[Pleuro-pulmonary disease and rigid spine syndrome (author's transl)]

A case of rigid spine syndrome associated with a pleuropulmonary disease is reported. This case is the ninth known rigid spine syndrome but it is the first associated with a diffuse pleural and pulmonary involvement. Clinical examination showed limitation of flexion of the spine since early infancy and limitation of extension of the elbows. Fibrosis probably involved both the lung and the pleura, although microscopic examination showed limitation of flexion of the spine since early infancy and limitation of extension of the elbows. Fibrosis probably involved both the lung and the pleura, although microscopic examination are not available. Severity of respiratory failure is explained by involvement of lungs, pleura and chest wall. Future is explained by involvement of lungs, pleura and chest wall. Future studies of similar case are needed to answer the questions whether or not this association is casual and if lung and muscular involvement are linked.