Extrapulmonary Findings Research Articles

Portosystemic Shunting in Superior Vena Cava Obstruction by Tumor Demonstrated on Ventilation-Perfusion Lung Scan

Extrapulmonary deposition of macroaggregated albumin particles on lung perfusion scintigraphy occurs in the presence of right-to-left shunting, as demonstrated in this case of portosystemic shunting related to superior vena cava obstruction by germ cell tumor of the mediastinum. The case demonstrates the importance of examining tracer deposition within the entire field of view on (99m)Tc-macroaggregated albumin perfusion lung scintigraphy for extrapulmonary findings.

Unusual Findings of Langerhans Cell Histocytosis in a Young Asymptomatic Patient: Case Report

Cyst is described on high-resolution computed tomography (HRCT) as focal round area of parenchymal hyperlucency with a well-defined interface with normal lung. A relatively small subset of lung diseases should be suspected when cystic pattern is seen on HRCT. In particular, the differential diagnosis of this pattern relies upon features such as: cranio-caudal distribution, cystic shape, and coexisting pulmonary or extra-pulmonary findings. Pulmonary Langerhans Cell Histocytosis (PLCH) is a smoke-related disease associated with cystic pattern. PLCH is characterized by infiltration of Langerhans cells in pulmonary interstitium with varying pathologic findings and depend on disease stage. The findings usually consist of a heterogeneous combination of lung nodules and cysts with upper lobes predominance. Notably, the HRCT pattern of PLCH is often striking and usually suggests confident diagnosis. Nevertheless, cases with atypical features can be seen. This case report describes the ambiguous imaging findings of a biopsy-proven PLCH.

Long term follow-up of infliximab efficacy in pulmonary and extra-pulmonary sarcoidosis refractory to conventional therapy

IntroductionInfliximab, a humanized, chimeric, monoclonal antibody against tumor necrosis alpha (TNF-α), has been shown to reduce the pulmonary and extra-pulmonary manifestations of sarcoidosis, however, there is little information regarding sustained efficacy with long-term use of infliximab. We retrospectively investigate whether a reduction in disease response is maintained, over a prolonged course of therapy (up to 85 months) with infliximab, and report on adverse events associated with its use. MethodsSubjects with multi-organ sarcoidosis were prescribed infliximab, between January 2000 to June 2010 due to failure of conventional therapy and were identified from the Drexel University College of Medicine sarcoidosis clinic. Retrospective patient reported symptom and objective clinical data analyses of pulmonary and extra-pulmonary findings were evaluated pre-infliximab and post or concurrent infliximab therapy. Any adverse events or reasons for discontinuation during infliximab therapy were reported. ResultsTwenty-six patients with biopsy proven sarcoidosis received anti-TNF therapy and met the criteria for study inclusion. Clinical evidence of sustained resolution or improvement was demonstrated in 58.5% of all organs assessed (p =<0.001). No clinical change in disease activity was seen in 35.8% of all organs evaluated. Despite infliximab treatment, 5.7% had progressive disease activity. Adverse events were seen in 57.7% of patients treated with infliximab over a 46.2 month average duration of therapy. Three (12%) patients had an adverse event that required permanent discontinuation. ConclusionsInfliximab is efficacious in the treatment of extra-pulmonary sarcoidosis and the efficacy is maintained with prolonged treatment. In patients with pulmonary sarcoid, sustained improvement in pulmonary imaging was seen after initiation of infliximab, however, post-treatment pulmonary function testing was not conclusive. Long-term infliximab therapy was well tolerated for our study group.

