Purpose A subset of patients with common variable immunodeficiency (CVID) develops granulomatous and lymphocytic interstitial lung disease (GLILD), a restrictive lung disease associated with early mortality. The optimal therapy for GLILD is unknown. This study was undertaken to see if rituximab and azathioprine (combination chemotherapy) would improve pulmonary function and/or radiographic abnormalities in patients with CVID and GLILD. Methods A retrospective chart review of patients with CVID and GLILD who were treated with combination chemotherapy was performed. Complete pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) scans of the chest were done prior to therapy and >6 months later. HRCT scans of the chest were blinded, randomized, and scored independently (in pairs) by two radiologists. The differences between pre- and post-treatment HRCT scores and PFT parameters were analyzed. Results Seven patients with CVID and GLILD met inclusion criteria. Post-treatment increases were noted in both FEV1 (p=0.034) and FVC (p=0.043). HRCT scans of the chest demonstrated improvement in total score (p=0.018), pulmonary consolidations (p=0.041), ground-glass opacities (p=0.020) nodular opacities (p=0.024), and both the presence and extent of bronchial wall thickening (p=0.014, 0.026 respectively). No significant chemotherapy-related complications occurred. Conclusions Combination chemotherapy improved pulmonary function and decreased radiographic abnormalities in patients with CVID and GLILD.

The superior vena cava (SVC) is the largest central systemic vein in the mediastinum. Imaging (ie, radiography, computed tomography [CT], magnetic resonance [MR] venography, and conventional venography) plays an important role in identifying congenital variants and pathologic conditions that affect the SVC. Knowledge of the basic embryology and anatomy of the SVC and techniques for CT, MR imaging, and conventional venography are pivotal to accurate diagnosis and clinical decision making. Congenital anomalies such as persistent left SVC, partial anomalous pulmonary venous return, and aneurysm are asymptomatic and may be discovered incidentally in patients undergoing imaging evaluation for associated cardiac abnormalities or other indications. Familiarity with congenital abnormalities is important to avoid image misinterpretation. Acquired abnormalities such as intrinsic and extrinsic strictures, fibrin sheath, thrombus, primary neoplasms, and trauma can produce mild narrowing to complete occlusion, the latter leading to SVC syndrome. Each imaging modality plays a role in evaluation of the SVC, helping to determine the site, extent, and cause of pathologic conditions and guide appropriate management. Commonly performed interventional procedures for fibrin sheath and benign and malignant strictures include low-dose thrombolytic infusion, fibrin sheath disruption, venous angioplasty, and stent placement.

CT pulmonary angiography and perfusion scanning have equivalent clinical negative predictive value (99% for CT pulmonary angiography; 100% for perfusion scanning) and image quality in the care of pregnant patients. Therefore, the choice of study should be based on other considerations, such as radiation concern, radiographic results, alternative diagnosis, and equipment availability. Reducing the amount of radiation to the maternal breast favors use of perfusion scanning when the radiographic findings are normal and there is no clinical suspicion of an alternative diagnosis.

The aim of this study is to determine the feasibility of using reduced scan range CT pulmonary angiography technique in pregnancy for pulmonary embolism (PE) and to quantify resulting dose reduction. This was a retrospective study. Eighty-four CTPA exams performed on pregnant women during 2004-2012. The scans were modified to create reduced anatomic coverage scans extending from aortic arch to base of heart. These were separately evaluated by two radiologists for PE and non-PE abnormalities. The results were then compared by the third radiologist with original radiology report and scans. Radiation dose reduction was evaluated prospectively in 36 patients as part of a quality control project. Two patients had PE and were successfully identified on reduced z-axis scans. Thirty-two exams were normal; rest had 60 pertinent and 16 had incidental findings. There were four incidental findings which included three benign thyroid nodules and one benign small lung nodule which were missed. None of these affected clinical outcome or management. There was 71 % radiation dose reduction. No PE or any important diagnoses are missed using reduced z-axis CTPA in pregnancy. There is a substantial radiation dose reduction. Hence, this technique is highly recommended in pregnancy.

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