Wegener's Granulomatosis: An Unfolding Enigma

First described by Klinger (1) and Rossale (2), the clinico-morphological entity named after Friedrich Wegener did not attract international attention until 1936 (3-6). Of unknown etiology, Wegener's granulomatosis (WG) is a systemic necrotising vasculitis associated with ulceration, granuloma formation, and glomerulonephritis (GN) (7,8), It involves predominantly the upper airways, the lungs and the kidneys; but also at times the eyes, ears, CNS, skin, joints, heart, gastrointestinal tract, liver, spleen, breast, salivary glands and gonads (9-11). It is remarkably similar all over the world (12-15). Classic WG is a triad of airway, lung and renal disease; but the disease may be confined to the lung and spare the kidneys (16). It is indeed possible to view WG within the framework of a continuously evolving spectrum of various permutations and combinations of organ involvement all easily identified except for isolated renal disease (17). It is often difficult to distinguish with certainty the glomerulonephritis of WG from other types using routine histology and immunofluorescence (17). Some patients present initially with isolated glomerulonephritis but later develop classic pulmonary WG (18). In such patients the diagnosis can be made only after the extrarenal manifestations appear (18). Some patients evolve from a single affected organ through a second one to the classic form of WG (7, 17). For nearly 30 years WG remained unresponsive to treatment (6,7). Patients with classic disease survived only a few months and rarely lived longer (7). For the limited form the prognosis was somewhat better but still remained grave (16). All this was changed with the introduction of cyclophosphamide (9, 11). Indeed the dramatic response to cyclophosphamide also led to a radical revision of the treatment protocols for other The International Journal Of Artificial Organs / Vol. 11 / no. 5, 1988/ pp. 322-324