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SESSION TITLE: Challenges in Cystic Fibrosis Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 1:45 pm INTRODUCTION: Pulmonary involvement in Systemic Lupus Erythematosus (SLE) is seen in 30-50% of patients (most commonly Nonspecific Interstitial Pneumonitis) but cystic lung disease is extremely rare (1). Lymphoid interstitial pneumonia (LIP) is an inflammatory lung disease that is characterized by infiltration of lymphocytes and plasma cells (2), and associated with lung cysts. Oftentimes, it is associated with HIV, lymphoma, and primary Sjogren's Syndrome (SS) (2), however there are rare reports of LIP associated with SLE (1). We present a case of a young male with incidental lung cysts who was found to have a new diagnosis of SLE. CASE PRESENTATION: A 24-year-old male with a past medical history of premature birth at 5 months and prior mild COVID-19 infection presented with 3 weeks of abdominal pain, nausea, vomiting, fever, and unintentional 15-pound weight loss. He endorsed dry mouth, frequent cavities, and a new rash involving his chest, face, and lower extremities. Physical exam was significant for malar rash and dry mucous membranes. Labs revealed pancytopenia, sedimentation rate 61 mm/hour and C-reactive protein 5.54 mg/L. Computed tomography (CT) of the chest showed several thin-walled cysts in all bilateral lung lobes (predominant in right upper lobe) and bilateral axillary lymph nodes [Figure 1]. CT abdomen and pelvis was unremarkable. Autoimmune work-up resulted in a positive antinuclear antibody >1:1280, double stranded DNA antibody elevated at 34, elevated SSA and SSB antibodies (>8.0 and 1.4 respectively), and decreased Complement 3 (59.5 mg/dl) and 4 (10.1 mg/dl) levels. Peripheral smear, right axillary lymph node and bone marrow biopsies were negative for malignancy. He was started on prednisone and Plaquenil with symptomatic improvement. There is high suspicion of LIP given the clinical and radiological findings. He will follow up in clinic to obtain PFTs and schedule a lung biopsy. DISCUSSION: Interstitial lung disease in SLE presents in middle-aged patients at a later part of their disease course, with a female preponderance (2,3). An initial presentation of SLE and secondary SS in a young male and associated cystic lung disease is rare. The suspicion for LIP in association with SLE is high in our patient given variable size and distribution of lung cysts and coexisting secondary Sjogren's syndrome, although no ground glass or nodular opacities were found on CT chest as reported in typical LIP (3). Though this patient has no pulmonary symptoms, cysts/LIP in SLE tend to progress and have a high incidence of developing lymphomas, gammaglobulinemia and amyloidosis (2,3). CONCLUSIONS: It is important to establish a histopathological diagnosis and obtain baseline PFTs to monitor pulmonary disease manifestations. In addition to controlling the primary disease with antirheumatic drugs, steroids have been found to be useful in acute pulmonary flares (2). Reference #1: Maeda R, Isowa N, Miura H, Tokuyasu H. Systemic lupus erythematosus with multiple lung cysts. Interact Cardiovasc Thorac Surg. 2009 Jun;8(6):701-2. doi: 10.1510/icvts.2008.200055. Epub 2009 Mar 12. PMID: 19282324. Reference #2: Yood RA, Steigman DM, Gill LR. Lymphocytic interstitial pneumonitis in a patient with systemic lupus erythematosus. Lupus. 1995 Apr;4(2):161-3. doi: 10.1177/096120339500400217. PMID: 7795624. Reference #3: Filipek MS, Thompson ME, Wang PL, Gosselin MV, L Primack S. Lymphocytic interstitial pneumonitis in a patient with systemic lupus erythematosus: radiographic and high-resolution CT findings. J Thorac Imaging. 2004 Jul;19(3):200-3. doi: 10.1097/01.rti.0000099464.94973.51. PMID: 15273618. DISCLOSURES: No relevant relationships by Matthew Fain No relevant relationships by Christina Fanous No relevant relationships by Rathnavali Katragadda No relevant relationships by CHRISELYN PALMA
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SESSION TITLE: Late Breaking Chest Infections Posters SESSION TYPE: Original Investigation Posters PRESENTED ON: 10/18/2022 01:30 pm - 02:30 pm PURPOSE: (1) Assess the characteristics of COVID-19 patients who developed pulmonary cysts, bullae, blebs, and pneumatoceles. (2) Investigate outcomes of patients who developed cystic lung disease from COVID-19. METHODS: A literature search using Pubmed, Cochrane, and Embase was performed for case reports from 2020 to 2022 describing COVID-19 patients who developed lung cysts, bullae, blebs and pneumatoceles. The following data were extracted: patient demographics, presence of underlying lung disease, history of smoking, maximum