Video-assisted Thoracoscopic Lung Biopsy Research Articles

Sequential development of diffuse panbronchiolitis and myasthenia gravis after thymectomy for thymic neoplasm: a case report

BackgroundMyasthenia gravis (MG) is the most common paraneoplastic disorder associated with thymic neoplasms. MG can develop after thymectomy, and this condition is referred to post-thymectomy myasthenia gravis (PTMG). Diffuse panbronchiolitis (DPB), is a rare form of bronchiolitis and is largely restricted to East Asia, has been reported in association with thymic neoplasms. Only three cases of combined MG and DPB have been reported in the literature.Case presentationA 45-year-old Taiwanese woman presented to our hospital with productive cough, rhinorrhea, anosmia, ear fullness, shortness of breath, and weight loss. She had a history of thymoma, and she underwent thymectomy with adjuvant radiotherapy 7 years ago. Chest computed tomography scan revealed diffuse bronchitis and bronchiolitis. DPB was confirmed after video-assisted thoracoscopic surgery lung biopsy, and repeated sputum cultures grew Pseudomonas aeruginosa. She has been on long-term oral azithromycin therapy thereafter. Intravenous antipseudomonal antibiotics, inhaled amikacin, as well as oral levofloxacin were administered. Three months after DPB diagnosis, she developed ptosis, muscle weakness, and hypercapnia requiring the use of noninvasive positive pressure ventilation. MG was diagnosed based on the acetylcholine receptor antibody and repetitive stimulation test results. Her muscle weakness gradually improved after pyridostigmine and corticosteroid therapies. Oral corticosteroids could be tapered off ten months after the diagnosis of MG. She is currently maintained on azithromycin, pyridostigmine, and inhaled amikacin therapies, with intravenous antibiotics administered occasionally during hospitalizations for respiratory infections.ConclusionsTo our knowledge, this might be the first case report of sequential development of DPB followed by PTMG. The coexistence of these two disorders poses a therapeutic challenge for balancing infection control for DPB and immunosuppressant therapies for MG.

Criteria for progressive fibrotic hypersensitivity pneumonitis in a Portuguese patient cohort.

Hypersensitivity pneumonitis (HP) is a syndrome caused by sensitisation to inhaled antigens that leads to an abnormal immune response in the airways and lung parenchyma. Some patients previously diagnosed with certain types of fibrotic interstitial lung diseases (f-ILDs), including fibrotic HP (f-HP), are susceptible to develop a progressive fibrosing phenotype (PF-ILD), despite initial state-of-the-art management. To characterise a cohort of patients with a multidisciplinary diagnosis (MTD) of chronic f-HP, who were followed up in an ILD outpatient clinic of a hospital in Portugal, and to assess the prevalence of PF-ILD criteria in these patients. Data were collected from all patients with a definite or provisional diagnosis of f-HP after a multidisciplinary team discussion. Patients were followed up between December 2014 and July 2019. Data included clinical characteristics, high-resolution chest tomography (HRCT) disease patterns, lung function tests, bronchoalveolar lavage and further immunological work-up, biopsy reports (conventional transbronchial lung biopsy, transbronchial lung cryobiopsy or surgical video-assisted thoracoscopic lung biopsy), all ILD multidisciplinary team records and diagnostic confidence levels. Patients were assessed according to PF-ILD criteria as defined in the INBUILD trial. We identified 83 patients with an MTD of HP, who had been followed up for at least 12 months. Of these, 63 (75.9%) were diagnosed with f-HP. Of the 63 f-HP patients, 33.3% (n=21) fulfilled the predefined criteria for PF-HP: 66.7% had a relative decline of ≥10% forced vital capacity (FVC); 5% a relative decline of 5 - 9% FVC, with worsening symptoms or increased fibrosis on HRCT; and 23.8% had worsening respiratory symptoms with radiological progression. This single-centre cohort study demonstrated that a third of f-HP patients presented with PF-ILD, as determined by progression during initial standard-of-care treatment. A usual interstitial pneumonia (UIP)/UIP-like pattern was present in >70% of patients with f-HP, and two-thirds of these patients had an FVC decline of ≥10%. PF-HP patients were also more exacerbation prone. According to recent trial data, this segment of patients can be considered possible candidates for antifibrotic treatment, with a reasonable prospect of effectiveness. Further efforts should focus on refining knowledge of longitudinal behaviour of large multicentric cohorts of f-HP patients, establishing a consensual and uniform definition of progression for use in clinical practice, as well as developing prognostic prediction tools to better (and early) inform the disease course.

