Enfermedad pulmonar relacionada con IgG4 - Tres casos no tratados con resultado benigno / IgG4-Related Lung Disease - Three Untreated Cases with a Benign Outcome

La enfermedad relacionada con IgG4 es una condición fibroinflamatoria en la cual los distintos órganos afectados comparten similitudes patológicas. Recientemente se ha caracterizado clínica y radiológicamente esta enfermedad en órganos torácicos. La mayoría de los casos descritos abogan por un tratamiento precoz con terapia inmunosupresora y observan una respuesta rápida y favorable. Describimos 3 casos de enfermedad pulmonar relacionada con IgG4 que no recibieron tratamiento farmacológico y que tuvieron una respuesta clínica y radiológica favorable o estable durante el seguimiento. La terapia inmunosupresora puede no ser necesaria en algunos casos de enfermedad pulmonar relacionada con IgG4

IgG4-related disease is a fibroinflammatory disease in which the organs involved share similar pathological findings. Chest disease has been recently clinically and radiologically characterized. Most reports advocate prompt immunosuppressive therapy and describe a fast and good response. We report 3 cases of untreated IgG4-related lung disease that on follow-up have been clinically asymptomatic and radiologically stable or improved. In some cases of IgG4-related lung disease immunosuppressive therapy may not be warranted

IgG4-Related Lung Disease - Three Untreated Cases with a Benign Outcome.

IgG4-related disease is a fibroinflammatory disease in which the organs involved share similar pathological findings. Chest disease has been recently clinically and radiologically characterized. Most reports advocate prompt immunosuppressive therapy and describe a fast and good response. We report 3 cases of untreated IgG4-related lung disease that on follow-up have been clinically asymptomatic and radiologically stable or improved. In some cases of IgG4-related lung disease immunosuppressive therapy may not be warranted.

Histoplasmosis in Israeli travelers.

Histoplasmosis is a common endemic human mycoses acquired mostly in the Americas. We reviewed 23 cases of histoplasmosis in Israeli travelers; 22 had traveled to Central or South America and one to North America. Fourteen cases had been exposed to bat habitats and were symptomatic, presenting ≤ 3 months after their return. Asymptomatic patients (N = 9) were diagnosed during the evaluation of incidental radiological findings or because a travel partner had been suspected of Histoplasma infection, 16-120 months after their return. Serological testing was positive in 75% of symptomatic cases but only 22% of asymptomatic cases. Histoplasmosis should be considered in travelers returning from the Americas with respiratory or febrile illness within weeks of return, particularly if exposed to bat habitats. Travel history is essential in patients presenting with pulmonary nodules, even years after travel to endemic countries.

Extra-cardiac findings on coronary computed tomography scanning.

BACKGROUND: Cardiac computed tomography scans include several extra-cardiac structures such as mediastinum, lung parenchyma and upper abdominal organs. A variety of abnormalities in those structures might be clinically important and in some cases might explain the patient's complaints. OBJECTIVES: To analyze consecutive CCT examinations for the prevalence and clinical significance of extra-cardiac findings. METHODS: Cardiac CT scans of 134 sequential patients (104 males, 30 females) aged 20-77 (mean 54 years) with suspected coronary artery disease were prospectively and independently reviewed by a consensus of two radiologists for the presence of lung, mediastinal, pleural, upper abdominal and skeletal abnormalities. CT scans with extra-cardiac abnormalities were divided into two groups: group A- defined as "clinically significant" or "potentially significant findings" - consisted of patients requiring further evaluation or follow-up, and group B - "clinically non-significant findings." RESULTS: Extra-cardiac abnormalities were found in 103 of the 134 patients (76.8%). Group A abnormalities were found in 52/134 patients (39%), while group B abnormalities were seen in 85/134 (63%). The most common abnormalities in group A were non-calcified lung nodules (> 4 mm) noted in 17/134 patients (13%), followed by enlarged mediastinal lymph nodes (> 10 mm) in 14/134 (10%), diaphragmatic hernia (2 cm) in 12/134 (9%), moderate or severe degenerative spine disease in 12/134 (9%), and emphysema and aortic aneurysm in 6 patients each (4.5%). A malignant lung tumor was noted in one patient. CONCLUSIONS: There is a high prevalence of non-cardiac abnormalities in patients undergoing CCT. Clinically significant or potentially significant findings can be expected in 40% of patients who undergo CCT, and these will require further evaluation and follow-up. The reporting radiologist should be experienced in chest imaging.

Cardiovascular calcifications after radiation therapy for Hodgkin lymphoma: computed tomography detection and clinical correlation.

