Pulmonary involvement in childhood-onset systemic lupus erythematosus: a report of five cases

Systemic lupus erythematosus (SLE) is a chronic systemic disease, which can involve multiple organs such as kidney, skin and brain. Lung is another organ that can be affected. A number of pulmonary complications including pleuritis, pneumonitis, infectious pneumonia, pulmonary haemorrhage, pulmonary hypertension and pneumothorax have been reported in patients with SLE. Pulmonary involvement is relatively frequent in adult patients; it has infrequently been reported in children with SLE. However, pulmonary manifestations may be an initial and/or life-threatening complication of SLE in children. In this paper we aim to emphasize the pulmonary involvement in childhood-onset SLE via description of our patients.

The patients, who were diagnosed with SLE at the Children's Hospital of Ankara University Medical School between 1993 and 2002, were retrospectively evaluated for evidence of pulmonary involvement. All patients fulfilled at least four of the classification criteria of the American Rheumatism Association. Using a standardized form, we obtained data regarding the age, sex and presenting complaints of the patients, previous therapies given, clinical and laboratory features, treatment and outcome. Informed consent was obtained from all patients.

During the 10-yr study period, 16 patients were diagnosed with childhood-onset SLE. Five of them (31%) had pulmonary involvement including acute lupus pneumonitis, invasive pulmonary aspergillosis, cytomegalovirus pneumonia and pulmonary haemorrhage (in two patients). These 5 patients with lupus lung disease are presented in more detail.