An unexpected diagnosis of a 57-year-old women with migratory pulmonary infiltrates.

A 57-year-old female presented with sudden shortness of breath and migratory pulmonary infiltrates on imaging, which corresponds with a diagnosis of cryptogenic organizing pneumonia. Initial treatment with corticosteroids showed only mild improvement during follow-up. BAL was performed and revealed diffuse alveolar hemorrhage. Immune testing showed positive P-ANCA with positive MPO, leading to a diagnosis of microscopic polyangiitis.

Imaging aspects of interstitial lung disease in patients with rheumatoid arthritis: Literature review.

OBJECTIVE: Interstitial lung disease (ILD) is a frequent and severe complication of rheumatoid arthritis (RA), resulting in pulmonary fibrosis (PF) and respiratory failure. METHODS: Chest computed tomography (CT-c) or high resolution CT (HRCT) is the main modality for assessment of ILD. We performed a systematic literature review on CT-c/HRCT findings in patients with ILD-RA, using the MEDLINE database for the period from 1991 to 2015. RESULTS: Findings on CT-c/HRCT attributed to ILD-RA are variable (ground glass opacities, reticular and nodular pattern, as well as a combined pattern of emphysema and PF). Correlation of CT-c/HRCT findings with clinical data is inconsistent. CONCLUSIONS: ILD-RA is part of a general autoimmune inflammation and should be integrated into the decision-making process for the treatment of RA. There is an unmet need to design an algorithm which will allow prediction of CT-c changes compatible with ILD-RA with a high probability. Hopefully, this will enable treating patients with ILD-RA early, with possible halting of the progression of ILD-RA toward PF.

Optimization of Endotracheal Tube Cuff Pressure by Monitoring CO2 Levels in the Subglottic Space in Mechanically Ventilated Patients: A Randomized Controlled Trial.

BACKGROUND: Many of the complications of mechanical ventilation are related to inappropriate endotracheal tube (ETT) cuff pressure. The aim of the current study was to evaluate the effectiveness of automatic cuff pressure closed-loop control in patients under prolonged intubation, where presence of carbon dioxide (CO2) in the subglottic space is used as an indicator for leaks. The primary outcome of the study is leakage around the cuff quantified using the area under the curve (AUC) of CO2 leakage over time. METHODS: This was a multicenter, prospective, randomized controlled, noninferiority trial including intensive care unit patients. All patients were intubated with the AnapnoGuard ETT, which has an extra lumen used to monitor CO2 levels in the subglottic space.The study group was connected to the AnapnoGuard system operating with cuff control adjusted automatically based on subglottic CO2 (automatic group). The control group was connected to the AnapnoGuard system, while cuff pressure was managed manually using a manometer 3 times/d (manual group). The system recorded around cuff CO2 leakage in both groups. RESULTS: Seventy-two patients were recruited and 64 included in the final analysis. The mean hourly around cuff CO2 leak (mm Hg AUC/h) was 0.22 ± 0.32 in the manual group and 0.09 ± 0.04 in the automatic group (P = .01) where the lower bound of the 1-sided 95% confidence interval was 0.05, demonstrating noninferiority (>-0.033). Additionally, the 2-sided 95% confidence interval was 0.010 to 0.196, showing superiority (>0.0) as well. Significant CO2 leakage (CO2 >2 mm Hg) was 0.027 ± 0.057 (mm Hg AUC/h) in the automatic group versus 0.296 ± 0.784 (mm Hg AUC/h) in the manual group (P = .025). In addition, cuff pressures were in the predefined safety range 97.6% of the time in the automatic group compared to 48.2% in the automatic group (P < .001). CONCLUSIONS: This study shows that the automatic cuff pressure group is not only noninferior but also superior compared to the manual cuff pressure group. Thus, the use of automatic cuff pressure control based on subglottic measurements of CO2 levels is an effective method for ETT cuff pressure optimization. The method is safe and can be easily utilized with any intubated patient.

