Natural history of asthma in patients requiring long-term systemic corticosteroids.

To study the natural history of corticosteroid-dependent asthma, we evaluated 40 randomly selected adult patients with severe asthma who were refractory to management with inhaled corticosteroids and bronchodilators and who required long-term prednisone therapy (mean duration, 6.2 +/- 5.1 years). During long-term observation, 13 patients (32.5%) significantly improved; ten (25%) of these tolerated discontinuation of long-term prednisone use and three (7.5%) had decreased prednisone requirements. Three patients (7.5%) had increased requirements for prednisone. Twenty-four patients (60%) had generally unchanged, long-term prednisone requirements; of note, eight of these had significant, but temporary intervals (mean, 3.2 years) when they could be managed without prednisone. Patients with mixed asthma were more likely to tolerate discontinuation of long-term prednisone; no other factors studied were predictive of the course of asthma. Although prior to our care many patients had a history of numerous emergency room visits and hospitalizations (some for life-threatening episodes of status asthmaticus), there were few emergency room visits and hospitalizations while under strict management by our service. Variations observed in the natural history of corticosteroid requirements in asthma must be considered in designing studies seeking to evaluate efficacy of new experimental therapies for asthma.

Allergic bronchopulmonary aspergillosis. Natural history and classification of early disease by serologic and roentgenographic studies.

Eighty-four patients with allergic bronchopulmonary aspergillosis (ABPA) were evaluated for a total of 294 patient-years with a mean observation period of 3.7 years and classified by the stage of ABPA. The largest percentage of patients were in the stage IV (corticosteroid-dependent asthma stage) group. The next largest percentage were in the stage V (fibrotic, end-stage lung disease) group. Of the latter 24 patients, eight had died. In addition, we describe 13 patients with all serologic characteristics of ABPA but without central bronchiectasis. We propose that these patients have seropositive ABPA and represent the earliest cases of it that can be diagnosed in contrast with ABPA with central bronchiectasis in which lung damage is already present.

Roentgenographic and clinical staging of allergic bronchopulmonary aspergillosis.

Allergic bronchopulmonary aspergillosis (ABPA) is a disease of asthmatics that follows a protracted course. When ABPA is treated with high dose corticosteroids, it presents a difficult problem in clinical management. Five stages, based on clinical, roentgenographic, and immunologic criteria, have been identified as follows: (I) acute, (II) remission, (III) exacerbation, (IV) corticosteroid-dependent asthma, and (V) pulmonary fibrosis. We studied 24 ABPA patients actively followed for up to 11 years at our institution. We conclude that while there are no unique roentgenographic findings to define a particular stage, clinicoroentgenographic staging does aid in therapeutic management. Two major roentgenographic contributions are (1) to establish the diagnosis by demonstrating proximal bronchiectasis, and (2) to provide a baseline for an individual patient against which to monitor progressive changes and remissions.

Elephantiasis nostras 1984.

Elephantiasis nostras is a rare condition that usually presents as a persistent swelling of the lower extremity secondary to recurrent lymphangitis. We present a patient, originally referred for "angioneurotic edema of the lip," who presented with a history of several months of persistent swelling of the upper lip. There was probable evidence for recent infection of the lip that is consistent with the diagnosis of elephantiasis nostras. This disease should be included in the differential diagnosis of chronic persistent angioedema of the lip.

A familial occurrence of allergic bronchopulmonary aspergillosis: a probable environmental source.

This is the first article of a familial occurrence of allergic bronchopulmonary aspergillosis in a father and daughter. Serologic studies revealed serum precipitins and elevated IgG or IgE antibody activity against certain Aspergillus species. Investigation of the home revealed an environmental source of exposure. Another family member demonstrated serum precipitins and an IgG antibody response to the isolated Aspergillus species without evidence of allergic bronchopulmonary aspergillosis.

Emergency administration of radiocontrast media in high-risk patients.

Patients with previous anaphylactoid reactions to radiographic contrast media (RCM) are at increased risk for subsequent reactions on repeat exposure. The most efficacious pretreatment regimens require administration of medications up to 13 hours before the anticipated procedure. Emergency administration of RCM in patients requiring essential procedures precludes prolonged pretreatment. We report results of pretreatment in nine patients requiring emergency administration of RCM. We recommend hydrocortisone, 200 mg intravenously, immediately, and every 4 hours until the procedure is completed, and diphenhydramine, 50 mg intravenously, 1 hour before the procedure. No reactions occurred in these patients, suggesting that this pretreatment regimen may be valuable in prophylaxis for patients requiring emergency administration of RCM who have experienced previous anaphylactoid reactions. Although used in only one additional patient, ephedrine, 25 mg orally, 1 hour before the procedure may also be valuable.

Prevalence of adverse effects in corticosteroid dependent asthmatics.

Eighty-five corticosteroid dependent patients with respiratory diseases requiring alternate day prednisone were studied for certain adverse effects that have been reported to be associated with corticosteroid therapy. The mean age of the patients was 52 years, the average years of prednisone therapy was 5.3, and the mean dose of alternate day prednisone was 26.2 mg. In this group of 85 patients the prevalence of hypertension, peptic ulcer disease, pathologic fractures and psychosis was not statistically increased over that of the general population. None of the patients was diagnosed as having steroid-induced psychosis, pancreatitis or tuberculosis. One patient developed aseptic necrosis of the hip; however, she received daily prednisone for approximately 3.2 years before being converted to an alternate day schedule. Our results demonstrate that alternate day corticosteroid therapy can be used without significant risk of adverse effects in patients in whom it is essential for control of respiratory disease.

Recurrence of allergic bronchopulmonary aspergillosis after seven years of remission.

A staging system has been presented previously to assist in the evaluation and management of allergic bronchopulmonary aspergillosis (ABPA). One of these stages is a remission stage in which patients are free from pulmonary infiltrates. It had been uncertain whether patients with ABPA in remission were at risk for recurrences. We now present a patient in whom recurrence of ABPA developed after 7 yr of remission that demonstrates that patients with ABPA are at risk for recurrences for a prolonged period of time.

Allergic bronchopulmonary curvulariosis.

A patient with clinical and roentgenographic findings suggestive of allergic bronchopulmonary disease who presented with recurrent pulmonary infiltrates with peripheral eosinophilia was identified. Sputum cultures were positive for Curvularia lunata. Total serum IgE was elevated, and intracutaneous skin testing with C. lunata was positive. Serum precipitins against C. lunata were present with specific IgE and IgG antibody indexes elevated. These studies provide further Immunologic characterization of this uncommon disorder.