Episodes Of Hemoptysis Research Articles

Solitary pulmonary nodule − Pulmonary hamartoma

Pulmonary hamartomas are rare, benign tumours of lung and are composed of an abnormal mixture of epithelial and mesenchymal elements. They usually present as single, round nodules with distinct boundaries and are mostly asymptomatic. We present a case of 45 years old female who presented with cough, chest pain and dyspnea with few episodes of hemoptysis. Computed tomography (CT) scan of thorax showed a well-defined non enhancing mass lesion measuring 4 × 3.2 cm seen in the left lower lobe. Wedge resection was done. Histopathological examination confirmed the diagnosis of pulmonary hamartoma.

P1728 Pulmonary hypertension in a pregnant Women - a rare anatomical aetiology

Abstract A 32 year old female patient, with a medical history of an ill-characterized Pulmonary Arterial Hypertension associated with congenital heart disease, lost in the follow up with no medical therapy, attended an emergency department for a gynecological hemorrhage at 16 weeks of pregnancy. Due to high maternity mortality risk, informed consent was obtained, and termination of pregnancy was performed. She was then referred to our pulmonary hypertension center. At our center she had complains of fatigue with moderate intensity exertion, classified in a class II of the World Health Organization (WHO) classification, but was otherwise asymptomatic, with no history of dyspnea, angina or syncope. There was allusion to a self-limited episode of hemoptysis in the past. On physical examination she had an increased pulmonary component of the second heart sound, continuous heart murmur in left sternal border and no cyanosis (O2 peripheral saturation in the upper and lower limbs of 99% at room air). The performed echocardiograms (both transthoracic and transesophageal) showed an estimated systolic pulmonary artery pressure of 120 mmHg with severe right ventricular hypertrophy and systolic dysfunction. There was dilatation of the trunk and right pulmonary artery. The left pulmonary artery was not seen. Biochemical evaluation and viral serologies were unremarkable. The pulmonary function tests and the arterial blood gases were normal. Cardiac MRI demonstrated the presence of a right aortic arch and a right patent arterial duct. An anomalous origin of the left pulmonary artery from the ascending thoracic aorta could be noted. Associated congenital cardiac defects were excluded. A right heart catheterization confirmed the presence of severe pulmonary hypertension with mean pulmonary artery pressure of 86 mmHg and Pulmonary vascular resistance of 11 Wood Units. A large persistent arterial duct to the right pulmonary artery was confirmed with persistent left to right shunt. The left pulmonary artery was visualized when injection was performed in the aortic root. Coronary arteries were normally implanted. The patient was started on Sildenafil and Bosentan (later replaced by Macitentan due to hepatic toxicity). After 3 years of follow up, there was an improvement in symptoms and in the 6 minutes walking test, remaining in a low risk category and on a WHO class I. This case reports a very rare congenital abnormality identified in an adult patient. Despite the complex anatomy and severe pulmonary hypertension, the patient is reasonably well under medical therapy and close follow up. Abstract P1728 Figure. Cardiac MRI Cine Sequences

Hemoptysis from the perspective of people with cystic fibrosis.

IntroductionPeople with cystic fibrosis (CF) are living longer, thus complications associated with age, such as hemoptysis, are increasing. The Institute of Medicine has emphasized the importance of patient‐centeredness. Although guidelines about hemoptysis in people with CF are available, these focus on management of the complication and not the patient perspective.ObjectiveWe sought to understand hemoptysis from the point of view of those who have experienced it.MethodsWe fielded an 11‐question survey to adults with CF and asked those who had hemoptysis to respond. Four questions had open‐ended options: (1) the person’s first experience with hemoptysis, (2) how that experience affected the way they approach their CF, (3) how they deal with hemoptysis when it occurs outside the home and (4) a free text box for general comments.ResultsThirty‐one of 132 adults with CF who were sent a survey completed it (23% response rate); 63% F), indicated that they had experienced hemoptysis and described their triggers. In response to open questioning, 77% of respondents found their first experience with hemoptysis to be ‘scary,’ ‘frightening,’ ‘worrying’ or ‘jarring.’ Half of respondents reported quality of life being negatively affected by worsening stress or anxiety, fear of bleeding in public or other life impacts.ConclusionsFocusing on how to cope with future episodes of hemoptysis and the associated anxiety can be helpful to patients. Proactive communication and sensitivity to patient experience may deepen physician‐patient rapport, increase self‐efficacy to cope with future episodes and lead to more comprehensive care of hemoptysis.

Diffuse alveolar haemorrhage associated with subsequent development of ANCA positivity and emphysema in three young adults

BackgroundDiffuse alveolar haemorrhage (DAH) is characterized by the diffuse accumulation of red blood cells within the alveoli, presence of ground glass opacities and/or consolidation on computed tomography (CT). Aside from identifiable non-immune causes, DAH is classically subdivided into idiopathic (idiopathic pulmonary haemosiderosis, IPH) and autoimmune DAH. Here we describe three cases presenting with recurrent pulmonary haemorrhage, initially classified as IPH, who, several years after first presentation, develop anti myeloperoxidase antibodies (MPO) positivity, emphysema on CT and, in one case, renal involvement.Case presentationPatient 1 was diagnosed with IPH aged 14. Her disease remained poorly controlled despite immunosuppression, although ANCA remained negative over the years. Nineteen years from initial presentation, she developed MPO-ANCA positive antibodies and mild renal impairment. She was treated with Rituximab with good response. From first presentation, the chest CT was consistently characterized by diffuse ground-glass opacities and interlobular septal thickening. Ten years later, cystic opacities consistent with emphysema, with a striking peribronchovascular distribution, developed. Patient 2 was diagnosed with IPH aged 32. He was treated with corticosteroids and methotrexate, with fluctuating response. At 11 years from initial presentation, MPO-ANCA positivity was identified, and emphysema with a peribronchovascular distribution was observed on CT, with subsequent significant increase in extent. Patient 3 was diagnosed with IPH at the age of seven, and had recurrent episodes of haemoptysis of varying degree of severity, treated with intermittent courses of corticosteroids until age 11, when he was intubated due to severe DAH. Eight years after the diagnosis emphysematous changes were noted on CT and MPO-ANCA positivity developed for the first time 11 years after initial diagnosis.ConclusionsWe believe these three cases highlight: 1) the possibility of development of ANCA positivity several years down the line from first DAH presentation 2) the possibility that DAH may lead to cystic/emphysematous changes with peribronchovascular distribution on CT. Moreover, the need for ongoing immunosuppressive treatment and the development of emphysema, emphasize a possible role played by autoimmune phenomena, even when DAH is initially diagnosed as “idiopathic”. Further studies are required to better understand the relationship between DAH, ANCA positivity and development of emphysema.

