge with normal PCWP is a critical clue pointing towards PVOD. Timely diagnosis is essential given the poor prognosis for this condition for which lung transplantation is the definitive treatment.

Pulmonary veno-occlusive disease in childhood-a rare disease not to be missed.

Pulmonary veno-occlusive disease (PVOD) is a rare disease leading to pulmonary hypertension and potentially death related to right heart failure and/or respiratory insufficiency. Clinical symptoms are heterogenous and nonspecific: fatigue, decreased exercise tolerance, shortness of breath on exertion, cough, dizziness, chest pain with exercise, palpitations, syncope, as well as nonspecific symptoms such as headache, poor appetite, pallor or perioral cyanosis. Mutations in the EIF2AK4 (eukaryotic translation initiation factor 2-alpha kinase 4) have been recently described, other risk factors include exposure to organic solvent and trichloroethylene, tobacco exposure and chemotherapy. Echocardiography helps to estimate right ventricular systemic pressure, but further diagnostic workup includes cardiac catheterization to confirm pulmonary hypertension and increased pulmonary vascular resistance. High-resolution computed tomography reveals typical findings: centrilobular ground-glass nodules or opacities, septal lines, thickened interlobular septa, mosaic perfusion, and lymphadenopathy. Histology remains the gold standard, but carries risks for the patient. Proper workup is essential in order to avoid incorrect diagnosis. Pulmonary hypertension targeted treatment has been used in patients with PVOD, however, experience is limited, vasodilatory effects on pulmonary vasculature may lead to deterioration of the patients and should be used with great caution. Lung transplantation is currently the only valid treatment option for patients with PVOD. With prolonged waiting time and progression of the disease mechanical support could be considered.

An interesting case of progressive dyspnoea and diffuse mediastinal adenopathy in a 25-year-old man.

Mediastinal adenopathy, septal line thickening, centrilobular ground glass opacities on CT and a markedly reduced TLCO in a young patient with pulmonary hypertension, should alert the clinician to this potential diagnosis https://bit.ly/3cfe9pX

PULMONARY CAPILLARY HEMANGIOMATOSIS: A RARE CASE OF STABLE DISEASE WITHOUT TREATMENT

SESSION TITLE: Medical Student/Resident Pulmonary Vascular Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Pulmonary capillary hemangiomatosis (PCH) is a rare cause of primary pulmonary hypertension caused by extensive proliferation of pulmonary capillaries within parenchymal, vascular, and bronchial tissue. Typically, PCH progresses rapidly with a median time to lung transplantation of two years post diagnosis. To our knowledge, we present the longest case of biopsy proven PCH that has been stable without treatment for seven years. CASE PRESENTATION: A healthy 23-year old non-smoking male presented to the emergency department (ED) with a 2-week history of hemoptysis associated with intermittent left-sided pleuritic chest pain, fatigue, and dyspnea. His physical exam, routine lab work, and chest X-ray were unremarkable. He was discharged home and prescribed clarithromycin for suspected bronchitis. He returned to the ED months later with ongoing symptoms and was referred for urgent outpatient consultation to pulmonology. Bronchoscopy was performed with no blood on bronchoalveolar lavage analysis. Further serum rheumatologic and vasculitis panels were negative. A non-contrast computed tomography (CT) scan of his chest revealed subtle bilateral upper lung predominant centrilobular ground-glass opacities. (Figure 1) With worsening hemoptysis, he returned to the ED six months later. A CT Chest angiogram was performed and showed progressive bilateral airspace opacities. (Figure 2) The patient was referred to Thoracic Surgery for lung biopsy. This demonstrated septal proliferation of capillaries consistent with PCH without evidence of vasculitis, venous occlusion or microorganisms. Immunofluorescence staining was also negative. (Figure 3) The patient remains stable with no signs of progression or pulmonary hypertension on echocardiogram. His enhanced CT and pulmonary function tests remain normal seven years from his initial presentation. DISCUSSION: The typical presentation of PCH includes progressive dyspnea, cough with or without hemoptysis, chest pain, and fatigue. (1) Chest X-ray can be normal or progress to demonstrate bilateral opacities in keeping with diffuse alveolar hemorrhage (DAH). CT chest often shows diffuse centrilobular ground-glass opacities. (1) Bronchoscopy may not reveal erythrocytes if the patient is not actively bleeding. Diagnosis of PCH is essential, as vasodilators used in pulmonary hypertension can cause hemodynamic instability in PCH. (2) Interestingly, an autopsy case series found incidental cases of PCH in patients who were asymptomatic during life. (3) This raises the possibility that subclinical cases of PCH may exist which do not require urgent referral for lung transplantation. CONCLUSIONS: This case represents the longest active case of PCH after confirmation with biopsy without treatment. Re-evaluating the expected prognosis for individuals diagnosed with PCH is important, as median survival may be longer than currently reported in the literature. Reference #1: O'keefe MC, and Post MD. Pulmonary Capillary Hemangiomatosis: A Rare Cause of Pulmonary Hypertension. Archives of Pathology & Laboratory Medicine, 2015;139(2):274–277. Reference #2: Almagro P, Julia J, Sanjuame M, Gonalez G, Casalots J, Heredia JL, et al. Pulmonary Capillary Hemangiomatosis Associated with Primary Pulmonary Hypertension. Medicine. 2002;81(6):417–24. Reference #3: Havlik DM, Massey LW, Williams WL, and Crooks LA. Pulmonary Capillary Hemangiomatosis- like foci: an autopsy study of 8 cases. Anatomic Pathology. 2000;113(5):665-662. DISCLOSURES: No relevant relationships by Meredith Chiasson, source=Web Response No relevant relationships by Jasper Johar, source=Web Response No relevant relationships by Daria Manos, source=Web Response No relevant relationships by Matthew Nunn, source=Web Response

