Severe Restrictive Lung Disease Research Articles

Combined General Anaesthesia with Retrolaminar Block for Breast Cancer Surgery in Pompe’s Disease

Pompe’s disease is a rare metabolic disease that results in progressive muscle weakness which culminates in respiratory failure in the late onset type. Patients with Pompe’s disease are at high risk of cardiac and respiratory complications under general anaesthesia owing to their premorbid cardiac and respiratory impairments, as well as the respiratory depressant effects of anaesthetic and analgesic agents. We present a high-risk case of a patient with late onset Pompe’s disease complicated by severe restrictive lung disease scheduled for breast cancer surgery under general anaesthesia with a single shot retrolaminar nerve block for analgesia.

Anaesthetic management of severe scoliosis correction in spinal muscular atrophy, severe restrictive lung disease and difficult airway.

Anaesthetic management of severe scoliosis correction in spinal muscular atrophy, severe restrictive lung disease and difficult airway.

STARTED FROM THE BOTTOM, NOW WE'RE HERE: THE UNRELENTING CASE OF CHARCOT MARIE TOOTH DISEASE FROM ANKLE WEAKNESS, TO VENOUS THROMBOEMBOLI, TO VENTILATOR DEPENDENCE

TYPE: Case Report TOPIC: Genetic and Developmental Disorders INTRODUCTION: Charcot Marie Tooth Disease Type 4J (CMT4J) is a rare inherited peripheral neuropathy caused by mutations of the lipid phosphatase FIG4 gene. Severe forms may lead to respiratory dysfunction. CASE PRESENTATION: A 44 year old male had an initial complaint of falls due to bilateral foot drop. Nerve conduction studies demonstrated axonal and demyelinating polyneuropathy. This raised suspicion for chronic inflammatory demyelinating polyneuropathy. He was started on intravenous immunoglobulin treatments and corticosteroids with no improvement, and soon he became wheelchair bound. 3 months after initial presentation, he developed shortness of breath and chest pain; he was diagnosed with provoked bilateral pulmonary emboli with pulmonary infarcts and started on rivaroxaban. Pulmonary function testing revealed severe restrictive lung disease with decreased respiratory muscle force necessitating a non-invasive auto-titration ventilation device (AVAPS-AE). Genetic testing performed at 4 months since initial symptoms revealed a heterozygous missense variant in the FIG4 gene consistent with CMT4J. 6 months after initial presentation, he went into cardiac arrest due to hypercapnic respiratory failure; return of spontaneous circulation was achieved, and eventually he underwent a tracheostomy and became ventilator dependent. DISCUSSION: CMT4J is a rapidly progressive pure motor neuron type of CMT which mimics amyotrophic lateral sclerosis. This case highlights the course of the disease as it relates to the timing of symptom onset, diagnosis, and the most crippling complication that is respiratory failure. CONCLUSIONS: Peripheral neuropathies, especially in the benign phases, should provoke early and broad screening, genetic testing, and advanced planning with regard to the devastating sequelae of ascending neuromuscular weakness. DISCLOSURE: Nothing to declare. KEYWORD: Charcot Marie Tooth

Protocol-driven early tracheal extubation in patients with flaccid neuromuscular scoliosis and pre-existing lung disease.

To review the results of a postoperative respiratory pathway for patients with muscular dystrophy (MD) and spinal muscular atrophy (SMA) undergoing spinal surgery. With IRB approval, a retrospective review was done on all patients with SMA and MD undergoing spinal surgery on a neuromuscular protocol. Baseline demographics, perioperative results, and long-term outcomes were collected. Per the protocol, patients remained intubated after surgery and were transported to the intensive care unit (ICU) for extubation. We present the results of protocol implementation and compare patients with MD to those with SMA. Twenty-four patients were treated using the protocol. Average age was 13.1years. Severe restrictive lung disease was present in 75% of patients. Nocturnal BiPAP was required in 68% of patients. Average number of instrumented levels was 17. All patients were immediately extubated upon entering the ICU. There were three respiratory complications and only was patient was re-intubated. Average ICU stay was 1.8days and average hospital length of stay was 6.7days. No differences in postoperative inspiratory or expiratory positive airway pressures were observed between the MD and SMA groups. Through a multidisciplinary neuromuscular protocol, excellent clinical outcomes were achieved in patients with neuromuscular scoliosis and restrictive lung disease, with complication rates and length of stay significantly lower than previously published data. IV.

