IgA Antibasement Membrane Nephritis with Pulmonary Hemorrhage

Abstract

Goodpasture's syndrome has characteristically been described as being mediated by IgG antibodies. We have recently seen a 55-year-old man who developed renal failure and hemoptysis; a renal biopsy showed linear deposits of IgA and C3 involving glomerular and tubular basement membrane. Serologic tests for detecting (IgG) antiglomerular basement membrane antibodies were negative. Elution studies of kidney and lung showed the presence of an IgA antibasement membrane antibody only. The patient's serum contained IgA, but not IgG, antibodies reactive with glomerular and tubular basement membrane of normal human kidney and alveolar basement membrane of normal human lung. Attempts to transfer disease with the patient's IgA antibody to a monkey and to Lewis and Brown-Norway rats were unsuccessful. Immunoglobulin A antibasement membrane antibody must be considered in the design of immunoserologic procedures for the diagnosis of Goodpasture's syndrome.

IgA Variant of Anti-glomerular Basement Membrane Glomerulonephritis Associated with Pulmonary Hemorrhage and Microangiopathic Hemolytic Anemia

“…There have been reports of four cases, including ours, of IgA anti-GBM disease with pulmonary involvement, of which all concomitantly had clinical signs of renal involvement. 2,3,9 MAHA has been reported also in association with anti-GBM disease.…”

“…Hypocomplementemia was present in two of nine patients with available data. There is also a broad spectrum of glomerular histological findings in IgA anti-GBM GN, including normal glomeruli, 5 focal mesangial GN, 5 thin basement membrane glomerulopathy, 7 focal segmental glomerulosclerosis, 9 focal necrotizing and crescentic GN, 2,3,10,12 and diffuse crescentic GN. 8,11 Organ damage in anti-GBM disease can be confined to the kidneys, the lungs, or both.…”

“…The occurrence of GN characterized by linear staining of non-IgG class immunoglobulin along the GBM is quite rare, with only 13 previously reported cases in the worldwide literature, including 11 IgA, 2 IgM, and 1 lambda light chain. [2][3][4][5][6][7][8][9][10][11][12] Herein, we report an unusual case of IgA anti-GBM disease presenting with rapidly progressive GN, pulmonary hemorrhage (PH), and microangiopathic hemolytic anemia (MAHA).…”

Task force on chronic interstitial lung disease in immunocompetent children

“…Laboratory tests in these patients should, therefore, include antibodies against basement membrane, antinuclear antibodies against double strand DNA, and antineutrophilcytoplasmatic autoantibodies. Although false positive results do occur, these antibodies are quite specific for these three entities [20][21][22][23][24]. In paediatric patients with sarcoidosis mild elevation of ACE, as well as hypercalcaemia and hypercalciuria, can be observed [25,26].…”

Anti-Glomerular Basement Membrane Disease

“…IgG1 is the predominant IgG subclass in staining of the glomerular capillary walls (Bowman et al, 1987;Segelmark et al, 1990). Linear staining for IgM and IgA is less common, but rare cases with anti-GBM disease have linear staining only for IgA and circulating IgA-class anti-GBM antibodies in the absence of IgG-class anti-GBM antibodies in the serum or staining in glomeruli (Border et al, 1979;Gris et al, 1991;Borza et al, 2005). Granular or discontinuous linear staining for C3 is observed in most cases with anti-GBM disease, but glomerular staining for C3 is negative for some cases (Wilson and Dixon, 1973).…”