Pneumoconiosis with a Sarcoid-Like Reaction Other than Beryllium Exposure: A Case Report and Literature Review.

Background: Chronic beryllium disease (CBD) is a granulomatous disease that resembles sarcoidosis but is caused by beryllium. Clinical manifestations similar to those observed in CBD have occasionally been reported in exposure to dusts of other metals. However, reports describing the clinical, radiographic, and pathological findings in conditions other than beryllium-induced granulomatous lung diseases, and detailed information on mineralogical analyses of metal dusts, are limited. Case presentation: A 51-year-old Japanese man with rapidly progressing nodular shadows on chest radiography, and a 10-year occupation history of underground construction without beryllium exposure, was referred to our hospital. High-resolution computed tomography showed well-defined multiple centrilobular and perilobular nodules, and thickening of the intralobular septa in the middle and lower zones of both lungs. No extrathoracic manifestations were observed. Pathologically, the lung specimens showed 5-12 mm nodules with dust deposition and several non-necrotizing granulomas along the lymphatic routes. X-ray analytical electron microscopy of the same specimens revealed aluminum, iron, titanium, and silica deposition in the lung tissues. The patient stopped smoking and changed his occupation to avoid further dust exposure; the chest radiography shadows decreased 5 years later. Conclusion: The radiological appearances of CBD and sarcoidosis are similar, although mediastinal or hilar lymphadenopathy is less common in CBD and is usually seen in the presence of parenchymal opacities. Extrathoracic manifestations are also rare. Despite limited evidence, these findings are similar to those observed in pneumoconiosis with a sarcoid-like reaction due to exposure to dust other than of beryllium. Aluminum is frequently detected in patients with pneumoconiosis with a sarcoid-like reaction and is listed as an inorganic agent in the etiology of sarcoidosis. It was also detected in our patient and may have contributed to the etiology. Additionally, our case suggests that cessation of dust exposure may contribute to improvement under the aforementioned conditions.

Beriliosis: una granulomatosis para no olvidar la importancia de la historia laboral / Berylliosis: A Granulomatosis to Remind Us of the Importance of Occupational History

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Beriliosis, la enfermedad que se esconde tras algunas sarcoidosis / Chronic beryllium disease, the disease hidden under some sarcoidosis

La enfermedad crónica por berilio (BC) es una enfermedad pulmonar ocupacional causada por una reacción de hipersensibilidad retardada a dicho metal. El número de trabajadores expuestos a berilio (Be) se ha incrementado en los últimos años (en Estados Unidos ha pasado de 30.000 en 1970 a 200.000 apenas 30 años después) 1. Estudios transversales llevados a cabo en trabajadores expuestos en distintos tipos de industrias han encontrado prevalencias de la enfermedad que varían de 0% a 7,8%2. En este contexto es llamativo el escaso número de beriliosis que se diagnostican, probablemente porque muchas de ellas son erróneamente diagnosticadas de sarcoidosis

Chronic beryllium disease (CDB) is an occupational retarded hypersensitivity disease elicited by beryllium exposure. The number of exposed workers has increased in the last years. (in the USA has risen from 30,000 in the 1970s to 200,000 thirty years later)1. Cross-sectional studies of exposed workers in various industries have found that the prevalence of this illness ranged from 0.0 to 7.8%2. In this context, it is remarkable that CBD is only rarely diagnosed, probably because many of them are misdiagnosed as sarcoidosis

Prostate-Specific Membrane Antigen-Positive Manifestations of Chronic Beryllium Lung Disease.

Three years after prostatectomy, a 78-year-old man with initial high-risk prostate cancer and new increasing prostate-specific antigen levels underwent Ga-prostate-specific membrane antigen (PSMA) PET/CT. Imaging revealed PSMA-positive pelvic, ascending retroperitoneal and left supraclavicular lymph nodes consistent with metastases. Additionally, there was PSMA-positive lymphadenopathy (hilar and mediastinal) and pulmonary changes (fibrotic and nodular) in which histopathology excluded metastases. A lymphocyte proliferation test was indicative of beryllium sensitization. Chronic beryllium lung disease-a multisystem granulomatous inflammatory disease mainly affecting the lungs-could be established in this patient with occupational exposure to beryllium. This case shows that manifestations of berylliosis can also be PSMA positive.

[Case Report with Differential Diagnostic Aspects of Sarcoidosis]. / Differenzialdiagnostische Aspekte der Sarkoidose anhand eines Fallberichts.

