Intrathoracic lymphadenopathy: an unusual manifestation of rheumatoid arthritis

Lung disease is the most frequent extra-articular manifestation of rheumatoid arthritis. It is detected in nearly 50% of patients with this multisystem affection, his knowledge has benefited from advances in computed tomography (CT). The inflammation can affect the pleura, the airways and the lung parenchyma. Intrathoracic lymphadenopathy complicating rheumatoid lung are not usual, and then pose the problem of differential diagnosis. We report a 51-year-old man, with a history of tobacco intoxication, suffering from rheumatoid arthritis who developed an interstitial lung disease at stage of fibrosis with mediastinal and hilar adenopathy. We will discuss the clinical, paraclinical, evolutionary and therapeutic particularities case.     

Bronchoscopic and roentgenographic correlates of a positive transbronchial needle aspiration in the staging of lung cancer

A study was conducted to determine the bronchoscopic and chest roentgenographic findings associated with a positive TBNA. One hundred fifty-seven of 465 patients who were diagnosed for the first time as having carcinoma of the lung had a positive aspirate. Bronchoscopic findings associated with a positive TBNA of N2 nodes were carinal widening and endobronchial disease, especially of the right upper lobe. Mediastinal adenopathy noted on chest roentgenograms and subcarinal nodes on CAT scans were associated with a positive aspirate as well. In 34 of 465 patients, TBNA was the only means of establishing the diagnosis of pulmonary malignancy. A useful, simple and safe procedure, TBNA can be used to stage the mediastinum in patients with lung cancer and is most likely to be positive with endobronchial and nodal disease. It can also facilitate therapeutic decision-making in patients whose surgical candidacy is marginal.     

Mediastinal adenopathy: finding the answer with endoscopic ultrasound-guided fine-needle aspiration biopsy

BACKGROUND: Endoscopic ultrasound-guided fine-needle aspiration biopsy (EUS FNA) is a relatively new imaging modality that has been reported to be useful for mediastinal nodal staging of lung cancer and for the evaluation of mediastinal adenopathy of unknown cause. However, the technique is not commonly used in Australia. METHODS: A retrospective review of all patients who had mediastinal EUS FNA was undertaken. Of a total of 787 patients who had undergone endoscopic ultrasound (EUS) studies from November 1999 to March 2004, 27 patients were identified to have had mediastinal EUS FNA. Details were recorded including study indication, history of malignancy, source of referral, prior attempts for tissue diagnosis, EUS and EUS FNA findings, complications, surgical pathology if available and clinical outcome after diagnosis. RESULTS: Mediastinal EUS FNA was performed on an outpatient basis and no complications were recorded. Diagnostic material was obtained from all patients with a mean number of three passes. Nodal stations sampled included left paratracheal, subcarinal, aortopulmonary window and inferior mediastinum. Indications for the studies included mediastinal adenopathy of uncertain cause (17), lung cancer staging (7) and gastrointestinal cancer staging (3). EUS FNA confirmed malignancy in 16/27 patients, sarcoidosis in three patients, tuberculosis in one patient and seven patients were deemed to have reactive adenopathy. Primary cytopathological diagnosis of malignancy was determined by EUS FNA in nine patients. CONCLUSIONS: EUS FNA is a safe, efficient and effective modality for mediastinal staging of lung cancer and for the diagnosis of mediastinal adenopathy of uncertain origin. EUS FNA has the potential to significantly impact on patient management, avoiding more invasive procedures as well as unnecessary operations.     

Distribution of thoracic lymphadenopathy in sarcoidosis using computed tomography

The authors used chest computed tomography to determine the distribution of pulmonary lymphadenopathy in 40 patients with sarcoidosis. Using the American Thoracic Society lymph node map, the number and distribution of significant lymph nodes was calculated. Overall, lymphadenopathy was identified in 39 of the 40 patients. Mediastinal adenopathy was present in 38 patients, and hilar adenopathy was present in 27. Commonly involved nodal stations were 4R, 5, 7, 10R, 11R, and 11L, and little involvement was seen in stations 1, 6, and 14. An understanding of the common sites of adenopathy in sarcoidosis is useful when assessing adenopathy in patients without a known diagnosis.     

