Onboard aircraft medical emergencies are on the rise as commercial air traffic is increasing. However, thoracic injury secondary to air travel is extremely rare and, most reported injuries are cases of pneumothoraces. Spontaneous pneumomediastinum and pneumopericardium have been barely reported in the medical literature as a complication of air travel. We are reporting a case of spontaneous pneumopericardium and pneumomediastinum in a patient with Gardner's Syndrome after a flight from Central America to New York City. The patient presented with chest discomfort. He was managed conservatively with oxygen therapy as he was hemodynamically stable throughout his stay in the hospital. A thorough work up in hospital including and esophagogram and a CT scan of the chest were none revealing of the cause. However, the patient was noted to have metastatic rectal cancer with lung involvement. The patient was discharged with instructions to avoid air travel.
In this study, few public school patients received epinephrine for anaphylaxis and the vast majority occurred in communities with rapid ALS response. The direct annual supply cost of the school EAI mandate is substantial.
Thoracic endometriosis is very rare. Usually, the thorax is the most frequent affected site outside the pelvis. Common symptoms include chest pain, dyspnea, and hemoptysis. Common manifestations include pneumothorax, hemothorax, and pulmonary or pleural nodules. In addition, symptoms and manifestations can be “catamenial” happening a few days after menstruation onset. This disease can be debilitating, causing a significant impact on the quality of life of young women. We present a case of a young female who was referred to our hospital with recurrent right-sided pleural effusions and pneumothoraces. Pleural fluid drainage was consistent with hemothorax. Transvaginal ultrasound showed mild intraperitoneal fluid in the Cul-de-Sac. Due to concerns for thoracic endometriosis, video-assisted thoracoscopic surgery was performed confirming the diagnosis by pathology. Therapeutic pleurectomy with diaphragmatic repair and pleurodesis was performed. The patient was started on medroxyprogesterone acetate injections two weeks after with great clinical response.
Swyer-James syndrome (SJS) is a rare lung condition characterized by abnormal lung growth secondary to childhood post-infectious bronchiolitis obliterans. Usually, one lung is affected more than the other leading to asymmetrical lungs with one lung being significantly smaller. The disease can lead to pulmonary obstructive airflow physiology, bronchiectasis, and fibrosis. Dyspnea usually presents early on in infancy and symptoms can mimic asthma, however, they can go unnoticed until adulthood. We present a case of SJS in a patient with adult polycystic kidney disease (ADPKD) and color vision deficiency. The patient presented to our clinic for evaluation for progressively worsening dyspnea and cough. His imaging revealed a hypoplastic left lung with fibrosis, cystic airway disease, and a small left pulmonary artery. His spirometry revealed an obstructive defect. A Ventilation-Perfusion scan (V/Q) showed a significant reduction of ventilation and perfusion to his left lung confirming the diagnosis of SJS. Both conditions – SJS and ADPKD-are not pathologically or genetically related and are very rare. Having both conditions is even rarer yielding interesting radiological imaging.