oxygen requirements during acute illness, imaging findings, complications, and patient mortality. RESULTS: 65 publications (11 case series and 54 case reports) with a total sample size of 76 patients were analyzed. The mean age of patients was 52.2 ± 15.8 years. A majority of the cases were males (n=67, 88.2%). Twelve (15.8%) cases had an underlying lung disease, such as COPD or asthma, and 16 (21.1%) cases had a history of smoking tobacco. We categorized severity of illness based on the levels of oxygen requirement defined as: (1) mild - 0 to 2 liters of oxygen, (2) moderate - greater than 2 liters of oxygen to face mask/venturi mask and (3) severe - high flow nasal cannula, non-invasive ventilation, or mechanical ventilation. The majority of patients (n=40, 52.6%) had severe illness while 7 (9.2%) and 17 (22.4%) presented with mild and moderate disease, respectively. Of the 25 (32.9%) patients who required invasive mechanical ventilation, duration of ventilator days was provided for 14 patients, with a median of 40 days (interquartile range=54). Twenty-one (27.6%) patients were found to have cysts on imaging, 26 (34.2%) were found to have bullae, 3 (3.9%) were found to have blebs, 15 (19.7%) were found to have pneumatoceles, and 11 (14.5%) were found to have more than one of the aforementioned findings. A total of 53 (69.7%) patients developed pneumothorax and 12 (15.8%) developed pneumomediastinum. Seventeen (22.4%) patients were on the mechanical ventilator while pulmonary complications occurred. Additionally, 41 (53.9%) required chest tube placement, 16 (21.1%) required surgical intervention including open thoracotomy or video assisted thoracoscopy. A total of 47 (61.8%) cases reported either resolution of symptoms and complications, or improved imaging findings following interventions. The rate of inpatient mortality was 11.8%. CONCLUSIONS: Patients with severe COVID-19 may have a higher risk for developing cystic lung disease, hence, increasing the risk for complications such as pneumothorax and pneumomediastinum. CLINICAL IMPLICATIONS: Patients who had severe COVID-19 may benefit from closer follow up and serial imaging for early detection of cystic lung disease. DISCLOSURES: No relevant relationships by Kavita Batra No relevant relationships by Rajany Dy No relevant relationships by Christina Fanous No relevant relationships by Wilbur Ji No relevant relationships by Max Nguyen No relevant relationships by Omar Sanyurah
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SESSION TITLE: Post-COVID-19 Infection Complications SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: COVID-19 has affected over 200 million people worldwide. Clinicians continue to observe unusual manifestations of this disease. In an attempt to improve our understanding of COVID-19 pneumonia, we present the details of one patient who developed large bilateral pulmonary cysts. CASE PRESENTATION: A 40-year-old woman with no known medical problems presented with the chief complaint of fever, nausea, vomiting, generalized weakness followed by difficulty breathing that developed over a few days. Her vital signs on admission included temperature 98.4° F, heart rate 104 beats/minute, respiratory rate 48 breaths/minute, O2 saturation 88 percent on 15 liters of oxygen through a non-rebreather mask, and Body Mass Index 42 kg/m2. The patient tested positive for COVID-19. Computed tomography (CT) of the chest to rule out a pulmonary embolism showed bilateral extensive ground-glass opacities and reticular and nodular opacities. She was intubated for acute hypoxic respiratory failure. Twenty days into the hospital admission, she was noted to have a bulla in the right lower lobe. A repeat CT chest on day 45 revealed an increase in the number and size of cysts bilaterally. Patient was discharged to rehab and later readmitted for worsening respiratory status. This time she tested positive for human metapneumovirus. A CT chest showed increase in the size of the right sided lung cysts;the left sided lung cysts had resolved. DISCUSSION: The first COVID-19 related pulmonary cystic lesions were reported in May 2020(1). Since then, several reports have now established a relationship between an infection and cyst formation. The most common distribution is peripheral in the lower lobes. The pathogenesis remains uncertain, but several mechanisms have been proposed. Microthrombi in the pulmonary circulation could lead to ischemia and subsequent remodeling of interstitial matrix and bronchial obstruction with distal hyperinflation due to check valve mechanism. (1,2). Hamad et al. propose that pneumatoceles are formed by air leaked in to the interstitium which causes stripping and