Pulmonary hypertension in patients with amyloidosis.

Pulmonary hypertension (PH) with right-sided cardiac failure is a rare complication of amyloidosis, and its natural history is not well-defined. The aim of our study was to evaluate patients who were seen at our institution who had PH and amyloidosis and to describe the natural history of this complication. The study was a retrospective chart review of patients seen at the Mayo Clinic with both PH and amyloidosis listed as major diagnoses between January 1, 1980, and December 31, 1999. Patients with known causes of PH were excluded. Five patients met our criteria (four women and one man). Four patients had light-chain amyloidosis and one had amyloid A deposition secondary to familial Mediterranean fever. All patients had symptoms related to PH without echocardiographic evidence of left ventricular dysfunction. The median survival time after the diagnosis of amyloidosis was 2.8 years, and PH was found a median of 73 days before death. Five patients died of cardiac complications, including one with sudden cardiac death. PH is an unusual complication of amyloidosis. Patients develop PH late in the disease process and do not have a worse prognosis compared to other patients with cardiac amyloidosis. PH is a marker of advanced amyloidosis.

Unexplained pulmonary hypertension in chronic myeloproliferative disorders.

AIM: To investigate the potential association between the chronic myeloid disorders (CMDs), including the chronic myeloproliferative disorders, and pulmonary hypertension (PH). METHODS: Retrospective chart review of patients who had received diagnoses of both CMD and PH. Patients with a known cause of PH were excluded. The diagnosis of a CMD was based on established criteria. The diagnosis of PH was based on echocardiographic data or right heart catheterization data. RESULTS: Twenty-six patients satisfied the criteria for both a CMD and PH. Twelve patients had myeloid metaplasia with myelofibrosis (MMM), 5 patients had essential thrombocythemia (ET), 6 patients had polycythemia vera, 2 patients had a myelodysplastic syndrome, and 1 patient had chronic myeloid leukemia. Twenty-two patients (92%) received treatment for their CMDs, which included therapy with hydroxyurea (18 patients), anagrelide (7 patients), and busulfan (3 patients). PH was diagnosed a median of 8 years after recognition of the CMD (range, 0 to 26 years). The median right ventricular systolic pressure (RVsys) was 71 mm Hg (range, 32 to 105 mm Hg). RVsys correlated with the platelet count in patients with MMM (r = 0.30) and ET (r = 0.6) and with the hemoglobin levels in patients with PV (r = 0.77). Treatment of CMD did not seem to affect the severity of the pulmonary artery pressures as measured by serial echocardiography. With a median survival time of 18 months after the diagnosis of PH, the cause of death in the majority of the patients was cardiopulmonary. CONCLUSIONS: The current study suggests a higher than expected incidence of PH in patients with MMM, PV, and ET. Prognosis in such a setting is poor and may not be influenced by aggressive treatment of the underlying hematologic disorder.

External-beam radiation therapy in the treatment of diffuse tracheobronchial amyloidosis.

Tracheobronchial amyloidosis is characterized by deposits of amyloid in airway walls. No effective treatment is known. We describe a 59-year-old woman who presented with increasing symptoms of airway obstruction due to diffuse deposition of amyloid throughout her tracheobronchial tree. She was treated with external-beam radiation therapy (20 Gy) with marked improvement in her symptoms, effort tolerance, bronchoscopic appearance, and forced expiratory volume in 1 second (1.39 L to 1.97 L [42%]). This improvement was maintained during 21 months of follow-up.

High short-term mortality following lung biopsy for usual interstitial pneumonia.

Usual interstitial pneumonia (UIP) is a specific histological pattern of interstitial pneumonia most often associated with the clinical syndrome of idiopathic pulmonary fibrosis (IPF). There is controversy regarding the use of surgical lung biopsy in the diagnosis of UIP, and the risk of lung biopsy in these patients is largely unknown. This study investigated the 30 day surgical mortality rate in patients undergoing surgical lung biopsy for UIP. Patients undergoing surgical lung biopsy over a 10-yr period from 1986-1995 with the ultimate diagnosis of UIP (with or without underlying connective tissue disease) were identified. Pathology, computed tomography, medical records, and survival were assessed. Ten of sixty patients with usual interstitial pneumonia were found to be dead within 30 days of surgical biopsy. All of these were patients with idiopathic UIP, unassociated with connective tissue disease (clinical condition of IPF). In conclusion, patients with usual interstitial pneumonia of the idiopathic type, who present with atypical features, may be at higher risk for death following surgical biopsy than patients presenting with more typical features or patients with other interstitial illnesses.