PULMONARY CAPILLARY HEMANGIOMATOSIS: A CONUNDRUM FOR EXPERTS

PULMONARY CAPILLARY HEMANGIOMATOSIS: A CONUNDRUM FOR EXPERTS

Diagnosis Yield and Safety of Surgical Biopsy in Interstitial Lung Diseases: A Prospective Study

BackgroundSurgical lung biopsy is essential in the diagnostic algorithm of interstitial lung disease (ILD) of unknown cause. Safety concerns have been recently reiterated. This study prospectively assessed the yield of diagnosis and safety of video-assisted thoracoscopic surgical lung biopsy (VATS-LB) for ILD diagnosis. MethodsThis prospective study, conducted in 6 ILD-referral Paris hospitals, included 103 patients with ILD. VATS-LB was proposed after initial multidisciplinary discussion. A final diagnosis was made after the procedure, during a second multidisciplinary discussion. The main outcome was to determine the final diagnoses and their proportion after VATS-LB. Other outcomes were the percentage of change in diagnosis and treatment propositions after VATS-LB and adverse events during 3 months after the operation, postoperative pulmonary function, quality of life, and pain. ResultsA definite diagnosis was reached in 87 patients (84.4%), and 16 remained unclassifiable (15.6%). After VATS-LB, the hypothesized diagnosis changed in 65 patients (63.1%) and treatment changed in 41 patients (39.8%). One patient died of acute exacerbation. In-hospital complications were predicted by a shorter preoperative 6-minute walking test distance and by forced vital capacity lower than 77%. Postoperative quality of life was not modified at 3 months, whereas forced vital capacity decreased slightly. Postoperative neuropathic pain was revealed in 5% of patients at 1 month and in 2% at 3 months. ConclusionsVATS-LB dramatically changed preoperative hypothetical diagnoses and treatment in ILD of unknown cause, with good patient survival in ILD referral centers.

Lymphangioleiomyomatosis associated with prolactinoma: A case report and literature review

The thirty-five-year-old woman had been experiencing dyspnea on exertion since her second child's birth five years before presenting to hospital A, where she was diagnosed with lymphangioleiomyomatosis (LAM) based on video-assisted thoracoscopic surgery lung biopsy and referred to our hospital. She was treated with sirolimus for one year. Although her subjective symptom of dyspnea improved, she found that her amenorrhea had persisted for six years. A blood test revealed high prolactin (PRL) levels of 98 ng/mL and head magnetic resonance imaging revealed a pituitary adenoma, which was complicated by a prolactinoma. We continued with follow-up observation without any pharmacotherapy for the prolactinoma. However, she was administered oral cabergoline (0.25 mg per week) when her PRL levels were elevated to 250 ng/mL 38 months after therapeutic intervention with sirolimus. For the next 14 months, her respiratory function and PRL concentration both demonstrated improvement and her condition did not worsen any further. It has been reported that prolactin may exacerbate LAM. Our case suggests that a clinical reduction in PRL levels may also improve LAM.

T-cell lymphoma with a granulomatous lesion of the lungs after autologous hematopoietic stem cell transplantation for Epstein\u2013Barr virus-positive diffuse large B-cell lymphoma: a unique rare case of metachronous B-cell and T-cell lymphoma

