ABSTRACT
STUDY OBJECTIVE: The object of the study was to longitudinally follow immune parameters of Aspergillus fumigatus sensitization so as to predict those at risk for developing allergic bronchopulmonary aspergillosis (ABPA). DESIGN: Patients were evaluated for 5 immune parameters (skin test [ST], positive precipitating antibody [PPN], total IgE, IgE anti-A fumigatus antibody [IgE-Af], and IgG anti-A fumigatus antibody [IgG-Af]) at yearly intervals over a 12-year time period. SETTING: Patients were enrolled and evaluated during routine visits to the cystic fibrosis (CF) clinic at Cardinal Glennon Children's Hospital, St. Louis. PATIENTS: One hundred eighteen patients with documented CF participated. INTERVENTIONS: None. MEASUREMENTS AND RESULTS: Six patients were diagnosed as having ABPA. In the non-ABPA patient group, 42% had a positive ST, 42% were PPN positive, 54% had IgE-Af, 61% had IgG-Af, and 10% had an IgE greater than 1,000 IU/mL at some point in time. However, on follow-up, 18% lost skin reactivity, 54% lost-PPN, 53% lost IgE-Af, 45% lost IgG-Af, and IgE greater than 1,000 IU/mL declined more than 72% in 64% of patients. These losses were spontaneous, without systemic corticosteroid intervention. CONCLUSIONS: Spontaneous diminution and loss of immune parameters in non-ABPA CF patients prevented us from defining a profile of sensitivity likely to result in ABPA. This variability highlights the importance of obtaining follow-up studies and including clinical symptoms when considering the diagnosis of ABPA in patients with CF.
Subject(s)
Aspergillus fumigatus/immunology , Cystic Fibrosis/immunology , Hypersensitivity/diagnosis , Adolescent , Adult , Antibodies, Fungal/analysis , Aspergillosis, Allergic Bronchopulmonary/complications , Aspergillosis, Allergic Bronchopulmonary/diagnosis , Child , Child, Preschool , Cystic Fibrosis/complications , Female , Humans , Immunoglobulin E/analysis , Immunoglobulin G/analysis , Longitudinal Studies , Male , Middle Aged , Risk Factors , Skin TestsABSTRACT
Patients with cystic fibrosis (CF) have demonstrated more arthritides with their longer life expectancy. The most common presentations have been that of an episodic arthritis or a hypertrophic osteoarthropathy. However, other arthropathies have been associated with CF. We now describe 3 new patients with CF and arthritis, and review the types of arthritis that may be found in patients with CF. This report is to increase awareness of the arthropathies that may be found in patients with CF so that proper evaluation, classification, and treatment may be facilitated.
Subject(s)
Arthritis/etiology , Cystic Fibrosis/complications , Adult , Arthritis/pathology , Female , Humans , Infant , MaleABSTRACT
Seventy-nine patients with cystic fibrosis (CF) were evaluated and were followed in a longitudinal, prospective fashion during a 6-year period for the development of immune parameters indicating Aspergillus fumigatus (Af) sensitization and allergic bronchopulmonary aspergillosis (ABPA). Although four patients developed frank ABPA, there was considerable variability in immune parameters in non-ABPA. Twenty-four patients became skin test positive to Af with none losing skin reactivity. Twenty-five patients developed serum precipitins to Af, whereas 12 patients lost their precipitins. Of 15 patients with an elevated total serum IgE of greater than or equal to 2 SD, five demonstrated a marked decline of at least 40%. Three of 16 patients with IgE-Af became negative, whereas eight of 27 patients lost their IgG-Af. None of these patients had received corticosteroid therapy that could have accounted for the findings. Thus, patients with CF frequently lose evidence of Af sensitivity spontaneously without corticosteroid intervention. The diagnosis of ABPA in CF should not be based solely on serology and skin test results, since at any point in time, patients with CF may demonstrate variable responses to Af.