824 Case Series on the Use of Volume Assured Pressure Support in Patients with Chronic Pulmonary Disease and Progressive Hypercapnia

Abstract Introduction Chronic hypercapnia results from destruction of lung parenchyma which occurs in chronic lung diseases including interstitial lung disease (ILD), bronchiectasis, and chronic lung transplant rejection. Many patients with these diseases will experience progressive respiratory failure eventually requiring consideration of transplantation or re-transplantation. Due to physiologic changes in sleep including reduction in tidal volume, worsening air tapping, and REM atonia, hypoventilation can be exacerbated during the sleeping hours. We present four patients who were prescribed nocturnal Volume Assured Pressure Support VAPS for their progressive hypercapnia. Report of case(s) Subject 1 is a 72 year old female with severe bronchiectasis and restrictive lung disease due to TB pneumonia at a young age. Subject 2 is a 45 year old male with history of pulmonary cavitation due to extensive TB disease when he was younger. Subject 3 is a 45-year-old woman with rheumatoid arthritis related ILD with associated pulmonary arterial hypertension. Subject 4 is a 74 year old patient with a bilateral lung transplant for IPF complicated by bronchiolitis obliterans syndrome who presented with progressive dyspnea and hypercapnia. Despite optimal therapy, all of these patients were admitted for hypercapnic and hypoxemic respiratory failure requiring treatment with BPAP then transitioned to nocturnal VAPS on discharge. For all patients, dyspnea and pCO2 improved as outpatients although all patients did eventually experience an exacerbation of their lung disease requiring repeat admission. Conclusion Due to the physiologic changes that occur with sleep, patients with severe lung disease may experience worsening CO2 retention while sleeping. There is little data assessing the use of chronic nocturnal non-invasive ventilation (NIV) to treat the hypercapnia of chronic lung diseases other than chronic obstructive pulmonary disease, extra-thoracic restriction, and neuromuscular disease. In this case series, nocturnal VAPS stabilized and/or reduced pCO2 in patients with pulmonary parenchymal disease of various etiologies. Additional studies are needed to assess long term effects of VAPS in these patients, including exacerbations, symptoms, and overall mortality. Support (if any):

The use of high flow nasal oxygen in a patient with Duchenne muscular dystrophy with severe restrictive lung disease requiring dental extractions: a case report

Presentation: A 22-year-old man with Duchenne muscular dystrophy (DMD) presented for removal of impacted third molars in his baseline health status. Comorbidities included decreased mobility, restrictive lung disease [forced vital capacity (FVC) 35% at baseline], adrenal insufficiency, and obstructive sleep apnea. Anesthetic challenge: General anesthetics are problematic in this population. The airway is typically compromised by macroglossia. Patients have reduced functional residual capacity, so time to desaturation after induction is reduced; in patients with FVC<50% there is high risk of postoperative pulmonary complications. Those with FVC<30% face further increased risk. The use of depolarizing neuromuscular blockade is contraindicated due to the risk of hyperkalemia. Residual nondepolarizing neuromuscular blockade could compromise postoperative pulmonary status further. Controversy remains regarding use of inhaled anesthetics, but they are generally avoided given the risk of rhabdomyolysis. In addition, DMD patients often have cardiomyopathy, further complicating management. The overall challenge was to avoid general anesthetics in this patient. How it was done: After a full discussion with the patient and family, we decided sedation with local anesthetic was the safest anesthetic plan. As a safeguard, inhaled anesthetics were removed, the machine was flushed, and filters were placed in the circuit; a Glidescope was available. We proceeded with moderate sedation with propofol and dexmedetomidine infusions. Given his reduced pulmonary function and need for oral access, the patient was placed on high-flow nasal cannula to provide supplemental oxygen and provide positive end-expiratory pressure. Opioids were kept to a minimum: 50 mcg fentanyl was given with local anesthetic injection. The anesthetic and dental teams talked the patient through his discomfort for much of the procedure. Music of the patient’s choosing was played. Overall the patient did well with a combination of sedation, local anesthesia, and coaching by the surgery and anesthesia teams. He was discharged same day without noted complications of anesthesia. Conclusion: Preparation for this apparently “simple” case in a complex patient was key to our success. By addressing the known pulmonary changes in DMD, we employed high-flow nasal cannula in a dental surgery—a new use for this device. By using all the tools available to us as anesthesiologists we were able to avoid an intubation and the possibility of a prolonged hospital stay for our patient.

