Permanent respiratory impairment and upper airway symptoms despite clinical improvement in patients with reactive airways dysfunction syndrome.

We previously reported clinical findings for 19 patients who developed symptomatic airways hyperactivity following an acute exposure to an inhaled irritant and who were given the diagnosis of reactive airways dysfunction syndrome (RADS). We now report on nine of these patients who have been followed for a mean of 9 years, allowing assessment of function, symptoms, and comorbidity beyond the early phase of acute airway injury and inflammation. None of the patients have resolved their airway hyper-responsiveness and symptoms completely, although only in one subject, who had a premorbid history of asthma, has the condition progressed. A common feature has been sinusitis and other upper-airway symptoms. We conclude that in this group of patients, RADS presented in a consistent pattern regardless of the cause of airway injury, resolved only partially, even in subjects without a premorbid history of respiratory disease, and was associated with significant secondary morbidity, especially affecting the upper airway. This pattern was evident regardless of smoking status or age at time of exposure. We conclude that for these subjects, RADS was a distinct entity with a consistent natural history that conferred permanent impairment, but did generally improve somewhat over time.

Disability evaluation.

A physician may be called upon to evaluate disability in the roles of impartial judge of impairment, expert witness in a legal case, or treating physician. Critical to these processes are discernment between disability and impairment, an understanding of legal responsibilities, and knowledge of the elements of examinations and reports.

The many facets of occupational asthma.

Occupational asthma is an all-encompassing term that describes asthma derived from, worsened by, or encountered in an occupational setting. The diagnosis can be elusive and is easily confused with other disorders. Nevertheless, clarity of the diagnosis is essential for legal purposes. To best serve the patient, it is important to be familiar with the legal distinctions as well as proper medical management techniques.

Quantitation of abnormal 67Ga uptake in pulmonary interstitial vascular disease--a new test to detect diffuse lung disease.

Gallium 67 has been used as a modality to diagnose and follow the clinical course of diseases such as tumors, infections, inflammatory disorders, and interstitial lung disease. It has been appreciated, however, that mild to moderate changes in scan activity, when these disorders are followed over time, are less than optimal. SPECT (single-photon emission computed tomography) scanning is a new technique designed to obviate this problem. SPECT scanning utilizes computer acquisition to provide three-dimensional scanning and the additional benefit of colorization to aid in discerning differences of uptake. SPECT scanning was performed on 22 patients with interstitial lung disease of various etiologies. Additionally, 7 patients had follow-up SPECT scanning to determine their response to treatment. Two patients are presented as examples.

Myocardial sarcoidosis unresponsive to steroids. Treatment with cyclophosphamide.

Sarcoidosis affecting the pleura, pericardium, and myocardium is described in a patient who demonstrated continued disease activity while on therapy with high-dose steroids. Cyclophosphamide was found to be highly effective in suppressing her disease over a period of six years.

Gas exchange across abnormal interstitial tissue in pulmonary sarcoidosis--results of therapy.

Sarcoidosis is a granulomatous, inflammatory disorder that can affect any organ in the body. The interstitium of the lung is a frequent area of involvement. This tissue comprises the alveolar-capillary unit of the lung with the pulmonary vasculature affected either on a primary or (usually) secondary basis. Measurements of gas exchange across this tissue should be expected to parallel disease activity. Five patients with histologic and/or radiographic evidence of interstitial lung disease were evaluated by serial exercise studies over time with respect to therapy. Preexercise and postexercise values were obtained, for exercise is felt to make these measurements more sensitive to changes in gas transfer. Good correlations were seen in all five patients. Exercise studies are concluded to be of value in patients with interstitial lung disease due to sarcoidosis and may prove to be a sensitive form of follow-up during therapeutic intervention.

Hyperventilation syndrome and asthma.

Hyperventilation syndrome is a common and often disabling condition. Traditional treatment consists of reassurance and anxiolytic drugs. Hyperventilation is known to precipitate an asthmatic reaction. A retrospective review of patients with hyperventilation syndrome was performed to ascertain the frequency of asthma as well as the response to bronchodilator medication. Forty-seven patients were seen. Thirty-eight were tested, and asthma was proved in 36. Two additional patients had positive clinical responses with bronchodilators. Thus, asthma was identified in 38 of 47 consecutive patients seen for hyperventilation syndrome (80 percent), and asthma was proved in 36 of 38 of patients tested (95 percent). Hyperventilation syndrome was eliminated in 29 of 35 patients (90 percent) treated with a combination of explanation and bronchodilator treatment.

Interstitial vasculitis--interstitial lung disease: case studies.

Six patients with interstitial lung disease were tested in a serial fashion to determine response to medications. All patients had biopsy-proven interstitial lung disease. Exercise testing with measurements of PaO2, PaCO2, and DLCO (the diffusing capacity to carbon monoxide) were made before and after exercise to determine the response to medications. Three patients were treated with corticosteroids and three patients with cytotoxic medications. Results of serial testing of gas transfer correlated with improvement of interstitial lung disease as characterized by resting PaO2, symptoms, and maximal duration of exercise. The value of serial exercise testing is of great help in some, but not all patients. The value of serial testing of patients with interstitial lung disease is discussed.

