Outcome in sarcoidosis. The relationship of relapse to corticosteroid therapy.

STUDY OBJECTIVE: To determine the demographic, clinical, and radiographic characteristics of corticosteroid-treated patients with sarcoidosis who developed relapse following a period of clinical stability lasting longer than 1 month, and to compare these characteristics with those of a group of patients with sarcoidosis who were not treated. DESIGN: Historic, concurrent and prospective, nonrandomized, observational study. SETTING: Ambulatory sarcoidosis clinic in a university city hospital. PATIENTS: Over a 4-year calendar period, 337 patients with sarcoidosis were prospectively enrolled in a registry. One hundred eighteen patients were assigned to a spontaneous remission group when symptoms resolved without treatment, and 103 were assigned to an induced remission group when symptoms resolved following corticosteroid therapy and successful discontinuation. In 116 patients assigned to a recalcitrant group, therapy could not be stopped for 1 month or more owing to severity of symptoms or lack of compliance. We defined relapse as a recurrence of symptoms of sufficient severity to warrant treatment with corticosteroids, following a remission without treatment lasting greater than 1 month. INTERVENTION: Patients who were judged to be sufficiently symptomatic to preclude observation without treatment or who failed to respond to conservative treatment with topical or inhaled corticosteroids or nonsteroidal anti-inflammatory agents were treated with systemic corticosteroids at a target dose of 20 mg prednisone per day for 1 year. MEASUREMENTS AND RESULTS: We observed a 74% relapse rate in the induced remission group, but only an 8% relapse rate in the spontaneous remission group (p < 0.01). Relapse occurred with similar frequency in whites and African-Americans (20% vs 28%), despite a lower treatment rate in white patients than in African-Americans (43% vs 76%; p < 0.01). White patients maintained a sustained remission with twice the frequency of African-Americans (58% vs 29%; p < 0.01). During relapse, 40% of chest radiographs showed no change in type, but there was a significant increase in interstitial profusion (p < 0.05). Initial presentation with asymptomatic chest radiographic abnormalities, erythema nodosum, or peripheral adenopathy portended a favorable prognosis, with sustained remission in 60% of such patients lasting 130 +/- 226 months from time of diagnosis. In contrast, patients who presented with musculoskeletal complaints were nine times, and those with symptoms from hepatic involvement were three times more likely to suffer relapse than to sustain remission without receiving corticosteroids. Most relapses (50%) occurred between 2 and 6 months after discontinuing steroid therapy, but late relapse was not unusual, occurring more than 12 months after discontinuing steroid therapy in 20% of patients with induced remission. CONCLUSIONS: Relapse occurred frequently in patients with sarcoidosis who had been treated with corticosteroids, and rarely occurred in patients who had not been treated with corticosteroids in the past. The striking difference in relapse rate between treated and untreated patients suggests that patients with disease that would later be severe and protracted were almost unerringly identified early in their course. One explanation is that severe presenting symptoms portend a protracted and recurrent course; an alternative explanation is that corticosteroids contributed to the prolongation of the disease by delaying resolution.

Inflammatory pseudotumor of the sternum. A complication of polymer application during sternotomy repair.

A case of inflammatory pseudotumor of the sternum resulting from the application of polyurethane foam is discussed. The past use of polymers in bone repair is reviewed.

Pseudoaneurysm of the ascending aorta following cardiac surgery.

Pseudoaneurysm of the ascending aorta is an unusual and potentially fatal complication of cardiovascular surgery. Most pseudoaneurysms are associated with aortic valve or coronary artery bypass graft surgery and are often mycotic in origin. Chest roentgenography and aortography have been the principle methods of diagnosis. Recently, contrast enhanced computed tomography (CT) has proven to be a useful means of diagnosis, providing a less invasive method of distinguishing pseudoaneurysm from other causes of fever and mediastinal widening in the postoperative cardiovascular patient. Thirty one cases of postoperative pseudoaneurysm of the ascending aorta occurring since 1963 are reviewed, including seven cases from our experience.