What a Differential a Virus Makes: A Practical Approach to Thoracic Imaging Findings in the Context of HIV Infection???Part 2, Extrapulmonary Findings, Chronic Lung Disease, and Immune Reconstitution Syndrome

The Centers for Disease Control and Prevention reported more than one million people with HIV infection in the United States in 2006, an increase of 11% over 3 years. Worldwide, nearly 34 million people are infected with HIV. Pulmonary disease accounts for 30-40% of acute hospitalizations of HIV-seropositive patients, underscoring the importance of understanding the range of cardiothoracic imaging findings associated with HIV infection. This article will cover extrapulmonary thoracic diseases, chronic lung diseases, and immune reconstitution inflammatory syndrome in HIV-infected patients. Our approach is focused on the radiologist's perspective by recognizing and categorizing key imaging findings to generate a differential diagnosis. The differential diagnosis can be further refined by incorporating clinical data, such as patient demographics, CD4 count, and presenting symptoms. In addition, with prolonged survival of HIV-infected patients in the era of highly active antiretroviral therapy, radiologists can also benefit from awareness of imaging features of a myriad of chronic cardiopulmonary diseases in this patient population. Finally, the change of imaging findings and clinical status in response to treatment provides important diagnostic information, such as in immune reconstitution syndrome. Developing a practical approach to key cardiothoracic imaging findings in HIV-infected patients will aid the radiologist in generating a clinically relevant differential diagnosis and interpretation, thereby improving patient care.

Extrapulmonary Malignancies Detected at Lung Cancer Screening

To retrospectively assess the detection rate, histologic characteristics, and clinical stage of screening-detected extrapulmonary malignancies in a population at high risk for lung cancer. In this institutional review board-approved study, 5201 asymptomatic heavy smokers aged 50 years or older underwent annual low-dose computed tomography (CT) for 5 consecutive years. The 5-year cumulative effective dose was 5 mSv. Subjects with at least one "potentially significant extrapulmonary incidental finding" (PS-IF) were extracted from the study database. An extrapulmonary finding was classified as potentially significant if it required further diagnostic and/or clinical evaluation. In retrospect all clinically relevant information, including findings from diagnostic work-up and final diagnosis of the PS-IF, was collected. On the basis of the information collected, only histologically proved screening-detected extrapulmonary malignancies were eventually included in this study. The percentages of volunteers with extrapulmonary malignancies were calculated, along with 95% confidence intervals (CIs), on the basis of a binomial distribution. After 5 years of CT screening, 27 unsuspected extrapulmonary malignancies were diagnosed, representing 0.5% (27 of 5201 subjects; 95% CI: 0.34%, 0.75%) of volunteers enrolled and 6.2% (27 of 436 findings; 95% CI: 4.12%, 8.88%) of PS-IFs. Eight malignancies were diagnosed at the 1st year of screening, nine at the 2nd year, four at the 3rd year, two at the 4th year, and four at the 5th year. Twelve of the 27 extrapulmonary tumors (44%) were renal carcinomas (n = 7) or lymphomas (n = 5). Twenty-four of the 27 subjects with a malignancy were alive at the most recent follow-up. A considerable number of unsuspected extrapulmonary malignancies can be detected in lung cancer screening trials. A careful evaluation of extrapulmonary structures, with particular attention to the kidneys and lymph nodes, is recommended.

Rapid clinical diagnosis of Legionnaires' disease during the “herald wave” of the swine influenza (H1N1) pandemic: The Legionnaires' disease triad

