Pulmonary hypertension in polymyositis-dermatomyositis: clinical and hemodynamic characteristics and response to vasoactive therapy.

The increased incidence of pulmonary hypertension and its association with decreased survival is well-recognised in patients with systemic sclerosis. This association is not widely appreciated in patients with polymyositis-dermatomyositis. We report clinical and hemodynamic characteristics and response to vasoactive therapy in three patients with polymyositis-dermatomyositis and pulmonary hypertension and discuss them in light of the available literature.

Challenges in the diagnosis and management of Nocardia infections in lung transplant recipients.

BACKGROUND: Nocardia infection occurs in 2.1-3.5% of lung transplant recipients, and may involve cavitary nodular pulmonary lesions, soft tissue infection, or other sites of dissemination. Nocardiosis can pose challenging clinical problems in the areas of diagnosis and treatment. Diagnostic delays may occur, and adverse reactions to therapy are common. This study reviews clinical and epidemiological aspects of nocardiosis in lung transplant recipients, with special attention to pitfalls in management. Clinicians should be alert for these possibilities in order to institute prompt therapy and to achieve successful outcomes. METHODS: A retrospective cohort study was conducted of 577 lung transplant recipients from January 1991 to May 2007. Demographics, reason for transplant, recent rejection, time from transplantation, site of infection, hypogammaglobulinemia, and/or neutropenia shortly before onset, Pneumocystis jiroveci prophylaxis, Nocardia species, radiographic findings, extrapulmonary lesions, nature and duration of treatment, adverse reactions, and outcomes were recorded. RESULT: Nocardia infection occurred in 1.9% (11/577). Mean onset was 14.3 months after transplant (range 1.5-39 months). N. asteroides was isolated in 55% (6/11). Emphysema was the most common reason for transplant (7/11, 64%). Six patients were receiving trimethoprim-sulfamethoxazole (TMP-SMX) prophylaxis at the time of diagnosis. Three patients had immune globulin G levels <400 mg/dL and 2 were neutropenic in the 3 months preceding diagnosis. Diagnosis was made by bronchoalveolar lavage (55%), skin abscess culture (18%), open lung biopsy (9%), pleural fluid (9%), and sputum culture (9%). Definitive diagnosis required a median of 9 days and a mean of 13.6 days (range 3-35 days) from the time of diagnostic sampling. Soft tissue lesions occurred in 3 and central nervous system involvement in 1 patient. Adverse reactions to therapy occurred in 9/10 (90%) of patients for whom information was available. Nocardia-related mortality occurred in 2/11 patients (18%). CONCLUSIONS: Nocardiosis occurred in 1.9% of lung transplant recipients and was associated with a mean of nearly 2 weeks to diagnosis and frequent adverse effects on therapy. TMP-SMX prophylaxis on a thrice weekly basis did not prevent all episodes of nocardiosis. Despite utilization of protocol bronchoscopies with cultures for Nocardia, this organism remains a source of clinical complexity in the lung transplant population.

Utility of transbronchial needle aspiration in the diagnosis of endobronchial lesions.

BACKGROUND: The utility of transbronchial needle aspiration (TBNA) in visible endobronchial lesions presenting as either an exophytic mass lesion (EML) or submucosal and peribronchial disease (SPD) is not well established. OBJECTIVE: To compare the yield of conventional diagnostic procedures (CDP) (bronchial washing, bronchial brushing, and endobronchial forceps biopsy) with that obtained from a combination of CDP and TBNA (CDP + TBNA). DESIGN: Prospective study of 55 patients. SETTING: Tertiary-care referral hospital. RESULTS: Of the 55 patients in whom malignancy was confirmed, CDP + TBNA identified 53 (96%) vs 42 (76%) identified by CDP (p = 0.001). The highest yield from any individual procedure was obtained by TBNA. Of the 23 patients with SPD, 22 (96%) were diagnosed using CDP + TBNA compared with 15 (65%) by CDP (p = 0.016); the yield from TBNA alone (22 of 23) in this group surpassed the combined yield from all other procedures. Although no statistically significant difference in yield was observed for EML, the use of TBNA identified four additional patients compared with CDP. CONCLUSION: We conclude that the addition of TBNA to CDP increases diagnostic yield in patients with visible endobronchial lesions.

Therapy for alpha1-antitrypsin deficiency: pharmacology and clinical recommendations.