COVID-19 in advanced Duchenne/Becker muscular dystrophy patients

Duchenne muscular dystrophy (DMD) is the most common childhood muscular dystrophy. As a result of progressive muscle weakness, pulmonary function decreases during the second decade of life and lung disease contributes significantly to morbidity and mortality in these patients. Corticosteroids are the current standard of care for patients with DMD, despite known adverse effects such as obesity and immunosuppression. Over the past year (2020), the novel coronavirus (COVID-19/SARS-CoV2) outbreak has caused a global pandemic. Restrictive lung disease due to low lung volumes, chronic immunosuppressive treatment with corticosteroids, and obesity are potential risk factors that may contribute to a more severe course of the disease. Out of 116 Duchenne/Becker muscular dystrophy patients treated in our tertiary neuromuscular center, six patients with DMD and one with advanced Becker muscular dystrophy were found to be positive for COVID-19 infection. Two of the DMD patients were admitted for hospitalization, of whom one was dependent on daily nocturnal non-invasive ventilation. All patients recovered without complications despite obesity, steroid treatment and severe restrictive lung disease.

Low Preoperative Lung Functions in Children With Early Onset Scoliosis Predict Postoperative Length of Stay.

Children with early onset scoliosis (EOS) undergoing spine surgery often have significant respiratory disease. Preoperative risk assessments that predict an increased length of hospital stay (LOS) for this group have not been previously evaluated. A voluntary protocol using preoperative lung function studies began among participants of a multicenter registry in 2016. Preoperative assessments were standardized to include spirometry, blood hemoglobin levels, serum bicarbonate, albumin and prealbumin; radiographic parameters of the spine, C-EOS classification and need for preoperative pulmonary assistance before initial growth friendly device insertion or "definitive" spine fusion. Primary outcome was LOS postoperatively. Data, including age, diagnosis, and type of surgery, was collected prospectively. Secondary outcomes measured included intensive care unit LOS, requirement for new pulmonary assistance on discharge, and pulmonary complications. Groups were compared using the Fisher exact tests. Of 525 children enrolled, 101 (20%) had preoperative spirometry. Median age was 8.9 years [interquartile range (IQR): 4.27]. Etiologies for EOS included 29 neuromuscular (28%), 33 idiopathic (32%), 19 syndromic (19%), and 22 congenital (21%) scoliosis. Eighty (78%) had growing rod (GR) insertions; 23 (22%) had spine fusion SF. Eighteen subjects (17%) were hospitalized ≥7 days (median=9 d); 83 had a LOS <7 days (median=3 d). Percentage of forced vital capacity (FVC%) predicted was inversely associated with LOS ≥7 days with a median of 75.3% (IQR: 41.7) for LOS <7 days and 51.7% (IQR: 41.6) (P=0.02). There were no detectable differences in LOS for other preoperative values. FVC predicted ≤50% preoperatively in children undergoing initial growth friendly rod insertion or definitive fusion after growth friendly treatment is associated with an increased risk of postoperative hospital stays ≥7 days. As demonstrated in previous studies, severe restrictive lung disease (FVC% predicted at or below 50%) is associated with increased risk of poorer outcomes for EOS patients.

A Woman in Her Forties with Six Months of Worsening Dyspnea

Uterine leiomyosarcoma (ULMS) is a rare cancer that originates from the smooth muscle of the uterine wall. ULMS is aggressive with a high rate of recurrence and mortality. Although the majority of ULMS are initially diagnosed within the uterus, they possess a high metastatic potential. Two thirds of metastatic ULMS sites involve the lung. However, the clinical presentations of such metastases are surprisingly not well described. In this case, we describe a case of a woman in her forties who presented with 6 months of worsening dyspnea and chronic cough. Her pulmonary function test demonstrated severe restrictive lung disease. Computed tomography (CT) of the chest was done which showed multiple pulmonary nodules. CT abdomen and pelvis identified multiple uterine masses with largest being 17 cm. Biopsy of uterine masses confirmed the diagnosis of UMLS. Her restrictive lung disease was diagnosed to be secondary to UMLS, as patient had no other identifiable risk factors.