BackgroundIn adults hospitalized with atypical community-acquired pneumonia (CAP), Legionnaires' disease is not uncommon. Legionnaire's disease can be differentiated from typical CAPs and from other atypical CAPs based on its characteristic pattern of extrapulmonary organ involvement. The first clinically useful diagnostic weighted point score system for the clinical diagnosis of Legionnaires' disease was developed by the Infectious Disease Division at Winthrop-University Hospital in the 1980s. It has proven to be diagnostically accurate and useful for more than two decades, but was time-consuming. Because Legionella spp. diagnostic tests are time-dependent and problematic, a need was perceived for a rapid, simple way to render a clinical, syndromic diagnosis of Legionnaires' disease pending Legionella test results. During the “herald wave” of the swine influenza (H1N1) pandemic in the New York area, our hospital, like others, was inundated with patients who presented to the Emergency Department with influenza-like illnesses (ILIs) for H1N1 testing/evaluation. Most patients with ILIs did not have swine influenza. Hospitalized patients with ILIs who tested positive with rapid influenza diagnostic tests (RIDTs) were placed on influenza precautions and treated with oseltamivir. Unfortunately, approximately 30% of adult patients admitted with an ILI had negative RIDTs. Because the definitive laboratory diagnosis of H1N1 pneumonia by reverse transcription-polymerase chain reaction(RT-PCR), testing was restricted by health departments, resulted in clinical and infection control dilemmas in determining which RIDT-negative patients did, in fact, have H1N1 pneumonia.ObjectiveAccordingly, a diagnostic weighted point score system was developed for H1N1 pneumonia patients, based on RT-PCR positivity by the Infectious Disease Division at Winthrop-University Hospital. This diagnostic point score system for hospitalized adults with negative RIDTs was time-consuming. As the pandemic progressed, a simplified diagnostic swine influenza (H1N1) triad was developed for the rapid clinical diagnosis of probable H1N1 pneumonia, which also differentiated it from its mimics as well as from bacterial pneumonia, eg, Legionnaires' disease. During the “herald wave” of the H1N1 pandemic, we noticed an unexplained increase in Legionnaires' disease CAPs. Because clinical resources were stressed to the maximum during the pandemic, it was critically important to rapidly identify patients rapidly with Legionnaire's disease who did not require influenza precautions or oseltamivir, but who did require anti-Legionella antimicrobial therapy.MethodsBased on the Winthrop-University Hospital Infectious Disease Division's diagnostic weighted point score system for Legionnaires' disease (modified), key indicators were identified and became the basis for the diagnostic Legionnaires' disease triad. The diagnostic Legionnaires' disease triad was used to make a clinical diagnosis of Legionnaires' disease until the results of Legionella diagnostic tests were reported. The diagnostic Legionnaires' disease triad diagnosed Legionnaires' disease in hospitalized adults with CAPs with extrapulmonary findings (atypical CAP) and relative bradycardia, accompanied by any three (ie, a triad) of the following: otherwise unexplained relative lymphopenia, early/mildly elevated serum transaminases (SGOT/SGPT), highly increased ferritin levels (≥2 × n), or hypophosphatemia. The diagnostic Legionnaires' disease triad provides clinicians with a rapid way to clinically diagnose Legionnaires' disease, pending Legionella test results.ResultsThe accuracy of the diagnostic Legionnaires' disease triad was confirmed in our 9 cases of Legionnaires' disease by subsequent Legionella diagnostic testing.ConclusionsThe diagnostic Legionnaires' disease triad is particularly useful in situations where a rapid clinical syndromic diagnosis is needed, ie, during an H1N1 pandemic.

Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Complicated With Diffuse Alveolar Hemorrhage