REPEAT PRESENTATION OF A FEBRILE RESPIRATORY ILLNESS SECONDARY TO HYPERSENSITIVITY PNEUMONITIS

SESSION TITLE: Medical Student/Resident Occupational and Environmental Lung Disease SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Dyspnea is one of the most common reasons for presentation to the hospital. Antigen mediated lung diseases are an important cause of dyspnea however due to the wide variation in clinical presentation, the large number of possible antigens, and a lack of diagnostic tests identification can be difficult.1 Thus, a high index of suspicion must be maintained to prevent missed or delayed diagnosis. CASE PRESENTATION: A 44-year-old nonsmoker with an insignificant past medical history originally presented to an outpatient urgent care with an acute onset of fever, dyspnea, productive cough, and myalgias. He was empirically treated for bacterial pneumonia with an oral antibiotic. After initial improvement, his symptoms progressed and he ultimately presented to the emergency room with fever and worsening dyspnea. He was admitted with acute hypoxic respiratory failure and sepsis secondary to presumed post-influenza pneumonia (standard chest radiograph showed left lower lobe infiltrate). He was fluid resuscitated and received broad spectrum antibiotics. With antibiotics his fever dissipated although he continued to remain hypoxic and was discharged on hospital day seven on three liters of oxygen via nasal cannula. At hospital follow up one week later he continued to endorse dry cough and severe dyspnea (being only able to ambulate household distances with oxygen prior to having to rest). Exam was significant for crackles in the bilateral lung bases along with hypoxia (oxygen saturation dropped to 83% with removal of his nasal cannula during rest). Further history revealed the patient owned seven parakeets which were kept in the same room that the patient slept. He also endorsed unintentional 12-pound weight loss over the past 3 months. CT imaging was pursued which showed diffuse ill-defined centrilobular micronodularity and ground glass opacities throughout both lungs. Based upon this new information serum hypersensitivity pneumonitis screen was performed and ultimately returned negative for the presence of six antigens (only bird antigen on the screen was pigeon serum). Based upon the patient meeting five out of six clinical criteria for a validated clinical predication rule a diagnosis of hypersensitivity pneumonitis was established (92% Likelihood).2 He was advised to avoid further exposure to his birds and outpatient pulmonology consultation was arranged. After a short course of prednisone and avoiding further exposure the patient's symptoms resolved. DISCUSSION: Hypersensitivity pneumonitis is an important disease to keep on the differential for a patient presenting with dyspnea. CONCLUSIONS: Early diagnosis is of the upmost importance as with avoiding further exposure early lung disease can be potentially completely reversed. This case also illustrates the importance of taking a complete history and how routine use of validated clinical predication rules can help you make a diagnosis. Reference #1: 1. Riario Sforza, G.G., Marinou, A. Hypersensitivity pneumonitis: a complex lung disease. Clin Mol Allergy. 2017; 15, 6. Reference #2: 2. Lacasse Y, Selman M, Costabel U, Dalphin JC, Ando M, Morell F, Erkinjuntti-Pekkanen R, Muller N, Colby TV, Schuyler M, Cormier Y. Clinical diagnosis of hypersensitivity pneumonitis. Am J Respir Crit Care Med. 2003;168(8):952. DISCLOSURES: No relevant relationships by Catherine Cheney, source=Web Response No relevant relationships by Chris Morris, source=Web Response

Hypersensitivity pneumonitis: clinical, radiological and pathological profile of 103 patients from North India.