FDG PET/CT for assessing the resectability of NSCLC patients with N2 disease after neoadjuvant therapy.

OBJECTIVE: Neoadjuvant chemoradiotherapy (CMRT) is the most effective treatment of stage III non-small-cell lung cancer (NSCLC). The present study aimed at assessing FDG PET/CT for defining the response of N2 disease to neoadjuvant CMRT, as surgical resection after such therapy significantly improves 5-year survival in responding N2 disease. METHODS: Forty-five patients with locally advanced NSCLC underwent both pre-neoadjuvant therapy FDG PET/CT and post-neoadjuvant therapy FDG PET/CT followed by anatomical resection of lung and ipsilateral mediastinal lymph nodes (LN). Seventeen of these patients who had PET/CT studies in our institution and were operated after CMRT were retrospectively included in the study group (12 males, ages 43-78 years; stage IIIA: 14 patients, stage IIIB: 3 patients). PET/CT response in N2 was visually scored per-lymph node station and per patient. Quantitative N2 response was evaluated by SUVmax and total lesion glycolysis (TLG) measurements after therapy alone and in comparison with pre-therapy values. PET/CT N2 response was confirmed at surgery. RESULTS: Seventeen NSCLC patients with 29 metastatic N2 lymph nodes (LN) were assessed. Histopathology confirmed 14 responders and 3 non-responders, and was available in 20/29 metastatic LN, showing complete response in 17 and residual disease in 3 LN. LN-based visual analysis of N2 response on PET/CT defined 3 TP, 16 TN and 1 FP, for sensitivity, specificity, accuracy, negative and positive predictive values (NPV and PPV) of 100, 94, 95, 100 and 75%, respectively. Patient-based visual analysis defined 3 TP, 13 TN and 1 FP study, for sensitivity, specificity, accuracy, NPV and PPV of 100, 93, 94, 100 and 75%, respectively. Nodal-based quantitative analysis of FDG uptake in N2 nodes revealed a significant difference between responding and non-responding LN only of SUVmax post-therapy (2.5 ± 1.21 vs. 3.5 ± 2.36, P = 0.04). CONCLUSION: FDG PET/CT after neoadjuvant therapy accurately defined response in metastatic N2 nodes of NSCLC patients, presenting very high sensitivity and NPV for detecting responding nodes. PET/CT may enable selection of candidates for curative resection of stage III NSCLC. Mediastinoscopy may not be mandatory in patients with a negative PET/CT after neoadjuvant therapy.

Long-lasting stable disease with mTOR inhibitor treatment in a patient with a perivascular epithelioid cell tumor: A case report and literature review.

Perivascular epithelioid cell tumor (PEComa) of the small intestine is extremely rare, and there is no established treatment at the present time. In 10% of patients with PEComas, genetic alterations of tuberous sclerosis complex have been reported. These genetic alterations activate mechanistic target of rapamycin (mTOR) in AMP-activated protein kinase and Ras/mitogen-activated protein kinase pathways, resulting in high mTOR activity. Since 2007, several cases of treatment with mTOR inhibitors in advanced PEComa have been reported. The current study presents the case of a patient with small bowel PEComa that metastasized to the brain and lungs. Following resection of the brain metastasis, the patient was treated with everolimus, a mTOR inhibitor, resulting in improvement if the patient's quality of life and a long period of stable disease. In conclusion, the use of mTOR inhibitors as a first-line treatment option in advanced PEComa patients appears to be reasonable, according to the increasing evidence from data observed from reported cases with this rare malignancy.

Long-term follow-up of patients with scleroderma interstitial lung disease treated with intravenous cyclophosphamide pulse therapy: a single-center experience.