To summarize the clinical features and therapeutic response of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis complicated with diffuse alveolar hemorrhage (DAH). A retrospective chart review of the patients having ANCA-associated vasculitis with DAH was made with regard to their clinical symptoms, laboratory test results, responses to therapy and outcomes. During January 1994 to June 2007, 131 ANCA-associated vasculitis patients were admitted to Peking Union Medical College Hospital. During this period, 12 of these cases consisting of 9 males and 3 females with a mean age of 59.9 +/- 16.7 years developed DAH. The mean duration of vasculitis before the onset of DAH was 9.0 +/- 14.3 months (range: 0-48 months). Dyspnea was the most consistent presenting symptom, while frank hemoptysis occurred in only 5 cases at the onset of DAH. The most common extrapulmonary findings associated with DAH were glomerulonephritis (100%). Symptoms including fever and arthralgia-myalgia (91.7%) as well as complications involving gastrointestinal (41.7%), mucocutaneous (25%), ear-nose-throat (25%), cardiovascular (16.7%), and nervous system (16.7%) were also frequently seen. The Birmingham Vasculitis Activity Score at the onset of DAH was 21.8 +/- 4.9. All patients were treated with systemic corticosteroids combined with cyclophosphamide including methylprednisolone pulse therapy in 7 patients. In addition, plasmapheresis (41.7%), dialysis (25%), and mechanical ventilation (41.7%) were applied. The overall mortality rate was 58.3% (7 patients). Three patients died of fulminant DAH and respiratory failure during the first week of treatment. The other 4 patients died of septic shock (2 patients), severe heart failure (1 patient), and systemic fungus infection with septic shock (1 patient) 10 to 32 days after the onset of DAH. DAH is the most serious complication of ANCA-associated vasculitis. The episode of DAH always occurs simultaneously with multiple system involvement. The most constant signs are newly developed dyspnea and new infiltration of bilateral lungs. Prompt bronchoalveolar lavage can be helpful for timely diagnosis of the patients without overt hemoptysis and a useful tool to exclude pulmonary infection.

Severe Q fever community-acquired pneumonia (CAP) mimicking Legionnaires' disease: Clinical significance of cold agglutinins, anti-smooth muscle antibodies and thrombocytosis

Atypical community-acquired pneumonia (CAP) may be caused by zoonotic or nonpulmonary pathogens. However, atypical pathogens are systemic infectious disease accompanied by pneumonia in contrast with typical bacterial pathogens with infection limited to the lungs and absent extrapulmonary findings. Clinically and radiologically, the atypical CAP pathogens that most closely resemble each other are psittacosis, Q fever, and Legionnaires' disease. Psittacosis can usually be readily suspected or eliminated on the basis of a recent psittacine bird contact history. The 2 atypical pneumonias that most closely resemble each other clinically are Q fever and Legionnaires' disease. The epidemiology of Q fever is related to livestock, and sporadic cases are related to contact to parturient cats. In nonendemic areas, Q fever CAP mimics Legionnaires' disease most closely. Both Q fever and Legionella CAP have several clinical and laboratory features in common. However, there are subtle but important differences that allow the astute clinician to differentiate between these 2 disorders on the basis of clinical and nonspecific laboratory findings before definitive diagnostic tests results are reported. We report a case of severe Q fever CAP mimicking Legionnaires' disease in a young adult normal host. Her initial zoonotic contact history was negative, and her clinical presentation suggested Legionnaires' disease as the most likely diagnosis. Against the diagnosis of Legionnaires' disease was the patient's age and occurrence of the disease in spring time. In contrast, Legionnaires' disease is usually an infection of older individuals and occurs in late summer/fall. Although the patient did not have splenomegaly, a common finding in Q fever CAP, she did have mild hepatomegaly. Hepatomegaly is a uncommon in Q fever CAP but is not a feature of Legionnaires' disease. In the absence of a positive zoonotic contact history, the cardinal findings pointing to the diagnosis of Q fever in this case were "multiple round opacities" on chest computed tomography scan and thrombocytosis during her hospitalization. Against the diagnosis of Legionnaires' disease was the absence of hypophosphatemia and highly elevated ferritin levels. In patients with atypical pneumonias in whom the clinical presentation and nonspecific laboratory findings suggest Legionnaires' disease, but in addition have findings not associated with Legionnaires' (eg, hepatomegaly, thrombocytosis), Q fever serology should be ordered. We conclude that Q fever may closely mimic Legionnaires' disease. Severe atypical CAP with "multiple round opacities" on chest x-ray/computed tomography chest scan with elevated anti-smooth muscle antibodies or thrombocytosis should suggest the diagnosis of Q fever and prompt specific testing for Q fever. Rarely, Q fever CAP may be associated with elevated cold agglutinin titers.

