Pulmonary renal syndrome in childhood: A report of twenty-one cases and a review of the literature

In adults, the term specific pulmonary renal syndrome describes disorders w ith pulmonary and glomerular manifestations and includes Wegener's granulom atosis, Goodpasture disease, and systemic lupus erythematosus. Nonspecific pulmonary renal syndrome refers to either pulmonary disease complicating gl omerular disease, or glomerular diseases following pulmonary disease. Since little is known regarding pulmonary renal syndrome in childhood, we review ed the charts of 21 pediatric patients with pulmonary renal syndromes treat ed by the Department of Pediatrics, University of Bern between 1991 and 199 8; we also reviewed the pediatric literature that deals with specific pulmo nary renal syndromes. Specific pulmonary renal syndrome was noted in 3 children with systemic vas culitis (Wegener granulomatosis, N = 2; microscopic polyangiitis, N = 1)and 2 with systemic lupus erythematosus. Nonspecific pulmonary renal syndrome was observed in 12 patients with pulmonary edema (N = 9), pulmonary thrombo embolism (N = 2), and pulmonary infection (N = 1) complicating the course o f a glomerular disease, and in 4 children with a pulmonary disease followed by a glomerular disease. Review of the literature disclosed 52 cases of sp ecific pulmonary renal syndrome other than systemic lupus erythematosus: We gener granulomatosis (N = 28), Goodpasture disease (N = 13), and Henoch-Sch onlein purpura (N = 11). In addition, hemolytic uremic syndrome complicated pneumococcal pneumonia in 32 cases. We conclude that pulmonary renal syndromes need to be looked for in childho od. Apart from Wegener granulomatosis, Goodpasture disease, and systemic lu pus erythematosus, Henoch-Schonlein purpura and hemolytic-uremic syndrome o ccasionally have both pulmonary and renal features. Pediatr Pulmonol, 2000; 29:382-388. (C) 2000 Wiley-Liss, Inc.