OBJECTIVE: To study cardiovascular calcifications, detected by computed tomography, in patients following mediastinal radiation for Hodgkin lymphoma, and correlate them with clinical findings. MATERIALS AND METHODS: Fifteen patients,

Experience with somatostatin receptor scintigraphy in the management of pulmonary carcinoid tumors.

BACKGROUND: Somatostatin receptor scintigraphy has been used widely for the evaluation of neuroendocrine tumors in the gastrointestinal tract. Its use for detecting and staging thoracic carcinoids is only sporadically reported. OBJECTIVES: To evaluate the possible roles of SRS in the management of proven or suspected pulmonary carcinoids. METHODS: We conducted a retrospective study of all patients undergoing SRS for known or suspected pulmonary carcinoids in a tertiary referral center during a 10 year period. During this period 89 patients underwent resection of pulmonary carcinoids and SRS was used for detection, staging or localization purposes in 8 of them (9%). Scans were labeled true positive, true negative, false positive, or false negative in comparison with histologic or follow-up results. RESULTS: SRS was true positive in 6/6 lung locations; true positive in 2/8, true negative in 4/8 and false positive in 2/8 lymph node locations; and true positive in 1/8, true negative in 6/8 and false negative in 1/8 distant locations. The sensitivity, specificity, positive and negative predictive values and accuracy were 90%, 83%, 83%, 91% and 87% respectively. The scans were strongly positive in the tumors and involved lymph nodes. SRS correctly localized an occult secreting pulmonary carcinoid. Granulomatous and reactive lymph nodes showed increased uptake. SRS was accurate in ruling out distant metastases. CONCLUSIONS: SRS is effective for visualizing and localizing pulmonary carcinoids. It assists in the staging of these tumors by detecting lymph node involvement and confirming or ruling out distant metastases. Inflammatory areas in the lung or lymph nodes may be falsely positive.

Cryptogenic organizing pneumonia: variety of radiologic findings.

BACKGROUND: Cryptogenic organizing pneumonia is increasingly being recognized as a major cause of diffuse infiltrative lung disease. The differential diagnosis of non-infectious diseases that resemble pneumonia should include this entity. Understanding the radiologic features of this entity will help in defining the correct diagnosis, although lung biopsy is needed to provide histopathologic confirmation. Treatment with steroids achieves an excellent response. OBJECTIVES: To present a variety of radiologic findings on high resolution computerized tomography in eight sequential patients with COP, together with clinical and pathologic correlation. METHODS: Sequential HRCT examinations of eight patients (four males) aged 53-80 years (mean 65.5 years) with pathologcally proven COP were retrospectively analyzed by a consensus of two experienced chest radiologists for the existence and distribution of airspace consolidation, ground-glass opacities, nodular thickening along bronchovascular bundles and small (<1 cm) and large (>1 cm) nodules. The distribution of radiologic findings was classified as unilateral or bilateral, located in the upper, lower or middle lobe, and central or peripheral. Also recorded was the presence or absence of mediastinal lymphadenopathy and pleural effusion. Correlation with clinical symptoms was analyzed. RESULTS: All eight patients had bilateral airspace consolidations. in two cases consolidations were limited to central fields, in four they were peripheral, and in the remaining two cases they were both central and peripheral. Small nodules were noted in six cases and large nodules in three. Ground-glass opacities were found ln four cases. All patients had enlarged lymph nodes (1-1.5 cm) in the mediastinum. Radiologic abnormalities resolved or improved after steroid treatment in all patients. CONCLUSIONS: HRCT findings of bilateral multiple heterogenic lung infiltrates and nodules associated with mild mediastinal lymphadenopathy in a patient with non-specific clinical symptoms are suggestive of COP; in such cases lung biopsy is indicated. Radiologic resolution of abnormalities correlates well with clinical improvement under adequate steroid treatment.

Coronary artery calcium measurement with multi-detector row CT and low radiation dose: comparison between 55 and 165 mAs.