BackgroundEpstein–Barr virus (EBV) is associated with the pathogenesis of a variety of malignancies, most notably lymphomas. Especially in the background of immunodeficiency, such as primary immunodeficiency disorder (PID) and post-transplant lymphoproliferative disorder (PTLD), the role of EBV might be crucial. PIDs are rare heterogeneous diseases affecting the development and/or the function of the innate and adaptive immune system. Malignancy is the second-highest cause of death after infection, and lymphoma accounts for about half of malignancies. The most frequently reported lymphoma type is diffuse large B-cell lymphoma (DLBCL) and the incidence of T-cell lymphoma is rare. PTLDs are also rare serious lymphoid and/or plasmacytic proliferative disorders that occur after undergoing solid organ or hematopoietic stem cell transplantation (HSCT). In the context of HSCT, most reported PTLDs have occurred in patients who received allogenic HSCT, but only a few cases have been reported in autologous HSCT (AutoHSCT) recipients.Case presentationA 53-year-old female patient initially presented with enlargement of the left cervical lymph nodes and was diagnosed with EBV-positive DLBCL. She was treated with R-CHOP, R-ACES, and AutoHSCT and went into remission. Four years later, computed tomography results revealed multiple lung nodules and abnormal infiltration, and sustained and progressing hypogammaglobulinemia was observed. The pathological specimen of video-assisted thoracoscopic surgical lung biopsy demonstrated extensive invasion of lymphocytes with notable granuloma findings. Flow cytometric immunophenotyping analysis showed that lymphocytes were positive for CD3 and CD5; especially, CD3 was expressed in the cytoplasm. Southern blot analysis revealed rearrangements of the T-cell receptor Cβ1 gene. She was diagnosed with peripheral T-cell lymphoma, not otherwise specified, accompanied by notable granulomatous lesions.ConclusionHere, as a unique case of metachronous B-cell and T-cell lymphoma, we report a rare case of T-cell lymphoma that mainly affected the lungs with the presentation of notable granulomatous findings following AutoHSCT for EBV-positive DLBCL at the age of 53 years. These lung lesions of granulomatous T-cell lymphoma could be related to the underlying primary immunodeficiency background associated with sustained hypogammaglobulinemia.

Non-necrotizing granulomatous pneumonitis and chronic pleuritis in soldiers deployed to Southwest Asia.

Reports of respiratory illnesses among soldiers returning from Southwest Asia have been described. During deployment to Southwest Asia, soldiers are exposed to various respiratory hazards, including dust storms, smoke from burn pits and industrial air pollutants. A few studies have reported increased rates of constrictive bronchiolitis and asthma in these patients. We sought to expand upon the pathological findings in this cohort. Lung biopsies from veterans of Southwest Asia were identified and re-reviewed. All patients had undergone pulmonary function tests and chest high-resolution CT imaging with no significant findings. Overall, 59patients with a history of inhalational exposure to at least one of the following were identified: smoke from burn pit, dust storm and sulphur plant fire. Samples included video-assisted thoracoscopic lung biopsies (57 of 59, 96.6%) and cryobiopsies (two of 59, 3.4%). Patients were predominantly male (54 of 59, 91.5%) with an age range of 24-55years (mean and median=35). Non-necrotising, poorly formed granulomas were identified in 22cases (22 of 59, 37.2%). The granulomas were mainly bronchiolocentric and were associated with chronic lymphoplasmacytic bronchiolitis, similar to hypersensitivity pneumonitis (HP). Pleural reaction in the form of focal chronic lymphocytic pleuritis and/or focal pleural adhesions were seen in 43 of 57 (75.4%) biopsies. To our knowledge, this is the first study to report pleural reaction as well as features of HP in this population, suggesting that pleural reaction and HP may be part of the spectrum of Southwest Asia deployment-related lung diseases.

Video-Assisted Thoracoscopic Lung Biopsy in Critically Ill Patients With Hematologic Malignancy and Acute Respiratory Distress Syndrome: A Case Series Report.

