sildenafil-citrate and Bronchiectasis

Pulmonary non-tuberculous mycobacterial (PNTM) disease has increased over the past several decades, especially in older women. Abnormal mucociliary clearance and abnormal nasal nitric oxide (nNO) have been associated with PNTM disease in other patient cohorts. Mucociliary clearance can be affected by NO-cyclic guanosine monophosphate signalling and, therefore, modulation of the pathway may be possible with phosphodiesterase inhibitors such as sildenafil as a novel therapeutic approach.. To define ex vivo characteristics of PNTM disease affected by sildenafil.. Subjects with PNTM infections were recruited into an open-label dose-escalation trial of sildenafil. Laboratory measurements and mucociliary measurements-ciliary beat frequency, nNO and 24-hour sputum production-were collected throughout the study period. Patients received sildenafil daily during the study period, with escalation from 20 to 40 mg three times per day.. Increased ciliary beat frequency occurred after a single dose of 40 mg sildenafil and after extended dosing of 40 mg sildenafil. The increase ciliary beat frequency was not seen with 20 mg sildenafil dosing. There were no changes in sputum production, nNO production, Quality of Life-Bronchiectasis-NTM module (QOL-B-NTM) questionnaire or the St George's Respiratory Questionnaire during the study period.. Sildenafil, 40 mg, increased ciliary beat frequency acutely as well as with extended administration.

Topics: Aged; Aged, 80 and over; Bronchiectasis; Cilia; Female; Health Surveys; Humans; Lung Diseases; Middle Aged; Mucociliary Clearance; Mycobacterium Infections, Nontuberculous; Nasal Mucosa; Nitric Oxide; Nontuberculous Mycobacteria; Phosphodiesterase 5 Inhibitors; Quality of Life; Sildenafil Citrate; Treatment Outcome

Primary ciliary dyskinesia (PCD) is an autosomal recessive heterogeneous group of conditions with variable clinical findings like recurrent respiratory tract infections, bronchiectasis, situs inversus, singly or in various combinations. Development of Pulmonary arterial hypertension can be a late complication of this disease. Here we present a case of PCD with recurrent respiratory tract infections, bronchiectasis and severe PAH, who responded to treatment with Oxygen, IV broad spectrum antibiotics and oral sildenafil.

Topics: Adult; Anti-Bacterial Agents; Bronchiectasis; Cardiotonic Agents; Ciliary Motility Disorders; Digitalis Glycosides; Familial Primary Pulmonary Hypertension; Humans; Hypertension, Pulmonary; Male; Oxygen Inhalation Therapy; Piperazines; Purines; Recurrence; Respiratory Tract Infections; Sildenafil Citrate; Sulfones; Tomography, X-Ray Computed; Treatment Outcome; Vasodilator Agents