PURPOSE: To prospectively compare the results of coronary artery calcium (CAC) measurements obtained with 55- and 165-mAs electrocardiographically gated multi-detector row computed tomography (CT). MATERIALS AND METHODS: Institutional clinical study review board approval and written informed consent were obtained. Fifty-one consecutive subjects (mean age, 59 years +/- 10) were scanned consecutively by using 165 and 55 mAs. For each examination, the number of lesions, total calcium score (TCS) calculated with Agatston algorithm (130-HU threshold), and calcium mass (in milligrams) were measured. Noise was measured by averaging 1 standard deviation of the CT attenuation values in five consecutive transverse sections of the ascending aorta. Paired t test and Pearson correlation were used to compare measurements between the examinations. RESULTS: By using 55 mAs, CAC was detected (TCS > 0) in all 33 subjects in whom CAC was initially detected with 165 mAs. The mean values of CAC measures with 165 and 55 mAs, respectively, were as follows: number of lesions, 6.2 +/- 9.6 and 6.1 +/- 9.4; TCS, 123 +/- 223 and 126 +/- 225; and calcium mass, 23.25 mg +/- 43 and 24.25 mg +/- 44 (P value was not significant for all parameters). Significant high correlation was found between the two methods for all measures (r > 0.90, P < .01). Similar results were obtained with analysis by coronary vessel. Image noise was 9.3 HU +/- 2.1 with 165 mAs and 14.7 HU +/- 3.9 with 55 mAs (P < .001), with a parallel decrease in the volume CT dose index from 12 to 4 mGy. CONCLUSION: Radiation dose can be reduced (eg, 55 mAs) for CAC detection and measurement at multi-detector row CT and provides results comparable to those obtained with 165 mAs.

Visibility of normal thoracic anatomic landmarks on storage phosphor digital radiography versus conventional radiography.

BACKGROUND: Chest radiography is still the most frequently performed radiologic imaging study. Digital radiography is gradually replacing the conventional systems. OBJECTIVES: To compare the subjective visibility of normal anatomic landmarks in the chest on storage phosphor-based digital radiographs versus conventional screen-film radiographs. METHODS: Digital phosphor-based and screen-film posteroanterior chest radiographs were obtained during 1 year in 140 asymptomatic patients without any known pulmonary disease (119 men, 21 women; mean age 52.1 years, range 23-86). Both sets of films were independently compared by two experienced radiologists in different sessions. The visibility of each of the following anatomic landmarks was graded from 1 to 3: pulmonary fissures, carina, bronchi to left upper lobe, right upper lobe and left lower lobe, bronchus intermedius, anterior and posterior junctional lines, and vessels behind the heart and diaphragm. Additionally, subjective general quality impression of each radiograph was graded similarly. Statistical analyses were performed using the chi-square test. A P value less than 0.05 was considered significant. RESULTS: Visibility with the digital images was statistically significantly higher for the carina, left lower lobe bronchus, bronchus intermedius, and vessels behind the heart and diaphragm. Subjective general quality impression of digital radiographs was also higher (P < 0.001). No significant visibility differences were found for pulmonary fissures or junctional lines. CONCLUSION: Subjective visibility of anatomic structures behind the heart and diaphragm and at the hilae is significantly improved with phosphor-based digital radiography compared with conventional screen-film radiography. This suggests that pathologic processes such as pulmonary nodules, masses or consolidations projected over those structures may be more easily and reliably depicted on digital than on conventional chest X-rays.

Accelerated progression of coronary calcification: four-year follow-up in patients with stable coronary artery disease.

PURPOSE: To prospectively assess the 4-year progression rate of coronary artery calcium (CAC) in patients with clinically stable coronary artery disease (CAD) with multi-detector row computed tomography (CT). MATERIALS AND METHODS: The study group consisted of 382 consecutive patients. All underwent baseline dual-sector spiral CT, and CT was repeated at 2 and 4 years later. Progression of CAC was assessed with measurement of the increase in total calcium score (TCS) and with repeated-measures analysis and multivariate linear regression models. Logistic regression model was used to predict incidence of new lesions. RESULTS: Eighty-seven percent (333 of 382) of the study group were men, with mean age of 65 years +/- 11, and 13% (49 of 382) were women, with mean age of 68 years +/- 11. The average TCS increased after 4 years by sixfold from baseline in the 1st quartile, and by four-, two- and 1.5-fold in the 2nd, 3rd, and 4th quartiles of baseline TCS (P <.01), respectively. Multiple linear regression analysis included age; sex; natural logarithm of baseline TCS; history of hypertension, diabetes mellitus, current smoking, hypercholesterolemia, and lipid-lowering therapy with cholesterol synthesis enzyme inhibitor (statin); and family history of premature CAD. Results demonstrated that natural logarithm of baseline TCS and history of current smoking were independent predictors of the 4th-year natural logarithm of TCS levels (R(2) = 0.85, P <.001). New lesions were diagnosed in 56 (15%) patients. History of statin therapy (odds ratio = 0.35; 95% confidence interval [CI]: 0.16, 0.77; P <.01), age with an increment of 5 years (odds ratio = 0.76; 95% CI: 0.64, 0.90; P =.01), and natural logarithm of baseline TCS (odds ratio = 0.73; 95% CI: 0.62, 0.86; P <.01) were independent predictors for new calcific lesions during 4 years. CONCLUSION: Accelerated progression of CAC during 4 years was found in clinically stable patients with CAD.