Q fever bioprosthetic aortic valve endocarditis (PVE) successfully treated with doxycycline monotherapy

Q fever is a zoonotic infection caused by Coxiella burnetii. The most common clinical manifestation of acute Q fever infection is as an atypical community-acquired pneumonia. The pulmonary findings are accompanied by extrapulmonary findings, most typically an increase in serum transaminases and splenomegaly. Because C. burnetii is difficult to culture, the diagnosis of Q fever is usually made serologically. The diagnosis of acute Q fever atypical community-acquired pneumonia is made by demonstrating a fourfold or greater increase in titer between acute and convalescent specimens or by demonstrating elevated immunoglobulin (IgM) (phase II) titers. Chronic Q fever is manifested as granulomatous hepatitis or more commonly as culture-negative endocarditis (CNE). Chronic Q fever (CNE) is a difficult diagnosis because of difficulty in culturing the organism from the blood and the vegetations with Q fever CNE are small or absent. The diagnosis of chronic Q fever CNE is based on serology. Such patients commonly have highly elevated IgM and IgG titers (phase I/II) titers. Chronic Q fever CNE may involve native or prosthetic heart valves. Q fever prosthetic valve endocarditis is rare compared with native valve Q fever endocarditis. Q fever prosthetic valve endocarditis usually requires valve replacement for cure. We present a case of chronic Q fever bioprosthetic aortic valve endocarditis that was successfully treated with doxycycline monotherapy that did not require aortic valve replacement.

Q Fever Community-Acquired Pneumonia in a Patient With Crohn’s Disease on Immunosuppressive Therapy

Community-acquired pneumonia (CAP) may be caused by typical or atypical pathogens. The three most common zoonotic atypical pathogens are Chlamydophila psittaci (psittacosis), Francisella tularensis (tularemia), and Coxiella burnetii (Q fever). Atypical CAPs are suggested by a distinctive pattern of extrapulmonary organ involvement. Zoonotic CAP may be differentiated from nonzoonotic CAP (Chlamydia pneumoniae, Mycoplasma pneumoniae, Legionnaire's disease) by a recent zoonotic vector contact history. Zoonotic atypical CAP occurs sporadically, but not randomly, and require close association with the appropriate zoonotic vector to transmit the infection. CAP accompanied by the extrapulmonary finding of splenomegaly in a normal host limits differential diagnostic possibilities to Q fever and psittacosis. Splenomegaly does not occur with other typical or atypical CAP. Another common extrapulmonary finding occurs with some atypical pneumonias, that is, Q fever, psittacosis, and Legionnaire's disease is early mild/transient elevations of serum transaminases indicative of (hepatic) extrapulmonary organ involvement. The case presented is a middle-aged man with longstanding Crohn's disease who was further immunosuppressed by chronic prednisone therapy. The patient presented with CAP and extrapulmonary findings, that is, splenomegaly and increased serum transaminases. He denied recent contact with birds or animals. Because Crohn's disease and Q fever CAP may be accompanied by splenomegaly, the cause of his splenomegaly was a diagnostic dilemma. The patient was treated with levofloxacin. Serologic tests for atypical pathogens (Q fever, psittacosis, Legionnaire's disease, C. pneumoniae, and M. pneumoniae) were ordered. Enzyme-linked immunosorbent assay serology for Q fever was positive with elevated acute immunoglobulin-M (phase II) titers. Re-questioning of the patient revealed a recent exposure to a neighbor's parturient cat, providing the necessary zoonotic vector contact history for Q fever. The patient responded to levofloxacin, which resulted in resolution of the patient's symptoms, right lower lobe pneumonia, and splenomegaly. Because a prior abdominal computed tomography scan indicated no splenomegaly and his splenomegaly resolved with antimicrobial therapy, the splenomegaly was related to Q fever CAP.

