Changes in standards of care

First study to test the validity of the treatment of idiopathic pulmonary fibrosis, which causes inflammation and fibrosis (scarring) of the lung tissue, with cotrimoxazole.

Cotrimoxazole may improve the clinical course of the disease through eradication of Pneumocystis jiroveci colonization and other mechanisms as inhibiting the activation of alveolar macrophages and producing alterations in the surfactant system which favours the persistent activation of the inflammatory response and the development of pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease that is clinically manifested by the appearance of effort dyspnea and impaired lung function.

The natural history of the disease is poorly understood and there is no clear consensus as to the most appropriate markers for predicting patient outcome.

This pilot controlled trial aims to test the efficacy and safety of cotrimoxazole in the treatment of IPF. This novel therapeutic strategy, with very encouraging preliminary data is based on its pathophysiological basis, primarily related to the elimination of Pneumocystis colonization. That elimination, could serve as a potent weapon for reducing morbidity and mortality and the cost associated with this devastating disease.

Inclusion Criteria:

• Patient, regardless of gender, aged 18 to 80 years.
• Well-established diagnostic criteria of the Idiopathic Pulmonary Fibrosis (IPF) as ATA/ERS/JRS/ALAT 2011.
• Ability to obtain a sample of sputum or oropharyngeal washing.
• Forced Vital Capacity (FVC) above 50% from the theoretical value expected.
• Patient compliance or legal guardian to participate in this study by signing the informed consent.

Exclusion Criteria:

• Allergy / hypersensitivity or known gastrointestinal intolerance to cotrimoxazole.
• Use of immunosuppressants or corticosteroids in the previous 90 days at baseline.
• Exacerbation of IPF and / or pneumonia in the 90 days prior to baseline.
• Presence of autoimmune diseases or asthma.
• Patients with other significant diseases other than IPF. It is considered significant disease any disease or condition that, in the investigator's opinion, may jeopardize the patient's health participating in the study or influence the results of the study or the patient's ability to participate in the study.
• Pregnant or lactating or of childbearing potential not using medically approved contraceptive methods at least three months before or during trial.
• Participation in another trial with an investigational drug within 30 days or six half-lives (the larger of the two) above the baseline.