Frequency and type of respiratory manifestations differ among individual rheumatic diseases in childhood as well as among children and adults. It is important to recognise respiratory complications of established diseases like juvenile systemic lupus erythematosus or dermatomyositis early enough to prevent serious disease outcomes. Nevertheless, it is of the same importance to reveal unusual characteristics of respiratory symptoms which rheumatic disease may present with, like in Wegeners granulomatosis. Therefore high degree of suspicion and good access to specialised paediatric care including paediatric rheumatology and intensive care units are often of vital importance. In this review respiratory symptoms are summarised firstly by anatomical location of affected area and secondly by individual rheumatic diseases. Where applicable, differences between paediatric and adult disease are shown and main diagnostic and therapeutic principles overviewed. Disease spectrum covers juvenile idiopathic arthritis, juvenile systemic lupus erythematosus and dermatomyositis, systemic scleroderma and primary systemic vasculitides.