BACKGROUND: Scleroderma lung disease (ILD-SSc) is treated mainly with cyclophosphamide (CYC). The effectiveness of CYC was judged after 12-24 months in most reports. OBJECTIVES: To analyze the effect of monthly intravenous CYC on pulmonary function tests including forced vital capacity (FVC) and diffusing lung capacity (DLCO), as well as Rodnan skin score (mRSS), during long-term follow-up. METHODS: We retrospectively collected the data on 26 ILD-SSc patients who began CYC treatments before 2007. Changes in FVC, DLCO and mRSS before treatment, and at 1,4 and 7 years after completion of at least six monthly intravenous CYC treatments for ILD-SSc were analyzed. RESULTS: Mean cumulative CYC dose was 8.91 ± 3.25 G. More than 30% reduction in FVC (0%, 8%, and 31% of patients), DLCO (15%, 23%, 31%), and mRSS (31%, 54%, 62%) at years 1, 4 and 7 was registered. During the years 0-4 and 4-7, annual changes in FVC, DLCO and mRSS were 3.2 vs. 0.42% (P < 0.040), 4.6 vs. 0.89% (P < 0.001), and 1.8 vs. 0.2 (P = 0.002). The greatest annual FVC and DLCO reduction over the first 4 years correlated with mortality (P = 0.022). There were no differences in the main variables regarding doses of CYC (< 6 G and > 6 G). CONCLUSIONS: In patients with ILD-SSc, CYC stabilized the reduction of FVC during treatment, but this effect was not persistent. The vascular characteristic of ILD-SSc (DLCO) was not affected by CYC treatment. CYC rapidly improved the mRSS. This effect could be achieved with at least 6 G of CYC. Higher rates of annual reduction in FVC and DLCO in the first 4 years indicate the narrow window of opportunity and raise the question regarding ongoing immunosuppression following CYC infusions.

Metabolic PET/CT-guided lung lesion biopsies: impact on diagnostic accuracy and rate of sampling error.

UNLABELLED: CT-guided fine-needle aspiration (FNA) of lung lesions is subject to sampling errors. The current study assessed whether information provided by (18)F-FDG PET/CT will decrease the false-negative (FN) rate and thus improve the accuracy of CT-guided FNA. METHODS: Data from 311 consecutive patients with lung nodules who underwent (18)F-FDG PET/CT and CT-guided FNA within an interval of less than 30 d were retrospectively assessed. In-house-developed software was used to register CT images performed for the FNA procedure (CT FNA) with corresponding slices of the PET/CT study. The quality of registration was rated on a scale of 1 (excellent) to 5 (misregistration). Only cases scored 1 or 2 were further evaluated. The software provided the highest standardized uptake value (SUV) within the lesion and at the location of the tip of the aspirating needle. The distance between the tip and the area with the highest SUV within the lesion was measured. The mean distance from the tip of the needle to the focus with the highest SUV, as well as the mean difference between the maximum SUV in the whole lesion and at the needle tip, was calculated and compared for cases with true-positive (TP) and FN FNA results. Anatomic and metabolic parameters of lesions included in these 2 groups were also compared. RESULTS: There were 267 patients (86%) with score 1 or 2 registration quality for CT FNA and PET/CT/CT images, including 179 TP (67%), 5 false-positive (FP, 2%), 49 true-negative (TN, 18%), and 34 FN (13%) FNA results. The distance between the location of the needle tip and the focus with the highest SUV in the lesion was significantly greater in the FN group (15.4 ± 14 mm) than in the TP group (5.9 ± 13.4 mm, P < 0.001). The maximum SUV at the location of the aspirating needle tip was significantly higher in the TP group, at 6.4 ± 6.4, than in the FN group, at 4 ± 4.7 (P < 0.05). CONCLUSION: The present results demonstrate a relationship between the degree of metabolism at the site of tissue-sampling aspiration in lung lesions and the accuracy of FNA results. Anatomy- and metabolism-based FNA guidance using information provided by both (18)F-FDG PET and CT may improve the accuracy of histologic examinations, decrease the rate of FN results, and thus increase the probability of achieving a definitive diagnosis.

Propranolol as a treatment option in Gorham-Stout syndrome: a case report.

