Task force on chronic interstitial lung disease in immunocompetent children

Abstract

Chronic interstitial lung diseases in children represent a heterogeneous group of disorders of both known and unknown causes that share common histological features. Despite many efforts these diseases continue to present clinical management dilemmas, principally because of their rare frequency that limits considerably the possibilities of collecting enough cases for clinical and research studies. Through a Task Force conducted by the European Respiratory Society, which comprised respiratory physicians and basic scientists from across Europe, 185 cases of interstitial lung diseases in immunocompetent children were collected and reviewed. The present report provides important clinically-relevant information on the current approach to diagnosis and management of chronic interstitial lung diseases in children. In addition, recommendations for the management of paediatric interstitial lung diseases, as well as new insights into interstitial lung diseases pathophysiology during childhood are discussed

Bronchiolitis obliterans after allo-SCT: clinical criteria and treatment options

“…HDPM treatment is recommended in other inflammatory lung diseases in children. 75,76 Reported side effects include transient flushing, headache, mood changes 77 and sinus bradycardia, 78 but no serious long-term events have been described in children. HDPM has been the standard treatment of BO since 1999 at our centre, and we here summarise the results.…”

Surfactant proteins in pediatric interstitial lung disease

“…Knowledge about their causes and pathomechanisms is scarce, and the therapeutic options for the affected patients are frequently very limited. In particular in children, the disease spectrum is even broader, with many diseases uniquely manifesting during infancy, prevalence being several times lower than that in adults (1), and the diseases occur in the environment of an immature immune system and a rapidly growing and developing lung (2,3). Special effort in recent years has successfully delineated the framework necessary for the investigation of entities grouped as children's interstitial lung diseases (chILD) or pediatric diffuse parenchymal lung diseases (DPLD) (4).…”

Paediatric interstitial lung disease

“…The peak time of presentation is the newborn period [8]. They have a very non-specific presentation (table 6) [9], usually with cough, tachypnoea and respiratory distress of at least 1 month in duration.…”

“…They have a very non-specific presentation (table 6) [9], usually with cough, tachypnoea and respiratory distress of at least 1 month in duration. Specific features that should be sought include: the presence of ILD in other family members [8]; any relationship to feeding or swallowing; possible inhalant triggers in the environment; or features suggestive of a multisystem disease, in particular affecting the skin, joints, eyes or kidneys. There may be failure to thrive.…”