An unusual variant of Wegener's granulomatosis and the role of integrin adhesion receptors for pulmonary lesion

We report a patient with Wegener's granulomatosis (WC) whose initial manifestation was acute renal failure. The open renal biopsy specimen revealed periglomerular granulomatosis with crescentic glomerulonephritis. Pulmonary lesion appeared late period of his illness. Both renal and pulmonary disease responded to prednisolone and cyclophosphamide treatment. Serial determination of adhesive receptors on the patient's peripheral blood lymphocytes showed upregulated expression and function when his pulmonary lesion had appeared and returned to normal when the pulmonary lesion subsided. We think that the patient represents an unusual variant of WG and discuss the role of upregulated adhesive receptors for the development of the pulmonary lesion.