A 73-year-old non-atopic patient had developed at the age of 29 shortness of breath on exertion, general malaise, enlarged axillary lymph nodes and nodular cutaneous eruptions. Based on the presence of bihilar lymphadenopathy, the diagnosis of sarcoidosis was made at that time without any histological investigations and without taking detailed case history. Administration of systemic steroids resulted in remission. However, 12 years later, there was a relapse with alterations of lung parenchyma, followed by a more chronic course of the disorder. Since this relapse, an obstructive-restrictive ventilation defect requiring treatment has persisted till today. About five years ago and at the insistence of the patient, clarifying diagnostics were performed. The case shows the important role of a detailed case history including occupational history. Its failure not only led to disadvantages to the patient but also to incorrect social insurance handling and missing appropriate preventive measures with regard to co-workers.

Thoracic lymphadenopathy in benign diseases: A state of the art review.

Lymphadenopathy is a common radiological finding in many thoracic diseases and may be caused by a variety of infectious, inflammatory, and neoplastic conditions. This review aims to describe the patterns of mediastinal and hilar lymphadenopathy found in benign diseases in immunocompetent patients. Computed tomography is the method of choice for the evaluation of lymphadenopathy, as it is able to demonstrate increased size of individual nodes, abnormalities of the interface between the mediastinum and lung, invasion of surrounding fat, coalescence of adjacent nodes, obliteration of the mediastinal fat, and hypo- and hyperdensity in lymph nodes. Intravenous contrast enhancement may be needed to help distinguish nodes from vessels. The most frequent infections resulting in this finding are tuberculosis and fungal disease (particularly histoplasmosis and coccidioidomycosis). Sarcoidosis is a relatively frequent cause of lymphadenopathy in young adults, and can be distinguished from other diseases - especially when enlarged lymph nodes are found to be multiple and symmetrical. Other conditions discussed in this review are silicosis, drug reactions, amyloidosis, heart failure, Castleman's disease, viral infections, and chronic obstructive pulmonary disease.

Chronic beryllium disease: computed tomographic findings.

Chronic beryllium disease is a rare multisystem granulomatous disease predominantly involving the lungs and resulting from an immunologic response to long-term occupational exposure. Computed tomography of the chest reveals important lung parenchymal and mediastinal findings and plays an important role in the diagnosis and follow-up assessment of patients with chronic beryllium disease. Its significance lies in the exact localization and evaluation of the extent of lesions. We present an overview of the subject and a pictorial review of the spectrum of computed tomographic features of beryllium disease.

Uncommon occupational lung diseases: high-resolution CT findings.

OBJECTIVE: The purpose of this article is to describe the high-resolution CT (HRCT) features of uncommon occupational lung diseases. CONCLUSION: HRCT plays an increasing role in the evaluation of occupational lung diseases. We present several cases of unusual occupational lung diseases and their HRCT findings. The diseases studied were siderosis, talcosis, berylliosis, calcicosis, hypersensitivity pneumonitis (due to wheat flour and isocyanates), and Ardystil syndrome. The characteristic HRCT findings together with clinical features and related occupational history improve the diagnostic accuracy of these diseases.

Long-term complications and prognosis of chronic beryllium disease.

BACKGROUND: Chronic beryllium disease (CBD) is a rare disease, and there are no previous reports that have followed CBD patients over several decades. Thus, the long-term complications and prognosis of this illness still remain unclear. OBJECTIVE: The aim of this study was to investigate long-term complications and prognosis of CBD patients. STUDY DESIGN AND METHODS: This was a retrospective study based on the medical records of all CBD patients diagnosed at Kyoto University Hospital between the period 1973 to the present day. Ultimately, ten patients whose diagnoses had been made during the period 1973 to 1977 were included. Long-term physiological and radiological change, complications and prognosis of these patients were investigated. RESULTS: Three patients completely remitted, and one died of cor-pulmonale. Among the remaining six patients, four have been followed up for more than thirty years in our institute. The majority developed mixed patterns of lung function impairment, cavity lesions of the lung, pneumothorax, and respiratory infections. CONCLUSIONS: Long-term prognosis of CBD was poor with several complications due to chronic parenchymal and airway lesions.

Current treatment of chronic beryllium disease.

The current mainstay of management of chronic beryllium disease involves cessation of beryllium exposure and use of systemic corticosteroids. However, there are no randomized controlled trials to assess the effect of these interventions on the natural history of this disease. Despite this limitation, it is prudent to remove patients with chronic beryllium disease from further exposure and consider treating progressive disease early with long-term corticosteroids. The effect of treatment should be monitored using pulmonary function tests and high-resolution computed tomography of the chest. However, once pulmonary fibrosis has developed, corticosteroid therapy cannot reverse the damage.

Beryllium sensitization and lung function among former workers at the Nevada Test Site.