Pulmonary complications are prevalent among patients with hematologic malignancies, who are at high risk of developing acute respiratory distress syndrome (ARDS). Although diffuse alveolar damage is considered the diagnostic hallmark of ARDS, there are plenty of other non–diffuse alveolar damage etiologies that can mimic ARDS and benefit from a specific therapy, therefore correcting the underlying cause. When the etiology remains unclarified despite noninvasive procedures, a surgical lung biopsy (either open via thoracotomy or video-assisted thoracoscopic surgery [VATS]) may be warranted. However, the role of surgical lung biopsy has not been extensively studied in patients with hematologic malignancy and ARDS and so doubt exists about the risk-benefit relationship of such procedures. In this article, we report a series of 8 critically ill patients with hematologic malignancies and ARDS, who underwent VATS lung biopsy, in a specialized institution in Cali, Colombia, from 2015 to 2019, with special emphasis on its diagnostic yield, modifications in treatment protocol, and safety. VATS lung biopsy is a minimally invasive procedure that appears to be a relatively safe with few postoperative complications and minimal perioperative mortality. It has a high diagnostic yield, resulting in a modification of treatment in a nondepreciable percentage of patients. However, this subset of patients was critically ill, with a high risk of mortality, and the lung biopsy did not appear to affect in this aspect. Future randomized controlled trials are needed to further clarify this topic.

Transbronchial Cryobiopsy in Interstitial Lung Disease: Safety of a Standardized Procedure.

Transbronchial lung cryobiopsy (TBCB) plays an increasing role in the evaluation of diffuse parenchymal lung disease with acceptable diagnostic yield and safety profile compared with that of video-assisted thoracoscopic surgical lung biopsy on most reports. However, published outcomes with regard to safety and complication rates vary. We aim to determine the safety profile of TBCB when performed using a standardized protocol consistent with recently published expert guidelines. We reviewed prospectively maintained databases from 5 Australian tertiary referral centers. The procedures were performed in accordance with a recent expert statement recommending standardization of TBCB procedures, in particular with fluoroscopy, a secured airway, and prophylactic bronchial blockers. Periprocedural complications were assessed along with clinical outcomes. A total of 121 patients underwent TBCB between August 2013 and August 2017 following a standardized protocol using general anesthesia. Of them, 84 patients (66.7%) were discharged on the day of the procedure. Pneumothorax occurred in 18 patients (14.9%), 13 (10.7%) of whom required chest tube drainage. Moderate bleeding occurred in 15 patients (13.2%) and severe bleeding in 1 (0.83%). Histopathologic diagnosis was made in 80 patients (66.1%). Pooled outcomes from Australian tertiary centers indicate that TBCB is safe when performed in a protocolized fashion. Active measures to anticipate and manage bleeding and to direct biopsy position result in low rates of major complications.

