Pulmonary Artery Dimensions as a Prognosticator of Transplant-Free Survival in Scleroderma Interstitial Lung Disease.

BACKGROUND: Systemic sclerosis is a chronic debilitating autoimmune disease characterized by endothelial dysfunction and multi-organ fibrosis. Interstitial lung disease, a common manifestation of SSc, is termed scleroderma-related interstitial lung disease (SSc-ILD) and along with pulmonary hypertension contributes to a majority of deaths in SSc. SSc-ILD patients frequently develop pulmonary hypertension, which prognosticates a poorer outcome. We investigated pulmonary artery dimensions as an outcome predictor in patients with SSc-ILD. METHODS: A retrospective chart review abstracting data from SSc-ILD patients evaluated at a large tertiary care center was performed. HRCT imaging was reviewed and pulmonary artery (PA) and ascending aorta (Ao) diameters were measured for calculation of the PA:Ao ratio. Additionally, demographics, vital signs, spirometric parameters, comorbidities, and mean pulmonary artery pressures were collected when available. Outcome analysis with lung transplant or death events within 4 years based on pulmonary artery size as well as PA:Ao ratio was performed. RESULTS: 70 SSc-ILD patients were identified. Mean pulmonary artery diameter and PA:Ao ratio was 31.17 and 1.07 mm, respectively. Patients with a pulmonary artery diameter ≥32 mm had higher risk of lung transplantation or death (p < 0.001) within 4 years. Patients with a PA:Ao ratio ≥1.1 also had higher risk of lung transplantation or death (p < 0.001) within 4 years. Unadjusted outcomes analyses also identified PA:Ao ratio ≥1.1 as an independent outcome predictor (hazard ratio 3.30, p < 0.001). CONCLUSIONS/CLINICAL IMPLICATIONS: In SSc-ILD patients, a PA:Ao ratio ≥1.1 is associated with higher risk of lung transplant or death. These data suggest that PA:Ao dimension may be used for prognostication in SSc-ILD.

Assessing the Safety and Clinical Impact of Thoracoscopic Lung Biopsy in Patients with Interstitial Lung Disease.

INTRODUCTION: The clinical relevance of surgical lung biopsy in Interstitial Lung Disease (ILD) is supported in the literature. Yet most reports reflect institutional or personal bias. AIM: To evaluate the validity of radiologic diagnosis and clinical impact of lung biopsy to help clarify which patient benefit most from biopsy. MATERIALS AND METHODS: We performed a retrospective analysis of a prospectively managed database. All patients who had a surgical lung biopsy for ILD within a period of four year (2009 to 2013) were included. Data included patient demographics, peri-operative variables and outcomes. Preoperative Computed Tomography (CT) imaging was reviewed by a thoracic radiologist blinded to the original report and pathologic information. RESULTS: A total of 47 patients were included. Lung tissue was obtained via a thoracoscopic approach in all but two that had mini-thoracotomy. Mean operating time was 51.1 minutes (18-123), median hospital stay was two days (1-18). Most (87.2%) of the patients were discharged within 72 hours. Thirty day mortality for elective surgery was 4.5% (2/44). Post-operative complications occurred in about one third of the patients. Complications in elective procedures included pneumothorax (10.4%), re-intubation (5.4%) and prolonged intubation (2.7%). Full concordance of radiographic diagnosis with the final diagnosis was significantly higher when reviewed by a cardiothoracic radiologist (60.5% vs. 21.3%). The preoperative clinical diagnosis was fully concordant with the final diagnosis in only 28.2% of cases. In 13.0% of patients the preoperative diagnosis was incorrect. Malignancy was the final diagnosis in two (4.3%) patients. In 51.1% of the patients, results of the biopsy did alter therapy. CONCLUSION: Diagnosis of specific ILD by a cardiothoracic radiologist is more specific and accurate and will probably lead to more appropriate therapy. Elective thoracoscopic surgical lung biopsy is a safe procedure, leads to a more accurate diagnosis of ILD and impacts therapy.

Biphasic Malignant Pleural Mesothelioma Masquerading as a Primary Skeletal Tumor.

Biphasic malignant pleural mesothelioma is a rare malignant tumor, usually presenting as a pleural-based mass in a patient with history of chronic asbestos exposure. We herein report a case of a 41-year-old man who presented with chest pain and had a chest computed tomography (CT) scan suggestive of a primary skeletal tumor originating from the ribs (chondrosarcoma or osteosarcoma), with no history of asbestos exposure. CT-guided core needle biopsies were diagnosed as malignant sarcomatoid mesothelioma. Surgical resection and chest wall reconstruction were performed, confirming the diagnosis and revealing a secondary histologic component (epithelioid), supporting the diagnosis of biphasic malignant mesothelioma.

A Rare Case of Streptococcus alactolyticus Infective Endocarditis Complicated by Septic Emboli and Mycotic Left Middle Cerebral Artery Aneurysm.

To date, S. alactolyticus endocarditis complicated by middle cerebral artery aneurysm has not been reported. We describe the case of a 65-year-old female with a history of hypertrophic cardiomyopathy with left ventricular outflow tract obstruction presenting with confusion and a apical holosystolic murmur. Angiography of the brain identified new bilobed left middle cerebral artery aneurysm. Serial blood cultures grew S. alactolyticus, and aortic and mitral valve vegetation were discovered on transesophageal echocardiography. The patient was treated with antimicrobial therapy, mitral and aortic valve replacements, and microsurgical clipping of cerebral aneurysm. This case serves to highlight the pathogenicity of a sparsely described bacterium belonging to the heterogenous S. bovis complex.

Pneumocystis Pneumonia Presenting as an Enlarging Solitary Pulmonary Nodule.

