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Biomedical subjects

Kristin B Highland

Publications and source records attributed to Kristin B Highland.

11 recordsLinked to original sources

Clinical aspects of lung involvement: lessons from idiopathic pulmonary fibrosis and the scleroderma lung study.

Interstitial lung disease (ILD) occurs frequently among patients with systemic slcerosis (SSc) and accounts for significant morbidity and mortality. SSc-ILD resembles idiopathic pulmonary fibrosis (IPF) in many respects; each is characterized by a restrictive ventilatory defect that results from an active fibrosing process in the lung interstitium, and each is characterized by ground glass opacification on high resolution computed tomography (HRCT) and inflammatory cells in bronchoalveolar lavage fluid (BALF). Differences in the classification of lung histo-pathology are noted frequently and may account for some of the difference in outcome for patients with SSc-ILD and IPF. Optimal treatment of SSc-ILD remains to be determined, but cyclophosphamide has been reported to be effective in a number of case series. A randomized controlled trial, the Scleroderma Lung Study (SLS), will be completed in 2005; the outcome of the SLS should define the efficacy of daily oral cyclophosphamide for SSc-ILD patients with alveolitis defined by BALF and HRCT.

Comorbidity↗

New developments in scleroderma interstitial lung disease.

PURPOSE OF REVIEW: To review the recent medical literature pertaining to interstitial lung disease found in association with systemic sclerosis remains a major contributor to morbidity and mortality. Significant progress is being made in terms of understanding the pathogenesis, the best approaches to clinical evaluation, and various options for therapy of systemic sclerosis patients whose disease course is complicated by interstitial lung disease. RECENT FINDINGS: Recent studies highlight the importance of microvascular disease, autoimmunity, and fibroblast differentiation/activation in the pathogenesis of systemic sclerosis-interstitial lung disease, particularly in the early phase of disease. It appears as if the balance between various pro-fibrotic/pro-inflammatory and anti-fibrotic/anti-inflammatory mediators may be central to interstitial lung disease pathogenesis, which presents potential opportunities for therapeutic intervention. The clinical approach to staging of disease activity remains controversial. High resolution computed tomography scans, bronchoalveolar lavage and various serum markers (e.g., surfactant protein D and KL-6) each may provide useful information about the degree of activity of the systemic sclerosis-interstitial lung disease. Currently, treatment recommendations are limited by a scarcity of well designed clinical trials, but the recently completed Scleroderma Lung Study is a model for future studies and is providing useful information about this important complication of systemic sclerosis. SUMMARY: Basic and clinical studies of systemic sclerosis patients with interstitial lung disease are yielding promising data that ultimately will be translated in to more effective diagnostic and therapeutic strategies.

Biopsy↗

Pulmonary hypertension in interstitial lung disease.

PURPOSE OF REVIEW: Exercise impairment and pulmonary hypertension are common features of interstitial lung disease. Antifibrotic therapies for interstitial lung disease remain unproved; therefore, some interest has been focused on treating the pulmonary vascular impairment these diseases. RECENT FINDINGS: Patients with pulmonary hypertension secondary to idiopathic pulmonary fibrosis may have normal resting pulmonary artery pressure, but it often rises with exercise. Exercise impairment in idiopathic pulmonary fibrosis has a strong correlation with the degree of pulmonary artery pressure elevation, and hypoxemia, a hallmark of pulmonary hypertension, strongly correlates with survival. SUMMARY: Small case series have shown that some patients improve on receiving therapy for pulmonary hypertension secondary to interstitial lung disease. These findings suggest that larger treatment trials for medications targeting pulmonary hypertension in interstitial lung disease are warranted.

Exercise Test↗

Interstitial lung disease in the patient who has connective tissue disease.

Interstitial lung disease is a common complication of many of the connective tissue diseases. Because the prognosis, degree of reversibility, and optimal therapy differs for each disease presentation, a thorough knowledge of the pulmonary presentations of each connective tissue disease is important. Additionally, the challenge of finding the patient who has occult connective tissue disease in an interstitial lung disease clinic is discussed.

Arthritis, Rheumatoid↗

Inhaled corticosteroids in chronic obstructive pulmonary disease: is there a long-term benefit?

PURPOSE OF REVIEW: The use of inhaled corticosteroids is one of the most controversial issues in COPD pharmacotherapy. Experts disagree about the benefits and harms of ICS for patients with COPD, yet a majority of patients with COPD are being treated with inhaled corticosteroids. This is a review of the most recent literature on this subject. RECENT FINDINGS: Evidence suggests that ICS, with or without a long-acting beta2-agonist, are cost-effective in reducing exacerbation rates and retarding the decline in health status of COPD patients, although they do not significantly modify the rate of decline in FEV1 or change mortality. This discrepancy is likely related to the differences in pathology of COPD when compared with asthma. Evidence also suggests that ICS may be safe regarding the effects on adrenals and bone mineral density. We have yet to identify reliable criteria for predicting a response to ICS in COPD, but it has become clear that in mild disease, no beneficial effect has been demonstrated. SUMMARY: In contrast to asthma, inhaled corticosteroids should not be used as a first-line medication in patients with COPD. Identification of patients with COPD who might benefit from long-term treatment with ICS remains paramount.

Administration, Inhalation↗

Pleural effusion in interstitial lung disease.