AN ATYPICAL PRESENTATION OF CYTOMEGALOVIRUS PNEUMONIA

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Cytomegalovirus (CMV) is a viral pathogen that is seen in 40-100% of adults by the fourth decade (1). Infection in an immunocompetent individual is usually subclinical, with severe life-threatening infection seen in immunocomprised individuals. Steroid use has a weak association for infection reactivation in an immunocompetent host. We present the case of a chronic kidney disease (CKD) patient on steroids who was diagnosed and treated for CMV interstitial pneumonia. CASE PRESENTATION: 78-year-old female with a past medical history of interstitial lung disease, idiopathic thrombocytopenia purpura on steroids, CKD stage III who presented from a nursing facility where she was recuperating from a recent hospitalization due to pneumonia. During her prior admission, she was treated with steroids, vancomycin and cefepime for a presumed bacterial pneumonia. Patient presented with shortness of breath. Vital signs were notable for a temperature of 98.4 F, heart rate 102 bmp, RR 24/min, and SaO2 90% on 7L NC. Physical exam revealed bibasilar rales. Labs showed a white blood cell count of 10.1 Thou/uL, creatinine of 1.5 mg/dL, lactic acid 2.8 mmol/L, procalcitonin of 0.12 ng/mL, lactate dehydrogenase (LDH) of 544 U/L. Computed tomography (CT) of the chest showed increasing ground glass changes compared to previous admission imaging. Patient was started on steriods, vancomycin and cefepime. Given worsening radiographical findings, history of ILD, and elevated LDH in the setting of chronic steroids, there was a suspicion for pneumocystis pneumonia. She underwent a video-assisted thoracoscopic surgery lung biopsy, bronchoalveolar lavage and wedge resection of the right lung. Pathology showed microscopic honey combing and extensive subpleural fibrosis, consistent with interstitial pneumonia. CMV cytopathic effect was confirmed with immunoperoxidase staining. PCR testing and tissue culture also returned positive for CMV; viral load was 81,581 IU/mL. Patient was started on a six-week course of valganciclovir with a plan to taper steroids as an outpatient. Patient was unfortunately lost to follow up thereafter. DISCUSSION: Severe life threatening CMV infection is seen in immunocomprised risk groups particularly transplant recipients, patients affected with AIDS, malignancy and neonates. Steroid use has a weak association with CMV reactivation; CKD also has been postulated as a risk factor for reactivation due to malfunctioning white cells. CONCLUSIONS: Although CMV pneumonia targets a subtype of immunosuppressed patients, it should be considered a differential in patient’s on chronic steroid treatment and CKD. This case emphasizes the importance of having a high degree of suspicion of CMV pneumonia in a patient on chronic high dose steroids, in clinical setting, such as this one. Without the correct diagnosis, obtained by PCR of a BAL sample, treatment opportunities will be missed, leading to poor outcomes. Reference #1: Grilli, Elisabetta, et al. “Cytomegalovirus Pneumonia in Immunocompetent Host: Case Report and Literature Review.” Journal of Clinical Virology : the Official Publication of the Pan American Society for Clinical Virology, U.S. National Library of Medicine, Dec. 2012. Reference #2: Al-Omari, A., Aljamaan, F., Alhazzani, W., Salih, S., & Arabi, Y. (2016). Cytomegalovirus infection in immunocompetent critically ill adults: literature review. Annals of intensive care, 6(1), 110 Reference #3: Carpani, G., Foresti, S., Dell'Oro, R., Grassi, G., & Bombelli, M. (2019). Severe systemic cytomegalovirus infection in an immunocompetent patient outside the intensive care unit: a case report. BMC infectious diseases, 19(1), 34. https://doi.org/10.1186/s12879-018-3621-8 DISCLOSURES: No relevant relationships by Antonie Auguste, source=Web Response No relevant relationships by Amreet Aujla, source=Web Response No relevant relationships by Sarah Banks, source=Web Response No relevant relationships by Manmeet Grewal, source=Web Response No relevant relationships by Ann Palmer, source=Web Response

Novel Lung Biopsy Surgical Technique for Definitive Diagnosis of Pulmonary Capillary Hemangiomatosis

The 2015 European Society of Cardiology/European Respiratory Society Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension state that lung biopsy is still the gold standard for confirming pulmonary capillary hemangiomatosis; however, the surgical lung biopsy is no longer recommended in most cases for its inherent risks. Nonintubated and uniportal video-assisted thoracoscopic surgery are the new developments in video-assisted thoracoscopic surgery technology. We combined these 2 technologies to create a novel approach for pulmonary capillary hemangiomatosis lung biopsy. A incision is made in chest wall. Sponge forceps are used to pull the lung out of the pleural space, and to resect target lung tissue. The incised margin is sutured with 3-0 Prolene (Ethicon, Somerville, NJ), and a negative pressure suction tube is used to fully expand the lung. Three patients were definitively diagnosed with pulmonary capillary hemangiomatosis by this technology. One patient developed a postoperative fever, with no other complications. The tubeless and uniportal video-assisted thoracoscopic surgery lung biopsy is a safe, effective, and feasible technology for definitively diagnosing patients with pulmonary capillary hemangiomatosis.

Preoperative Evaluation of Patients With Interstitial Lung Disease

Preoperative Evaluation of Patients With Interstitial Lung Disease

Clinical and Radiological Features of Various Variants of Extrinsic Allergic Alveolitis