RECURRENT AND MASSIVE HEMOPTYSIS: A CONSEQUENCE OF A RARE ETIOLOGY

SESSION TITLE: Wednesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: Fibrosing mediastinitis is an inflammatory reaction that results in excessive fibrous tissue formation in the mediastinum. It is usually attributed to histoplasmosis. It may affect the airways, great vessels, and other mediastinal structures secondary to robust inflammatory reaction to fungal antigens including granuloma formation and calcifications. The clinical presentation depends on which mediastinal structures being involved and the extent of the damage. Hemoptysis in fibrosing mediastinitis can be the result of bronchial invasion, necrotizing pneumonia, pulmonary or bronchial venous or arterial invasion. CASE PRESENTATION: 68-year-old Caucasian female with past medical history of chronic sinusitis, paroxysmal atrial fibrillation and fibrosing mediastinitis who presented to the emergency room (ER) complaining of three episodes of hemoptysis for 6 hours. The hemoptysis was estimated to be around 100 ml each time, bright red blood and associated with mild shortness of breath. Initial vitals were 104/54 mm Hg, heart rate of 110 bpm, respiratory rate of 22 cpm and Spo2 95% on 4L nasal cannula. The patient received one unit of PRBC for acute drop in hemoglobin from 14 gm/dl to 11 gm/dl. The patient was admitted to the intensive care unit (ICU) due to a concern of airway compromise and potential need for endotracheal intubation. The patient had episodes of massive hemoptysis over the last year and received corticosteroids intermittently without significant improvement. In the ICU, The clinical condition was relatively stable with normal vital signs yet continued to have hemoptysis. CT scan of the chest revealed mediastinal widening with diffuse calcified lymph nodes, broncholith extending into right main stem bronchus, superior vena cava stent and dilated main pulmonary arteries. Conventional angiography demonstrated dilatation of the two right sided intercostal bronchial trunks and multiple dilated intercostal arteries. Successful embolization of the above-mentioned arteries with microspheres was performed until achieving hemostasis. Subsequently, the patient did not experience further episodes of hemoptysis. The patient was discharged home in a stable clinical condition. DISCUSSION: Recurrent and massive hemoptysis is a consequence of numerous etiologies. We are reporting a rare case of fibrosing mediastinitis that presented as recurrent and massive hemoptysis. Diagnostic approach indicated the involvement of the bronchial and intercostal arteries. The involved vessels were treated successfully by embolization CONCLUSIONS: Recurrent massive hemoptysis is an uncommon clinical feature in these patients and associated with poor prognosis along with other local invasive process by the calcified mediastinal masses. Reference #1: Mediastinal fibrosis complicating histoplasmosis, Loyd JE1, Tillman BF, Atkinson JB, Des Prez RM. Reference #2: Chronic fibrous mediastinitis and superior vena caval obstruction due to histoplasmosis, Dis Chest. 1959 Apr;35(4):364-77. DISCLOSURES: No relevant relationships by Mukhtar Al-Saadi, source=Web Response No relevant relationships by Sai Katta, source=Web Response

HEMOPTYSIS IN EISENMENGER SYNDROME-ASSOCIATED PULMONARY ARTERIAL HYPERTENSION (PAH): FORGOT ABOUT BRONCHIAL ARTERY EMBOLIZATION