Gorham-Stout syndrome is a very rare syndrome characterized by progressive angiomatosis and lymphangiomas involving multiple organs. We describe herein a girl with progressive Gorham-Stout syndrome since the age of 13 years. Her disease involved the mediastinum, pericardium, vertebrae, ribs, and skull and did not respond to interferon and bisphosphonates. Propranolol administration was initiated at the age of 18 years and was associated with improvement in pulmonary function tests and involution of the mediastinal and hilar hemangiomatous lesions. The possible beneficial effect of propranolol in Gorham-Stout syndrome should be further investigated.

Collision tumor of the mediastinum: a rare entity.

A collision tumor is a rare entity consisting of 2 different tumors that, because of proximity, merge together. The diagnosis of a collision tumor requires that the 2 tumors be histologically distinct. This phenomenon has been described mostly at the esophagogastric junction, where a squamous cell carcinoma of esophageal origin collides with a gastric adenocarcinoma. The present case is of 2 histologically distinct tumors with aggressive features occurring at the mediastinum.

Rituximab: rescue therapy in life-threatening complications or refractory autoimmune diseases: a single center experience.

Rituximab (RTX) is a chimeric anti-CD20 antibody, approved for rheumatoid arthritis (RA) patients who failed anti-Tumor Necrosis Factor therapy. It has been used occasionally for life-threatening autoimmune diseases (AID). We report our center experience in the use of RTX in life-threatening complications or refractory AID. Clinical charts of patients treated with RTX at our center were reviewed, cases treated for life-threatening complications or refractory AID were analyzed. Acute damage to vital organs such as lung, heart, kidney, nervous system with severe functional impairment were defined as life-threatening complications; treatment failure with high-dose corticosteroids, cyclophosphamide, IVIG, plasmapheresis was defined as refractory autoimmune disease. During the years 2003-2009, 117 patients were treated with RTX, most of them for RA. Nine patients (6 females, mean age 51.5 years, mean disease duration 6.3 years) answered the criteria. The indications were as follows: pulmonary hemorrhage (1 patient with cryoglobulinemic vasculitis, 1 with systemic sclerosis, 1 with ANCA-associated vasculitis), catastrophic anti-phospholipid syndrome (2 SLE patients), non-bacterial endocarditis and pulmonary hypertension (1 patient with mixed connective tissue disease), vasculitis and feet necrosis (1 patient with systemic lupus erythematosus), severe lupus demyelinative neuropathy and acute renal failure (1 patient), and severe rheumatoid lung disease with recurrent empyema and pneumothorax (1 patient). B cell depletion was achieved in all patients. The median time since starting of complications to RTX administration was 3 weeks (range 2-15 weeks). Complete remission (suppression of the hazardous situation and return to previous stable state) was seen in 7 out of 9 patients. Partial remission (significant improvement) was achieved in the remained. The median time to response was 3 weeks (range 1-8 weeks), mean follow-up 47.2 months (range 6-60 months). A rapid tapering off of steroids was achieved in all patients. Two patients relapsed and were successfully retreated with RTX: the patient with severe RA lung relapsed after 3 years, one of the patients with ANCA-associated pulmonary alveolar hemorrhage relapsed after 10 months. There were no side effects during RTX infusion. Two episodes of serious infections were registered: fatal Gram-negative sepsis 6 months after RTX treatment, and septic discitis 4 months after receiving RTX. RTX serves as a safe, efficient, and prompt rescue therapy in certain life-threatening conditions and resistant to aggressive immunosuppression AID. RTX when administrated at an earlier stage, prevented irreversible vital organ damage, and allowed rapid steroid tapering off in already severe immunodepressed patients.

Severe influenza A (H1N1): the course of imaging findings.