Objective . To optimize diagnosis of various variants of morphologically verified extrinsic allergic alveolitis (EAA), by determining the most significant clinical and radiological indicators of its development. Material and methods . Examinations were made in 82 patients with morphologically confirmed EAA, including 10 people with acute EAA, 38 with subacute EAA, 26 with chronic EAA, and 8 with recurrent EAA. Their medical history data were studied; physical examination, clinical blood, pulmonary function (PF), and lung diffusing capacity tests, and high-resolution computed tomography (HRCT) of the chest were performed. Transbronchial and/or video-assisted thoracoscopic lung biopsies were used for morphological studies. Results . Four disease course variants were identified. Disease duration, adherence to a specific antigenic effect, and the clinical, radiological, and morphological patterns of the disease were determined for its each variant. Conclusions . 1. Acute EAA is characterized by an abrupt onset and rapid (no more than 3 months) development with a preponderance of intoxication syndrome (Cumulative Index (CI), 2.1 ± 0.2 scores); subacute EAA is characterized by a slower (8.5–10 months) development with a predominance of respiratory symptoms (CI, 2.5 ± 0.1 scores), the steady progression of which is observed in chronic EAA (CI, 2.9 ± 0.2 scores). Recurrent EAA in previously cured patients is accompanied by both syndromes simultaneously (CI, 2.7 ± 0.3). 2. The characteristic feature of acute EAA on HRCT of the chest is the extent of ground-glass opacity in both lungs (more than 3 segments) and perivascular infiltrates; that of subacute EAA is microfocal dissemination; that of chronic EAA is macrofocal changes in the presence of interlobular septal thickening, as well all the development of cystic changes in the subpleural zones. In recurrent EAA, all the listed symptoms may appear. 3. Enlarged intrathoracic lymph nodes are most common in subacute EAA in 45.9% of cases and less common in acute and chronic EAA in 20 and 23.8% of cases, respectively. In all disease variants, lymph node hypertrophy affects the bifurcation and paratracheal groups and less often the bronchopulmonary and tracheobronchial groups; the sizes vary from 11 to 20 mm with means of 13.0 ± 1.2 mm.

Lung Bioposy Without Pleural Drainage.

Video-assisted thoracoscopy and atypical resection of lung parenchyma is a surgical procedure that is carried out very commonly around the world, mainly to determine the degree of malignancy of a suspect pulmonary nodule. A pleural drain is routinely inserted at the end of the procedure. The goal of our study was to evaluate the outcomes of this procedure with and without pleural drainage. From June 2015 to January 2018, 74 patients were prospectively randomized to either the chest-tube group (CT group, 37 patients) or the no-chest-tube group (NCT group, 37 patients) and were followed up until the seventh day after surgery. The postoperative duration of hospital stay was the primary endpoint; the secondary endpoints were the rates of pneumothorax and repeated chest drainage, pain intensity, and analgesic consumption. Blinding was not possible. An intention- to-treat analysis was performed. (Study registration; DRKS00008194, www.drks.de/drks.). Hospital stays were significantly shorter in the NCT group (means and first and fourth quartiles: 1.5 [1.5; 1.5] versus 2.5 [2.5, 2.5] days, p<0.001). The two groups did not differ significantly with respect to the frequency of postoperative complications. There were two occurrences of postoperative pneumothorax in the NCT group, with one patient needing drainage via chest tube and the other needing no treatment. Pain intensity and analgesic consumption were markedly lower in the NCT group; the cumulative oral intake of metamizole and acetaminophen was also lower in the NCT group (mean ± standard deviation: 3.7 ± 2.2 g in the NCT group versus 10.0 ± 4.2 g in the CT group, p<0.001). Not inserting a chest tube after video-assisted thoracoscopic lung biopsy significantly shortens the postoperative hospital stay, and the complications in the chest-tube and no-chest-tube groups are similar. Postoperative pain and analgesic consumption are markedly less when no chest tube is inserted.

Lung Diseases in Inflammatory Myopathies.