Alpha1-antitrypsin (A1AT) deficiency is inherited as an autosomal codominant disorder characterised by reduced levels of A1AT in the serum. Low levels of A1AT in blood perfusing the lung cause low levels in the lung interstitium, making it susceptible to proteolytic damage from resident neutrophil elastase. A 'protective threshold' serum A1AT level of 11 micromol/L has been identified by epidemiological studies as a minimum value below which there is an increased risk of emphysema. Intravenous augmentation therapy for patients with severe deficiency of A1AT has been shown to have biochemical efficacy. Although the clinical efficacy of intravenous augmentation therapy has not been demonstrated in a randomised clinical trial, available studies suggest that augmentation therapy is associated with a slowed rate of decline of lung function and enhanced survival. The criteria for patient selection include: age >18 years, serum A1AT level

Infection control in the bronchoscopy suite. A review.

Bronchoscopy can occasionally transmit disease. Infection control in the bronchoscopy suite is especially important because of the risk of transmitting HIV or tuberculosis. Many case reports, patient series, and small studies have been published, but little comprehensive guidance is available for clinicians who wish to learn more about the problem and prevent it. We review the literature and describe three ways in which bronchoscopy can cause disease: by transmitting infections between patients, by transferring microorganisms within a patient, and by triggering coughing that can cause airborne infection of patients or health-care workers. Recommendations for infection control are listed; they include installing powerful air filters, using disposable bronchoscope suction valves, manually cleaning all equipment before disinfection, controlling patient coughing, and in some cases, giving patients prophylactic antibiotics.

Manifestations of scleroderma pulmonary disease.

Scleroderma is a multisystem disease of unknown cause characterized by synthesis and deposition of excessive extracellular matrix and vascular anti-GBM antibodies, leading to pulmonary hemorrhage and glomerulonephritis with rapidly progressive renal insufficiency. Recent advances in the understanding of disease pathogenesis and diagnosis and treatment have significantly improved our ability to recognize the syndrome, distinguish it from other similar disorders, and offer successful treatment. This article focuses on the pathogenetic features, clinical manifestations, diagnostic strategies, and therapeutic principles of anti-GBM disease.

Pulmonary involvement in Niemann-Pick disease: case report and literature review.

Niemann-Pick disease (NPD) is a rare, inherited, autosomal recessive, lipid storage disease. The pathognomonic intracellular accumulation of sphingomyelin results in the production and accumulation of 'foam cells'. Interstitial lung disease is a rare manifestation of NPD. We present the case of a 48-year-old white female with NPD involving the lungs, liver and spleen. The chest radiograph showed bilateral, predominantly basal reticulonodular infiltrates and serial pulmonary function tests over a period of years showed preserved expiratory airflow and a severely decreased diffusion capacity for carbon monoxide (DLCO). In view of her visceral involvement, lack of neurological symptoms and survival into adulthood, we believe our patient represents a case of type B NPD. In this type of NPD, aside from prominent hepatosplenomegaly and sexual immaturity, significant pulmonary infiltration with 'Pick cells' has been reported. To date, no therapeutic modality has been shown to alter the natural history of this disease, which results in progressive debilitation and death. This case is unique in that it provides the longest physiological follow-up in the literature, and provides data on the natural history of pulmonary involvement in NPD.

Coccidioidomycosis in non-endemic areas: a case series.

Coccidioidomycosis is a systemic infection caused by the soil fungus Coccidioides immitis, which is endemic to the south-western United States. Manifestations range from flu-like illness to pneumonia and septic shock. Diagnosis may be delayed or missed in non-endemic areas because of the low index of suspicion. We describe a series of 23 patients with coccidioidomycosis at one institution in a non-endemic area. Diagnosis was often delayed. In two patients, the route of exposure could not be determined, but 20 patients had a history of residence or travel to endemic areas, and the remaining patient had an occupational history of exposure to fomites from an endemic region. Five patients were immunosuppressed. Most patients responded well to medical therapy, surgery, or both. Although coccidioidomycosis is rare in non-endemic areas, physicians must keep it in mind when evaluating patients who have traveled to endemic areas or who are immunosuppressed.

Hernia of the lung: a case report and review of literature.

Lung hernia is a protrusion of pulmonary tissue through an abnormal defect in the wall of the thoracic cavity. Though the hernias may be congenital, they are usually acquired following a penetrating injury and surgical intervention. Symptoms are usually vague and infrequent. Pain and discomfort may be present at the site of a chest swelling that increases with forced expiration and valsalva and is readily reducible. Diagnosis may be confirmed radiologically by an oblique chest roentgenogram or a computed tomographic scan. Lung hernias only occasionally require surgical repair. We report the case of a 38-year-old man with acquired lung hernia, through a thoracotomy scar, and presenting as chest pain. A review of the literature is presented.