DHORI VIRUS (ORTHOMYXOVIRIDAE: THOGOTOVIRUS) INFECTION IN MICE: A MODEL OF THE PATHOGENESIS OF SEVERE ORTHOMYXOVIRUS INFECTION

After intranasal, subcutaneous, or intraperitoneal infection with Dhori virus (DHOV), adult mice developed a fulminant and uniformly fatal illness with many of the clinical and pathologic findings seen in mice infected with H5N1 highly pathogenic avian influenza A virus. Histopathologic findings in lungs of DHOV-infected mice consisted of hemorrhage, inflammation, and thickening of the interstitium and the alveolar septa and alveolar edema. Extra-pulmonary findings included hepatocellular necrosis and steatosis, widespread severe fibrinoid necrosis in lymphoid organs, marked lymphocyte loss and karyorrhexis, and neuronal degeneration in brain. Similar systemic histopathologic findings have been reported in the few fatal human H5N1 cases examined at autopsy. Because of the relationship of DHOV to the influenza viruses, its biosafety level 2 status, and its similar pathology in mice, the DHOV-mouse model may offer a low-cost, relatively safe, and realistic animal model for studies on the pathogenesis and management of H5N1 virus infection.

CT of the chest in the evaluation of idiopathic pulmonary arterial hypertension in children

Idiopathic pulmonary arterial hypertension (IPAH) is a rare disease in children. By definition it is a diagnosis of exclusion, and CT of the chest is primarily performed to exclude other causes. Previous studies have defined CT features suggestive of the diagnosis of IPAH, but these have all been limited to the adult population. Contrast-enhanced chest CT and high-resolution CT findings in IPAH were evaluated in an attempt to define features consistently seen in children with this condition. The chest CT scans performed at initial presentation were reviewed in 17 children with echocardiographic or angiographic evidence of IPAH. There were nine boys and eight girls, ranging in age from 1 month to 17 years. The extrapulmonary findings included cardiomegaly with right-sided cardiac enlargement, which was seen in 13 children. The central pulmonary arteries were enlarged in 15 children, with peripheral enlargement in two. In six children this resulted in bronchial compression. In addition, mediastinal and hilar lymphadenopathy was noted in three children. Prominent intrapulmonary features included a peripheral vasculopathy, with enlarged tortuous vessels, seen in eight children. Ill-defined ground-glass centrilobular opacities were also noted in eight children, representing the most common parenchymal abnormality. Other findings included septal lines in five, diffuse ground-glass opacification in four and focal hyperlucent zones in three. Mosaic attenuation was seen in one child. A variety of imaging findings are identified in IPAH. Features particularly consistent with the diagnosis include peripheral vasculopathy and centrilobular opacities in the setting of cardiomegaly and central pulmonary arterial enlargement.

Extrapulmonary findings detected in a low-dose spiral computed tomography (sCT) early detection study for lung cancer (LC)

7206 Background: In 2001 a feasibility study testing low-dose sCT as an early diagnosis tool in asymptomatic volunteers at high risk for lung cancer was planned and initiated at our institution with an evaluation at baseline and then annually for 5 years. Subjects &amp; Methods: We enrolled 520 asymptomatic volunteers, 73% males, aged 55 years or older (median age 60 yrs), with at least 20 pack-years of cigarette smoking and no previous cancer. Results: At baseline, one hundred forty-four extra-pulmonary findings (28%) were detected. The more common findings were: radiologically-detected adrenal enlargements (19%); thyroid struma (8%); hepatic abnormalities, like angiomas, cysts, calcifications, steatosis (29%); renal alterations, like cysts, lithiasis, vascular calcifications and one case of stage I renal carcinoma (21%); spleen abnormalities, like cysts, calcifications, accessory spleen (5%); cholelithiasis (4%); pancreatic alterations (1%); thymic nodules (1%); axillary adenopathy (5%); aortic ectasia or aneurysm (5%) and one breast nodule. In 2 cases (renal carcinoma and breast nodule) a surgical resection was performed. All these findings were followed up through a letter to the general practitioner. In 62 subjects the initial findings were considered worth of further imaging investigations or were suggestive for a clinically definite disease (e.g. renal carcinoma). In the remaining 82, mainly based on personal history data, the abnormalities were already known and previously diagnosed. Globally, the cumulative number of extrapulmonary findings from 2nd to 5th year was 275 and the more common findings were: cysts (hepatic 9%, renal 13%), lithiasis (renal 1%, hepatic 5%), thyroid struma 4%, adrenal adenomas 3%, vascular calcifications 37%, aneurysm 3%, vertebral artropathy 2%. Conclusions: Even if early detection of lung cancer will result in a survival benefit, in the cost-effectiveness analyses the additional costs from the detection of extra-pulmonary findings should also be carefully considered because in many cases they may request substantial outpatient clinic admissions and further diagnostic investigations. No significant financial relationships to disclose.