Lung involvement is the leading cause of mortality in inflammatory myopathy. A careful assessment of clinical and serologic manifestations especially myositis-associated autoantibodies allows precise classification of the different phenotypes of inflammatory myopathy and stratification of the risk of lung involvement. About three out of four patients with inflammatory myopathy develop interstitial lung disease (ILD), which represents the main cause of morbidity and mortality. In patients with a confirmed diagnosis of inflammatory myopathy, the approach to the diagnosis of ILD includes assessment of clinical and functional severity, evaluation of the high-resolution computed tomography pattern of disease, which often suggests nonspecific interstitial pneumonia or organizing pneumonia. Bronchoalveolar lavage to rule out infection is often performed; however, video-assisted thoracoscopic lung biopsy is now generally discouraged, unless malignancy is suspected. The so-called antisynthetase syndrome characterized by the combination of mechanics' hands, Raynaud' phenomenon, myositis often mild or absent, and presence of one of the anti-tRNA synthetase antibodies is associated with a 70% risk of ILD, especially in subjects with antibodies other than anti-Jo1 antibodies (i.e., anti-PL7 or -PL12 antibodies). Treatment depends on both severity and progression of ILD, often including a combination of corticosteroids and immunosuppressive therapy. Rituximab-based regimen has showed promising results in retrospective studies for the management of refractory or rapidly progressive forms of ILD. Clinical trials are ongoing to evaluate the actual efficacy of this strategy on mortality related to lung disease. Secondary pulmonary complications of inflammatory myopathy include opportunistic infections, aspiration pneumonia, pneumomediastinum, ventilatory failure due to diaphragmatic muscular weakness, drug-induced pneumonitis, and rarely pulmonary hypertension.

CASE OF A GRANULOMATOUS-LYMPHOCYTIC INTERSTITIAL LUNG DISEASE IN A PATIENT WITH COMMON VARIABLE IMMUNODEFICIENCY DISORDER

SESSION TITLE: Pulmonary Manifestations of Systemic Disease 1 SESSION TYPE: Med Student/Res Case Report PRESENTED ON: 10/09/2018 03:45 PM - 04:45 PM INTRODUCTION: Common variable Immunodeficiency Disorder (CVID), is the most common primary immunodeficiency disorder. Approximately 30% of patients with CVID develop autoimmune and granulomatous disease. Interstitial lung disease (ILD) manifests in 5-15% of CVID patients and represents a diagnostic challenge. It may present with sarcoid like granulomas, organizing pneumonia, lymphocytic interstitial pneumonia and nonspecific interstitial pneumonia. We present one such case of granulomatous-lymphocytic interstitial lung disease (GL-ILD). CASE PRESENTATION: 60 year old African American non-smoking female with no significant underlying medical history presented with cough in March 2006. Family history consisted of mother with stomach cancer, aunt with bone cancer, and grandmother with liver cancer. On physical exam, she had palpable axillary and inguinal lymph nodes. She had an abnormal CT chest showing bilateral lung nodules along with mediastinal lymphadenopathy. CT abdomen/pelvis showed retroperitoneal and pelvic adenopathy. PET scan showed uptake in multiple regions in neck, chest, abdomen and pelvis. PPD and HIV tests were negative.Workup for connective tissue disease came back negative except for low IgA, low normal IgG, and reduced IgM levels. She had minimally elevated ACE level. Inguinal and left axillary lymph node biopsies were non-diagnostic, showing benign lymph nodes with reactive follicular hyperplasia. Flow cytometry was negative. Video-assisted thoracoscopic left lung biopsies showed nodular airway-centered lymphoid hyperplasia with associated scattered small non-necrotizing granulomas. Follicular bronchiolitis was present. Biopsies did not suggest sarcoidosis. Mycobacterial and fungal cultures were negative. She had serial CT chest, abdomen and pelvis for the next two years showing no progression of her disease. After 2008, she had slow progression of lymphadenopathy and was evaluated at a tertiary care center in 2012. Definitive diagnosis of GL-ILD was given at that point. She was placed on steroids for about a year with stabilization of her disease. Subsequently, she had worsening of her lymphadenopathy off steroids and has been maintained on IgG infusions. DISCUSSION: CVID is present in a considerable number of patients with a small subset presenting with cryptic ILD. Exhaustive diagnostic workup is usually needed including open lung biopsy. Patients with GL-ILD may remain stable over years after optimization of their immunoglobulin levels. Following this, steroids remain first line treatment and response may be variable. In progressive disease, immunosuppressive treatment is recommended. CONCLUSIONS: This case illustrates an example of a form of interstitial lung disease associated with CVID. When lung biopsies demonstrate lymphocytic infiltrate and/or granuloma in the lung, CVID should be considered in the differential. Reference #1: British Lung Foundation/United Kingdom PrimaryImmunodeficiency Network Consensus Statement. J Allergy Clin Immunol Pract. 2017 Jul - Aug;5(4):938-945. https://doi.org/10.1016/j.jaip.2017.01.021. Epub 2017 Mar 25. Reference #2: Granulomatous-lymphocytic interstitial lung disease as the first manifestation of common variable immunodeficiency. Clin Respir J. 2018 Jan;12(1):337-343. https://doi.org/10.1111/crj.12511. Epub 2016 Jun 22. DISCLOSURES: No relevant relationships by Riju Dasgupta, source=Web Response No relevant relationships by Asok Dasgupta, source=Web Response No relevant relationships by Chelsea Leipold, source=Web Response No relevant relationships by David Taylor, source=Web Response No relevant relationships by Paresh Timbadia, source=Web Response