Diffuse contralateral pulmonary metastases in malignant mesothelioma. An unusual radiographic presentation.

We describe a patient with malignant pleural mesothelioma involving the lung parenchyma bilaterally in a diffuse nodular fashion. This pattern of metastasis is seldom reported for this tumor.

Analysis of cell type and radiographic presentation as predictors of the clinical course of patients with bronchioalveolar cell carcinoma.

STUDY OBJECTIVES: Bronchioloalveolar carcinoma is a primary lung neoplasm of variable histopathologic, radiologic, and clinical expression. There are three cell types described in bronchioloalveolar carcinoma: Clara cells, mucin-producing cells, and alveolar type II epithelial cells. It is unclear whether these three tumor cell types are associated with a specific radiologic presentation and clinical course. In this study, we investigated whether tumor cell type, identified by transmission electron microscopy, correlated with a specific radiologic pattern, and whether tumor cell type or radiologic presentation correlated with the patient's clinical course and outcome. DESIGN: Transmission electron microscopy was used to restudy tissue blocks from the original surgical histopathologic specimens in 54 patients with primary bronchioloalveolar carcinoma diagnosed over a 10-year period (1980 to 1990). The pretreatment radiographs were reviewed in each case, and the first chest radiograph obtained at the time of the discovery of the tumor in each patient was compared with the results of the ultrastructural study. The medical records of each patient were examined to obtain pertinent radiologic, clinical, and patient outcome information. MEASUREMENT AND RESULTS: There were 32 Clara cell tumors, 10 mucin-producing cell tumors, and 1 alveolar type II epithelial cell tumor in this series. Eleven additional tumors had mixtures of two or more cell types. No statistically significant relationship was detected between tumor cell type and radiologic presentation or patient mortality pattern. There was increased mortality among patients who presented radiologically with segmental, lobar, multifocal, or diffuse disease compared with those patients exhibiting a solitary pulmonary nodule at presentation. CONCLUSION: Radiologic presentation, rather than tumor cell type, provides prognostic information that aids in predicting patient outcome.

Course of chronic hilar sarcoidosis in relation to markers of granulomatous activity.

Studies of the course of sarcoidosis have emphasized that patients with hilar or mediastinal adenopathy usually recover within several years or develop dissemination to the lungs. Chronic hilar and mediastinal adenopathy persisting with little or no change for many decades is an important subgroup that has not received adequate attention. Twelve such patients have been studied. Seven remained asymptomatic, despite persistent adenopathy, for a mean period of 16 years; two with disfiguring facial sarcoids received corticosteroids for 18 and 27 years, respectively, and three patients after ten years of stable adenopathy developed pulmonary infiltrates. Tests performed on patients with hilar adenopathy to evaluate cellular activity after a mean interval of over 16 years included Kveim reaction (positive in nine of ten), serum angiotensin converting enzyme (elevated in eight of 12), and gallium-67 scanning (hilar uptake in all eight tested). Results were similar for patients who remained well and for those who had symptomatic or progressive disease, indicating that these parameters of granulomatous activity do not reflect the duration of the disease, its outcome, or the need for treatment.

The practice of chest radiology in departments with residency programs.