Transdermal scopolamine in the treatment of asthma: a preliminary report.

The use of atropine has emerged over the past few years as a treatment for asthma. Scopolamine is pharmacologically similar to atropine and, as such, was used in a study of nine patients as a bronchodilator. These nine patients had diverse types of lung diseases. Two patients were intolerant to the medication and were not able to participate in the study. The other seven patients had variable responses with significant improvement in baseline pulmonary function testing occurring in four patients. Five of the seven patients had symptomatic relief and are now employing transdermal scopolamine on a once-every-3-days basis for the treatment of their bronchoconstriction. Baseline testing was performed to indicate response to parasympatholytic medications. Transdermal scopolamine appears to be a safe and effective means of achieving bronchodilation in some patients with asthma.

Intra-pulmonary artery streptokinase.

Fibrinolytic agents have been advocated for the treatment of massive pulmonary emboli. The usual mode of infusion is via a peripheral vein. Theoretically, infusion into the pulmonary artery would be expected to be more efficacious. A case representing the failure of intra-pulmonary artery infusion of fibrinolytic agents is presented. However, a literature review suggests that this mode of therapy may achieve a greater success in clot lysis than does peripheral infusion. Additionally, this form of treatment may allow the clinician to lower the administered dose which may represent an advantage to the patient.

Upper extremity thrombosis: etiology and prognosis.

Upper extremity thrombosis is responsible for 1-2% of all cases of deep venous thrombophlebitis (DVT). Little has changed concerning the clinical presentation, diagnostic investigation, or treatment of the disorder over the last forty years. However, a changing pattern in the natural history of the disease seems to be emerging with the advent of more invasive procedures and prolonged medical care of critically-ill individuals. Case histories of sixteen (16) patients with seventeen (17) episodes of upper extremity thrombosis are reviewed. Fewer cases of "primary" thrombosis, and fewer acute or chronic sequelae were noted in the present study than would be implied in previous reviews of the subject. It is our opinion that DVT of the upper extremity, especially when due to "secondary" thrombosis, is a more benign and self-limiting disease than has been traditionally reported.

Pulmonary manifestations of vinyl and polyvinyl chloride (interstitial lung disease). Newer aspects.

Newer varieties of occupational lung diseases primarily due to the vast increase in industrial technology have been reported recently. Preeminent among such newer agents are vinyl chloride (VC) and polyvinyl chloride. Very few cases have been reported, in Europe only, with descriptive histopathologic changes. To our knowledge, no pathologic studies of VC exposure have been described in the American literature. The biopsy abnormalities in our patients disclosed desquamation of alveolar macrophages into the alveolar lumina and minor interstitial and alveolar inflammatory changes. Pulmonary function abnormalities included restrictive insufficiency. Preventive therapy consists of the avoidance of further exposures, frequent industrial hygiene monitoring, and total avoidance of tobacco smoke, as well as associated atmospheric pollutants. Thus far, none of these patients has exhibited evidence of pulmonary neoplasms. All three patients survived their occupational injuries, and two are still disabled to varying degrees. Urine and blood levels of phthalic acid derivatives were elevated in two patients, the exact significance of which is not fully known. It probably represents a toxicologic response, but must be further pursued before conclusions can be reached.

Aortobronchial fistula: keys to successful management.

Aortobronchial fistula is a rare but highly lethal condition. If diagnosed, its survival rates are greater than 80%; if not, the condition is uniformly fatal with death most commonly due to massive hemoptysis. A review of the world's literature and an illustrated case are presented. A total of 30 cases have been described, but the published incidence may be low. Of the cases presented to sufficient detail for analysis, 92% (23 of 25) were associated with an aneurysm of the thoracic aorta. The most consistent symptom was hemoptysis (22 of 23, 96%), especially massive hemoptysis (15 of 19, 79%). Plain chest radiographs demonstrated an aneurysm in 46% (6 of 13 evaluable cases). Either an aneurysm on the chest x-ray or massive hemoptysis, or both were seen in 94% of cases (16 of 17). The most rewarding test (in 5 of 7, 71%) was the thoracic aortogram. Surgical correction was followed by an 82% survival rate (9 of 11). Untreated, the eventual mortality was 100%, although one patient died as late as 6 months after the initial presentation with massive hemoptysis.

Aortobronchial Fistula: Keys to Successful Management.

Aortobronchial fistula is a rare but highly lethal condition. If it is diagnosed,survival rates are greater than 80%; if not, the condition is uniformly fatal with death usually due to massive hemoptysis. A high index of suspicion is necessary to establish the diagnosis. The most consistent symptoms are hemoptysis(96%) and massive hemoptysis (79%). The most rewarding test is a thoracic aortogram (with a sensitivity of 71 %). Characteristics of this condition, as described in the literature, and an illustrative case are presented.