Blastomycosis in a Young African Man Presenting with a Pleural Effusion

Blastomyces dermatitidis is a dimorphic fungus endemic to north-western Ontario, Manitoba and some parts of the United States. The fungus is also endemic to parts of Africa. Pulmonary and extrapulmonary findings of a 24-year-old African man who presented with weight loss, dry cough and chronic pneumonia not resolving with antibiotic treatment are presented. The unusual occurrence of pulmonary blastomycosis associated with skin lesions and a moderate pleural effusion is reported.

P-267 Extrapulmonary findings in a low-dose spiral CT scan early detection study for lung cancer in a high risk population

P-267 Extrapulmonary findings in a low-dose spiral CT scan early detection study for lung cancer in a high risk population

Pulmonary involvement with Erdheim-Chester disease: radiographic and CT findings.

We determined the chest radiographic and CT findings of Erdheim-Chester disease with pulmonary involvement. We retrospectively reviewed the radiologic images of 15 patients with biopsy-proven Erdheim-Chester disease. Nine patients had chest radiographic images and CT scans that were available for review. Six men and three women were studied (age range, 25-70 years; mean age, 56 years). Two radiologists interpreted all images by consensus. Lung parenchyma was assessed for the type and distribution of disease. Bronchi, pleurae, hila, and mediastinal and extrathoracic structures were evaluated for abnormalities. Pathologic specimens from all patients were reviewed and correlated with radiologic findings. Eight of nine patients had thoracic images with abnormal findings. The most common radiographic pattern was reticular interstitial opacities with fissural and interlobular septal thickening. CT revealed regions of ground-glass attenuation and centrilobular nodular opacities. Typically, extrapulmonary findings included pleural effusions (6/8 patients), pericardial fluid or thickening (4/8), and extrathoracic infiltrative soft-tissue masses (4/8). The most common findings of Erdheim-Chester disease with pulmonary involvement include an interstitial process characterized by smooth interlobular septal thickening and centrilobular nodular opacities, fissural thickening, and pleural effusions. On CT, six of nine patients had pericardial fluid and thickening or extrathoracic soft-tissue masses. Such findings are characteristic of Erdheim-Chester disease with pulmonary involvement. Definitive diagnosis requires correlating skeletal findings and lung biopsy findings.