Therapeutic bronchoscopy in broncholithiasis.

Bronchoscopy is considered the most important diagnostic test for broncholithiasis. However, its role in the treatment of broncholithiasis in a large group of patients has not been studied. To evaluate the therapeutic role of bronchoscopy, we retrospectively reviewed the clinical data of patients with broncholithiasis who also underwent bronchoscopy at Mayo Clinic. Bronchoscopy revealed 127 broncholiths (free or partly eroded calcified material in the airway lumen) in 95 patients (49 men and 46 women) evaluated between 1954 and 1994. Bronchoscopic removal of 71 (56%) broncholiths was attempted in 48 patients (50.5%) during 61 bronchoscopy sessions. Forty-eight of the broncholiths selected for removal were partly eroding into the tracheobronchial lumen and 23 were free. Forty-eight percent (23 of 48) of the partly eroding broncholiths were successfully removed bronchoscopically, with a greater percentage removed with the rigid bronchoscope (67%) than with the flexible bronchoscope (30%). All free broncholiths were completely extracted regardless of the type of bronchoscope used. Complications occurred in only two patients (4% of the bronchoscopic broncholithectomy group), both with partially eroded broncholiths, and consisted of hemorrhage in one patient requiring thoracotomy and acute dyspnea in another patient, caused by a loose broncholith lodged in the trachea. We conclude that flexible and/or rigid bronchoscopic extraction of partly eroded or free broncholiths in the tracheobronchial tree can be considered safe and effective.

Lung volume reduction surgery for emphysema: out on a limb without a NETT.

Lung volume reduction surgery (LVRS) has recently been rediscovered and offers the potential of improving the quality of life of patients with advanced emphysema. In this article, we discuss the historical and contemporary versions of LVRS. Although initial enthusiasm has been substantial, existing data seem insufficient to demonstrate the safety and efficacy of the procedure in comparison with conventional medical therapy. Fundamental questions remain regarding the long-term effects of an operation versus medical therapy, the optimal selection criteria, the best measures of efficacy, the mechanisms of improvement, the cost-effectiveness of the procedure, and the optimal surgical technique. Until such questions are answered, advising patients about the best management their emphysema will be difficult. The National Emphysema Treatment Trial will address many of these issues and should be embraced by both health-care providers and patients.

Thoracic cross-sectional imaging of amyloidosis.

OBJECTIVE: Our objective was to determine the thoracic manifestations on cross-sectional imaging of patients with tissue-proven amyloidosis. MATERIALS AND METHODS: We reviewed the records of 300 patients with the diagnosis of amyloidosis on whom cross-sectional imaging was done at our institution between 1985 and 1995. After exclusions, 19 patients with tissue-proven amyloidosis and cervicothoracic cross-sectional imaging were included. Seven patients had localized amyloidosis and 12 patients had systemic amyloidosis. Eighteen patients underwent CT scans and the remaining patient, MR imaging. RESULTS: Two patients with systemic amyloidosis had widespread noncalcified adenopathy. A third patient had an infiltrative soft-tissue process in the mediastinum and axillae containing thick linear and focal calcifications. Five patients with localized amyloidosis had pulmonary nodules: Three patients had solitary nodules, one patient had two nodules, and one patient had 10 nodules. Nodules ranged in size from 8 mm to 3 cm. Eight patients with systemic amyloidosis had diffuse lung disease. One patient with systemic amyloidosis had recurrent right pleural effusions. Two patients with localized amyloidosis had laryngotracheobronchial amyloidosis. One of the two patients had focal thickening of the right aryepiglottic fold. The other patient had diffuse concentric soft-tissue thickening within the trachea. CONCLUSIONS: Localized amyloidosis can occur in patients as pulmonary nodules or as laryngotracheobronchia involvement. Nodules are typically solitary (60%) with a smooth or lobular contour and are frequently in a subpleural or peripheral location. Calcification is not common (20%). Systemic amyloidosis can occur in patients as a combination of adenopathy (75%), multiple pulmonary nodules (50%), and diffuse irregular lines or interlobular septal thickening (50%).

Sarcoidosis presenting as a central nervous system mass lesion.