Whipple disease revealed by lung involvement: a case report and literature review

We report the case of a man with a history of intermittent fever and arthritis who presented with a dry cough and associated lung involvement, who was eventually given the diagnosis of Whipple disease. The pulmonary symptoms preceded the development of GI manifestations. Five years later, periodic acid-Schiff (PAS)-positive macrophages were identified in duodenal biopsy specimens and polymerase chain reaction for Tropheryma whipplei was positive in the duodenum, stools, saliva, and cerebrospinal fluid. Pulmonary T whipplei was retrospectively confirmed by positive PAS staining and immunoreactivity to specific antibodies in endobronchial biopsy specimens. Antibiotic treatment was followed by remission. A literature review identified eight other cases of Whipple disease presenting with lung parenchymal involvement, predominantly interstitial lung disease (ILD), and without initial GI symptoms. In the absence of GI symptoms, a diagnosis of Whipple disease should be considered in middle-aged men presenting with ILD or lung nodules, if the patient has a history of unexplained arthralgia and/or fever. The association of mediastinal adenopathy or pleural effusion offers additional concern. Whipple disease may be fatal in the absence of treatment, but prolonged antibiotic treatment often leads to complete remission.     

Advances in the treatment of rheumatic interstitial lung disease

PURPOSE OF REVIEW: Interstitial lung disease frequently complicates the rheumatic diseases. The purpose of this review is to outline recent advances and current concepts regarding the management of these interstitial lung diseases. RECENT FINDINGS: Several histologic lesions cause interstitial lung disease in rheumatic diseases, including nonspecific interstitial pneumonia, usual interstitial pneumonia, organizing pneumonia, lymphocytic interstitial pneumonia, desquamative interstitial pneumonia, and acute interstitial pneumonia. Although the relative frequency of occurrence of these histopathologic lesions is not definitively established, it seems that nonspecific interstitial pneumonia accounts for a large proportion of rheumatic disease-associated interstitial lung diseases. Although usual interstitial pneumonia generally responds poorly to corticosteroid therapy, other forms of interstitial pneumonia are often steroid responsive and have a more favorable long-term prognosis. Pulmonary hypertension is increasingly recognized as a complication of these interstitial lung diseases. Treatment of pulmonary hypertension in these patients provides clinical benefit and may suppress pulmonary inflammation and fibrosis. Lung transplantation is a treatment option for selected patients with severe pulmonary involvement and limited life expectancy. SUMMARY: Interstitial lung disease is common in the rheumatic diseases, may be caused by a variety of lesions that respond differently to treatment, and may lead to the development of pulmonary hypertension. Whether the prognosis of interstitial lung disease associated with rheumatic disease is similar to that associated with the idiopathic interstitial pneumonias is not known. Treatment of these interstitial lung diseases should take into account the specific histologic lesion, the activity of the underlying rheumatic disease, and associated pulmonary hypertension, if present. The diagnosis of a rheumatic disease is no longer an absolute contraindication to lung transplantation.     

Interstitial lung disease in polymyositis and dermatomyositis

PURPOSE OF REVIEW: The purpose of this review is to discuss current concepts regarding management of interstitial lung disease in polymyositis and dermatomyositis. RECENT FINDINGS: Interstitial lung disease seems to be a more frequent manifestation in patients with polymyositis and dermatomyositis than previously reported. Modern technology, including high-resolution computerized tomography in combination with pulmonary function tests provides sensitive tools to detect early signs of interstitial lung disease. By systematic use of these investigations in newly diagnosed polymyositis and dermatomyositis, up to two thirds of patients were discovered to have signs of interstitial lung disease in a recent study. Clinical symptoms such as cough and dyspnea may not be sensitive enough to detect interstitial lung disease. Awareness of this complication in patients with myositis is important, because early diagnosis and management of interstitial lung disease may prevent development of chronic pulmonary fibrosis and thereby prolong patient survival and improve quality of life. Treatment recommendations of interstitial lung disease in polymyositis and dermatomyositis are still limited by absence of controlled trials and could only be based on experiences from small case series and case reports. At least some patients with interstitial lung disease improve with immunosuppressive treatment, but data are limited, and longitudinal studies are needed. SUMMARY: Interstitial lung disease seems to be a common manifestation in patients with polymyositis and dermatomyositis already at diagnosis of the muscle disease. When present, interstitial lung disease has a major effect on morbidity and mortality and should be looked for in these patients using high-resolution computerized tomography and pulmonary function tests early in the disease course, because immunosuppressive treatment may change the course of the lung disease.     