Papillary adenocarcinoma in a thymic cyst: a pitfall of thoracoscopic excision.

Carcinoma arising in a thymic cyst is very rare. We performed thoracoscopic subtotal resection of an assumed benign cyst. The patient had a reoperation when the diagnosis of papillary adenocarcinoma was established. Complete resection and pleural thermochemotherapy were performed. The patient died 26 months later from systemic metastases. Caution must be exercised when managing nontypical thymic cysts.

Congenital pulmonary venolobar syndrome: spectrum of helical CT findings with emphasis on computerized reformatting.

The term congenital pulmonary venolobar syndrome refers to a wide spectrum of pulmonary developmental anomalies that may appear singly or in combination. The main components of congenital pulmonary venolobar syndrome are hypogenetic lung (including lobar agenesis, aplasia, or hypoplasia), partial anomalous pulmonary venous return, absence of pulmonary artery, pulmonary sequestration, systemic arterialization of lung, absence of inferior vena cava, and accessory diaphragm. The recent introduction of multisection helical computed tomography (CT), combined with use of advanced postprocessing graphic workstations, allows improved noninvasive delineation of complex congenital anomalies. A single fast (5-15-second) CT scan now enables the radiologist to (a) generate angiogram-like images of the anomalous pulmonary arteries and veins; (b) demonstrate tracheobronchial abnormalities by generating simulated bronchographic or bronchoscopic images; and (c) depict associated parenchymal abnormalities on axial, coronal, or sagittal images, which once represented an important advantage of magnetic resonance imaging over CT. Multisection helical CT is a helpful diagnostic tool in the preoperative evaluation of patients with suspected congenital pulmonary venolobar syndrome.

Are referring clinicians aware of patients at risk from intravenous injection of iodinated contrast media?

AIM: The purpose of our study was to assess the level of awareness of referring clinicians to populations at risk for complications of intravascular administration of iodinated contrast media. SUBJECTS AND METHODS: Two hundred and three physicians from three university hospitals completed an anonymous questionnaire regarding risk factors and contraindications to the intravenous administration of iodinated contrast media. The questionnaire included medical conditions with increased risk for anaphylactoid reaction (asthma, hay fever and food allergy) as well as chemotoxic (ischaemic heart disease, phaeochromocytoma and myasthenia gravis) adverse reactions, some with dependence on renal function (metformin treatment, diabetes mellitus and multiple myeloma). Two additional multiple-choice questions addressed pre-medication protocols and risk of nephrotoxicity in diabetic patients. RESULTS: Asthma, food allergy and hay fever were recognized as risk factors by 81.3%, 77.8% and 61.6% of respondents respectively, while ischaemic heart disease, phaeochromocytoma and myasthenia gravis were defined as such only by 9.8%, 30.0% and 28.6% respectively. Metformin treatment, diabetes mellitus and multiple myeloma, in the presence of normal renal function, were considered as risk factors by 46.3%, 38.9% and 58.1% of respondents respectively. One of the generally accepted pre-medication protocols was selected by 89.8%. The risk of nephrotoxicity in a diabetic patient was correctly assessed by 63.5% of respondents. CONCLUSION: We found a relatively high awareness among referring clinicians of a potential anaphylactoid reaction and nephrotoxicity due to iodinated contrast media. However, additional chemotoxic adverse reactions are less well known. Future efforts to improve communication between clinicians and radiologists should be focused in this direction.

Pulmonary alveolar proteinosis: step-by-step perioperative care of whole lung lavage procedure.

BACKGROUND: Pulmonary alveolar proteinosis is a rare disease characterized by the accumulation of surfactant-like material within the alveolar spaces that causes progressive respiratory failure. Improvement can be achieved with whole lung lavage. OBJECTIVE: Our objective was to conduct a study of the feasibility of treating pulmonary alveolar proteinosis in a community hospital. METHODS: Five patients were treated. We assessed procedure pulmonary functions. RESULTS: No major sequelae occurred. Each lung was lavaged with 12 to 20 L of normal saline in cycles of 970 +/- 150 mL each (mean +/- standard deviation), over 106 +/- 49 minutes. Extubation was performed when compliance of the lavaged lung was restored. All patients showed subjective improvement. Resting and exercise oxygen saturation improved within 1 week after the lavage. A significant improvement was also noted in forced expiratory volume in 1 second, forced vital capacity, and maximal oxygen uptake, whereas total lung capacity and carbon monoxide single-breath diffusion capacity remained unchanged. CONCLUSION: Although retrospective and based on a small sample size, our results suggest that whole lung lavage may be performed safely even in medical centers that have limited experience, if strict adherence to a protocol is maintained.