Spondylocostal Dysplasia in a 7-Year-Old Sri Lankan Girl Causing Restrictive Lung Disease: A Case Report and Review of the Literature

Spondylocostal dysplasia (SCD) is a rare costovertebral malformation characterised by short-trunk short stature. It is a recessively inherited disorder, and commonly identified disease-causing mutations are in DLL3 gene. The reported prevalence is 1 : 200,000 worldwide, and none was reported from Sri Lanka. We report a 7-year-old Sri Lankan girl with spondylocostal dysplasia presenting with short stature and scoliosis. Disproportionate short stature was noted with short upper segment and small thoracic cavity. Skeletal survey revealed fused vertebra involving T5-T6, T9-T10, and L3-L4. Butterfly vertebrae were noted in T2, T4, T6, and T9. Diagnosis of SCD was made based on classic radiological features including vertebral fusion and rib abnormalities. Spirometry was performed due to small thoracic cavity which showed results compatible with moderate to severe restrictive lung disease. The child did not report respiratory difficulties or recurrent chest infections up to the presentation. She was referred to an orthopaedic team which recommended conservative management with close follow-up. In conclusion, spondylocostal dysplasia should be considered in short-trunk short stature with rib abnormalities in the absence of limb shortening. Appropriate treatment and follow-up for restrictive lung disease would determine the long-term outcome.

CHILDHOOD-ONSET INTERSTITIAL LUNG DISEASE: A RARE CASE OF IDIOPATHIC, NONSPECIFIC INTERSTITIAL PNEUMONIA IN A TEENAGER

SESSION TITLE: Fellows Diffuse Lung Disease SESSION TYPE: Fellow Case Reports PRESENTED ON: October 18-21, 2020 INTRODUCTION: Childhood interstitial lung disease (chILD) is remarkably rare with a reported prevalence from 0.13 per 100,000 children under 17 years to 16.2 per 100,000 children under 15 years of age (1). Here we present a case of a teenager with idiopathic nonspecific interstitial pneumonia (NSIP). CASE PRESENTATION: A 15-year old previously healthy female presented with a three-week history of dry cough, fever, malaise and shortness of breath which progressed to dyspnea on exertion despite a course of outpatient antibiotics. She had no environmental exposures, history of smoking or vaping nor family history of pulmonary pathology. Initial vital signs were significant for hypoxemia to 80% and a chest x-ray and CT scan with bilateral consolidations and ground glass opacities. Initial laboratory tests showed elevated inflammatory markers, other tests including SARS-CoV-2 PCR were negative . Bronchoscopy, bronchoalveolar lavage, and transbronchial biopsies yielded no definitive diagnosis. Following bronchoalveolar lavage, she developed rapid clinical deterioration necessitating mechanical ventilation, paralytics, and inhaled nitric oxide. Given elevated inflammatory markers and after infection was excluded, intravenous steroids were initiated with partial improvement. After extubation, a protracted inability to wean off oxygen support, and spirometry showing ongoing, severe restrictive lung disease, lung biopsy via video-assisted thorascopic surgery was performed lending to the ultimate diagnosis NSIP. Hypersensitivity pneumonitis panel, ILD panel and genetic testing were unrevealing, as such her case was classified as idiopathic NSIP. DISCUSSION: NSIP in adolescents is very rare. A meta-analysis (2) reports the average age of patients with NSIP to be 54.8 years. In the pediatric population, NSIP can be associated with systemic disease processes such as collagen vascular disease, including systemic lupus erythematosus, juvenile rheumatoid arthritis, and dermatomyositis associated with antibodies to melanoma differentiation-associated gene 5 (anti-MDA5), as well as a complication of blood stem cell transplants and surfactant dysfunction mutations, although most cases are idiopathic (3). Given that the etiology of NSIP is broad and in most cases idiopathic, response to corticosteroids is also variable. Our patient had a partial response to corticosteroids early on in her treatment, manifested by augmented gas exchange and improved symptomatology. The relatively slow response to treatment in the first several weeks is likely consistent with her biopsy findings which showed fibroblast proliferation and fibrosis. CONCLUSIONS: ILD and particularly NSIP are rare conditions in adolescents but should be suspected in patients with hypoxemia and abnormal imaging in the absence of infectious or autoimmune etiologies. An extensive workup is recommended to determine association with systemic conditions, treatment and prognosis. Reference #1: (1) Griese M, Haug M, Brasch F, et al. Incidence and classification of pediatric diffuse parenchymal lung diseases in Germany. Orphanet J Rare Dis. 2009;4:26. Published 2009 Dec 12. doi:10.1186/1750-1172-4-26 Reference #2: (2) Ebner L, Christodoulidis S, Stathopoulou T, et al. Meta-analysis of the radiological and clinical features of Usual Interstitial Pneumonia (UIP) and Nonspecific Interstitial Pneumonia (NSIP). PLoS One. 2020;15(1):e0226084. Published 2020 Jan 13. doi:10.1371/journal.pone.0226084 Reference #3: (3) Vece TJ, Fan LL. Interstitial Lung Disease in Children Older Than 2 Years. Pediatr Allergy Immunol Pulmonol. 2010;23(1):33-41. doi:10.1089/ped.2010.0008 DISCLOSURES: No relevant relationships by Giuliana Cerro, source=Web Response No relevant relationships by Gary Goulin, source=Web Response No relevant relationships by Jack Green, source=Web Response No relevant relationships by Michael Lewis, source=Web Response No relevant relationships by Tim Shen, source=Web Response