"Crack" cocaine-induced syndrome mimicking sarcoidosis.

A 39-year-old man with a history of frequent "crack" cocaine use of several years' duration presented with progressive dyspnea. Evaluation revealed bilateral interstitial pulmonary infiltrates and hilar adenopathy, diffuse pulmonary uptake of gallium, and markedly elevated serum angiotensin-converting enzyme activity. Open lung biopsy revealed interstitial and perivascular collections of histiocytes containing refractile, polarizable material, presumably inhaled along with the cocaine. Paratracheal lymph nodes were enlarged, reactive, and contained similar polarizable material. The well-formed, non-necrotizing granulomata characteristic of sarcoidosis were not present in either tissue specimen. To our knowledge, the association of chronic crack cocaine inhalation with this constellation of clinical findings, typically seen in sarcoidosis, has not previously been described.     

Interstitial lung diseases in polymyositis and dermatomyositis

PURPOSE: Interstitial lung disease is one of the most common respiratory manifestations in polymyositis and dermatomyositis. It still remains a severe complication of the disease, leading to death related to ventilatory insufficiency in 30-66% of patients. CURRENT KNOWLEDGE AND KEY POINTS: Time onset of interstitial lung disease is variable, although interstitial lung disease onset precedes initial manifestations of polymyositis/dermatomyositis in roughly half of the patients. Moreover, clinical presentation of interstitial lung disease can be dichotomized, according to patients' pulmonary manifestations, into: 1) both acute and aggressive lung disease similar to Hamman-Rich syndrome; 2) slowly progressive lung disease; and 3) an asymptomatic pattern. The methods of choice adopted for early diagnosis of interstitial lung disease are high-resolution computed tomography scan and pulmonary function tests, which should be performed during both initial evaluation of polymyositis/dermatomyositis and follow-up. Because anti-JO1 antibody is considered to be a marker of interstitial lung disease in polymyositis/dermatomyositis, close pulmonary follow-up of anti-JO1-positive patients with polymyositis is therefore required for early detection of subclinical impairment. Furthermore, histological lung findings provide prognostic data; patients with bronchiolitis obliterans organizing pneumonia (BOOP) indeed appear to have a more favorable outcome than those with usual interstitial pneumonia or diffuse alveolar damage. Finally, as a guide to both the severity and progress of interstitial lung disease, the significance of other investigations, notably bronchoalveolar lavage, remains controversial. FUTURE PROSPECTS AND PROJECTS: Specific therapy of interstitial lung disease has not yet been clearly established in polymyositis/dermatomyositis patients. Corticosteroid therapy is considered the first line of therapy for polymyositis/dermatomyositis patients with interstitial lung disease. The association of cyclophosphamide and corticosteroids may be the most effective in patients with steroid-resistant interstitial lung disease. Early diagnosis and management of this disease is therefore of the utmost importance.     

Drug-induced interstitial lung diseases

Any discussion of drug-induced interstitial lung disease is fraught with the problem of having a syndrome in which information is composed predominantly of case reports. When the information is taken as a whole, however, the picture becomes clearer: (1) Some drugs can produce significant pulmonary toxicity; (2) the clinical history, physical examination, and chest roentgenogram are not unique or specific for drug-induced interstitial lung disease; and (3) the lung reacts in limited ways to various insults, producing pathologic changes that are not unique for drug-induced interstitial lung disease. The work of Crystal et al. has shed new light on interstitial lung disease. Their concept that the alveolitis is the key initiating step in interstitial lung disease fits with the pathologic findings in many situations of drug-induced interstitial lung disease. Furthermore, as they proposed, if the primary alveolitis has not progressed to derangement of alveolar structures, then the process can be reversed. This may explain the variable response seen with drug withdrawal and the use of corticosteroids. These concepts have not been extensively studied in drug-induced interstitial lung disease and this needs to be done to fully evaluate their ideas. A keen awareness of the potential for any drug to cause drug-induced interstitial lung disease can be of immense benefit to the patient, for early discontinuation of the agent is likely to increase the chance of reversing the pulmonary toxicity.     