OBJECTIVES: The authors assessed to what extent radiology teaching programs employed dedicated chest radiologists and their functions. METHODS: Information regarding the clinical, teaching, and research role of the chest radiologist was available from responses to a survey of radiology departments with residency training programs in the United States and Canada. Emphasis was placed on the role of chest subspecialists, "dedicated chest radiologists" (DCRs), who spent at least two thirds of their clinical time interpreting and directing chest-related imaging studies and procedures. RESULTS: Among the 171 residency programs that responded, 118 had DCRs. There were 262 full-time and 233 part-time DCR positions, of which 43 were not filled. Among departments with DCRs, 66% were medical school programs while the remaining 34% were independent or medical-school-affiliated programs; 30% were organized by organ system, 5% by technology, and 65% had a combination of both; and 78% had more than 12 residents. DCRs interpreted routine chest radiographs in 96%, critical care radiographs in 94%, chest computed tomography (CT) studies in 72%, and chest magnetic resonance imaging (MRI) studies in 44% of their departments. Departments without DCRs were usually smaller, 70% having 12 or fewer residents. Their designated chest radiologists interpreted all chest radiographs in 21%, all critical care radiographs in 19%, all chest CT studies in 13% and all chest MRI studies in 8% of these departments. CONCLUSIONS: Dedicated chest radiologists were more involved than non-DCRs in all aspects of chest imaging, teaching, and research including analysis of image quality and acquisition of new technology. The highest quality of chest radiology training, defined as programs in which the chest fellowship positions were filled in 1991, was found in departments in which DCRs interpreted all chest radiographs, all chest CT studies, and most MRI studies.

Single breath-hold pulmonary magnetic resonance angiography. Optimization and comparison of three imaging strategies.

RATIONALE AND OBJECTIVES: Ultrafast gradient-recalled-echo techniques for obtaining high-quality pulmonary magnetic resonance angiograms within a single breath-hold were optimized. METHODS: Fourteen subjects were imaged with both the body coil and a phased-array surface coil, using three gradient-recalled-echo pulse sequences: 1) two-dimensional sequential; 2) two-dimensional interleaved; and 3) volumetric acquisitions. Image quality was assessed with varied flip angle, receiver bandwidth, slice thickness/number, and matrix size. Cardiac compensation diminished ghost artifacts in the interleaved sequence. Individual sagittal sections and maximum intensity projections were reviewed. RESULTS: Pulmonary magnetic resonance angiograms acquired with volumetric and two-dimensional interleaved gradient-recalled-echo pulse sequences benefit greatest from intravenous gadolinium and result in greater pulmonary arterial visualization than traditional time-of-flight techniques. Phased-array coils result in improved vessel detection. CONCLUSIONS: High-quality breath-held pulmonary magnetic resonance angiography can be obtained with an intravenous contrast-enhanced gradient-recalled-echo acquisition; however, image quality is dependent on the pulse sequence.

Bronchoalveolar cell carcinoma of the lung.

A multivariable analysis was performed of all patients registered and confirmed to have bronchoalveolar cell carcinoma of the lung in the Tumor Registry of Thomas Jefferson University Hospital between 1969 and 1983. These 122 patients were reviewed for age, sex, smoking history, occupational exposure, symptoms, radiographic findings, methods of diagnosis, clinical and pathologic staging, methods of treatment, survival, and complications of treatment. No correlation could be found in this series between a patient's age, sex, smoking history, or occupational exposure and the incidence or outcome of the disease. Seventy-one of the 122 patients in this series were asymptomatic, and the carcinoma was discovered in them by routine chest roentgenogram. Of these asymptomatic patients, 50 were seen with pathologic stage I disease. Of the 51 symptomatic patients, 32 (65%) were seen with stage IIIm0 or IIIm1 disease. Despite medical evaluations, 77% of the T1 and T2 lesions required thoracotomy for diagnosis. The overall five-year survival rate was 42.3%, ranging from 75% for those with stage I disease to 8.7% for those with stage IIIm1 disease.

Rounded atelectasis: a pulmonary pseudotumor.

Rounded atelectasis is a benign entity that is often misinterpreted as a pulmonary neoplasm. The roentgenologic appearance of a mass is due to an infolding of atelectatic tissue intermingled with pleura, blood vessels, and bronchi. Rounded atelectasis is usually asymptomatic and is commonly associated with chronic pleural disease or pleural effusions. The distinctive radiologic features include a rounded, pleural-based opacity associated with adjacent pleural thickening and volume loss of the affected lobe. The pathognomonic sign is the "comet tail" that results from the crowding of vessels and bronchi as they enter the atelectatic region. Many authors consider this constellation of findings diagnostic. Rounded atelectasis usually remains stable over time; however, slow growth, as well as diminution in size, has been described. A retrospective analysis revealed 7 cases of rounded atelectasis at our institution over a 9-year period. Three were operated on to exclude malignancy, one was confirmed at operation performed for other reasons, and 3 were followed up expectantly. We conclude that recognition of this entity and its radiologic features can be diagnostic and render further workup, including thoracotomy, unnecessary.