Amyopathic dermatomyositis with diffuse lung disease

A 38-year-old woman presented with progressively increasing breathlessness, recurrent productive cough, and intermittent fever of 1 year duration. Examination revealed cutaneous eruptions on the dorsal aspects of the hands and on face. Histopathologic features of skin biopsy revealed acanthosis, hyperkeratosis with focal vacuolar alteration of the basal-cell layer, and perivascular inflammatory infiltrates in upper dermis. CT scan showed diffuse lung disease and pulmonary function tests showed severe restrictive lung disease. There was no muscular involvement clinically or on electromyography and magnetic resonance imaging. She was diagnosed as a case of amyopathic dermatomyositis with diffuse lung disease and managed with topical and systemic steroid and topical sunscreen with fairly good response.

Correlation between Serum Krebs von den Lungen-6 Levels with Forced Vital Capacity and Modified Rodnan Skin Score of Patients with Restrictive Lung Disease in Diffuse-Type Systemic Sclerosis

Background Pulmonary fibrosis / intersitial lung disease (ILD) in systemic sclerosis (SSc) is a complicated restrictive pulmonary disease and the leading cause of disease-related mortality. Progressive skin fibrosis in diffuse-type SSc (dSSc) is associated with decreased forced vital capacity (FVC). Modified Rodnan Skin Score (mRSS) examination is used as a parameter to assess skin fibrosis, while high-resolution computed tomography (HRCT) and pulmonary function tests (PFTs) are used to assess pulmonary fibrosis. The HRCT test remains as the gold standard in diagnosing ILD. However, it costs a lot and is not available in all healthcare facilities. Krebs Von den Lungen-6 (KL-6) is a biomarker to evaluate pulmonary fibrosis. The aim of this study was to analyze the correlation of serum KL-6 levels with FVC and mRSS value of patients with restrictive lung disease in dSSc.&#x0D; Method This was a cross-sectional study that used primary data from dSSc patients who visited rheumatology outpatient clinic in Hasan Sadikin Hospital Bandung, Indonesia, during the period of June-July 2019. History taking, physical examination, mRSS, spirometry, and serum KL-6 levels were performed. Data were analyzed using the Rank Spearman correlation test. &#x0D; Result There were 27 subjects with the mean age of 42 ± 12 years. Based on FVC (%) restrictive lung disease criteria, the majority of subjects (74.1%) had severe restrictive lung disease and the rest of all subjects (25.9%) were non severe restrictive lung disease. Serum KL-6 levels ranged from 0.545 to 8.138 ng/ml. The results showed that there was no correlation between serum KL-6 levels and FVC values (r = -0.118, p = 0.279) and mRSS (r = 0.101, p = 0.312 ).&#x0D; Conclusion There is no correlation between serum KL-6 levels with FVC and mRSS value of patient with restritive lung disease in diffuse type systemic sclerosis.&#x0D; Keywords : diffuse type systemic sclerosis, Forced Vital Capacity, KL-6, mRSS, restrictive lung disease.&#x0D; &#x0D; &#x0D;