separation of a thin layer of lung parenchyma with further injury to the small blood vessels and bronchioles. The rate of barotrauma in non-COVID-19 related ARDS is 0.5%;the rate in COVID-19 ARDS is 15% (3). This suggests a close relation between COVID-19 pneumonia and subsequent development of pulmonary cysts. Our patient had no preexisting pulmonary disease and was noted to have pulmonary cysts after being on mechanical ventilation for almost 2 weeks. The patient later contracted the human metapneumovirus infection and CT showed that the right-sided lung cysts had become bigger in size. However, the left-sided cysts which had a maximum diameter of 4.8 cm had resolved. CONCLUSIONS: We need to follow patients with COVID 19 induced lung cysts clinically and radiologically to understand the clinical course and best management strategies. Reference #1: Kefu Liu et al. COVID 19 with cystic features on Computed tomography;Medicine (Baltimore) 2020May;99(18): e20175. PMCID: PMC7486878 Reference #2: Galindo J, Jimenez L, Lutz J et al. Spontaneous pneumothorax with or without pulmonary cysts, in patients with COVID 19 Pneumonia. Journal of infections in developing countries 2021;15(10);1404-1407 Reference #3: McGuinness G, Zhan C, Rosenberg N, Azour L, Wickstrom M, Mason DM, Thomas KM, Moore WH. Increased incidence of barotrauma in patients with COVID-19 on invasive mechanical ventilation. Radiology. 2020;297(2): E252–E262. doi: 10.1148/radiol.2020202352 DISCLOSURES: No relevant relationships by Arunee Motes No relevant relationships by Kenneth Nugent No relevant relationships by Tushi Singh No relevant relationships by Myrian Vinan Vega
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CASE: A 52-year-old male with a past medical history of asthma and uncontrolled OSA presented to the ED ten days after diagnosis of COVID-19 with worsening dyspnea. He had a history of fluticasone propionate and fluticasone salmeterol use for asthma exacerbations. He endorsed cough, fever, chills, and diarrhea, and denied chest pain, leg edema, and anosmia.Vitals showed oxygen saturation of 65%. CBC demonstrated leukopenia consistent with COVID- 19 infection. Blood labs showed hyperglycemia (blood sugar 182 mg/dL, hemoglobin A1c 9.6%). Bilateral crackles were noted on exam. He was placed on high-flow nasal cannula (HFNC) immediately due to critical hypoxemia. CT PE was negative;CXR revealed bilateral opacities consistent with COVID-19 pneumonia. He started on dexamethasone and remdesivir and was admitted to the MICU for acute hypoxemic respiratory failure. Notably, the patient had no known diagnosis of diabetes mellitus and was started on sliding scale insulin and Lantus. Barcitinib was added in the MICU in addition to linezolid and cefepime for fear of bacterial superinfection but were discontinued after receiving negative cultures. He was transferred out of the MICU four days later after successful weaning of oxygen but soon returned due to worsening oxygen needs. New leukocytosis prompted a repeat respiratory culture, which grew mold on the preliminary read. Voriconazole was initiated due to concern for Aspergillus infection and was continued with confirmation on the final read. Repeat CT showed left pneumomediastinum, right apical pneumothorax, and worsening bilateral opacities. Despite ongoing treatment, the patient required NC at rest and HFNC with minimal exertion. He was discharged home with HFNC. IMPACT/DISCUSSION: CAPA is a result of opportunistic fungal infection, causing devastating disease in the immunocompromised. A crucial risk factor is the use of high-dose corticosteroids for a prolonged period. The diagnosis of CAPA is based on a combination of imaging, microbiology, and clinical presentation. Peripheral nodules, air crescent, reverse halo sign, nodular consolidation, ground-glass opacities, crazy paving pattern, pleural effusion, and pulmonary cysts have been reported among CAPA patients. A fungal culture and galactomannan test from respiratory specimens can aid in early diagnosis. The usual presenting symptoms of CAPA include refractory fever, pleuritic chest pain, or dyspnea. Voriconazole is a first-line anti-Aspergillus agent. CONCLUSION: Clinical presentation of CAPA is often subtle but associated with high morbidity and mortality. Multiple reports add support to our observation that CAPA can be a result of worsening COVID-19 pneumonia. Early diagnosis and treatment are vital to prevent worse clinical outcomes. Physicians should demonstrate a heightened awareness of the risk of developing CAPA in critically ill COVID-19 patients. Clinicians should exercise low thresholds to identify and treat CAPA, especially in patients on high-dose steroids long-term.
