Immunocompetence , Nocardia Infections/microbiology , Nocardia/pathogenicity , Respiratory Tract Infections/microbiology , Administration, Inhalation , Adrenal Cortex Hormones/administration & dosage , Adrenal Cortex Hormones/adverse effects , Anti-Bacterial Agents/therapeutic use , Bronchiectasis/complications , Bronchiectasis/diagnosis , Bronchiectasis/drug therapy , Female , Humans , Middle Aged , Nocardia/isolation & purification , Nocardia Infections/diagnosis , Nocardia Infections/drug therapy , Nocardia Infections/immunology , Pseudomonas Infections/complications , Pseudomonas Infections/diagnosis , Pseudomonas Infections/drug therapy , Respiratory Tract Infections/diagnosis , Respiratory Tract Infections/drug therapy , Respiratory Tract Infections/immunology , Risk Factors , Sputum/microbiology , Tomography, X-Ray Computed , Treatment Outcome
Vasculitis is a well recognised complication of Cystic Fibrosis. Corticosteroids are the mainstay of treatment but some cases can be resistant and may require additional disease modifying agents. We describe a case of steroid resistant cutaneous vasculitis which was successfully treated with chloroquine in addition to corticosteroids and a subsequent relapse with chloroquine alone.
Antirheumatic Agents/administration & dosage , Chloroquine/administration & dosage , Cystic Fibrosis/complications , Vasculitis/drug therapy , Vasculitis/etiology , Adult , Exanthema/drug therapy , Exanthema/etiology , Exanthema/pathology , Humans , Male , Skin/blood supply , Skin/pathology , Vasculitis/pathology
BACKGROUND: Gastroesophageal reflux is one of the most common causes of chronic cough in the general population. Reflux occurs frequently in patients with cystic fibrosis (CF). We undertook laparoscopic Nissen fundoplication in adult CF patients with a clinical diagnosis of reflux cough who had failed conventional medical therapies. OBJECTIVE: We determined the response to the surgical route in the treatment of intractable reflux cough in CF. METHOD: Patients with refractory cough were assessed by 24 h pH monitoring and oesophageal manometry. Pre-and post-operation cough, lung function and exacerbation frequency were compared. Cough was assessed by the Leicester Cough Questionnaire (LCQ), lung function by spirometry and exacerbation frequency was defined by comparing the postoperative epoch with a similar preoperatively. RESULTS: Significant abnormalities of oesophageal function were seen in all patients studied. 6 patients (2 females), with the mean age of 34.5 years consented to surgery. Their mean number of reflux episodes was 144.4, mean DeMeester score was 39.2, and mean lower oesophageal sphincter pressure 12.4 mmHg. There was a small change in the FEV1 from 1.03 L to 1.17 (P = 0.04), and FVC improved from 2.62 to 2.87 (P = 0.05). Fundoplication lead to a marked fall in cough with the total LCQ score increasing from 11.9 to 18.3 (P = 0.01). Exacerbation events were reduced by 50% post operatively. CONCLUSION: Whilst there is an obvious attention to respiratory causes of cough in CF, reflux is also a common cause. Fundoplication is highly effective in the control of reflux cough in CF. Significant reduction in exacerbation frequency may indicate that reflux with possible aspiration is a major unrecognised contributor to airway disease.