INTRODUCTION:Sarcoidosis is a multi-system granulomatous disorder with characteristic pulmonary manifestations and typical histopathologic features. Treatment of sarcoidosis is typically based on an assessment of the extent, severity and activity of the disease. Corticosteroids are considered the first-line therapy for many decades; however, early initiation of steroid-sparing agents is gaining popularity especially in progressive cases that do not respond well to steroids [1]. We present a case of refractory sarcoidosis requiring multiple cytotoxic and biologic therapies. CASE PRESENTATION:A 56-year-old Caucasian female presented with dyspnea, fever and leukocytosis and was found to have bilateral large masses and mediastinal and hilar adenopathy concerning for infection or malignancy. She did not respond to antibiotic therapy and underwent endobronchial ultrasound with transbronchial needle aspiration (EBUS-TBNA) of the mediastinal lymph nodes twice followed by CT guided biopsy of one of the pulmonary masses, and all showed characteristic noncaseating granulomas with absence of malignancy and infectious agents on multiple stains and cultures. Atypical presentation of pulmonary sarcoidosis was considered especially with bronchoalveolar lavage (BAL) cellular analysis showing 36% lymphocytes and CD4/CD8 ratio of 4.24. Serologic work up showed no connective tissue diseases or vasculitis. The patient was started on corticosteroids but continued to progress with persistent fevers and leukocytosis. Azathioprine was started then changed to methotrexate because of intolerance. Despite adequate doses of methotrexate and prednisone, the patient progressed and became oxygen dependent. Repeat CT scan showed enlargement of the lung masses and development of large cavitations. Repeat EBUS-TBNA and BAL still showed non-caseating granulomas and absence of infectious agents. The patient also had elevated interlukin-2 receptors suggesting increased activity of the disease. Infliximab was started which led to improvement in symptoms and stability of the disease; however, the patient could not tolerate the last infusion because of hypersensitivity reaction. Mycophenolate mofetil was later added, but patient progressed on it which led to the final decision to start patient on rituximab. Her symptoms, lung function and CT findings stabilized on rituximab and she is being evaluated for lung transplant.DISCUSSION: Sarcoidosis diagnosis can be challenging when the presentation and clinical response is atypical. Treatment decision should always be guided by the individual patient's needs and response. Poor response to steroids and cytotoxic agents should prompt reevaluation, investigation for other causes and consideration of biologic agents [2].CONCLUSIONS: Refractory sarcoidosis, where multiple treatments fail, remain a real challenge with a poorly understood pathophysiology.
INTRODUCTION: Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a multi-organ vasculitis. However, the hallmarks of the disease are eosinophilia, chronic sinusitis, and asthma. Vasculitic organ involvement might be absent early on, making the diagnosis challenging1. We present the case of a patient with atypical presentation and serology for EGPA. CASE PRESENTATION:A 71-year-old male with a history of non-asthmatic eosinophilic bronchitis, chronic sinusitis, and peripheral eosinophilia (peak 936 cells/uL; 17.6%) was admitted to the hospital with dyspnea and rapidly progressive acute hypoxemic respiratory failure requiring transfer to the intensive care unit. He was started on high flow nasal cannula (60LPM; FiO2 90%-100%). Bilateral crackles noted with no pitting edema. Chest CT showed bilateral airspace disease and ground glass opacities. He tested negative twice for SARS-CoV-2. White blood cell count, kidney function, and brain natriuretic peptide were normal. Autoimmune workup was ordered. He was started on empiric broad-spectrum antibiotic, and gently diuresed. As his presentation was suggestive of eosinophilic lung disease, and in light of significant oxygen requirement, the decision was made to hold on bronchoscopy and start solumedrol at 125mg/day. On ICU Day 3, autoimmune workup showed elevated anti-nuclear antibody titer of 1:160 with a speckled pattern, elevated anti-myeloperoxidase (MPO) antibody titer of 49.4 (p-ANCA), and elevated anti-proteinase-3 (anti-PR3) antibody titers of 18.8 (c-ANCA). Infectious workup was negative. The patient was diagnosed with possible EGPA. He was started on cyclophosphamide in addition to steroids. Oxygen requirement continued to improve. He was discharged home on oxygen at 4LPM and oral steroid with a plan for monthly cyclophosphamide infusion. Follow-up in 2 months with repeat chest CT showed marked improvement. DISCUSSION:The manifestations of EGPA are dynamic, with a few manifestations presenting earlier than others2. With the temporal spacing of signs and symptoms, it is often diagnosed late when more clues are evident. In 1990, the American College of Rheumatology (ACR) established a set of 6 criteria: asthma, peripheral eosinophilia >10%, mononeuropathy or polyneuropathy, migratory pulmonary infiltrates, paranasal sinus abnormality, and biopsy containing blood vessel with extravascular eosinophils2. At least 4 out of six of these criteria must be met to have a diagnosis of EGPA with a specificity of 99.7%. p-ANCA (anti-MPO) is present in around 70% of patients with active EGPA, whereas c-ANCA (anti-PR3) is nearly absent3. Our patient met 3 out of the 6 criteria, and his serology was atypical, showing high titers for both p-ANCA and c-ANCA.CONCLUSIONS: EGPA diagnosis in our patient was not a robust one. However, establishing a presumed diagnosis early and starting immunosuppressive therapy likely saved his life.
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