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Computed Tomography has played a vital role in Coronavirus Disease 2019 (COVID-19) infection, caused by Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) over the last two years. The typical features of COVID-19 on High Resolution Computed Tomography (HRCT) of chest including ground glass opacities and consolidation with a peripheral and lower lobar predilection have been very well documented in literature worldwide. However, thin-walled lucencies in the lung parenchyma called cysts is not very well documented. Authors thus present a case series comprising six SARS-CoV-2 Reverse Transcription-Polymerase Chain Reaction (RT-PCR) positive patients admitted to the hospital during the period 1stApril 2021 to 31stMay 2021 with lung cysts on HRCT. It was a retrospective study wherein details of the patients were drawn from the case record sheets and the clinical parameters along with HRCT chest findings were analysed, and correlations were drawn to study the cause, timing and significance of these cysts. In this study, the cysts were found to be thin-walled, varying in size from 5-20 mm in diameter and subpleural in distribution with no obvious lobar predilection.The immediately surrounding lung parenchyma showed features of maximal involvement by the atypical pneumonitis. All six cases had moderate to severe lung involvement entailing oxygen therapy. The high flow oxygen therapy and its duration along with degree of lung involvement, are important determinants of cystic degeneration. In the present case series, cystic changes were observed somewhere between day 15 to day 40 of the disease and thus a part of postacute fibrosis in COVID-19 infection.
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Rationale: Barotrauma during mechanical ventilation more frequently occurs in COVID-19 patients than other diseases. Some previous studies reported that barotrauma was a risk factor for death;however, immortal bias due to its time-dependent nature was not considered in these studies. Therefore, we surveyed each prognosis of barotrauma whose onset was divided by time.Materials and Methods: We retrospectively reviewed intubated COVID-19 patients from March in 2020 to May in 2021. We compared 90-day survival prognosis of the barotrauma group (pneumothorax and pneumomediastinum/subcutaneous emphysema) with that of the non-barotrauma group. We also performed landmark survival analysis at day 7, 14, 21, and 28. Barotrauma occurred within 7 days on and before the landmark was considered as the exposure. The patients who were extubated before the exposure period were excluded. Additionally, we assessed the clinical data of surgically treated pneumothorax secondary to COVID-19 in our institution.Results: A total of 192 patients were included. Barotrauma occurred in 44 patients (22.9 %) (only pneumomediastinum/subcutaneous emphysema: 27 cases, only pneumothorax: 11 cases, both of them: 6 cases). The 90-day survival rate of the barotrauma group was significantly worse compared to the non-barotrauma group (47.7 % vs 82.4 %, p < 0.001). In the 7-day landmark analysis, there was no significant difference in the 90-day survival rate from the landmark between the barotrauma group and the non-barotrauma group (75.0 % vs 75.7 %, p = 0.79). On the other hand, in the 14, 21, and 28-day landmark analysis, the survival rate of the barotrauma group was significantly worse than that of the non-barotrauma group (at day 14, 41.7 % vs 69.1 %, p = 0.044;at day 21, 16.7 % vs 62.5 %, p = 0.014;at day 28, 20.0 % vs 66.7 %, p = 0.018).Pathological specimens of surgical cases showed subpleural hematoma and pulmonary cyst in the background of heterogenous lung inflammation.Conclusions: Barotrauma was a poor prognostic factor in ventilated COVID-19 patients. The impact of barotrauma after 7 days from intubation could be more important than within 7 days. Barotrauma might be an important clinical sign of lung destruction by COVID-19.