BACKGROUND: Beryllium use at the Nevada Test Site (NTS) was not acknowledged until the late 1990's. Subsequently, the ongoing U.S. DOE funded medical screening program, which began in 1998, started testing former workers of the NTS for beryllium sensitization (BeS) in 2001 to identify individuals who may be at higher risk of developing chronic beryllium disease (CBD). METHODS: An observational study was conducted to identify work-related factors associated with the odds of having BeS. Work history questionnaires were administered and principal components analysis was used to identify categories of related tasks associated with BeS. RESULTS: Of the 1,786 former workers tested for BeS, 23 had a confirmed positive result. An increased risk of BeS was found among workers who performed cleanup (OR = 2.68, 95% CI: 1.10, 6.56) and those who worked in Building B where beryllium parts were machined (OR = 2.52, 95% CI: 1.02, 6.19), though no significant increased risk was found when categories of related tasks were used. Additionally, the number of years worked at the NTS was associated with increased risk of BeS. There was no difference in pulmonary function, chest X-ray abnormalities, or respiratory symptoms between those who were sensitized and normal. CONCLUSIONS: The prevalence of BeS among former workers of the NTS who participated in our screening program was 1.3%. Former workers who performed specific job tasks may be at greater risk of developing BeS.

Ground-glass computed tomography pattern in chronic beryllium disease: pathologic substratum and evolution.

Five cases of chronic beryllium disease with predominant or isolated diffuse ground-glass lung opacities on computed tomography are reported with correlation to lung pathology. The ground-glass attenuation was either homogeneous and isolated (n = 1) or patchy and clearly predominant (n = 4) over linear and nodular opacities. In 4 cases, histologic samples showed a prominent diffuse involvement of the alveolar-capillary walls by florid granulomas. Evolution under corticosteroid was remarkable by the appearance of small-sized cysts in 3 cases.

[Asthma, alveolitis, aspergillosis, berylliosis. What to do when there is allergic reaction of the lung?]. / Asthma, Alveolitis, Aspergillose, Berylliose. Was tun, wenn die Lunge allergisch reagiert?

Among the major allergic pulmonary disorders are bronchial asthma, extrinsic allergic alveolitis, allergic aspergillosis and berylliosis. Asthma is diagnosed on the basis of clinical symptoms (wheezing, respiratory distress, tight chest, coughing) and lung function tests possibly supplemented by allergic and provocative testing. Asthma treatment is differentiated into long-term medication and as-required medication. Specific immunotherapy is considered the sole causal therapy. Extrinsic allergic alveolitis is work- or hobby-related (farmer's/cheese worker's/bird-fancier's lung) and manifests as diffuse pneumonitis with dyspnea, coughing and fever. For the diagnosis, the antigen provocative test in particular plays a major role. In the main, treatment comprises strict avoidance of allergens. The diagnosis of allergic pulmonary aspergillosis is based on the history, clinical findings, skin tests, serology and radiography. Treatment is stage-related by means of immunosuppressive agents. In terms of radiographic and pulmonary function findings, berylliosis is similar to sarcoidosis. Here, too, immunosuppressive agents are to the fore.

Clinical approach to chronic beryllium disease and other nonpneumoconiotic interstitial lung diseases.

Exposures in the workplace result in a diverse set of diseases ranging from the pneumoconiosis to other interstitial lung diseases to acute lung injury. Physician awareness of the potential disease manifestations associated with specific exposures is important in defining these diseases and in preventing additional disease. Most occupational diseases mimic other forms of lung disease, including pulmonary fibrosis, sarcoidosis, adult respiratory distress syndrome (ARDS), and bronchiolitis. A "sarcoidosis"-like syndrome, usually limited to the lungs, may result from exposure to bioaerosols and a number of metals. Exposure to beryllium in the workplace produces a granulomatous lung disease clinically indistinguishable from sarcoidosis, chronic beryllium disease (CBD). Beryllium's ability to produce a beryllium-specific immune response is used in the beryllium lymphocyte proliferation tests to confirm a diagnosis of CBD and exclude sarcoidosis. Exposure to other metals must also be considered in the differential diagnosis of sarcoidosis. When an individual presents acutely with ARDS or acute lung injury, an acute inhalational exposure must be considered. Exposure to a number of irritant substances at high levels may cause a "chemical pneumonitis" or acute lung injury, depending on the solubility and physicochemical properties of the substance. Some of the most notable agents include nitrogen and sulfur oxides, phosgene, and smoke breakdown products. Ingestion of paraquat may also result in an ARDS syndrome, with pulmonary fibrosis eventually resulting. Bronchiolitis is a rare manifestation of inhalational exposures but must also be considered in the clinical evaluation of inhalational exposure.

High-resolution CT in the evaluation of occupational and environmental disease.