Impact of switching from epoprostenol to IV treprostinil on treatment satisfaction and quality of life in patients with pulmonary hypertension.

BACKGROUND: Transition from intravenous (IV) epoprostenol to IV treprostinil in patients with pulmonary hypertension (PH) has traditionally been performed by gradually decreasing the epoprostenol dose while increasing the treprostinil dose. Preliminary data suggest that this transition can be performed more rapidly without the need for epoprostenol weaning. We conducted a single center, prospective clinical trial to assess the safety, efficacy, and treatment satisfaction of rapidly switching from epoprostenol to IV treprostinil. METHODS: This study included patients with PH who had rapidly transitioned from epoprostenol to IV treprostinil. Data collected included clinical status, adverse events, PH symptoms, and previously validated measures of quality of life and treatment satisfaction. RESULTS: Ten patients were enrolled in this study. Exercise capacity measured by mean 6-min walk distance was maintained from baseline throughout follow-up. Severity of disease as assessed by WHO functional class was maintained or improved for the majority of patients. Adverse events were minimal during the transition, and all patients remained on IV treprostinil throughout the follow-up period. A favorable impact on quality of life and treatment satisfaction measures was observed by eight weeks following the transition from epoprostenol to IV treprostinil. Specifically, time spent on drug preparation activities decreased by 39.5% with treprostinil compared to epoprostenol. CONCLUSIONS: Rapidly switching from epoprostenol to IV treprostinil can be achieved without safety concerns, with minimal patient monitoring and without the need for extended hospitalization, while favorably impacting on patients' quality of life.

Estimating pulmonary artery pressures by echocardiography in patients with emphysema.

In patients with emphysema being evaluated for lung volume reduction surgery, Doppler echocardiography has been used to screen for pulmonary hypertension as an indicator of increased peri-operative risk. To determine the accuracy of this test, the present authors compared the results of right heart catheterisations and Doppler echocardiograms in 163 patients participating in the cardiovascular substudy of the National Emphysema Treatment Trial. Substudy patients had both catheterisation and Doppler echocardiography performed before and after randomisation. In 74 paired catheterisations and echocardiograms carried out on 63 patients, the mean values of invasively measured pulmonary artery systolic pressures and the estimated right ventricular systolic pressures were similar. However, using the World Health Organization's definitions of pulmonary hypertension, echocardiography had a sensitivity of 60%, specificity of 74%, positive predictive value of 68% and a negative predictive value of 67% compared with the invasive measurement. Bland-Altman analysis revealed a bias of 0.37 kPa with 95% limits of agreement from -2.5-3.2 kPa. In patients with severe emphysema, echocardiographic estimates of pulmonary artery pressures correlate very weakly with right heart catheterisations, and the test characteristics (e.g. sensitivity, specificity, etc.) of echocardiographic assessments are poor.

Role of Tc-99m MIBI in the evaluation of single pulmonary nodules: a preliminary report.

BACKGROUND: Survival in bronchial carcinoma is closely related to the stage of the disease at the time of diagnosis and a single pulmonary nodule represents a potentially curable stage. This study was conducted to assess the feasibility of using Tc-99m labelled 2-methoxy isobutyl isonitrile (MIBI) to differentiate benign from malignant single pulmonary nodules. METHODS: A prospective study was conducted in the outpatient pulmonary clinic at the Cleveland Clinic Foundation. Twenty five patients with single pulmonary nodules considered indeterminate by their physicians and undergoing a procedure for tissue diagnosis were evaluated by Tc-99m MIBI SPECT scanning prior to definitive testing. Assessment of MIBI uptake was done qualitatively (subjectively) and quantitatively and correlated with the histopathology and nodule size. RESULTS: Of the 21 patients with malignant lesions, 18 had increased uptake of MIBI corresponding to the location of the nodule and were considered positive. The predominant tumour types were large cell (n = 5) and adenocarcinoma (n = 10). All four patients with benign lesions had negative MIBI scans. For malignancy the overall specificity was 100%, sensitivity was 85.7%, positive predictive value was 100%, and negative predictive value was 57%. Quantitative uptake of MIBI correlated with the diameter of the nodule with a correlation coefficient of 0.61 by Spearman's rank sum test. This relationship was statistically significant (p = 0.02). CONCLUSION: This preliminary study suggests that Tc-99m MIBI has a very high specificity and positive predictive value for malignant single pulmonary nodules and might be a useful non-invasive diagnostic modality in their management.