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Introduction: In 1977 Birt, Hogg and Dube described Birt-Hogg-Dubé syndrome (BHDS)as an dermatological syndrome.It is an autosomal dominant inherited disease, the gene responsible for the syndrome was cloned in 2002. Only 663 affected families have been reported in literature, To date, 152 unique pathogenic FLCN gene mutations in 616 families have been reported in worldwide;approximately 90% of these mutations were reported in Europe and the United States. It consists of the typical triad - skin lesions, pulmonary cysts, and renal tumors. This syndrome usually goes underdiagnosed since all these different clinical features treated by different specialities. This leads to longdelay in making the correct diagnosis from the first onset of the symptoms. There are many studies which have shown that the prevalence spontaneous pneumothorax ,bullous emphysema, thin-walled cysts are increased in these patients. Case report: A 39 year old male presented with complaints of mild fever for 5 days duration and shortness of breath for 1 day. Fever was continuous in nature, not associated with chillls and rigor. He denied history of diabetes mellitus, hypertension, tuberculosis , bronchial asthma .He was evaluated for covid 19. RT PCR for covid 19 came positive. HRCT chest was done. HRCT showed bilateral peripheral and peribronchovascular non lobar distribution of ground glass opacities with septal thickening in bilateral lung parenchyma. The CT severity score was 14/25. HRCT also showed multiple thin walled cyst in both lungs, largest measuring 22 X 27mm. Few subcentrimetric pre/paratracheal , prevascular and sub carinal lymph nodes was noted.His oxygen saturation was 89% on room air. He was admitted and started on oxygen inhalation via face mask and was managed medically. Relevant blood investigation and bio markers for COVID 19 were done. Reports were total count 3570, differential count N51L35M11E1.8B0.6., Hb 15.4 G/dl, platelet count of 2.14 lakhs/mm3, ESR 16 mm/hr, d dimer 0.77mg/l, LDH 203 U/L, CRP 21.774mg/l, procalcitonin 0.122ng/ml. 3 days later dyspnea aggravated and he was started on non invasive ventillation ( BiPAP). His clinical condition was stable on NIV. On the fifth day of NIV, he started developing subcutaneous surgical emphysema. HRCT chest was done, showed gross left and mild right pneumothorax, moderate pneumomediastinum, multiple focal patchy and confluent ill defined areas of ground glass and reticular densities predominant in basal and peripheral distribution involving bilateral lung parenchyma, diffuse subcutaneous emphysema extending into neck spaces.CT severity had increased to 17/25. He was managed by Intercostal chest tube insertion on left 5th intercostal space in mid axillary line connected to a underwater seal. He improved clinically with subcutaneous emphysema resolving in the next 3 days. He was later discharged and his chest tube was removed on followup. We conclude that Birt-Hogg-Dubé syndrome can be a rare cause of secondary pneumothorax in a patient with multiple skin follicles and having multiple cyst in HRCT.