Hypersensitivity pneumonitis (HP) is an interstitial lung disease, commonly occurring due to exposure to various inciting agent related to occupation. Few studies have shown that it can also occur without any occupation exposure. In this study we are presenting clinical, radiological and bronchoscopic finding of 103 HP patients. We retrospective analysis of 5½ years HP patient's data from a chest institute of India. The diagnosis of HP was considered with following criteria: i) known exposure to an inciting antigen; ii) presence of respiratory symptoms; iii) radiologic evidence of diffuse lung disease; iv) no other identifiable cause; v) lung biopsy specimen that demonstrated features of HP; and vi) bronchoalveolar lavage lymphocytosis (≥30%). The mean ±SD age was 47±12.8 years; 67% were female. The common symptoms were cough (97%) and dyspnea (91%). History of exposure to inciting agent was present in 61% with pigeon exposure being the most common (56%). Majority of patients (86%) were having chronic symptoms for >6 months. On 6MWT oxygen desaturation >4% was seen in 57% patients. Centrilobular nodules (61%) and ground glass opacity (47.5%) were common finding on HRCT chest. Bronchoalveolar lavage (BAL) lymphocytosis >30% was present in 48.5% and histopathological diagnosis HP on transbronchial lung biopsy (TBLB) and/or endobronchial lung biopsy (EBLB) was in 50% patients. HP is exposure related environmental disease, as it can occur without any occupational history. Bronchoscopy with BAL and lung biopsy should do in all suspected cases to confirm diagnosis in our country as it is less invasive, day care procedure with less complication.

Follicular Bronchiolitis: Two Cases with Varying Clinical and Radiological Presentation

Follicular bronchiolitis (FB) is a rare bronchiolar disorder associated with hyperplasia of the bronchial-associated lymphoid tissue (BALT). It is characterized by the development of lymphoid follicles with germinal centers in the walls of small airways. It falls under the category of lymphoproliferative pulmonary diseases (LPDs) and commonly occurs in relation to connective tissue disease, immunodeficiency, infections, interstitial lung disease (ILD), and inflammatory airway diseases. Computerized tomography (CT) findings include centrilobular nodules with patchy ground glass infiltrate, tree-in-bud findings, and air trapping. It can very rarely present as diffuse cystic lung disease. We present two cases of FB. The first case is associated with Human Immunodeficiency Virus (HIV) infection and asthma with diffuse cystic changes on the CT. The second case is associated with reactive airway disease and gastroesophageal reflux disease (GERD) with the classic centrilobular nodules and ground glass opacities on the CT.

E-cigarette, or vaping, product use-associated lung injury in adolescents: a review of imaging features.

There has been a recent increase in recognition of lung disease related to the use of electronic cigarettes (called "vaping"). These patients present with acute respiratory illness following exposure to vaporized cannabis or nicotine products and sometimes require hospitalization and intensive care. We describe the imaging findings of this disease entity in the pediatric population. To describe the radiologic findings of lung injury associated with electronic cigarette use (vaping) in the adolescent pediatric population. We identified all adolescents with acute respiratory illness and a history of electronic cigarette use who presented at our institution within a 3-month period (June 2019 through August 2019). We excluded adolescents with potential intercurrent pulmonary disease. We reviewed the charts for symptomatology and laboratory and pathology data. In addition, we reviewed the chest radiographs and chest CTs of these adolescents. The review group consisted of 12 teenage pediatric patients (10 boys and 2 girls; mean age 16.9years, range 16.0-17.7years) with acute respiratory illness found to have a temporal association with electronic cigarette use for cannabis products, nicotine, or both. Other etiologies for illness in these adolescents had been excluded by clinical and laboratory evaluation. All of the adolescents were admitted to the hospital for treatment. The clinical presentations included dyspnea, abdominal pain and constitutional symptoms. Pulmonary function testing that was performed in all patients during admission or follow-up demonstrated reduced diffusion capacity in 4/12 (33%), an obstructive ventilatory pattern in 4/12 (33%), a restrictive pattern in 1/12 (8%), and a mixed obstructive and restrictive pattern in 2/12 (17%) adolescents. Bronchoalveolar lavage studies, performed in 9 of the 12 adolescents, revealed inflammatory cells and lipid-laden macrophages. All of the patients underwent CT of the chest; the findings were notable for centrilobular ground-glass nodules (11/12; 92%) and confluent ground-glass opacities (12/12; 100%), with frequent subpleural sparing (9/12; 75%). Additionally, 6/12 (50%) adolescents demonstrated small pleural effusions; 6/12 (50%) had mild bronchial wall thickening; 9/12 (75%) had enlarged hilar or mediastinal lymph nodes; and 2/12 (17%) had a small pericardial effusion. As seen in our teenage population, e-cigarette, or vaping, product use-associated lung injury (EVALI) is characterized by centrilobular ground-glass nodules and ground-glass opacities with subpleural sparing. The imaging findings are most consistent with acute lung injury resulting from toxic inhalation. Because adolescent pediatric patients might not be forthcoming with their history of electronic cigarette use, it is important for the pediatric radiologist to be aware of the imaging patterns of this disease.