SESSION TITLE: Pulmonary Vascular Disease SESSION TYPE: Med Student/Res Case Report PRESENTED ON: 10/23/2019 8:45 AM - 9:45 AM INTRODUCTION: Anticoagulation in Eisenmenger syndrome (ES) associated PAH (ES-PAH) presents a clinical challenge, as there is both an increased prevalence of pulmonary artery thrombosis and hemoptysis. We present a case of ES-PAH complicated by hemoptysis and treated successfully with multi-vessel bronchial artery embolization (BAE). CASE PRESENTATION: A 52-year-old female with an atrial septal defect complicated by ES-PAH treated with continuous epoprostenol infusion, ambrisentan, supplemental oxygen, and thrombophilia previously managed with warfarin; presented with a one-week history with multiple episodes of hemoptysis and chest tightness. She reported a similar episode of hemoptysis one-year previously with no specific source of bleeding identified. At the time of her hemoptysis had resolved with discontinuation of warfarin.On this presentation, her vital signs were unremarkable on 3 LPM nasal cannula. Physical examination demonstrated a right ventricular heave, a loud and prominent P2, and a third heart sound. Electrocardiography revealed normal sinus rhythm and bifascicular block (RBBB and LPFB). CT angiography (CTA) of the chest demonstrated dilated bronchial arteries and patchy ground glass opacities within the left upper and lower lobes, consistent with alveolar hemorrhage.Otorhinolaryngology and interventional pulmonology found no discrete bleeding in the upper or lower respiratory tract. After discussion, prophylactic embolization of the dilated right and left bronchial arteries was performed successfully. No further episodes of hemoptysis were reported post embolization. On outpatient follow-up the patient has remain hemoptysis free. DISCUSSION: Eisenmenger physiology results from chronic left-to-right shunting of blood, leading to increased right-sided pressures, and reversal of shunt flow. Patients with ES-PAH are at increased risk of pulmonary artery thrombosis with concurrent increased risk of hemoptysis and pulmonary hemorrhage(1). Thrombo-embolic risks include atrial fibrillation and indwelling central catheters. Hemoptysis is often due to bronchial artery dilatation and/or rupture. The use of anticoagulation in ES-PAH patients is controversial due to balancing risk between thrombosis and hemoptysis, and survival benefits are unclear(2). BAE in PAH patients is more complex than in patients without PAH, due to frequent higher numbers of culprit arteries and increased anatomical/morphological variability(3). However, BAE has been demonstrated to be a safe and reliable intervention for hemoptysis in PAH3. There is no definitive treatment and repeated BAE for reoccurrence is common. CONCLUSIONS: Anticoagulation in ES-PAH should be used conservatively in an individualized approach. Hemoptysis is an uncommon in ES-PAH and may result from bronchial artery dilatation and rupture. We demonstrated the safety of multi-vessel BAE in ES-PAH and encourage its consideration within this cohort. Reference #1: Wan D, Tsui C, Kiess M, et al. Anticoagulation for Thromboembolic Risk Reduction in Adults With Congenital Heart Disease. Canadian Journal of Cardiology 2017; 33: 1597–1603. Reference #2: Sandoval J, Santos LE, Córdova J, et al. Does Anticoagulation in Eisenmenger Syndrome Impact Long-term Survival?: Anticoagulation in Eisenmenger Syndrome. Congenital Heart Disease 2012; 7: 268–276. Reference #3: Rasciti E, Sverzellati N, Silva M, et al. Bronchial artery embolization for the treatment of haemoptysis in pulmonary hypertension. La radiologia medica 2017; 122: 257–264. DISCLOSURES: No relevant relationships by Olayiwola Amoran, source=Web Response no disclosure on file for Christine Archer-Chicko; Advisory Committee Member relationship with Actelion/Janssen Please note: $5001 - $20000 Added 02/07/2019 by Harold Palevsky, source=Web Response, value=Honoraria Advisory Committee Member relationship with United Therapeutics Please note: $1001 - $5000 Added 02/07/2019 by Harold Palevsky, source=Web Response, value=Honoraria DSMB relationship with Eiger Please note: $1001 - $5000 Added 02/07/2019 by Harold Palevsky, source=Web Response, value=Honoraria No relevant relationships by cyrus vahdatpour, source=Web Response

THE CASE OF THE FUNGUS IN THE HONEYCOMB

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: Aspergillomas are fungal balls that typically develop in pre-existing pulmonary cavities such as those caused by tuberculosis, sarcoidosis, histoplasmosis, cystic fibrosis and lung abscesses but are rarely associated with UIP. Aspergillomas are typically distributed as either multiple bilateral pulmonary aspergillomas or as an isolated aspergilloma. The most commonly reported cases of aspergillomas have been found in post-tuberculosis cavities, accounting for 11 to 17% of cases, with an increased incidence in areas of high tuberculosis prevalence. For this reason, aspergillomas are typically found in the upper lobes with lower/middle lobe findings often associated with other cavitary pulmonary diseases. CASE PRESENTATION: We present an unusual case of multiple, unilateral aspergillomas in a non-immunocompromised patient with usual interstitial pneumonia (UIP). Patient is a 73-year-old female with seropositive rheumatoid arthritis (not on immunosuppressive therapy) who was evaluated for weight loss and minor hemoptysis. Chest CT showed classic bibasilar honeycomb changes typical of UIP. There were nodular opacities within the honeycomb changes suggestive of mycetomas seen exclusively in the right lung base. These were subsequently identified as aspergillomas by correlating the chest CT with bronchoscopic cultures that grew A. fumigatus. The patient’s symptoms resolved spontaneously after 6 months without treatment for either aspergillomas or UIP. At follow up after 2 years, the patient remained symptom free without further episodes of hemoptysis and her weight was stable. DISCUSSION: This case is a unique presentation for several reasons. First, aspergillomas are rarely associated with UIP. Second, multiple aspergillomas usually occur in immunocompromised patients whereas our patient was not immunocompromised. Third, despite the bilateral distribution of honeycombing in this patient, the aspergillomas were strictly unilateral. Finally, whereas aspergillomas are usually upper lobe predominant, our patient’s aspergillomas were localized to the right lower lobe. CONCLUSIONS: Chronic aspergillomas increase the risk of local tissue invasion which may result in potentially life-threatening hemoptysis. Fortunately, this patient’s hemoptysis spontaneously resolved and she subsequently remained asymptomatic. Such a presentation should be kept in mind when taking care of similar patients. Reference #1: Kumar N, Mishra M, Singhal A, Kaur J, Tripathi V. Aspergilloma coexisting with idiopathic pulmonary fibrosis: A rare occurrence. J Postgrad Med 2013;59:145-8 Reference #2: Moodley L, Pillay J, Dheda K. Aspergilloma and the surgeon. J Thorac Dis. 2014;6(3):202-9. Reference #3: Regis Gothard Bopaka, Hasna Jabri, Wiam El Khattabi, Hicham Afif European Respiratory Journal 2015 46: PA2650; DISCLOSURES: No relevant relationships by Nina Chan, source=Web Response No relevant relationships by Peter Sloane, source=Web Response