Pneumocystis pneumonia is a life threatening infection that usually presents with diffuse bilateral ground-glass infiltrates in immunocompromised patients. We report a case of a single nodular granulomatous Pneumocystis pneumonia in a male with diffuse large B-cell lymphoma after R-CHOP therapy. He presented with symptoms of productive cough, dyspnea, and right-sided pleuritic chest pain that failed to resolve despite treatment with multiple antibiotics. Chest X-ray revealed right lower lobe atelectasis and CT of chest showed development of 2 cm nodular opacity with ground-glass opacities. Patient underwent bronchoscopy and biopsy that revealed granulomatous inflammation in a background of organizing pneumonia pattern with negative cultures. Respiratory symptoms resolved but the solitary nodular opacity increased in size prompting a surgical wedge resection which revealed granulomatous Pneumocystis pneumonia infection. This case is the third documented report of Pneumocystis pneumonia infection within a solitary pulmonary nodule in an individual with hematologic neoplasm. Although Pneumocystis pneumonia most commonly occurs in patients with HIV/acquired immunodeficiency syndrome and with diffuse infiltrates, the diagnosis should not be overlooked when only a solitary nodule is present.

Pulmonary Artery Invasion Caused by Mycobacterium tuberculosis.

Extrapulmonary tuberculosis refers to Mycobacterium tuberculosis involving organs other than the lungs (eg, pleura, lymph nodes, genitourinary tract, abdomen, skin, joints and bones, or meninges). In non-HIV-endemic areas, where reactivation is the predominant mechanism of tuberculosis, pleural involvement occurs in 4% of cases. We present an extremely rare case of a 62-year-old immunocompetent patient with pleural tuberculosis confirmed by surgical pleural biopsies, who presented with a large mediastinal mass and evidence of pulmonary artery invasion on CT scanning and endobronchial ultrasonography imaging, highlighting a unique and malignant-like character of the disease.

TASER(®) Electronic Control Device-Induced Rhabdomyolysis and Renal Failure: A Case Report.

Many law enforcement agencies around the United States are employing the use of TASER(®) electronic control devices (TASER(®) International Inc.) to subdue combative suspects. Since its inception the TASER(®) has had a temporal association with reports of rhabdomyolysis. Case reports have reported TASER(®) induced rhabdomyolysis as mild but serious cases have also been reported. Herein we present the case of a single patient who was admitted to our health network with severe rhabdomyolysis after receiving TASER(®) shocks and review the pertinent literature. No direct link has been established between clinically significant rhabdomyolysis and TASER(®) device application but this case serves as an example of a sparsely documented but serious complication that may occur in patients who are at risk for restraint by an electronic control device.

The Rapid Initiation, Titration, and Transition from Intravenous to Oral Treprostinil in a Patient with Severe Pulmonary Arterial Hypertension.

In patients who require urgent initiation of pulmonary arterial hypertension medications due to disease progression, it is customary to start intravenous prostacyclin therapy, typically during a hospital admission. If there are complicating factors or relative contraindications to intravenous and subcutaneous prostanoids, oral treprostinil provides another pathway to prostanoid therapy, but this usually requires a prolonged titration. We describe the case of a thirty-six-year-old male with severe pulmonary arterial hypertension and contraindication to intravenous and subcutaneous prostanoid therapy due to congenital deafness and the risk of not hearing the intravenous pump alarms. Intravenous treprostinil was initiated, titrated to high dose, and then rapidly transitioned to oral treprostinil. A rapid initiation, titration, and transition from intravenous to oral treprostinil can be safely performed under watchful supervision in order to achieve higher and more efficacious doses of oral treprostinil in a timely manner.

Combined Intrathoracic and Subcutaneous Splenosis Discovered 51 Years after Abdominal Trauma.

Splenosis is a rare condition that results from the autotransplantation of splenic parenchyma into unexpected locations such as the abdomen or subcutaneous tissue. In the presence of coexisting injury to the diaphragm intrathoracic transplantation can occur emerging as single or multiple pleural-based masses. This occurs after traumatic rupture of the spleen and is usually asymptomatic, only to be discovered incidentally on routine thoracic or abdominal imaging. To our knowledge this is the third documented case of combined intrathoracic and subcutaneous splenosis found in English literature. This occurred in a 71-year-old male involved in a motor vehicle accident at age 19 requiring urgent splenectomy. He has a significant cigarette smoking history and was referred to our hospital for further evaluation of an abnormality seen on shoulder X-ray.

Pulmonary Sequestration: A 29 Patient Case Series and Review.

INTRODUCTION: Pulmonary sequestration also known as bronchopulmonary sequestration is a rare disease, with very few case series reviewed in literature. In this study, we review the demographics, presentation, imaging and treatment of pulmonary sequestration in 29 patients from our institution, and provide comparison data from previously published series with an overview of the disease history. MATERIALS AND METHODS: Records reviewed for all patients evaluated and treated in our institution with a pathological proven diagnosis of pulmonary sequestration from January 2004 through December 2013. Collected data included demographics, clinical presentation, diagnostic imaging, location of the lesion, type of sequestration, and subsequent treatment. RESULTS: Of the 29 patients reviewed 8 (28%) were children 0-2 years, 1 adolescent age 17, and 20 (69%) adults 21-70 years with a mean age of 42 among adults. Systemic arterial supply to the sequestered segment was demonstrated with computed tomographic angiography (CTA) in 25 patients (86%). In 19 patients (66%), the sequestered segment was located in the left lower lobe, and 16 (55%) were intralobar. CONCLUSION: Diagnostic delays of pulmonary sequestration were common among the adult population as the presenting symptoms often mimicked other common pulmonary diseases, such as pneumonia and asthma. These findings were consistent among previously published series. CTA was the preferred imaging modality for preoperative planning with high sensitivity and specificity in identifying the lesion.