Idiopathic Crescentic Glomerulonephritis

Smoking and Immunity: an Additional Player in the Mosaic of Autoimmunity

“…This clinical picture is observed in 75% of the patients harbouring antibodies to glomerular basement membrane (GBM) [36]. Anti-GBM antibodies are actively involved in the initiation of pulmonary lesions.…”

“…Anti-GBM antibodies are actively involved in the initiation of pulmonary lesions. This is manifested by the detection of linear deposits of IgG and complement in the alveolar septa of the affected lungs [36,37]. The so-called Goodpasture's antigen towards which anti-GBM antibodies form has been found to reside in the 3 chain of type IV collagen [38].…”

Idiopathic Rapidly Progressive Glomerulonephritis

“…In several other studies a majority of patients had IF and electron microscopic (EM) findings more consistent with immune complex disease [2,7,8,[13][14][15][16]. However, most of these studies also included variable numbers of patients with negative [3,7,[13][14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29] or 'sparse' [8] glomerular immune deposits by'IF and EM that did not clearly fit into either the anti-GBM or immune complex categories. In 1978, we described the clinical and pathologic features of 16 such patients who had idiopathic RPGN with no significant glomerular immune deposits by IF or EM and no detect able circulating anti-GBM antibody or immune complexes [29], Thus, idiopathic RPGN occurs de novo as a primary renal disease probably mediated by at least three patho genetic mechanisms: glomerular immune complex forma tion, anti-GBM antibody deposition, and some additional process (or processes) apparently not related to glomerular immunoglobulin deposits.…”

“…However, it has been more frequent in some other series [2,7,8,13,16,32). Among the 20% of patients with RPGN due to anti-GBM antibody deposition, patients without pulmonary hemor rhage represent only 10-30% of the total, the remainder having Goodpasture's syndrome [2,3,7,32]. Thus, patients with idiopathic anti-GBM disease also account for less than 10% of the total group with RPGN.…”

“…Idiopathic rapidly progressive glomerulonephritis (RPGN) accounts for only 1-2% of renal biopsies in most centers [1][2][3], Despite its infrequency, however, it is the syndrome among glomerular diseases which most com mands the respect and attention of the clinical nephrolo gist. The usual fulminant course, and recent therapeutic advances which may alter that course, compel an urgency in making an accurate diagnosis and initiating therapy.…”