Use of 5-amino-2,3-dihydro-1,4-phthalazinedione in the treatment of rare chronic inflammatory pulmonary diseases

A composition for use in the prophylaxis or treatment of a chronic inflammatory pulmonary disease having a worldwide prevalence of 1:1500 or less, wherein said composition contains 5-amino-2,3-dihydro-1,4-phthalazinedione or one of its pharmaceutically acceptable salts, a carrier and at least one pharmaceutically acceptable excipient, and said chronic inflammatory pulmonary disease is selected from a group consisting of bronchiectasis, pulmonary alveolar microlithiasis, coalworker's pneumoconiosis, asbestosis, pneumoconiosis due to talc dust, silicosis, aluminosis of lung, bauxite fibrosis of lung, berylliosis, graphite fibrosis of lung, siderosis, stannosis, pneumoconiosis associated with tuberculosis, byssinosis, flax-dresser's disease, cannabinosis, farmer's lung, bagassosis, bird fancier's lung, suberosis, maltworker's lung, mushroom-worker's lung, maple-bark-stripper's lung, air-conditioner lung, humidifier lung, cheese-washer lung, coffee-worker lung, fishmeal-worker lung, furrier lung, sequiosis, allergic alveolitis, hypersensitivity pneumonitis, respiratory conditions due to inhalation of chemicals, gases, fumes and vapors, pneumonitis due to solids and liquids, radiation pneumonitis, fibrosis of lung following radiation, chronic drug-induced interstitial lung disorders, pulmonary permeability edema, high-altitude pulmonary edema, eosinophilic asthma, Löffler's pneumonia, tropical pulmonary eosinophilia, alveolar and parietoalveolar conditions, Hamman-Rich syndrome, abscess of lung with pneumonia, pyothorax, pleural plaque, pneumothorax, chylous effusion, fibrothorax, hemothorax, hemopneumothorax, hydrothorax, chronic pulmonary insufficiency following surgery, host-versus-graft disease after lung transplantation, graft-versus-host disease after lung transplantation, chronic lung allograft dysfunction, chronic lung allograft dysfunction—bronchiolitis obliterans syndrome, lung ischemia reperfusion injury, primary graft dysfunction after lung transplantation, Mendelson's syndrome, pulmonary collapse, atelectasis, interstitial emphysema, mediastinal emphysema, compensatory emphysema, mediastinitis, disorders of diaphragm, transient tachypnoea of newborn, congenital pneumonia due to viral agent, congenital pneumonia due to Chlamydia, congenital pneumonia due to Staphylococcus, congenital pneumonia due to Streptococcus group B, congenital pneumonia due to Escherichia coli, congenital pneumonia due to Pseudomonas, congenital pneumonia due to Haemophilus influenzae, congenital pneumonia due to Klebsiella pneumoniae, congenital pneumonia due to Mycoplasma, neonatal aspiration of meconium, interstitial emphysema originating in the perinatal period, pneumothorax originating in the perinatal period, pneumomediastinum originating in the perinatal period, pulmonary hemorrhage originating in the perinatal period, and Wilson-Mikity syndrome.

A composition comprising a combination of 5-amino-2,3-dihydro-1,4-phthalazinedione or one of its pharmaceutically acceptable salts and at least one further active agent for use in the prophylaxis or treatment of a chronic inflammatory pulmonary disease having a worldwide prevalence of 1:1500 or less, wherein said chronic inflammatory pulmonary disease is selected from a group consisting of bronchiectasis, pulmonary alveolar microlithiasis, coalworker's pneumoconiosis, asbestosis, pneumoconiosis due to talc dust, silicosis, aluminosis of lung, bauxite fibrosis of lung, berylliosis, graphite fibrosis of lung, siderosis, stannosis, pneumoconiosis associated with tuberculosis, byssinosis, flax-dresser's disease, cannabinosis, farmer's lung, bagassosis, bird fancier's lung, suberosis, maltworker's lung, mushroom-worker's lung, maple-bark-stripper's lung, air-conditioner lung, humidifier lung, cheese-washer lung, coffee-worker lung, fishmeal-worker lung, furrier lung, sequiosis, allergic alveolitis, hypersensitivity pneumonitis, respiratory conditions due to inhalation of chemicals, gases, fumes and vapors, pneumonitis due to solids and liquids, radiation pneumonitis, fibrosis of lung following radiation, chronic drug-induced interstitial lung disorders, pulmonary permeability edema, high-altitude pulmonary edema, eosinophilic asthma, Löffler's pneumonia, tropical pulmonary eosinophilia, alveolar and parietoalveolar conditions, Hamman-Rich syndrome, abscess of lung with pneumonia, pyothorax, pleural plaque, pneumothorax, chylous effusion, fibrothorax, hemothorax, hemopneumothorax, hydrothorax, chronic pulmonary insufficiency following surgery, host-versus-graft disease after lung transplantation, graft-versus-host disease after lung transplantation, chronic lung allograft dysfunction, chronic lung allograft dysfunction—bronchiolitis obliterans syndrome, lung ischemia reperfusion injury, primary graft dysfunction after lung transplantation, Mendelson's syndrome, pulmonary collapse, atelectasis, interstitial emphysema, mediastinal emphysema, compensatory emphysema, mediastinitis, disorders of diaphragm, transient tachypnoea of newborn, congenital pneumonia due to viral agent, congenital pneumonia due to Chlamydia, congenital pneumonia due to Staphylococcus, congenital pneumonia due to Streptococcus group B, congenital pneumonia due to Escherichia coli, congenital pneumonia due to Pseudomonas, congenital pneumonia due to Haemophilus influenzae, congenital pneumonia due to Klebsiella pneumoniae, congenital pneumonia due to Mycoplasma, neonatal aspiration of meconium, interstitial emphysema originating in the perinatal period, pneumothorax originating in the perinatal period, pneumomediastinum originating in the perinatal period, pulmonary hemorrhage originating in the perinatal period, and Wilson-Mikity syndrome, and said at least one further active agent is selected from a group comprising steroidal and non-steroidal anti-inflammatory drugs;

• immunomodulators;
• immunostimulatory agents;
• immunosuppressive agents;
• antibiotics;
• antiviral agents;
• antifungal agents;
• antiprotozoal agents;
• analgesics;
• anticoagulants;
• antiplatelet drugs;
• bronchodilators;
• pulmonary vasodilators;
• mucolytic agents;
• pulmonary surfactants;
• antioxidants;
• ENaC-activating agents;
• HMG-CoA reductase inhibitors, calcium antagonists or AT1 receptor antagonists.