BACKGROUND: An outbreak of respiratory illness caused by a novel swine-origin influenza virus (influenza A/H1N1 2009) that began in Mexico was declared a global pandemic by the World Health Organization in June 2009. The pandemic affected many countries, including Israel. OBJECTIVES: To compare the course of chest radiographic and computed tomography findings in patients who survived and those who died following admission to the intensive care unit (ICU) or intubation due to severe laboratory-confirmed swine-origin influenza A/H1N1 2009. METHODS: We retrospectively reviewed the patient records (267 radiographs, 8 CTs) of 22 patients (10 males, 12 females) aged 3.5-66 years (median 34) with confirmed influenza A/ H1N1 2009, admitted to the ICU and/or intubated in five major Israeli medical centers during the period July-November 2009. We recorded demographic, clinical, and imaging findings--including pattern of opacification, extent, laterality, distribution, zone of findings, and presence/absence of nodular opacities--at initial radiography and during the course of disease, and compared the findings of survivors and non-survivors. Statistical significance was calculated using the Wilcoxon (continuous variables) and Fisher exact tests. RESULTS: The most common findings on the initial chest radiography were airspace opacities, which were multifocal in 17 patients (77%) and bilateral in 16 (73%), and located in the lower or lower and middle lung zones in 19 patients (86%). Large airspace nodules with indistinct margins were seen in 8 patients (36%). Twelve patients survived, 10 died. Patients who died had multiple background illnesses and were significantly older than survivors (P = 0.006). Radiologic findings for the two groups were not significantly different. CONCLUSION: Airspace opacities, often with nodular appearance, were the most common findings among patients with severe influenza A/H1N1 2009. The course of radiologic findings was similar in patients with severe influenza A/ H1N1 2009 who survived and those who died.

Pulmonary arteries involvement in Takayasu's arteritis: two cases and literature review.

OBJECTIVES: To review pulmonary arteritis (PA) complicated by pulmonary arterial hypertension (PAH) in Takayasu's arteritis (TA). METHODS: Two cases of PA and PAH in TA patients and similar cases published in the Medline database from 1975 to 2009 were reviewed. RESULTS: Forty-six cases (females 89.1%, Asians 65%, mean age 34.6 years) were analyzed, 42.2% of which had PAH. Isolated PA was reported in 31.8%. Respiratory symptoms were presented as dyspnea (75.5%), chest pain (48.9%), hemoptysis (42.2%), and cough (17.7%). Hypertension, vascular bruits, and diminished/absent pulses were reported in 48.9% of patients. A diagnosis of PA was based on abnormal uptake on pulmonary perfusion scan and a finding of stenosis, narrowing, occlusion, and irregularity on computed tomography or magnetic resonance imaging, and/or pulmonary angiography. Patients were treated with glucocorticoids (77.5%), disease-modified antirheumatic drugs (35%), and warfarin (20%); only a few were treated with biological agents. Vascular procedures were performed in 52.5% of cases, on pulmonary arteries in 37.5% with good results. The outcome was death in 20.5% of PA patient and 33.3% in PAH patients. CONCLUSIONS: TA may be complicated by life-threatening PA and PAH. Clinical signs are not specific and may be masked by involvement of the aorta and its branches. Treatment with glucocorticoids and disease-modified antirheumatic drugs has only partial effect, which may be intensified by biological agents. Invasive procedures on pulmonary arteries may be a complementary option. PA and PAH in TA patients should be recognized early and treated promptly for prevention of irreversible vascular damage.

Pulmonary outcome of Alport syndrome with familial diffuse esophageal leiomyomatosis.

X-linked Alport syndrome is associated in some families with diffuse leiomyomatosis. We describe herein, the pulmonary complications and outcome of three family members (mother, daughter, and son). The three underwent esophagectomy at different ages (22 years, three years, and 15 months respectively). Their current forced expiratory volume in the first second (FEV1) ranged from 33% in the mother to 60% in the daughter and 97% in the son. It is suggested that earlier intervention may lead to improved pulmonary function tests.

Catastrophic antiphospholipid syndrome presented with abdominal, pulmonary, and bone marrow complications.

We are presenting a case of catastrophic antiphospholipid syndrome in an adult female manifesting with abdominal thrombosis, pancytopenia, and alveolar hemorrhage. Alveolar hemorrhage is infrequently reported as it is difficult to diagnose, but it is considered as a life-threatening condition. The diagnosis should be made promptly based on clinical symptoms coupled with radiological features. Once this diagnosis is suspected, treatment with corticosteroids and anticoagulation must be initiated as soon as possible in order to reduce severe morbidity and high mortality.