UNUSUAL CASE OF INTERSTITIAL LUNG DISEASE

SESSION TITLE: Diffuse Lung Disease 1 SESSION TYPE: Fellow Case Reports PRESENTED ON: 10/07/2018 03:30 pm - 04:30 pm INTRODUCTION: Interstitial Lung Disease can be associated with a broad range of diseases, exposures, drugs, and can be idiopathic. Silicosis has been understood as a cause of pulmonary disease for centuries. Silicosis inhalation is seen by occupational exposure such as sandblasting, mining, and masonry. Injected silicone is a liquid form of silicone and is commonly used in plastic and reconstructive surgery. We present a case of silicone injection that caused pulmonary fibrosis. CASE PRESENTATION: A middle aged Transgender female underwent breast implants and silicone thigh, gluteal, and hip injections. The injections were done in by unlicensed non-physician practitioners located in a home. Two weeks after the procedures the patient developed a severe skin reaction causing infection and skin breakdown. Several weeks after recovery the breast implants opened and caused skin necrosis and infection in the breasts. A bilateral mastectomy had to be completed. After recovery the skin became thickened and fibrotic. Two years later the patient developed worsening shortness of breath, dyspnea on exertion, and hypoxia. She presented to the emergency room where a chest xray showed bilateral upper lobe infiltrates. A CT chest showed severe fibrotic changes in middle and upper lung zones. Associated groundglass opacities, mild bronchiectasis, and interlobular connective-tissue thickening in bilateral lower lobes was also present. Pulmonary function tests noted severe restrictive lung disease with severely low oxygen exchange. Pathology of surgical biopsy showed extensive multinucleated foreign body giant cells. Many of the giant cells included non-polarizable microdroplets which were suggestive of silicone droplets. DISCUSSION: Acute lung injury from venous or arterial injection is most understood complication in literature. Chronic injury causing progressive massive fibrosis is rare. When silicone is mixed with soft tissue a fibroblastic response is generated. With history, clinical findings, imaging, and pathology our suspicion of silicone injection induced lung fibrosis was confirmed. CONCLUSIONS: Silicone is considered as inert agent, physicians especially pulmonologists need to be aware of its complications which may range from local tissues necrosis to progressive massive fibrosis1. Risk of complications increases with illicit injections or if performed by unskilled practitioners. Most of the pulmonary complications due to silicon implants, described in literature are acute like acute pneumonitis or diffuse alveolar damage and only few case reports are available for chronic pulmonary pathology like Granulomatous pneumonitis and progressive massive fibrosis2, 3.Recognition of acute and chronic complications is important as treatment of chronic complications is limited. Reference #1: Narins RS, Beer K. Liquid injectable silicone: a review of its history, immunology, technical considerations, complications, and potential. Plast Reconstr Surg. 2006;118(3 suppl):77S–84S. Reference #2: Chastre J, Basset F, Viau F, et al. Acute pneumonitis after subcutaneous injections of silicone in transsexual men. N Engl J Med. 1983;308(13):764–767. Reference #3: Clark RF, Cantrell FL, Pacal A, et al. Subcutaneous silicone injection leading to multi-system organ failure. Clin Toxicol (Phila). 2008;46(9):834–837. DISCLOSURES: No relevant relationships by Dipen Kadaria, source=Web Response No relevant relationships by Adnan Khan, source=Web Response No relevant relationships by Anthony Mattox, source=Web Response No relevant relationships by April McDonald, source=Web Response

Combined Selective Nerve Blockade and Local Infiltration Analgesia in a Total Shoulder Arthroplasty Patient With Chronic Pain and Severe Restrictive Lung Disease: A Case Report: Erratum.

Combined Selective Nerve Blockade and Local Infiltration Analgesia in a Total Shoulder Arthroplasty Patient With Chronic Pain and Severe Restrictive Lung Disease: A Case Report: Erratum.

Role for Assessment of Diaphragm Function Prior to Lung Transplant

Post-operative diaphragm dysfunction is a potential complication of lung transplant. This is most commonly associated with intraoperative phrenic nerve injury, but surgery itself is not the only risk factor for diaphragm dysfunction. It has been postulated that patients with interstitial lung disease (ILD) are at increased risk for diaphragm dysfunction. This could be due to malposition of the diaphragm in the setting of severe restrictive lung disease or from subpleural fibrosis affecting innervation and motility of the diaphragm. The goal of this study is to assess the prevalence of pre-operative diaphragm abnormalities in patients with ILD who underwent lung transplant and to determine if there is a correlation with prolonged ventilator dependence post-operatively.

Brief Report: Anti-RNPC-3 Antibodies As a Marker of Cancer-Associated Scleroderma.