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Background: Schwannomaare benign tumors arising from Schwann cells of nerve root sheaths. Schwannomasare mostly solid / heterogeneous solid tumours, rarely cystic. Presentation of cystic schwannomainthorax is extremely rare. Case Study: A 62-year-old female came to our hospital withthe complaint of right sided chest pain for 2 months, which is dull, non-radiating. She had history of fever 4 months back, diagnosed to have covid and took treatment at home. Chest x-ray showed a homogenous opacity in the right upper lobe extending up to 1stintercostal space. Ct reported well-defined thick-walled cyst, hypodensewith central low attenuation, forming acute angles with lung parenchyma in apical segments of right upper lobe towards mediastinum. Mild perilesional atelectasis is seen.On contrast, cyst is non enhancing. Patient is evaluated for the possibilities of Foregut duplication cyst, Hydatidcyst, Neoplasm. Endoscopic ultrasonography revealed a 5cms×5cms cyst, which is not communicating, infiltrating to esophagus. Patient was referred to ctvswhere excision of cyst is done by right thoracotomy .A 6cms×6cms×2cms unilocularcyst with wall thickness ranging from 0.1- 0.4cms. Its outer surface is congested, inner surface showed dark-brown hemorrhagic contents. Histopathological examination revealed cystic schwannoma. Discussion: Schwannomasare mostly benign in nature .Treatment includes excision of Bronchogenic cyst, Brachial cyst, neurogenic tumours, apical lung tumour, hydatid cyst, foregut duplication cyst are to be considered as differential diagnosis. It is important to consider cystic schwannomasin the differential diagnosis of thoracic cystsince the best surgical outcome is strongly related to earlier diagnosis and total resection of the lesion.
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Background: Lung transplantation has been performed as a life-saving treatment in a small number of post-acute COVID- 19 patients who develop severe pulmonary insufficiency. We report a detailed clinicopathologic analysis of 7 such patients that underwent bilateral orthotopic lung transplantation at our institution. Design: The time interval between initial diagnosis of Covid-19 infection and lung transplantation, other clinical findings, and imaging features of the 7 patients were reviewed. The pathologic findings in the 14 explants were assessed and histologic abnormalities were classified into parenchymal, airway and vascular changes. The extent (1+-to 4+ based on # of slides with the abnormality) and severity (mild, moderate, marked) of histologic abnormalities were recorded. Results: Patients ranged in age from 34 to 55 years old and were transplanted at 10.4 to 24 weeks after initial diagnosis of Covid- 19 (median 16 weeks). Six of these patients had been previously treated with ECMO for 82-145 days. Other clinical and imaging features are summarized in Table 1. All 14 explants showed diffuse marked interstitial fibrosis with a nonspecific interstitial pneumonia (NSIP) pattern. Other pathologic changes included extensive alveolar hemosiderosis (n=7 patients);prominent peribronchial metaplasia (n=7);focal multinucleated giant cells in either the parenchyma, vascular wall or peribronchiolar location (n=6);focal thrombosis involving medium to large vessels (n=4) and lung cysts associated with delicate calcifications and multinucleated giant cells (n=1). No diffuse alveolar damage changes (DAD) or vasculitis were identified. Conclusions: Lung explants from patients with post-acute COVID-19 syndrome show severe NSIP pattern of fibrosis and other pathologic changes that do not resemble the extensive fibrotic changes resulting from organizing phase of DAD or the extensive vascular changes seen in patients dying during the acute or subacute phases of COVID-19 infection. Further studies with lung biopsies are needed to understand the mechanism of fibrosis in post-acute COVID-19 syndrome and identify individuals who are likely to develop severe pulmonary fibrosis requiring lung transplantation.
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Background: Cavities and cystic lesions on chest CT, a rare presentation in COVID-19, can be caused by various causes including COVID-19 infection itself or secondary to other concurrent illnesses. Case Study: A 37-year-old female with no comorbidities presented with complaints of fever and breathlessness for 1 week duration and tested RT-PCR positive for COVID-19, with bilateral peripheral ground-glass opacities with basal predominance in CT thorax. Repeat CT thorax after one month showed multiple cysts in bilateral lung fields with air-fluid levels. The patient was managed conservatively. Subsequently performed cross-sectional imaging of the chest shows a significant reduction in the size and thickness of the cysts with near complete resolution at 6 months. Discussion: Cysts may complicate the course of COVID-19 disease. The alveolar epithelial cell damage caused by SARS-CoV2 together with the associated cytokine storm makes the alveoli prone to rupture with subsequent air leakage and formation of cystic lesions, the majority of which may resolve spontaneously over time. Conclusion: This case emphasises the necessity of recognising cyst formation in COVID-19 pneumonitis and understanding that patient worsening may be caused by pneumothorax or pneumomediastinum.