Increased Expression of Interleukin-9, Interleukin-9 Receptor, and the Calcium-Activated Chloride Channel hCLCA1 in the Upper Airways of Patients With Cystic Fibrosis

• Published in: The Laryngoscope 2003-06, Vol.113 (6), p.1037-1042
• Main Authors: Hauber, Hans-Peter, Manoukian, John J., Nguyen, Lily H. P., Sobol, Steven E., Levitt, Roy C., Holroyd, Kenneth J., McElvaney, Noel G., Griffin, Siobhan, Hamid, Qutayba
• Format: Article
• Language: English
• Subjects: Airway; Biological and medical sciences; Biopsy; Chloride Channels - metabolism; cystic fibrosis; Cystic Fibrosis - immunology; Cystic Fibrosis - pathology; Ent. Stomatology; Epithelial Cells - immunology; Epithelial Cells - pathology; hCLCA1; Humans; Immunoenzyme Techniques; interleukin-9; Interleukin-9 - metabolism; interleukin-9 receptor; Investigative techniques, diagnostic techniques (general aspects); Medical sciences; Mucins - metabolism; mucus; Mucus - immunology; Nasal Mucosa - immunology; Nasal Mucosa - pathology; Nasal Polyps - immunology; Nasal Polyps - pathology; Non tumoral diseases; Otorhinolaryngology. Stomatology; Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques; Receptors, Interleukin - metabolism; Receptors, Interleukin-9; Reference Values; Respiratory Mucosa - immunology; Respiratory Mucosa - pathology; Sinusitis - immunology; Sinusitis - pathology; Upper respiratory tract, upper alimentary tract, paranasal sinuses, salivary glands: diseases, semeiology
• ISSN: 0023-852X; 1531-4995
• DOI: 10.1097/00005537-200306000-00022

Objectives/Hypothesis Mucus overproduction is commonly found in airway disease in patients with cystic fibrosis. Interleukin‐9 (IL‐9) has been shown to mediate airway hyper‐responsiveness and mucus overproduction. Recently, the calcium‐activated chloride channel hCLCA1 has been described to be upregulated by IL‐9 and has been thought to regulate the expression of soluble gel‐forming mucins. We sought to examine the expression of IL‐9, interleukin‐9 receptor (IL‐9R), and hCLCA1 in the upper airway of patients with cystic fibrosis in comparison to healthy control subjects and to demonstrate the relationship of IL‐9, IL‐9R, and hCLCA1 expression with mucus production. Study Design Prospective design. Methods Biopsy samples from nasal polyps of four patients with cystic fibrosis, nasal mucosa of six patients with cystic fibrosis, sinus mucosa of eight patients with cystic fibrosis, and nasal mucosa of six healthy control subjects were stained with periodic acid‐Schiff (PAS) to identify mucus glycoconjugates. IL‐9, IL‐9R, and hCLCA1 expression was determined by immunocytochemical study. Results We demonstrated significant increases in IL‐9, IL‐9R, and hCLCA1 immunoreactivity in the mucosa of patients with cystic fibrosis compared with that found in control subjects (P .05). We also observed a significant increase in the number of mucus‐producing cells in biopsy specimens from patients with cystic fibrosis in comparison to control subjects. A positive correlation was found between hCLCA1‐positive cells and IL‐9‐positive cells (correlation coefficient [r] = 0.79, P