Shrinking lung syndrome (SLS) is an uncommon feature of systemic lupus erythematosus (SLE) characterized by dyspnea, pleuritic chest pain, diaphragmatic elevation, restrictive ventilatory defect and reduced respiratory muscle strength as measured by volitional tests. We report the case of a 28-year-old woman with overlapping features of SLE and Sjögren syndrome who developed severe SLS while receiving corticosteroids and azathioprine for severe polyarthritis. She was treated with a combination of rituximab and cyclophosphamide, which led to a dramatic improvement in her clinical condition and respiratory function tests. The increase in vital capacity was one of the highest among 35 published cases of SLS. Thus, restoring a near-normal lung function is an achievable goal in SLS, and the use of rituximab, with or without concomitant cyclophosphamide, certainly deserves further study in this setting.

Résumé La compression médullaire métastatique est une urgence médicale régulièrement rencontrée dans un milieu de soins palliatifs. L’IRM permet un diagnostic sensible, spécifique et non invasif. Le traitement de première intention reste actuellement la radiothérapie associée aux corticostéroïdes, la chirurgie – laminectomie ou corpectomie – étant réservée à des indications particulières. La situation clinique neurologique constituant le facteur pronostique principal, le diagnostic précoce et une prise en charge rapide sont essentiels.