Medico-legal issues in occupational lung disease litigation

We present a case of a 76 year-old, non-smoking Honduran female who was referred to our clinic for years of persistent dry cough. Cardiac evaluation was unremarkable. She denied symptoms of heartburn, allergic rhinitis, and there was no personal or family history of asthma. Her physical exam demonstrated wheezing over the right mid-posterior chest. Spirometry was within normal limits. CT-imaging of the chest demonstrated right middle lobe bronchus and lingular segmental bronchus narrowing with bibasilar atelectasis and mild interlobular septal thickening with prominent mediastinal adenopathy. Bronchoscopy showed diffuse airway hyperpigmentation, right middle lobe medial segmental bronchial stenosis and lingular segmental bronchial stenosis. Endobronchial ultrasound demonstrated enlarged mediastinal lymph nodes, and transbronchial needle aspirates revealed necrotic tissue with black anthracotic pigment. Cultures were negative. Post-procedurally, the patient revealed regular use of a wood stove in an enclosed, poorly-ventilated kitchen. Anthracosis is the black discoloration of bronchial mucosa due to deposition of carbon-based particles in the airway, usually related to chronic environmental exposures. It can eventually result in endobronchial and parenchymal fibrosis and is mostly seen in non-smokers. Burning biomass fuel from wood smoke is a risk factor in the developing world. Symptoms include cough, dyspnea, and wheezing. Spirometry will usually demonstrate obstruction. CT-based imaging can show intraluminal narrowing, predominantly affecting the right middle and right upper lobe bronchi, resulting in distal segmental collapse. Extrapulmonary findings are rare, but usually are in the form of calcified mediastinal adenopathy on CT-based imaging, which allows for differentiation from lung cancer. There is no reliable treatment though bronchodilators can achieve clinical benefit.

Extrapulmonary Sites of Radiogallium Accumulation in Sarcoidosis

In an effort to detect extrapulmonary sites of radiogallium accumulation in cases of sarcoidosis, 145 separate Ga-67 citrate studies of 114 patients with biopsy-proven sarcoidosis were examined. The most characteristic extrapulmonary radiogallium uptake pattern was the "panda sign" in 47 patients (41%). The most common site of prominent extrapulmonary radiogallium uptake was the lacrimal glands in 101 patients (88%). Second most common was activity in one or more superficial lymph node regions such as the cervical, axillary, femoral, or inguinal in 19 patients (17%). Other extrapulmonary sites included breast uptake in 6 out of 80 women (8%), prominent splenic and nasal uptake in 9 (8%) patients, periportal accumulation in 7 (6%), and cutaneous/subcutaneous activity in 4 (4%). Because many of these individuals were receiving corticosteroids, the natural (untreated) prevalence of extrapulmonary findings may be even higher. Although the sensitivity and specificity of extrapulmonary radiogallium accumulation has still to be determined, many of the sites may be accessible to biopsy both for diagnostic purposes and to follow the effects of medications. It is therefore suggested that whole-body imaging be performed when radiogallium is administered to patients with suspected or known sarcoidosis.

Sporadic Legionnaires' Disease: Clinical Observations on 87 Nosocomial and Community-Acquired Cases

To add information about sporadic Legionnaires' disease, 87 cases of L. pneumophila pneumonia were reviewed. Twenty cases were nosocomial infections and 67 cases were community-acquired. Most cases (64%) occurred between July and October.The mean age of patients was 51.4 years and males outnumbered females 2.5:1.0. Thirty-one percent of patients were receiving corticosteroid, immunosuppressive, or antineoplastic chemotherapy when illness began. Immunosuppression at onset of illness was more common in nosocomial infections (90%) than in community-acquired infections (14%). Seventy percent of patients had underlying diseases. Malignancies, renal failure, and transplantation were the most common conditions underlying nosocomial infections. Chronic lung disease and malignancies were the most common diseases underlying community-acquired infections. The case-fatality rate in nosocomial infection (70%) was greater than that in community-acquired disease (22%).Clinical, laboratory, and radiologic features of the cases were examined. Illness ranged from mild to severe. Extra pulmonary findings of encephalopathy and renal failure were more common in fatal than in non-fatal cases. Indirect immunofluorescent and microagglutination antibody responses plateaued by the fourth week of illness.Twenty-nine patients died. The case-fatality rate of patients receiving erythromycin (6%) was less than that of patients receiving penicillin (36%), ampicillin (28%), cephalosporin (32%), or aminoglycosides (41%). Despite erythromycin therapy, the case fatality rate for nosocomial L. pneumophilia pneumonia was unacceptably high (25%).