Neurosarcoidosis occurs in approximately 5% of patients with systemic sarcoidosis. A review of medical literature shows that intracranial mass-like lesions secondary to sarcoidosis are quite rare. CT and MRI scanning would suggest that this manifestation of neurosarcoidosis may be more common than previously realized. We discuss five cases of neurosarcoidosis presenting as an intracranial mass. Empiric corticosteroid treatment can be recommended for cases with CNS mass in the context of systemic sarcoidosis.

Pulmonary amyloidosis. The Mayo Clinic experience from 1980 to 1993.

OBJECTIVE: To define the prognosis for and radiographic presentation of patients with pulmonary amyloidosis. DESIGN: Retrospective review of the Mayo Clinic experience with biopsy-proven pulmonary amyloidosis from 1980 to 1993. SETTING: Tertiary care center. PATIENTS: Patients with pulmonary biopsy specimens showing amyloid deposition. MEASUREMENTS: Medical records were reviewed, and pertinent information was recorded, including demographic data, type of pulmonary biopsy, results of biopsies of nonpulmonary sites and of immunoelectrophoresis, and other clinical, radiographic, and laboratory information necessary for distinguishing localized pulmonary amyloidosis, primary systemic amyloidosis, secondary amyloidosis, and familial amyloidosis. RESULTS: 35 of 55 patients with pulmonary amyloidosis had primary systemic amyloidosis that presented radiographically as an interstitial or reticulonodular pattern with or without pleural effusion. The median survival after diagnosis was 16 months. Nodular pulmonary "amyloidomas" (nodular amyloid lesions) were not associated with systemic disease and were associated with a benign prognosis. Three of 4 patients with localized tracheobronchial amyloidosis required Nd:YAG (neodymium:yttrium-aluminum-garnet) laser therapy for obstructive symptoms. "Senile" amyloid deposition was an incidental finding in some patients at autopsy. CONCLUSIONS: Localized amyloidomas are characterized by a benign course and are not associated with systemic amyloidosis. Despite its localized nature, tracheobronchial amyloid deposition may be asymptomatic or may result in significant morbidity due to obstructive phenomena. Pulmonary amyloidosis associated with primary systemic amyloidosis generally presents as a diffuse interstitial pattern with or without pleural effusion. Complete survival data indicate that long-term outcome is poor after diagnosis. We describe the largest series of patients diagnosed by bronchoscopic lung biopsy. Despite reports to the contrary, we have found bronchoscopic lung biopsy to be a safe and effective diagnostic technique.

Cardiac echinococcosis: case report of unusual echocardiographic appearance.

An unusual echocardiographic appearance of a cardiac echinococcal lesion confirmed intraoperatively is described in a patient with nonspecific cardiac complaints. Although previous reports have emphasized the echolucent, often multiseptated nature of echinococcal lesions, such characteristics were absent in this case. Thus, in the appropriate clinical setting, echinococcal infection should be included in the differential diagnosis of solid mass lesions of the heart because the surgical approach may need to be altered.

Cavitary pulmonary infarct in immunocompromised hosts.

Pulmonary disease in immunocompromised patients is common, but cavitary lung disease is less common and is usually associated with a fungal or mycobacterial infection. Pulmonary embolism is a noninfectious cause of a cavitary pulmonary process. Pulmonary embolism causes infarction in fewer than 15% of cases, and only about 5% of infarctions cavitate. Herein we describe two cases of cavitary infarcts in immunocompromised patients and review the clinical aspects of pulmonary infarcts and cavitation. Cavitary pulmonary infarction has been reported only rarely in immunocompromised patients. It is a dangerous but treatable pulmonary disease that must be considered in the differential diagnosis of immuno-compromised patients with lung disease.

The role of transcarinal needle aspiration in the staging of bronchogenic carcinoma.