开胸肺活检对肺间质疾病的诊断价值

目的探讨开胸肺活检对肺间质疾病的诊断作用。方法对１９９３～１９９８年６月２４例开胸肺活检的肺间质疾病患者进行回顾性分析。结果２４例患者均获病理确诊,其中普通型间质性肺炎（ＵＩＰ）７例,闭塞性细支气管炎伴机化性肺炎（ＢＯＯＰ）３例,结节病３例,弥漫性泛细支气管炎（ＤＰＢ）２例,肺结核２例。其他急性间质性肺炎（ＡＩＰ）、呼吸性细支气管炎伴肺间质病（ＲＢＩＬＤ）、肺组织细胞增生症Ｘ、炎性结节、多发性肺脓肿、肺组织炎症和肺泡细胞癌各１例。结论开胸肺活检作为一种诊断方法,能获得足够的肺组织,具有很高的敏感性和特异性,能明确病变的部位和程度。对常规和纤维支气管镜未能确诊的病例,尤其是一些罕见病和不典型的病例,具有较大的价值。     

Mediastinal transthoracic needle and core lymph node biopsy: should it replace mediastinoscopy?

STUDY OBJECTIVES: Primary assessment of mediastinal lymph nodes (N2 or N3) for staging lung cancer by transthoracic needle with or without core biopsy. Mediastinoscopy only performed after FNA failed to yield a diagnosis. DESIGN AND SETTINGS: A retrospective study in a university setting. PATIENTS: Eighty-nine patients with mediastinal lymphadenopathy (> 1.5 cm in short-axis diameter) by CT. METHODS: Mediastinal transthoracic fine-needle aspiration (FNA) with or without core biopsy was performed prior to mediastinoscopy in 89 patients with mediastinal lymphadenopathy (lymph node > 1.5 cm in short-axis diameter) or masses by CT. RESULTS: Mediastinal transthoracic FNA was used alone in 39 of 89 patients, or with core biopsy in 50 of 89 patients. Mediastinal transthoracic FNA with or without core biopsy was diagnostic in 69 of 89 patients (77.5%) for cancer cell type, sarcoidosis, or caseating granulomas with or without tuberculosis. Transthoracic FNA with or without core biopsy of nodal stations (total, 94 biopsies) judged readily accessible by mediastinoscopy (n = 59) included paratracheal (n = 56) and highest mediastinal (n = 3); those more difficult (n = 26) included subcarinal (n = 20) and aorticopulmonary window (n = 6); and those impossible (n = 9) included paraesophageal and pulmonary ligament (n = 6), parasternal (n = 2), and para-aortic (n = 1). Innovative lung protective techniques for CT-guided biopsy access windows included "iatrogenic-controlled pneumothorax" (n = 10) or saline solution injection creating a "salinoma" (n = 11). Pneumothorax was detected in only 10% with a "protective" technique but 60% when traversing lung parenchyma. Transthoracic FNA with or without core biopsy failed to yield a diagnosis in 20 of 89 patients (22.5%); all then underwent mediastinoscopy, with 11 of 20 procedures (55%) diagnostic for cancer, and 9 of 20 procedures diagnostic of benign diagnosis or no cancer. CONCLUSION: Transthoracic FNA with or without core biopsy accesses virtually all mediastinal nodal stations is diagnostic in 78% of cases with mediastinal adenopathy or masses, and should precede mediastinoscopy in the staging of lung cancer or workup of mediastinal masses.     

High resolution computed tomography in early scleroderma lung disease.