Pseudopancreatitis in trauma patients.

OBJECTIVE: The purpose of our study was to review the significance of intra- and peripancreatic fluid in trauma patients who have no other signs of pancreatic injury. CONCLUSION: We propose that intra- and peripancreatic fluid may be the consequence of hypovolemic shock treated with hyperhydration when there is significant delay between injury and imaging.

Left-sided perforated acute appendicitis in an adult with midgut malrotation: the role of computed tomography.

We report a case of left-sided perforated acute appendicitis in the patient with midgut malrotation. Embryology, clinical findings, and radiological presentation are discussed. Highly prevalent disease presents here in the unusual location and thus in the unusual presentation. The emergency room physician and radiologist should be aware of these unique clinical presentations so that appropriate surgical intervention may be initiated promptly.

Accelerated pulmonary nodulosis and sterile pleural effusion in a patient with psoriatic arthropathy during methotrexate therapy: a case report.

Pulmonary nodulosis and sterile pleural exudates are well-known extra-articular manifestations in rheumatoid arthritis patients with a positive rheumatoid factor. In some patients, treatment with methotrexate has been postulated as the trigger of these complications. We report a patient with psoriatic arthropathy, negative RF, negative anticyclic citrulinated peptide antibodies but positive antibodies to cardiolipin who developed massive sterile pleural empyema and multiple cavitary pulmonary nodules during methotrexate treatment. We suggest that awareness of methotrexate-induced lung and pleural complications should be extended to other than rheumatoid arthritis diseases, not necessarily accompanied by rheumatoid factor or anticyclic citrulinated peptide antibodies.

Noninvasive diagnosis of solitary pulmonary lesions in cancer patients based on 2-fluoro-2-deoxy-D-glucose avidity on positron emission tomography/computed tomography.

BACKGROUND: 2-Fluoro-2-deoxy-D-glucose (FDG) imaging is highly accurate for assessing solitary pulmonary nodules (SPNs) in patients without known malignancy. In the current study, the authors evaluated FDG-positron emission tomography/computed tomography (PET/CT) for the characterization of SPN in cancer patients. METHODS: FDG-PET/CT was performed in 56 cancer patients to evaluate SPNs that measured 15 +/- 8 mm in greatest dimension. The diagnosis was confirmed by histology (n = 34 patients), or by CT or clinical follow-up (n = 22 patients). The performance of PET/CT was calculated for visual and semiquantitative assessment and was related to SPN size, location, histology, and time after initial cancer diagnosis. RESULTS: Malignancy was diagnosed in 27 of 56 SPNs (48%; 18 second primary tumors and 9 metastases). There were 26 true-positive PET/CT studies (17 second primaries and 9 metastases), 5 false-positive studies, 24 true-negative studies, and 1 false-negative study. The sensitivity of PET/CT for diagnosing malignant SPN in patients with cancer was significantly greater for visual analysis than for semiquantitative analysis (96% vs 89%, respectively; P < .05). Specificity and accuracy were similar for both methods (83% and 89% vs 93% and 91%, respectively). The presence of low-intensity FDG uptake increased the detection rate of malignancy from 4% in non-FDG-avid SPNs to 40%, mainly in second primary tumors. False-positive results were more frequent with lower than mediastinal uptake versus higher than mediastinal uptake (3 of 5 SPNs vs 2 of 26 SPNs, respectively; P < .01) and in SPNs >10 mm. CONCLUSIONS: FDG imaging was highly accurate for the diagnosis of malignant SPNs in patients with cancer, similar to the general population. The presence of any FDG avidity had significantly greater sensitivity than semiquantitative analysis. The current results indicated that lower than mediastinal uptake should be explored cautiously, particularly for second primary tumors, whereas no FDG avidity was a better predictor of SPN benignity than very low uptake.