Goodpasture's disease with late presentation of renal abnormality and anti-GBM autoantibody

Abstract

Goodpasture's disease without circulating autoantibodies is a rare presentation of a rare diagnosis. We present the case of a man aged 17 years who had 3 hospital admissions over a 2-month period with haemoptysis and shortness of breath. Throughout his first 2 admissions, his renal function was normal and anti-glomerular basement membrane (GBM) antibodies were negative. CT pulmonary angiogram during his second admission revealed florid and diffuse alveolar infiltrates. However, high-resolution CT chest performed 4 weeks later showed complete resolution of these changes. On his third admission, he developed acute kidney injury. A repeat CT chest revealed the reappearance of initial findings and anti-GBM antibodies were now positive. Goodpasture's disease was subsequently confirmed with renal biopsy. Our case, with delayed onset of renal impairment, initial seronegativity for anti-GBM antibodies and relapsing and remitting CT findings, emphasises the need to consider this diagnosis in the setting of otherwise unexplained pulmonary haemorrhage.

The Role of Monocytes/Macrophages In The Pathogenesis of Immune Associated Diffuse Alveolar Hemorrhage

“…SpC dysfunction with the I73T mutation was also found to develop autoimmunity accompanying with DAH [24] . Besides that, the DAH cases who had excluded the non-immune mediated pathogenic factors should be highly suspected for immune mediated either, in which some were found to had pulmonary capillaritis [25] or deposition of immune substances in the basement membranes of alveolar walls in the lung biopsy [26] , some were found to be the autoimmune diaseases or develop autoantibodies during the follow-up [27,28] , and some cases were the idiopathic pulmonary hemosiderosis (IPH). IPH was classi ed as the non-immune mediated DAH in the Tobias Peikert's classi cation system, as there was no pulmonary capillaritis or autoantibody in the IPH.…”

Rapidly Progressive Glomerulonephritis with Delayed Appearance of Anti-Glomerular Basement Membrane Antibody Successfully Treated with Multiple Courses of Steroid Pulse Therapy

“…Typically, the anti-GBM antibody titer is also a good indicator of disease activity [4, 9], but occasionally it cannot be detected in the early stages of disease [10, 11, 12]. In these cases, delays in appearance of anti-GBM antibodies in serum varied from 4 to 11 weeks [10, 11, 12].…”

“…Typically, the anti-GBM antibody titer is also a good indicator of disease activity [4, 9], but occasionally it cannot be detected in the early stages of disease [10, 11, 12]. In these cases, delays in appearance of anti-GBM antibodies in serum varied from 4 to 11 weeks [10, 11, 12]. In two out of three cases, only lung involvement was initially evident and the positive antibody status was associated with the onset of renal involvement [10, 12].…”

“…In these cases, delays in appearance of anti-GBM antibodies in serum varied from 4 to 11 weeks [10, 11, 12]. In two out of three cases, only lung involvement was initially evident and the positive antibody status was associated with the onset of renal involvement [10, 12]. Therefore, a delay in appearance of anti-GBM antibodies in serum mostly is associated with delayed renal involvement.…”