Seventeen patients with early systemic sclerosis (SSc) underwent high resolution computed tomography (HRCT) of the chest to evaluate dyspnea and/or abnormal pulmonary function tests (PFT). All patients were assigned a dyspnea score and each had routine chest radiography (CXR). Bronchoalveolar lavage (BAL) was performed on 10 patients. HRCT was abnormal in 15 patients (88%), while CXR was abnormal in only 10 patients (59%). Mediastinal lymphadenopathy was detected in 7 patients (41%). Disease duration, dyspnea score, and forced vital capacity (FVC) did not correlate with HRCT score. However, trends toward higher total BAL cell counts and higher BAL neutrophil counts were noted in patients with ground glass opacities on HRCT, and BAL lymphocyte counts were significantly higher in such cases. HRCT is superior to CXR for detecting early interstitial lung disease in SSc, but patient history and FVC correlate poorly with HRCT findings. Ground glass opacities on HRCT may reflect active alveolitis, and mediastinal lymphadenopathy associated with SSc lung disease may be a consequence of pulmonary inflammation.     

The radiographic appearance of tuberculosis in patients with the acquired immune deficiency syndrome (AIDS) and pre-AIDS

We reviewed the medical records and chest radiographs of 23 adult patients with culture-proved tuberculosis and verified acquired immune deficiency syndrome. Seventeen patients, including 8 with disseminated tuberculosis, had positive sputum or bronchial washing cultures for Mycobacterium tuberculosis. Their initial pretreatment radiographs revealed hilar and/or mediastinal adenopathy in 10 patients (59%), localized pulmonary infiltrates limited to the middle or lower lung fields in 5 patients (29%), localized pulmonary infiltrates involving an upper lobe in 3 patients (18%), diffuse miliary or interstitial infiltrates in 3 patients (18%), no pulmonary infiltrates in 6 patients (35%), and no abnormalities in 2 patients (12%). Pulmonary cavitation was not seen. Only 1 patient (6%) had a chest radiograph typical of adult onset reactivation tuberculosis (i.e., localized pulmonary infiltrate involving the upper lung fields without hilar or mediastinal adenopathy). Six patients (35%) had pulmonary infiltrates that may have been caused by concomitant nontuberculous infection. Six patients had positive cultures for M. tuberculosis from extrapulmonary sites only. Three (50%) of these patients had hilar and/or mediastinal adenopathy. None of them had pulmonary infiltrates on their initial chest radiograph.     

Fibrinopeptide A reactive peptides and procoagulant activity in bronchoalveolar lavage: relationship to rheumatoid interstitial lung disease

Extravascular, primarily, alveolar fibrin deposition is commonly associated with the alveolitis of many interstitial lung diseases including the interstitial lung disease associated with rheumatoid arthritis (RA). We therefore hypothesized that coagulation pathways, which promote fibrin formation, would be activated in the alveolar lining fluids of patients with rheumatoid interstitial lung disease. To test this hypothesis, we studied the bronchoalveolar lavage (BAL) fluids from patients with rheumatoid interstitial lung disease (n = 7) and patients with RA unassociated with interstitial lung disease (n = 10) to characterize and quantitatively compare the BAL procoagulant material and levels of fibrinopeptide A (FPA), which is cleaved from fibrinogen by thrombin. FPA reactive peptide concentrations were significantly greater in rheumatoid interstitial lung disease than RA when normalized per ml of concentrated BAL fluid (p = 0.02), per mg BAL total protein (p = 0.01) or BAL albumin content (p = 0.03) and correlated with BAL antigenic neutrophil elastase concentrations (r = 0.87). Procoagulant activity was present in similar concentration of BAL of patients with RA and rheumatoid interstitial lung disease and was mainly attributable to tissue factor associated with factor VII (or VIIa). Our results demonstrate that tissue factor and factor VII are endogenous in the alveoli of subjects with RA and interstitial lung disease and could interact with distal coagulation substrates which may enter the alveoli in interstitial lung disease to locally promote fibrin deposition.(ABSTRACT TRUNCATED AT 250 WORDS)     

Lung transplantation in interstitial lung disease

Interstitial lung disease is a heterogeneous group of illnesses, some of which may progress to a fibrosing stage and cause respiratory failure. For selected candidates, lung transplantation is the ultimate therapeutic option. We review data on lung transplantation for various interstitial lung diseases. We address indications, procedures, and outcomes for patients undergoing transplantation. Unique issues affecting morbidity, mortality, and recurrence of disease are discussed. We review the literature of transplantation for specific interstitial lung diseases and the outcomes of transplantation for interstitial lung diseases. Candidates with idiopathic pulmonary fibrosis experience high mortality on the waiting list, but derive significant survival benefit from lung transplantation. Recurrence is reported for several interstitial lung diseases after lung transplantation. Survival with lung transplantation for interstitial lung diseases is comparable with that attained in recipients with other indications. Lung transplantation is a well-tolerated, effective therapy for respiratory failure in interstitial lung disease.     

