1/ A story about anti-GBM disease for #ThrowbackThursday — starting with the initial case report by Dr. Ernest Goodpasture himself. pubmed.ncbi.nlm.nih.gov/19680020/
2/ In late September 1918, Dr. Ernest Goodpasture admitted a patient to the hospital with what initially appeared to be a quote “typical attack of influenza.” He presented with a 3 day history of cough, dyspnea and fever to 103.0.
3/ He had no clinical evidence of pneumonia. His fever resolved and he was discharged home 3 days later. He felt ill after discharge, but returned to work. His cough persisted though, and weight loss and fatigue worsened.
4/ He was readmitted to the hospital on Novemer 6, 1918 with cough, pleuritic chest pain, bloody sputum, anemia, signs of RLL pneumonia and fever. Temp was 100.4; HR 120; RR 28; WBC 17.6, UA showed trace protein.
5/ His condition worsened and he died 3 days after admission. Autopsy was performed. Lung gross pathology and histology was consistent with diffuse pulmonary hemorrhage and light microscopy of the kidney suggested glomerulonephritis with crescents.
6/ The case was described as quote “a more unusual one, being the only instance of its kind observed by us, and I have not seen a similar one described,” but he did attribute the case to influenza though.
7/ Over the following years, similar cases were described and these were collected to form a case series of 9 patients which was published by Stanton and Tange in1958. It was this paper which coined the term Goodpasture’s syndrome. pubmed.ncbi.nlm.nih.gov/13546112/
8/ It is now thought that the initial patient had ANCA vasculitis, but the name Goodpasture Syndrome stuck
9/ The pathogenicity of anti-GBM antibodies in humans was proven by Lerner, Glassock, and Dixon in a paper published in 1967. pubmed.ncbi.nlm.nih.gov/4964566/
10/ Several experiments were described in this paper, but the most notable one involved isolation of IgG from the kidney of patient with Goodpasture’s disease.
11/ The isolated IgG was then injected into unilaterally nephrectomized monkeys. Kidney biopsies, taken just 24h after injection, showed cellular proliferation of the glomerulus. The monkeys were anuric by day 4 and died on day 6.
12/ We now know much more about the pathogenesis of anti-GBM disease including the main circulating antibody target: the NC1 domain of the alpha-3 chain of type IV collagen. This NEJM paper gives a great overview of the subject. nejm.org/doi/full/10.10…
13/ Over time, the treatment has stayed consistent with aggressive treatment. This 2001 paper, by Levy et al, outlining the prognosis of anti-GBM patients has given us great information on these patients. pubmed.ncbi.nlm.nih.gov/11388816/
14/ More recently, a subgroup of patients have been shown to have slightly different presentations and have been dubbed to have atypical anti-GBM disease. This case series by Nassir, et all outlines their clinical presentation and biopsy findings nicely. pubmed.ncbi.nlm.nih.gov/26994577/
15/ The arc of research from astute clinical observation, to elucidation of the pathogenesis, to defining the prognosis and variant phenotypes is interesting story in nephrology. Tweetorial brought to you by @ghobby, creator of @Saltwebsite
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