PRESENTATION OF MYCOBACTERIUM ABSCESSUS INFECTION FOLLOWING A SARCOIDOSIS DIAGNOSIS: A CASE REPORT

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: Sarcoidosis is an idiopathic, multiorgan, inflammatory disease characterized by the presence of noncaseating granulomas. Although mycobacterial infection has long been a candidate as a causative agent in sarcoidosis, only recently has strong evidence supported a causal connection. Mycobacterium abscessus is a rapidly growing mycobacterium (RGM), found worldwide in the environment. It is increasingly being recognized as a cause of human disease, including pulmonary infections. Clinical features of progressive RGM lung disease include bilateral lung involvement and fibrocavitary disease. Here, we describe a case of Mycobacterium abscessus infection detected two years after sarcoidosis. CASE PRESENTATION: A 63 year old Chinese female, who worked as a farmer, presented with episodes of hemoptysis for 33 years, which was treated empirically with azithromycin on multiple occasions, resulting in good response according to the patient. She sought medical advice in the United States several times for the past 5 years. Workup during that period included: Quantiferon, ANA, ANCA, Anti-GBM, which were negative, chest CT remarkable for diffuse tree-in-bud opacities in the left lung, bronchoscopy with bronchoalveolar lavage (BAL) and pathology demonstrating noncaseating granulomas. She was started on steroids resulting in resolution of fever and hemoptysis. The patient stopped steroids and was lost to follow up. She remained asymptomatic until two years later when hemoptysis recurred. During that time, she was taking antibiotics from China. Repeat chest CT showed several scattered nodular and cavitary densities throughout the left lower lobe in a tree-in-bud morphology (Figure 1). Repeat rheumatological, vasculitis, and fungal panels were negative. Quantiferon TB Gold and MTB PCR were negative. Based on these results, as well as the previous biopsy demonstrating noncaseating granuloma, and the previous response on steroids, she was restarted on high dose steroids. Chest CT repeated 3 days after steroids demonstrated decrease in size and number of the pulmonary nodules. Bronchial washings and BAL samples were positive for M. abscessus. Steroids were tapered, and she was referred to infectious disease for treatment of pulmonary M. abscessus. She received Doxycycline with complete resolution of her symptoms. DISCUSSION: Although the patient's clinical and CT findings were not typical for sarcoidosis, the histopathology and improvement with steroids supported the diagnosis. However, noncaseating granulomas are nonspecific; as it can be seen in various conditions. The histological similarity between sarcoidosis and atypical mycobacterial infection raises the possibility that this patient’s Mycobacterial infection had previously evaded detection. CONCLUSIONS: Mycobacterial infection should be investigated in all patients with sarcoidosis as it could be a causative agent an infection that evaded detection. Reference #1: James DG, Sharma OP. From Hutchinson to now: a historical glimpse. Current opinion in pulmonary medicine. 2002;8(5):416-23. Reference #2: Brownell I, Ramirez-Valle F, Sanchez M, Prystowsky S. Evidence for mycobacteria in sarcoidosis. American journal of respiratory cell and molecular biology. 2011;45(5):899-905. Reference #3: Primm TP, Lucero CA, Falkinham JO, 3rd. Health impacts of environmental mycobacteria. Clinical microbiology reviews. 2004;17(1):98-106. DISCLOSURES: No relevant relationships by Meily Arevalo, source=Web Response No relevant relationships by Cinthya Carrasco Barcenas, source=Web Response My spouse/partner as a Speaker/Speaker's relationship with Salix Please note: $1001 - $5000 Added 03/13/2019 by Ebtesam Islam, source=Web Response, value=Speaker fee No relevant relationships by John Makram, source=Web Response No relevant relationships by Haneen Mallah, source=Web Response No relevant relationships by Ximena Solis, source=Web Response No relevant relationships by Wasawat Vutthikraivit, source=Web Response

