[Cardio-respiratory involvement in adult-onset Still's disease]

Abstract

Cardiopulmonary involvement in adult-onset Still's disease is not as common as cutaneous and articular involvement. Pleuropericarditis is the most frequent thoracic manifestation. Although difficult, diagnosis of other thoracic manifestations, which may reveal the disease, is crucial, due to the high risk to life and the efficacy of new immunosuppressive agents. The pathophysiology involves essentially immunological factors, Still's disease being increasingly seen as an autoimmune inflammatory disease. Pro-inflammatory cytokines such as interleukine (IL) 1, 6 and 18 play a crucial role in macrophage activation, which is central in the pathophysiology of adult Still's disease. The classification of cardiopulmonary manifestations is based on anatomy. Cardiac lesions may involve all the tissues of the heart and the pulmonary arteries. Respiratory lesions may involve the pleura, the lung parenchyma (organizing pneumonitis, infiltrative lung disease, alveolar damage, amyloidosis), and the respiratory muscles, including the diaphragm. Finally, some manifestations may be provoked by the treatment itself. Steroids, the first-line treatment, are very effective in pleuropericarditis. Methotrexate used to be prescribed when steroids failed, but biotherapies such as IL1 and IL6 inhibitors have transformed the prognosis of forms resistant to these drugs.