Severe pulmonary sarcoidosis

Mediastinal and pulmonary localizations are found in 90% of al patients with sarcoidosis. In half the cases, the disease is not severe and is reversible without treatment. In the other half of cases, early or late respiratory complications can be seen. Early complications include subacute respiratory insufficiency by interstitial lung disease or by bronchial airway obstruction. Among late complications, the most frequent is pulmonary fibrosis. Four computed tomography patterns are found with variable functional impairments and course. Chronic obstructive respiratory insufficiency can be the consequence of specific bronchial lesions or pulmonary fibrosis surrounding proximal bronchi. Cor pulmonale is seen in 5% of cases. Aspergilloma seen in fibroemphysematous lesions can be the cause of major hempoptysis. Respiratory complications account for half of the 5% of deaths due to sarcoidosis. Respiratory complications are most often seen in radiographic stage III and IV disease. Treatments, mainly corticosteroids, only exert a suspensive effect but reduce the incidence and severity of respiratory manifestations. The gain obtained by treatment depends on the choice of the best time of institution and on the quality of monitoring. Lung transplantation is useful in most severe cases unresponsive to medical treatment.     

Polymyositis and interstitial lung disease in a patient with anti-Jo1 prototype

The most common marker autoantibody among patients with polymyositis is anti-Jo₁. The patient (John P.) providing the prototype serum for this specificity had both interstitial lung disease and polymyositis. A preliminary survey by Ouchterlony analysis and counter immunoelectrophoresis of serum from 15 patients with idiopathic interstitial lung disease revealed no anti-Jo₁ or other precipitating autoantibodies. This provides no evidence to suggest that anti-Jo₁ has specificity for interstitial lung disease per se. However, this autoantibody may serve as a possible marker for some patients with overlap of polymyositis and interstitial lung disease. The several interesting features about this patient's diseases and course are discussed.     

Pulmonary coccidioidomycosis.

Coccidioidomycosis is a fungal disease endemic in the southwestern desert area of the United States. The infection is acquired by inhalation of arthrospores, and 60% of the infections are asymptomatic. Chest radiographic abnormalities are common and may even be seen in asymptomatic cases. In patients with acute infection, segmental or lobar consolidation and nodular or patchy pulmonary opacities are frequent. Hilar and mediastinal adenopathy may be present in 20% of cases, usually with parenchymal findings. A small pleural effusion may occur in 20% of cases. Approximately 5% of patients with primary disease are left with chronic, residual lesions of the lung. These consist of nodules, cavities, pneumonia, adenopathy, pleural effusion, fibrosis, bronchiectasis, and calcification. Rarely, in about 0.5% of cases, the infection may disseminate to any organ. The chest radiograph demonstrates a miliary or reticulonodular pattern and mediastinal adenopathy. Overall the disease is benign in nature; but patients with severe, progressive pulmonary or disseminated disease often require medical and occasionally surgical management.     

间质性肺病合并肺癌研究进展

间质性肺病和肺癌是呼吸内科常见疾病.近年来2种疾病的发病率都有明显升高的趋势,而且预后不良,特别是间质性肺病合并肺癌的出现,在其诊断和治疗方面都存在一定的难度和挑战.一方面,2种疾病的发病机制在一定程度上有共同之处;另一方面,肺癌的治疗会诱发间质性肺病的发生和急性加重.本文通过搜集近年来有关间质性肺病合并肺癌的流行病学、发病机制、临床特征、治疗和预后等相关问题的研究进展,并进行总结,以提高临床医师对间质性肺病合并肺癌的诊治水平.