Prior studies have demonstrated an increased risk of cancer-associated scleroderma in patients with anti-RNA polymerase III (anti-RNAP III) autoantibodies as well as in patients who are triple-negative for anticentromere (anti-CENP), anti-topoisomerase I (anti-topo I), and anti-RNAP III (also known as anti-POL) autoantibodies (referred to as CTP negative). In a recent study of 16 CTP-negative scleroderma patients with coincident cancer, 25% of the patients were found to have autoantibodies to RNPC-3, a member of the minor spliceosome complex. This investigation was undertaken to validate the relationship between anti-RNPC-3 antibodies and cancer and examine the associated clinical phenotype in a large sample of scleroderma patients. Scleroderma patients with cancer were assayed for anti-CENP, anti-topo I, anti-RNAP III, and anti-RNPC-3 autoantibodies. Disease characteristics and the cancer-scleroderma interval were compared across autoantibody groups. The relationship between autoantibody status and cancer-associated scleroderma was assessed by logistic regression. Of 318 patients with scleroderma and cancer, 70 (22.0%) were positive for anti-RNAP III, 54 (17.0%) were positive for anti-topo I, and 96 (30.2%) were positive for anti-CENP. Twelve patients (3.8% of the overall group or 12.2% of CTP-negative patients) were positive for anti-RNPC-3. Patients with anti-RNPC-3 had a short cancer-scleroderma interval (median 0.9 years). Relative to patients with anti-CENP, patients with anti-RNPC-3 and those with anti-RNAP III had a >4-fold increased risk of cancer within 2 years of scleroderma onset (for anti-RNPC-3-positive patients, odds ratio [OR] 4.3, 95% confidence interval [95% CI] 1.10-16.9 [P = 0.037]; for anti-RNAP III-positive patients, OR 4.49, 95% CI 1.98-10.2 [P < 0.001]). Patients with anti-RNPC-3 had severe restrictive lung disease, gastrointestinal disease, Raynaud's phenomenon, and myopathy. Anti-RNPC-3 autoantibodies, similar to anti-RNAP III autoantibodies, are associated with an increased risk of cancer at the onset of scleroderma. These data suggest the possibility of cancer-induced autoimmunity in this subset of patients with scleroderma.

Paecilomycosis Lilacinus Infection of the Immunocompromised Host

SESSION TITLE: Student/Resident Case Report Poster - Chest Infections II SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Although ubiquitous in distribution the fungus Paecilomycosis lilacinus (P lilacinus) is an extremely rare cause of human disease. It has been reported to cause skin and subcutaneous tissue infection as well as oculomycosis, cutaneous and subcutaneous infection not only in immunocompromised but also in immunocompetent hosts. The number of reported cases of lung infection with this pathogen is very scarce. We report a case of P lilacinus presenting with severe restrictive lung disease. CASE PRESENTATION: A 69 year old female with long term history of extensive psoriasis treated with methotrexate and adalimumab presented for evaluation of chronic cough and sputum production for 2 years. The cough was present throughout the day, often with prolonged coughing fits, and occasionally woke the patient up from sleep. She had been bringing up 4-5 teaspoons of green to yellow sputum almost every day for 2 years. She denied any fever, night sweats, hemoptysis, weight loss or history of treatment for any lung disease. Chest X-ray showed chronic interstitial disease. Pulmonary function tests revealed a severe restrictive ventilator defect with moderate reduction in gas exchange. High resolution computed tomography (HRCT) of the chest was significant for bronchiectasis with multiple small nodules, suspicious for infection but no fibrosis. Labs, including ANA, rheumatoid factor, serum immunoglobulin, CFTR gene mutation were normal. A subsequent bronchoscopy with lavage was done and the fungal culture came back positive for Paecilomycosis lilacinus. DISCUSSION: P lilacinus is a filamentous fungus that is responsible for devastating oculomycosis especially in patients with intraocular lens implantation or ocular surgery. It also causes cutaneous and subcutaneous infection in both immunocompromised and immunocompetent patients. Very rarely this fungus has been reported to cause infection at other body sites, with sinusitis being the most common. Lung involvement is extremely rare and only one case of lung abscess has been reported in the literature. To our knowledge, this is the first case of chronic lung infection by P lilacinus presenting as restrictive lung disease. Although it causes a non fatal infection, the treatment is very difficult as the fungus is often resistant to commonly used antifungal medications. CONCLUSIONS: P lilacinus is an emerging pathogenic mold that poses potential threat particularly in the ever expanding population of immunocompromised patients. The incidence in immunocompetent patient is also increasing. This pathogen might become a challenge in managing immunocompromised patients with structural lung disease in near future. Reference #1: Nayak DR, Balakrishnan R, Nainani S, Siddique S: Paecilomyces fungus infection of the paranasal sinuses. Int J Pediatr Otorhinolaryngol. 2000, 52: 183-187. 10.1016/S0165-5876(00)00282-2. DISCLOSURE: The following authors have nothing to disclose: Biplab Saha, Himani Sharma, Kristin Fless, Paul Yodice, Fariborz Rezai, Nirav Mistry, Vagram Ovnanian No Product/Research Disclosure Information