Diffuse parenchymal pulmonary amyloidosis associated with multiple myeloma: a case report and systematic review of the literature

BackgroundPulmonary is an uncommon site of extramedullary involvement in multiple myeloma (MM). Diffuse parenchymal amyloidosis as pulmonary manifestation of MM is even rarer. We report a rare case of diffuse parenchymal pulmonary amyloidosis associated with MM diagnosed by video-assisted thoracoscopic lung biopsy (VATLB).Case presentationA 58-year-old woman complained of cough and shortness of breath. HRCT disclosed diffuse ground-glass opacifications with interlobular septal thickening in bilateral lungs. A lung-biopsy sample obtained by VATLB revealed Congo Red-positive amorphous eosinophilic deposits in the alveolar septa. Surgical biopsy of abdominal wall skin and subcutaneous fat was also performed, which showed the apple-green birefringence with polarized light on Congo red stain was demonstrated in dermis. The serum immunoelectrophoresis showed monoclonal lambda light chains. A bone marrow biopsy specimen comprised 11.5% plasma cells. She was therefore diagnosed with diffuse parenchymal pulmonary amyloidosis accompanied by MM. The patient was referred to the hematology department for further chemotherapy.ConclusionsIt is important to recognize diffuse parenchymal pulmonary amyloidosis to avoid misdiagnosis.

Non-intubated video-assisted thoracoscopic lung biopsy for interstitial lung disease: a single-center experience.

The mortality and morbidity associated with video-assisted thoracoscopic (VATS) lung biopsy for interstitial lung disease (ILD) are not negligible. We evaluated whether non-intubated VATS lung biopsy, which avoids intubation and general anesthesia, can be safely performed in ILD subjects. This retrospective study compared the incidence of complications and surgical mortality between 25 consecutive intubated subjects and 10 non-intubated subjects (a total of 35 consecutive subjects) at a single institution. No major surgical complications or deaths were reported in either group, and non-intubated VATS biopsies were safely performed in subjects with relatively low carbon monoxide diffusing capacity (P=0.08) or poor American Society of Anesthesiologists physical status scores (ASA) (P=0.02). These preliminary results suggest that non-intubated VATS lung biopsy is a safe and feasible option in patients with ILD.

IgG4-related lung disease on the horizon

Abstract Immunoglobulin G4 (IgG4) related lung disease is an emerging entity. We report a case of a 42-year-old man presented with fever and cough with minimal sputum. Chest X-ray revealed diffuse reticulonodular shadows. Extensive investigations were performed, including video-assisted thoracoscopic lung biopsy, which confirmed the diagnosis of IgG4-related disease (IgG4-RD) with lung involvement. This case report aims to illustrate that IgG4-related lung involvement can present as diffuse lung nodules and can affect different pulmonary structures. IgG4-RD should always be considered when a similar scenario is encountered.

IgG4-related lung disease on the horizon

Abstract Immunoglobulin G4 (IgG4) related lung disease is an emerging entity. We report a case of a 42-year-old man presented with fever and cough with minimal sputum. Chest X-ray revealed diffuse reticulonodular shadows. Extensive investigations were performed, including video-assisted thoracoscopic lung biopsy, which confirmed the diagnosis of IgG4-related disease (IgG4-RD) with lung involvement. This case report aims to illustrate that IgG4-related lung involvement can present as diffuse lung nodules and can affect different pulmonary structures. IgG4-RD should always be considered when a similar scenario is encountered.