Noninvasive diagnostic imaging in hemoglobinopathies.

We have provided a global review of the current applications of newer modalities, particularly MR imaging, as applicable to the major manifestations of sickle cell disease, the most common hemoglobinopathy. There is a need for well-designed prospective longitudinal studies utilizing MR imaging to gain further insight into the pathophysiology of this intriguing disease. Additionally, MR imaging, because of lack of ionizing radiation, is ideally suited for long-term sequential studies and may thus provide an objective means for monitoring response to therapy.

Filter wheel equalization for chest radiography: a computer simulation.

A chest radiographic equalization system using lung-shaped templates mounted on filter wheels is under development. Using this technique, 25 lung templates for each lung are available on two computer controlled wheels which are located in close proximity to the x-ray tube. The large magnification factor (> 10X) of the templates assures low-frequency equalization due to the blurring of the focal spot. A low-dose image is acquired without templates using a (generic) digital receptor, the image is analyzed, and the left and right lung fields are automatically identified using software developed for this purpose. The most appropriate left and right lung templates are independently selected and are positioned into the field of view at the proper location under computer control. Once the templates are positioned, acquisition of the equalized radiographic image onto film commences at clinical exposure levels. The templates reduce the exposure to the lung fields by attenuating a fraction of the incident x-ray fluence so that the exposure to the mediastinum and diaphragm areas can be increased without overexposing the lungs. A data base of 824 digitized chest radiographs was used to determine the shape of the specific lung templates, for both left and right lung fields. A second independent data base of 208 images was used to test the performance of the templates using computer simulations. The template shape characteristics derived from the clinical image data base are demonstrated. The detected exposure in the lung fields on conventional chest radiographs was found to be, on average, three times the detected exposure behind the diaphragm and mediastinum.(ABSTRACT TRUNCATED AT 250 WORDS)

Radiographic manifestations of congenital heart disease in the adult patient.

Improvements in the preoperative evaluation, surgical treatment, and postoperative care of patients with congenital cardiac disease have allowed a large patient population with congenital cardiac abnormalities to reach adolescence and adulthood. Noninvasive diagnostic imaging procedures (e.g., plain film radiography, echocardiography, computed tomography, and magnetic resonance imaging) are playing an increasingly important role in the evaluation and management of adults with both treated and untreated congenital cardiac disease. The role of plain film radiology is emphasized.

Occupationally induced neoplasms of the lung and pleura.

The relationship between asbestos and mesothelioma has been well delineated in the past. The epidemiologic, clinical, radiologic, and pathologic features of mesothelioma are discussed with reference to the diagnostic evaluation of asbestos-exposed patients. The extensive epidemiologic data correlating asbestos, tobacco smoke, and induction of lung carcinoma are also reviewed. These data provide a model for evaluation of other occupationally induced lung carcinogens such as organic and metallic industrial inhalants.

Magnetic resonance imaging of diffuse bone marrow disease.

MR is a highly sensitive alternative to plain films, CT, and radionuclide studies for the imaging of normal and abnormal marrow and can characterize differences between fatty, fibrotic, cellular, hypercellular, and hemosiderotic marrow. MR is helpful in depicting the extent of disease and has been a useful method to follow the clinical course of many disorders. It has been found to be particularly useful in explaining the unrepresentative biopsy, as the distribution of many diseases is frequently heterogeneous as exemplified by the mixed fatty and cellular patterns of aplastic anemia, myeloma, lymphoma, and skeletal metastases. Patterns of cellular and fatty marrow in the epiphysis and apophysis after marrow reconversion were not completely understood prior to the introduction of MR scanning. Because it has the advantage of imaging the entire bone marrow compartment (unlike the situation with biopsy on aspiration), MR allows a better understanding of the distribution of skeletal disease.