The most common of the pneumoconioses are silicosis, CWP, and asbestosis. The former two are characterized by the presence of small nodular opacities predominantly distributed in the upper zones of the lung. The small nodular opacities are classified into two patterns on HRCT: (1) ill-defined fine branching lines and (2) well-defined discrete nodules. Asbestosis demonstrates thickened interlobular and intralobular lines, subpleural dot-like or curvilinear opacities, and honeycombing on HRCT, predominantly distributed in the bases of the lungs. Although HRCT findings of other pneumoconioses are variable and nonspecific, there are predominant and characteristic findings for each type of pneumoconiosis. HRCT is useful in achieving more accurate categorization of the parenchymal changes in each type of pneumoconiosis.

A 65-year-old factory worker with dyspnea on exertion and a normal chest x-ray.

Chronic beryllium disease is an occupationally acquired granulomatous lung disease similar to sarcoidosis. It is caused by exposure to beryllium in genetically susceptible persons. It should be suspected in patients with beryllium exposure who present with pulmonary symptoms or have a positive screening blood beryllium-specific lymphocyte proliferation test. The diagnosis is confirmed by the finding of granulomas on transbronchial biopsy in the appropriate clinical and epidemiologic setting. Although there is no cure, treatment with corticosteroids is usually beneficial. In view of the potential side effects, treatment is reserved for patients with symptoms or a decline in pulmonary function.

Association of man-made mineral fibre exposure and sarcoidlike granulomas.

It is assumed that sarcoidosis is caused by inhalation of air borne agents in susceptible persons triggering the inflammatory reaction. The association of metallic dust exposure, such as beryllium and aluminium, and sarcoidlike pulmonary disorders is well known. The ability of man-made mineral fibres (MMMF) to cause granulomatous lung disease has not been appreciated until now. Recently, we observed the association of sarcoidlike granulomatous reaction and occupational history of glass fibre exposure. We hypothesized that there might be a relationship between MMMF exposure and the development of sarcoidlike granulomas. Therefore, the records of 50 sarcoidosis patients-who visited our outpatient clinic between 1996 and 1999 were reviewed. This revealed that 14 cases recalled a history of exposure to either glass fibres or rock wool, both MMMF fibres. The available obtained tissue specimens (n = 12) were reviewed. In six cases electron microscopy qualitative analysis of small fragments of the tissue revealed among others silica, aluminium and sometimes titanium. A distinct relation between fibre deposits fibre deposits and granulomas was found. These findings indicate that in susceptible people MMMF exposure might be related to a chronic granulomatous disease similar to chronic beryllium disease.

Observer variation and relationship of computed tomography to severity of beryllium disease.

Although high resolution computed tomography (HRCT) is commonly used to assess interstitial lung disease (ILD), relatively little is known about interrater reliability and construct validity of HRCT-reported nodules, ground-glass opacity, or other typical findings. We determined the interobserver and intraobserver variability of HRCT findings and correlated HRCT abnormalities with physiologic measures in 57 patients with chronic beryllium disease (CBD). Reliability of HRCT scan measurements were assessed using weighted kappa (K(W)) and intraclass correlation coefficients. We correlated HRCT with spirometry, body plethysmographic lung volumes, diffusing capacity for carbon monoxide (DL(CO)), maximal exercise testing with measurement of arterial blood gases, and bronchoalveolar lavage (BAL). Interobserver agreement for three of the HRCT abnormalities found in CBD was moderate: the K(W) for nodules, septal lines, and ground-glass attenuation were 0.53, 0.44, and 0.53, respectively. Agreement was poor for bronchial wall thickening (K(W) = 0.15). HRCT scores correlated significantly with DL(CO), gas exchange at rest and at maximal exercise, and lung volume. This study demonstrates that HRCT has good interrater reliability and correlates with indices of the severity of granulomatous lung diseases such as CBD.

[Chronic pulmonary berylliosis: imaging with high-resolution computerized tomography]. / Chronische pulmonale Berylliose: Darstellung mit der hochauflösenden Computertomographie.

Chronic pulmonary berylliosis presents both in computed tomography, including high resolution techniques, and in plain radiographs of the chest with unspecific results: subpleural micronodular changes, thickening of interlobular septa, occurrence of intralobular septa, traction bronchiectasis, and honeycombing. Conspicuous in high resolution expiratory scans are density inhomogeneities which are interpreted as expiratory obstructions in small airways with ventilation-perfusion mismatches that accompany respiratory partial insufficiency. A differentiation from other pneumoconioses or other interstitial pulmonary diseases is not possible. The significance of CT lies in the exact documentation of the localization and extent of the lesions and in evaluation.