A Mistaken Case of Sarcoid

SESSION TITLE: Fellow Case Report Poster - Diffuse Lung Disease II SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Chronic hypersensitivity pneumonitis (HP) is a diffuse parenchymal lung disease caused by repeat exposure to a variety of antigens. It can mimic several interstitial lung diseases radiologically and pathologically making it difficult to differentiate. CASE PRESENTATION: A 40 year old female was referred to our pulmonary clinic for ILD evaluation after recently immigrating from Pakistan. She reported years of debilitating cough progressive dyspnea. She lived in an urban region and denied any significant organic or chemical exposures. Her initial pulmonary function testing (PFTs) suggested severe restrictive lung disease and her CT Chest revealed coarse upper lobe reticulation and architectural distortion. Despite non-revealing trans-bronchial and EBUS biopsies at the time, she was started on prednisone therapy for presumed sarcoidosis. Notably, her steroids were begun just months before her move and she reported persistent improvement in her symptoms since, despite the lack of objective changes in PFTs and CT chest on our assessment. Extensive serologic evaluation for an etiology revealed only antibodies to pigeon droppings which were not consistent with her history. She underwent VATs biopsy which revealed many poorly formed granulomas, severe diffuse interstitial fibrosis, cholesterol clefts, and Schumann bodies. Her final diagnosis was deemed to be chronic HP with fibrosing NSIP pattern. DISCUSSION: A definitive diagnosis for HP requires exposure to a known antigen and the assemblage of clinical, radiologic, laboratory, and pathologic findings. Due to the similar pathological features of NSIP and Chronic HP, the diagnosis remains a challenge. While acute HP may respond to immunosuppressive therapy, it is less clear whether patients with severe disease with an unclear precipitant would benefit from such therapy. In our case, it is unclear if the steroids or the environmental change (or both) may have halted the progression of her already severe disease. Thereby making the decision for long term immunosuppression a more difficult one. CONCLUSIONS: There remain large gaps in our knowledge with regards to the management of chronic HP, necessitating the need for larger case series or prospective trials. Reference #1: Churg A, Sin DD.Pathologic patterns and survival in chronic HP. Am J Sur Path.2009Dec;33(12)1765-70. Reference #2: Selman M, Pardo A. HP:insights in diagnosis and pathobiology. Am J Respir Crit Care Med 2012;186:314-324. Reference #3: Vourlekis JS, Schwarz MI.NSIP as the sole histologic expression of HP.Am J Med 2002;112:490-493. DISCLOSURE: The following authors have nothing to disclose: Mohammed Siddiqui, Omid Savari, Yasir Tarabichi No Product/Research Disclosure Information

G397(P) Hypoxic challenge testing – which children are we assessing?

Aim Hypoxic challenge testing (HCT) is the method used to assess whether a patient with stable respiratory disease requires in-flight oxygen. A national guideline, published by British Thoracic Society in 2011, makes recommendations on which children ought to undergo HCT prior to undertaking air travel. Identified are: infants under 1 year with neonatal history of chronic lung disease, children with CF/chronic lung disease old enough for spirometry whose FEV1 This audit aimed to define which patients had undergone hypoxic challenge tests and compared this against the patient groups identified in the BTS guideline. Methods A large tertiary children’s hospital in the UK conducted a retrospective audit of hypoxic challenge tests carried out between 2012 and 2014. Information was collected on age, diagnosis, when the patient last required long-term oxygen, lung function around the time of the test and the outcome of the test. Results 31 HCTs from 25 patients were audited. The average age was 6.3 years (6 months-16.5 years). Of the 22 patients who passed the HCT, three were recommended in the guideline to have HCT. Of the nine patients who did not pass HCT, two were within the recommendations. These five patients, who were within the recommendations, were three children with CF/chronic lung disease with FEV1 Of the nine children who did not pass the HCT, seven fell outside the recommended groups. Five were ex-preterm children older than one year had history of neonatal chronic lung disease, one had SMA Type 2, one had muscular dystrophy, one had severe kyphoscoliosis and one had severe restrictive lung disease following PNET. Conclusion Results show that clinicians are choosing to preform hypoxic challenge tests on patient outside of the recommendations. A larger proportion who failed the hypoxic challenge test were not defined within the BTS guidelines. With advancing technology the demographics of our respiratory paediatric patient population is evolving. It is therefore prudent to reconsider the paediatric recommendations.