The functional and anatomic evaluation of the cardiovascular system with rapid-acquisition computed tomography (cine CT).

Cine CT combines the advantages of digital cross-sectional imaging with those of angiography. It provides vivid portrayal of complex anatomic relationships as well as important functional data not easily obtainable elsewhere, including quantitation of myocardial wall thickening and regional myocardial blood flow. The blood flow in major vessels following coronary artery bypass graft surgery and cardiac output have been quantified with accuracy. The future of cine CT will depend ultimately on controlled studies comparing this technique with other modalities, including echocardiography, magnetic resonance imaging, radionuclide angiocardiography, and contrast-enhanced catheter angiography.

The value of ultrasound-guided fiberoptic bronchoscopy.

Flexible fiberoptic bronchoscopy is the procedure of choice for the diagnosis of endobronchial neoplasm. Because of technical limitations of fiberoptic bronchoscopy, the use of ultrasound assistance was initiated and evaluated. This article reviews the technique, study design, and clinical results of ultrasound-assisted fiberoptic bronchoscopy.

Femoral head avascular necrosis in sickle cell anemia: MR characteristics.

Since considerable expansion of hematopoietic marrow occurs in patients with sickle cell anemia (SCA), magnetic resonance images of 20 hips in 10 patients with known homozygous SCA were reviewed to determine a) if low signal hematopoietic marrow extended into the femoral capital epiphysis and b) if the MR characteristics of avascular necrosis (AVN) differed depending on the type of epiphyseal marrow. Our results revealed variable epiphyseal marrow type; mixed (fatty and hematopoietic) marrow (42%), fatty marrow (32%), hematopoietic marrow (16%) and hemosiderotic marrow (10%). AVN occurred irrespective of the underlying marrow. Segmental areas of low signal intensity in variable shapes (ring, band, crescent or large homogeneous area) was the most consistent MR manifestation of AVN in SCA. A low signal intensity peripheral rim surrounding a central zone, isointense with epiphyseal marrow on T1 and T2 weighted images, was most frequently observed similar to that described in patients without hemoglobinopathy. The notable difference, however, was of segmental areas within the same femoral head that demonstrated variable central zone signal on T2 weighted images. Further, while an increase in hip joint fluid is commonly seen with both early and advanced AVN in patients without hemoglobinopathy; it was increased in only one hip in patients with SCA. The observed differences in MR characteristics may be due to different pathophysiology of AVN in patients with SCA.

Marrow infarction in sickle cell anemia: correlation with marrow type and distribution by MRI.

Ischemic necrosis of bone is believed to occur exclusively in areas of predominantly fatty marrow. Sickle cell disease is unusual in that marrow infarction occurs in areas of active hematopoiesis. MR images of long bone obtained in ten patients with sickle cell anemia (SCA) were analyzed to correlate the distribution and appearance of marrow infarction with the type of marrow. While the hematopoietic marrow predominated in metaphyseal and diaphyseal regions of femurs and tibias, the fatty or mixed marrow was the most common pattern in epiphyses. Infarcts occurred in fatty as well as hematopoietic marrow. Marrow infarcts were isointense or minimally hyperintense on T1 weighted images with the hematopoietic marrow and therefore difficult to detect. On T2 weighted images, the infarcts showed very high signal. T2 weighted images are essential for detection of marrow infarction. Soft tissue changes seen as low signal on T1 and high signal on T2, may be secondary to intramuscular injections of analgesics or muscle ischemia occurring during sickle crisis.

Aneurysm of saphenous vein coronary bypass graft: diagnosis by computed tomography.

A patient with a history of coronary artery bypass graft surgery underwent computed tomography scanning for evaluation of a lung mass. A heterogeneous mediastinal mass discovered incidentally on computed tomography scanning was shown to be a saphenous vein bypass graft aneurysm.