IT CAN'T BE LUPUS...UNTIL IT IS: A CASE OF DIFFUSE ALVEOLAR HEMORRHAGE IN SYSTEMIC LUPUS ERYTHEMATOSUS

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Diffuse Alveolar Hemorrhage (DAH) is a rare, but well-documented phenomenon in systemic lupus erythematosus (SLE). DAH affects only 4% of patients with SLE but has a 50% mortality. CASE PRESENTATION: A 67 year-old female with SLE complicated by lupus nephritis requiring four renal transplants (last in 2015), but currently in remission on maintenance tacrolimus and mycophenolate mofetil with recurrent episodes of hemoptysis and hypoxic respiratory failure. In October 2018 she was admitted with dry cough, scant hemoptysis and developed rapidly progressive respiratory failure requiring intubation. Workup showed normal immunosuppressive levels, elevated white blood count and inflammatory markers. CT revealed diffuse ground glass opacities. Antinuclear antibody (ANA) and anti-double stranded DNA were negative with normal complement levels. Bronchoscopy showed mononuclear predominance with unrevealing microbiology and cytology and no active bleeding despite sequential BAL. She rapidly improved with antibiotics and steroids. In January 2019 she was admitted with similar symptoms of cough, scant hemoptysis, multilobar infiltrates. Again, serologic and bronchoscopic workup were unrevealing, without classical evidence of DAH leading to a broad differential and needing to determine the etiology for the recurrent episodes of hemoptysis. An open lung biopsy was performed and results returned from Mayo Clinic showing acute alveolar hemorrhage, secondary to SLE. Patient was started on high dose steroids. However, 24 hours later she had frank hemoptysis, this time with bronchoalveolar lavage confirming DAH. Unfortunately, despite steroids, dialysis and plasmapheresis, hospital course was complicated by multi-organ dysfunction and she ultimately expired. DISCUSSION: DAH due to SLE is usually in the setting of a flare with thrombocytopenia, hypocomplementemia as well as elevated lupus antibodies. It is often seen within the first 5 years of diagnosis. Our patient had well-controlled disease on immunosuppressive therapy and had consistently negative antibodies and normal complement levels. Her only known risk factor was recurrent lupus nephritis, which is the most associated extrapulmonary manifestation in patients with SLE and DAH. This patient was given high-dose steroids and plasmapheresis; in reviews of DAH in SLE nearly 100% were given steroids, 55% cyclophosphamide and 31% plasmapheresis. Utilizing recombinant factor VII during the massive hemoptysis was considered, but the data to support it was not well-supported. CONCLUSIONS: This may be the first documented patient with well-controlled Lupus, without evidence of a flare, who developed recurrent episodes of DAH from SLE without classical symptoms highlighting need for broad differentials and exact diagnosis. Given the high mortality associated with DAH, it must be recognized that this can occur and recur despite ongoing immunosuppressive therapy. Reference #1: Kusunoki et al. Severe Progressive Diffuse Alveolar Hemorrhage in a Patient with Systemic Lupus Erythematosus. Case Reports in Critical Care. 2018 Reference #2: Kazzaz NM, Coit P, Lewis EE, McCune WJ, Sawalha AH, Knight JS. Systemic lupus erythematosus complicated by diffuse alveolar haemorrhage: risk factors, therapy and survival. Lupus Sci Med. 2015;2(1):e000117. Published 2015 Sep 23. https://doi.org/10.1136/lupus-2015-000117 Reference #3: Ednalino, Christina & Yip, Julie & Carsons, Steven. (2015). Systematic Review of Diffuse Alveolar Hemorrhage in Systemic Lupus Erythematosus: Focus on Outcome and Therapy. Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases. 21. 305-10. 10.1097/RHU.0000000000000291. DISCLOSURES: No relevant relationships by Achal Dhupa, source=Web Response No relevant relationships by Jamie Felzer, source=Web Response No relevant relationships by Shazia Jamil, source=Web Response No relevant relationships by Lakshmi Koripella, source=Web Response

11. Accelerated UIP: a special challenge

IntroductionInterstitial lung disease (ILD) is a progressive fibrotic disease of the lung parenchyma. The lungs are the most common site in rheumatic diseases. ILD may be idiopathic or caused by connective tissue diseases, rheumatoid arthritis, drugs, and infection and radiation therapy. The severity of lung involvement may vary considerably, and, in some cases, it can lead to irreversible fibrosis leading to respiratory failure and eventually death. Although progress has been made in understanding of the disease, there are still significant challenges with the diagnosis and treatment of ILD. This case illustrates the diagnostic and treatment dilemmas.Case description67-year-old gentleman presented with a 4-year history of gradual onset of shortness of breath and dry cough with worsening over 18 months. Additionally, he developed ENT problems including nasal obstruction, polyposis, intermittent crusting with epistaxis and change in voice. He was treated with nasal douches, intermittent short courses of Prednisolone and antibiotics. He then developed joint pains with stiffness and weight loss. He had no other features of CTD or vasculitis. He was a BT Telecom office worker with no exposure to environmental agents, no family history and was an ex-smoker. Other medical history included type 2 diabetes and benign prostate hypertrophy. He was on metformin, finasteride, tamsulosin, fluoxetine and omeprazole.He had grade 2 finger clubbing with inspiratory bibasal crepitations. There was mild joint synovitis and systemic review was otherwise unremarkable. CT scan showed interstitial lung disease with UIP pattern. Initial lung function tests showed normal spirometry with TLCo 84% and KCO 86% predicted. Bloods revealed ACE 100, RF 16, MPO-ANCA 6.0, Eosinophilia 0.82 and CRP 7. A CT scan of the sinuses revealed bilateral ethmoidal polyps and moderate deviation of the nasal septum to the right.Initial treatment with glucocorticoids improved his joints, ENT symptoms significantly and respiratory symptoms marginally. However, his breathlessness and cough deteriorated over the next 6 months alongside lung function and DLco. Repeat CT scan showed progression of ILD with interval worsening of the honeycomb with reducing lung volume. He was tried on MMF but unfortunately deteriorated rapidly which led to hospital admission. CT scan showed new ground glass changes; it was thought to be acute exacerbation of underlying ILD with possible super added infection. He received high dose steroids and antibiotics with no improvement. Serial CXR showed rapid progression consistent with diffuse alveolar damage and he died 8 days after hospital admission.DiscussionThis case wasn’t straightforward. It was difficult to make a unifying diagnosis based on clinical presentation and the investigations. Given the predominant ENT and respiratory symptoms with low levels of MPO the most likely unifying diagnosis considered was primary systemic ANCA vasculitis. Although rare, ILD changes are also seen in MPO positive ANCA vasculitis. The association between interstitial lung disease (ILD) and ANCA-associated vasculitis (AAV), particularly myeloperoxidase MPO - ANCA has been described in the last 2 decades. Given the mild clubbing and rapidly progressing UIP, the alternative diagnosis could have been IPF. However, ENT symptoms and joint pains are usually not associated with IPF. Given the initial response to steroids, further immunosuppression with MMF was started. However, a few days later he was hospitalised due to severe dyspnoea and episodes of haemoptysis. The CT showed new GCO changes on the background of UIP changes and no PE. The dilemma was whether it was drug-induced acute exacerbation of interstitial pneumonitis or natural acceleration of UIP. It was unclear whether he had 2 different pathologies. Disease stratification and early identification of patients who are more prone to progress, and thus in need of more aggressive treatment, is important in the field of ILD.Key learning pointsThere is still significant challenge in the diagnosis and management of ILD. It was unclear whether this case was IPF or MPO vasculitis. There are no specific diagnostic markers and there are no consensus clear guidelines on managing ILD. Although considerable progress has been made in understanding ILD, curing and eliminating interstitial lung disease is still a distant goal. A clearer understanding of how the cells fail to adequately repair the lung and key pathogenetic pathways are much needed and require further exploration. Current clinical trials are studying agents that reduce the fibrotic signalling within the lung, reduce pulmonary hypertension associated with interstitial lung disease, and alleviate oxidative stress. Early diagnosis, monitoring and MDT input are essential to improve overall outcomes. This case illustrates the complexity faced by clinicians in the management of ILD patients in the real-life setting and raises a number of points outside of the clinical guidelines.Conflict of interestThe authors declare no conflicts of interest.