Metastatic spread to subcarinal lymph nodes in patients with bronchogenic carcinoma generally indicates unresectability. Transcarinal needle aspiration of the main carina (TCNA) has been used to obviate the need for more invasive procedures, particularly thoracic surgery. Of 510 transbronchial needle aspirations performed at our institution from 1983 to 1991, 88 (17 percent) were from the main carina in patients with bronchogenic carcinoma. We reviewed these 88 TCNA procedures to assess our experience with TCNA in the staging of lung cancer. The TCNA results were positive in 32 of 88 (36 percent) patients (20 non-small-cell cancers, 12 small-cell lung cancers). Following bronchoscopy, TCNA was the only evidence of unresectability in all 20 patients with non-small-cell lung cancer and was the only mode of diagnosis in 5 of 12 (42 percent) patients with small-cell lung cancer. Thirteen patients with non-small-cell lung cancer and positive TCNA also had positive bronchial secretion cytologic studies. Five of these patients had further subcarinal sampling and in all cases metastatic involvement was confirmed. TCNA was positive in 29 of 67 (43 percent) patients with radiographic evidence of mediastinal adenopathy. Of the remaining 38 patients with radiographic evidence of mediastinal adenopathy and negative TCNA results, 23 patients had further mediastinal sampling with mediastinoscopy or thoracotomy and in all cases mediastinal spread of cancer was established. Nineteen of 58 (33 percent) patients with an endoscopically normal-appearing main carina had a positive TCNA, while 13 of 30 (43 percent) patients with broadening or widening of the main carina had positive TCNA results. There were no complications. We conclude that TCNA is often a safe and useful staging modality in patients with bronchogenic carcinoma.

Pulmonary lymphoproliferative disorders.

Pulmonary lymphoproliferative disorders are a diverse group of conditions that primarily or secondarily involve the lungs. Modern diagnostic modalities, including immunologic and molecular genetics techniques, have contributed substantially to the continuously evolving understanding of these complex lesions. In this review, we classified pulmonary lymphoproliferative disorders into benign, intermediate or indeterminate, and malignant categories and described the clinical, radiographic, and pathologic features for the entire spectrum. Our emphasis is on the clinical findings and the role of tissue biopsy as well as ancillary techniques in diagnosis. Various treatments and expected responses are also discussed.

Pediatric tracheobronchial foreign bodies. A case report.

Pediatric tracheobronchial foreign bodies (PTBFBs) remain a significant cause of childhood morbidity and mortality. Because physicians do not always obtain a history of aspiration, and because the signs and symptoms of a PTBFB are nonspecific, diagnosis may be delayed, which increases the risk of complications when the foreign body is removed. Flexible fiberoptic and rigid bronchoscopy are often used in tandem for the identification and removal of PTBFBs. Recent advances in bronchoscopic equipment and technique have made removal of PTBFBs safer and more successful. Education of parents, child care providers, and medical personnel can reduce morbidity and mortality due to PTBFBs.

Risk of osteoporotic fractures in women with breast cancer: a population-based cohort study.

A population-based study was undertaken to assess the risk of osteoporotic fractures in an inception cohort of breast cancer patients and an age-matched cohort of women from the community. Prior to the index date, 9.4% of cases and 12.3% of controls (p = 0.30) had one or more osteoporotic fractures. After the index date, these proportions were 16.2 and 20.0% (p = 0.28), but follow-up was shorter for cases due to reduced survival. When duration of follow-up was accounted for in a person-years analysis, the relative risk of any fracture was 1.0 and for any osteoporotic fracture was 0.9 (95% C.I. 0.7-1.2). After adjusting for other factors in a proportional hazards model, the relative risk of any osteoporotic fracture in breast cancer cases compared to controls was 0.9. Despite contentions that breast cancer patients have more often been exposed to estrogenic factors, we found little to suggest that such women are substantially protected from osteoporotic fractures.

Epidemic of pulmonary blastomycosis (Namekagon fever) in Wisconsin canoeists.

Epidemics of pulmonary blastomycosis have rarely been reported. The following epidemic occurred in a Minnesota family and several of their acquaintances after a canoeing trip in northwestern Wisconsin. The common exposure area was most likely a campsite, located along the upper reaches of the Namekagon River. The Namekagon River Valley is a known endemic area of Namekagon fever (blastomycosis) in dogs. Approximately one month after returning home, five of the eight members of the group had positive direct microscopic examinations and cultures of Blastomyces dermatitidis from their sputa, as well as abnormalities on their chest roentgenograms. Among these five patients, four were symptomatic, with fever, cough, and pleuritic chest pain. Of the three others, one had pleuritic chest pain with a transient lung infiltrate, the second was asymptomatic with a transient lung infiltrate, and the third was asymptomatic with a normal chest roentgenogram. Results of acute serologic tests (complement fixation and immunodiffusion) were negative in all five patients evaluated. None of the patients received antifungal therapy. Follow-up five years after the epidemic revealed that all eight were in excellent health, and none had evidence of continuing pulmonary or extrapulmonary disease.