PURPOSE OF REVIEW: This review reports recent information on the occurrence of pleural effusions in association with disorders that produce interstitial parenchymal lung disease. RECENT FINDINGS: The occurrence of effusions has been expanded to include systemic sclerosis, polymyositis-dermatomyositis, several drugs, and several miscellaneous causes of interstitial lung disease (ILD). SUMMARY: Pleural effusions occur in patients with various forms of interstitial lung disease. The effusions require a clinical evaluation to exclude complications of therapy and coexisting conditions unrelated to the underlying ILD.

Connective Tissue Diseases↗

Long-term effects of inhaled corticosteroids on FEV1 in patients with chronic obstructive pulmonary disease. A meta-analysis.

BACKGROUND: There is no consensus on the effectiveness of inhaled corticosteroids for the treatment of chronic obstructive pulmonary disease (COPD). PURPOSE: To evaluate the long-term effects of inhaled corticosteroids on the rate of FEV1 decline in patients with COPD. DATA SOURCES: MEDLINE, EMBASE, CISCOM, and AMED databases and the Cochrane Library (1966 to December 2002), reference lists from identified articles, and consultation with experts. Searches were not limited to the English language. STUDY SELECTION: Randomized, placebo-controlled trials that examined the rate of FEV1 decline as a primary outcome in patients with COPD. DATA EXTRACTION: Two reviewers independently extracted the data by using predetermined criteria. DATA SYNTHESIS: For the six studies that met the inclusion criteria, the summary estimate for the difference in FEV1 decline between the placebo and treatment groups was -5.0 +/- 3.2 mL/y (95% CI, -11.2 to 1.2 mL/y; P = 0.11). CONCLUSIONS: The use of inhaled corticosteroids was not associated with the rate of FEV1 decline in 3571 patients followed for 24 to 54 months.

Administration, Inhalation↗

Chronic obstructive pulmonary disease in geriatric critical care.

COPD is a progressive disorder that is punctuated in its later stages with acute exacerbations that present a risk for respiratory failure. COPD has a disproportionate impact on older patients. In the ICU, therapy is directed toward unloading fatigued respiratory muscles, treating airway infection, and prescribing bronchodilatory drugs. Most patients survive hospitalization in the ICU for an episode of respiratory failure. The severity of the underlying lung disease, however, underlies the poor outcomes of patients in terms of postdischarge survival and quality of life.

Aged↗

Treatment of pulmonary arterial hypertension: a preliminary decision analysis.

STUDY OBJECTIVE: New therapies for pulmonary arterial hypertension (PAH) improve functional status, quality of life (QOL), and survival. Clinicians must chose between very different therapies without the availability of comparison studies. We constructed a "virtual" clinical trial to help inform these treatment choices. DESIGN: We compare key outcomes related to survival, costs, and QOL using a Markov-type decision model to estimate the expected outcomes and costs for PAH patients treated for 1 year with bosentan and treprostinil compared to patients treated with epoprostenol, as well as patients treated with bosentan compared to those treated with treprostinil. The allowed transitions in the model were between World Health Organization functional class I to IV and death. Transition probabilities were based on observed transitions for bosentan. Treatment effect was estimated using 6-min walk data for treprostinil and epoprostenol. Utilities were calculated from estimated EuroQol health states. Cost was estimated from average wholesale price and Medicare reimbursement data. The effects of changing values of input variables on the key outcomes were calculated. RESULTS: Treatment with bosentan compared to treatment with either epoprostenol or treprostinil was less costly and resulted in a greater gain in quality-adjusted life years (QALYs). Conversely, treprostinil was significantly more expensive than epoprostenol, without an appreciable gain in QALYs. These findings were not substantially affected by the reasonable adjustments of transition probabilities, utility values, or tachyphylaxis to epoprostenol. CONCLUSION: Treatment with bosentan is more cost-effective than treatment with either treprostinil or epoprostenol. In addition, a net improvement in quality-adjusted survival may be expected.

Antihypertensive Agents↗

A "story" of a woman with cystic fibrosis.

Munchausen syndrome is a pattern of abnormal behavior in which patients repeatedly present with symptoms that have no organic basis and are intentionally simulated or produced in order to assume the sick role and gain admission to the hospital. A 25-year-old white woman presented to our adult cystic fibrosis (CF) program and gave a detailed history consistent with a diagnosis of CF, a complicated disease with multiorgan involvement that is difficult to feign. However, discrepancies in her history were noted and objective measures of disease were inconsistent, including evidence of a fabricated sweat chloride result. We describe the classic characteristics of Munchausen syndrome that our patient exhibited, and report this unusual presentation of Munchausen syndrome to be consistent with the recommendation of reporting such cases to other physicians. Such knowledge is likely to result in less harm to the patient through unnecessary testing and treatment.

Adult↗

Critical care challenges in the adult ECMO patient.

Extracorporeal membrane oxygenation (ECMO) represents an alternative method of pulmonary support for the critically ill patient with severe respiratory distress. It is commonly used in the neonatal and pediatric populations and is being used with increasing frequency in adults. Although ECMO is not new to the intensive care unit setting, it is usually considered a last resort measure in the adult population. ECMO may save a life and present an awarding challenge to the intensive care unit nurse.

Acute Disease↗