Etiology and outcome of hemoptysis in Qatar, a high-resource country with a large expatriate population: A retrospective study.

Background: Hemoptysis is an alarming symptom in clinical practice. We reviewed ten years of experience with hemoptysis in a tertiary hospital in Qatar to identify hemoptysis etiologies, patient characteristics, and associated factors. Methods: Hemoptysis was defined based on severity as mild ( < 50 ml or streaks of blood), moderate (50–150 ml) and massive (>150 ml) in the 24 hours before admission. Hemodynamically unstable is considered when systolic BP < 100 mmHg,tachycardia with HR>110/min, tachypnea with RR>22/min, or SpO2 < 92% on room air. Results: A total of 102 patients (41 females and 61 males) with 133 episodes of hemoptysis were identified in this study. Among the hemoptysis patients with co-morbidities, 19 patients had hypertension, 17 patients had cardiovascular disease, and 66 patients with other co-morbidities. COPD patients had a significant (p < 0.02) association with hemoptysis. Chest X-ray was used in most patients and other modalities like CT scan and bronchoscopies were used less frequently. Pneumonia (12.8%), bronchiectasis (11.8%) and cardiovascular disorders (11.8%) are the primary causes of hemoptysis. Malignancy was less frequent (7.8%), and bronchogenic carcinoma was uncommon (2%). There were 24 (23.5 %) no identified causes of hemoptysis. The overall mortality was 9.8% in this study. Conclusions: Population demographics played a significant role in the severity of hemoptysis and prognosis. Most patients had benign etiologies, lower severity of hemoptysis and good prognosis. Differences in the etiology, initial severity, and prognosis of patients with hemoptysis were found significantly different when compared with those reported in previous studies.

Bronchial arterial embolization using a gelatin sponge for hemoptysis from pulmonary aspergilloma: comparison with other pulmonary diseases.

To evaluate the clinical outcomes of bronchial artery embolization (BAE) using a gelatin sponge for hemoptysis from pulmonary aspergilloma and compare them with treatment outcomes for hemoptysis from other diseases. Fifty-two patients underwent BAE using a gelatin sponge. The etiology of hemoptysis was pulmonary aspergilloma in 8 (PA group) and other diseases in 44 (control group). The technical success rate, clinical success rate, hemoptysis-free rate, and complication rate were compared between the PA group and control group. Technical success was defined as the complete cessation of the targeted feeding artery as confirmed by digital subtraction angiography, and clinical success as the cessation of hemoptysis within 24h of BAE. Recurrent hemoptysis was defined as a single or multiple episodes of hemoptysis causing > 30ml of bleeding per day. Technical and clinical success rates were 100% in both groups. Hemoptysis-free rates were 85% at 6months and 72% at 12-60months in the control group, and 38% at 6-12months and 25% thereafter in the PA group (P = 0.0009). No complications were observed following BAE in any case in the two groups. BAE using a gelatin sponge may not be effective for hemoptysis from pulmonary aspergilloma.

Sleeve lobectomy for an arteriovenous malformation in the bronchus intermedius in a child

Abstract Arteriovenous malformations within the tracheobronchial tree in pediatric patients uncommonly present as hemoptysis, and can usually be treated with percutaneous transcatheter embolization. When this fails, parenchymal-sparing operations are needed. While these have been popularized for the management of bronchial neoplasms in children and adults, such bronchoplastic techniques are not commonly performed in children with tracheobronchial vascular malformations and acute hemoptysis. Here, we present a patient with recurrent hemoptysis who was found to have an arteriovenous malformation in the bronchus intermedius, and had failed numerous attempts at coil embolization. Via a right thoracotomy approach, we successfully performed a right middle lobectomy and sleeve resection of the bronchus intermedius. The post-operative recovery was uneventful, and there were no further episodes of hemoptysis. Thus, by applying the principles of bronchoplastic resection, such parenchymal-preserving surgery can be successfully used to manage symptomatic bronchial arteriovenous malformations with hemoptysis.

Antifibrinolytic Agents for Hemoptysis Management in Adults With Cystic Fibrosis

Hemoptysis is a major cause of morbidity and mortality in patients with cystic fibrosis (CF). Antifibrinolytic agents have shown efficacy in a broad range of bleeding disorders and conditions. The goal of this study was to examine the use of antifibrinolytic agents in managing hemoptysis in CF. We developed a clinical treatment pathway for inpatient and outpatient use, and rates of admission for bleeding prior to and following implementation of the pathway are reported. All adult patients with CF treated with systemic antifibrinolytic agents over a 54-month period according to the treatment pathway were analyzed. Data collected included demographic characteristics, baseline CF-related characteristics, and bleeding and treatment parameters. Effectiveness of the pathway was evaluated via comparison of annualized hemoptysis admission rates prior to and following pathway enrollment. Seventy-two distinct episodes of hemoptysis treated with antifibrinolytic agents were analyzed in a total of 21 adult patients with CF. Two-thirds of episodes treated involved moderate or massive hemoptysis. Bleeding ceased following a median of 2days. Outpatient treatment was associated with a 50%reduction in the annualized hemoptysis admission rate following pathway enrollment (2.44 vs1.23 admissions per year; P= .0024) that was independent of other changes in management. Antifibrinolytic therapy was well tolerated. One central catheter-associated upper extremity DVT was observed in a patient with previous thrombosis in the same vessel. A pathway using systemic antifibrinolytic therapy to treat hemoptysis in patients with CF was associated with a reduction in hospital admissions. No serious adverse events were observed. Additional studies are needed to further define the benefits of systemic antifibrinolytic use in patients with CF.

Idiopathic pulmonary hemosiderosis: A differential diagnosis of pulmonary tuberculosis in a young child

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease diagnosed primarily in children and young adults, characterized by recurrent diffuse alveolar hemorrhage which leads to the deposition of hemosiderin-laden macrophages in the lung. It is a diagnosis of exclusion and requires a high level of clinical suspicion. It may remain undiagnosed for years, hence often mistreated. Here, we describe such a case of IPH in a 15-year-old male who was initially mistreated twice as clinically diagnosed pulmonary tuberculosis first with Category 1 and then Category 2 antitubercular drugs. He had a history of two episodes of hemoptysis, recurrent episodes of paleness, persisting weakness, and shortness of breath. He also had bilateral reticulonodular opacities on chest skiagram. His history raised the suspicion, the presence of hemosiderin-laden macrophages in bronchoalveolar lavage, and transbronchial lung biopsy strongly suggested the diagnosis. However, the final diagnosis came out after ruling out all the probable causes.

Coil embolization of intralobar pulmonary sequestration - an alternative to surgery: a case report

BackgroundPulmonary sequestration is a congenital lung disease characterized by nonfunctioning pulmonary tissue that lacks normal communication with the bronchial tree and is supplied by a nonpulmonary systemic artery. Symptomatic bronchopulmonary sequestration is uncommon, seen more frequently in the pediatric population than in adults. It has traditionally been treated with surgical resection; however, a limited but growing number of cases have been treated with angiographic embolization. Given the inherent risks of cardiothoracic surgery, embolization of the anomalous vessel is an enticing alternative treatment. We present a case of a 56-year-old woman with known, symptomatic, intralobar pulmonary sequestration that was successfully treated with coil embolization.Case presentationA 56-year-old Pacific Islander woman with a history of chronic myeloid leukemia was admitted to the hospital with an episode of hemoptysis. Computed tomography of the chest demonstrated left lower lobe intralobar pulmonary sequestration fed by a large tortuous vessel branching off of the descending thoracic aorta. Surgical resection of the sequestration is the current standard treatment strategy of symptomatic intralobar pulmonary sequestration. The cardiothoracic surgeon noted that given the size and location of arterial blood supply, intervention would involve thoracotomy and lobectomy. The interventional radiologist offered embolization of the lesion as an alternative to surgery. Multiple coils, 6–13 mm in size, were used to embolize the sequestration. No considerable flow distal to the coils was noted postembolization.ConclusionsIntralobar pulmonary sequestration is a rare condition that typically requires surgical management. This case demonstrates the efficacy of coil embolization as an alternative management strategy. To date, limited case reports of adults treated with endovascular embolization exist. Treatment of symptomatic pulmonary sequestration with embolization can be considered as an alternative to surgical resection.

A Rare Case of Congenital Pulmonary Airway Malformation Associated with Intralobar Pulmonary Sequestration in an Adult - Case Report

Abstract The congenital pulmonary airway malformations (CPAM) are extremely rare malformations which are most frequently diagnosed before birth or during childhood and are extremely rarely diagnosed in adults. Sometimes, type II CPAM can be associated with pulmonary sequestrations (PS), especially those extralobar. We present the case of a 39 years old female without any known previous respiratory symptoms until this age, who has having for approximatively 2 months repeated episodes of haemoptysis. The angio-CT exam showed the existence of multiple cyst-like formations in the left inferior pulmonary lobe (LLL) and 2 arteries starting from thoracic aorta that participated at the vascularization of the LLL. We performed a left inferior lobectomy and the postoperative evolution was favorable. The histopathological exam confirmed the diagnosis of type I CPAM associated with intralobar PS. The particularity of the presented case is given by the fact that this patient of 39 years old had no previous respiratory symptoms and no cases of type I CPAM associated with intralobar PS were cited before.

Case report and management approach in idiopathic pulmonary arteries aneurysm

BackgroundIdiopathic pulmonary artery aneurysm is a rare anomaly with only a handful reports in the literature. It is often asymptomatic, while the first presentation could be severe hemoptysis or death.Surgical intervention needs to be planned carefully with a multidisciplinary team approach to secure optimal outcome. We hope to spread our experience with such cases and to encourage other surgeons worldwide to deal surgically with these cases when its indicated.Case presentationA 47 years old man presented to our institution after three episodes of hemoptysis, echo demonstrated good left ventricle (LV) systolic function, normal right ventricle (RV) size and function, Chest computed tomography (CT) revealed aneurysmal dilatation with pending rupture of the pulmonary artery trunk (4.5 cm), the left pulmonary artery (6 cm) and the right pulmonary artery (2.3 cm). The patient successfully underwent replacement of Pulmonary artery trunk, left pulmonary artery and right pulmonary artery by Wovex Prosthetic graft (28 mm). The patient discharged home on the eight postoperative day in good clinical condition .ConclusionWith this case report we wish to emphasize the need for a careful multidisciplinary approach given the complex and rare nature of the reported pathology.

Dysphagia: The Primary Indicator of Esophageal Compression by a Pericardial Effusion

Introduction: Dysphagia is a relatively common symptom seen in late adulthood and can be classified as oropharyngeal or esophageal. Esophageal dysphagia typically presents due to scarring from acid reflux, strictures within the lumen, a tumor causing obstruction or an infection causing inflammation. A rare occurrence of dysphagia is reported here as an outcome of extrinsic compression of the esophagus by a large pericardial effusion. Case: A 61-year-old male with a history of DM, epilepsy, schizophrenia, vertigo and hyperlipidemia presented with an episode of hemoptysis and dysphagia for 3 weeks duration. Although this was the first account of hemoptysis, he admitted to a dry cough for a few months. He noted his dysphagia was predominantly to solids and unless he ate slowly, he would regurgitate. He denied any fever, shortness of breath, chest pain, weight loss or night sweats. On initial examination the patient was an obese male with stable vitals except for a slightly low oxygen saturation of 94%. Inspection of the neck revealed no masses. On auscultation, heart sounds were difficult to appreciate. Labs initially revealed an elevated WBC of 15.3, platelet count of 110, a blood sugar of 222 and the remaining results were within normal limits. Chest X-Ray showed infra-hilar infiltrates suggesting pneumonia and cardiomegaly. Modified barium swallow was negative. An esophagogastroduodenoscopy revealed mild extrinsic compression in the mid-esophagus but no strictures. CT of the chest showed a large pericardial effusion of 3.3 cm compressing the esophagus and chronic atelectasis with small bilateral pleural effusions. An echocardiogram confirmed a worsening pericardial effusion from 1 year previous and a dropping left ventricular ejection fraction (40-45% to 20-25%). Initial option for treatment was a pericardial window, but a cardiac catheterization revealed 2-vessel CAD with left main involvement. The patient ended up having a CABG and having his pericardial effusion drained. Follow up echocardiogram one month later revealed no pericardial effusion and a complete resolve of his dysphagia. Discussion: While there are documented cases of pericardial effusion resulting in compression of the esophagus, it is a rare occurrence. This case emphasizes that while dysphagia is often an esophageal complaint, the origin of the symptom may be non-GI and communication between GI and cardiac specialists is essential.

EARLY SURGICAL INTERVENTION CAN BE NECESSARY: PERSISTENT MASSIVE HEMOPTYSIS DESPITE EMBOLIZATION AND BLOCKADE

SESSION TITLE: Critical Care 5 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Massive hemoptysis is a life-threatening condition due to the potential for asphyxiation or exsanguination. Commonly causes are bronchiectasis, bronchogenic carcinoma and infectious etiologies such as tuberculosis and pneumonia. Management involves localization of the bleeding source via bronchoscopy or computerized tomographic (CT) angiography with subsequent non-surgical or surgical intervention to control the bleeding. We present a case of massive hemoptysis secondary to bronchiectasis, unresponsive to bronchoscopic intervention and bronchial artery embolization (BAE), requiring definitive surgical lobectomy. CASE PRESENTATION: A 67-year-old male was admitted for evaluation of persistent hemoptysis in setting of moderate chronic obstructive pulmonary disease (COPD) and recent percutaneous biopsy of liver mass. Examination revealed rales in right lower lung base. CT of the thorax showed emphysematous changes with multiple blebs and lower-lobe honeycomb fibrotic thickening. Continued episodes of hemoptysis prompted bronchoscopic evaluation. Right lower lobe visualization revealed active bleeding from lateral and posterior segments. Despite epinephrine administration and iced saline lavage, bleeding persisted. Balloon tamponade with an endobronchial blocker was introduced into the right lower lobe and left in place. Interventional radiology consulted for arteriographic embolization. Balloon deflation after 24 hours revealed active persistent bleed. Cardiothoracic surgery was consulted and performed an emergent lobectomy. Gross pathology revealed no pleural disruption to support the notion that liver biopsy might have precipitated hemoptysis. Microscopy revealed reactive pneumocytes and significant airway ectasia. DISCUSSION: Angiography followed by BAE is the preferred management for massive hemoptysis in poor surgical candidates. In COPD both bronchiectatic and cryptogenic hemoptysis responds favorably to BAE. It has been reported as definitive treatment in both cystic and non-cystic fibrosis bronchiectasis. BAE is effective in controlling hemoptysis with success rates ranging from 70-90%. Recurrence rates vary from 10 to 57%.(1) Early recurrences occur secondary to lack of localization and inadequate embolization of all the feeding vessels. Despite high operative risks, our patient was managed with emergent lobectomy due to persistent bleeding. Emergent surgery in massive hemoptysis is associated with mortality rates up to 40%. BAE can be used as a bridge to stabilize patients before surgical treatment with an elective mortality rate 18%. (2) A retrospective analysis comparing the mortality and morbidity cohort of patients who underwent surgery after BAE vs those with early surgical intervention found no difference in outcomes.(3) CONCLUSIONS: Early surgical consultation is warranted for life-threatening massive hemoptysis in the setting of unsuccessful endobronchial blockade and BAE. Reference #1: Panda A, Bhalla AS, Goyal A. Bronchial artery embolization in hemoptysis: a systematic review. Diagn Interv Radiol. 2017;23(4):307-17. Reference #2: Fruchter O, Schneer S, Rusanov V, Belenky A, Kramer MR. Bronchial artery embolization for massive hemoptysis: long-term follow-up. Asian Cardiovasc Thorac Ann. 2015;23(1):55-60. Reference #3: Alexander GR. A retrospective review comparing the treatment outcomes of emergency lung resection for massive hemoptysis with and without preoperative bronchial artery embolization. Eur J Cardiothorac Surg. 2014;45(2):251-5. DISCLOSURES: No relevant relationships by Francis Christian, source=Web Response No relevant relationships by Sardar Ijaz, source=Web Response No relevant relationships by Kellie Jones, source=Web Response No relevant relationships by Erin Shirley, source=Web Response