Thrombotic thrombocytopenic purpura associated with anti-glomerular basement membrane disease.
Goodpasture's disease is associated with circulating anti-glomerular basement membrane (anti-GBM) antibodies. Thrombotic thrombocytopenic purpura (TTP) is a disease related to platelet clumping and microthrombosis in the circulation. We report an unusual case where both entities coexist in the same patient. The patient was a 43-year-old Caucasian male, with a recent history of inhalational hydrocarbon exposure for ~10 weeks. He initially presented with confusion, persistent fever and acute oliguric renal failure. In addition, he was found to be thrombocytopenic and had concurrent microangiopathic haemolytic anaemia. All presenting signs, symptoms and laboratory findings had a temporal relationship within 3 weeks. In addition, he was also found to have active pulmonary hemorrhage and positive anti-GBM antibody. During his stay, the patient underwent treatment with plasmapheresis, and an open lung biopsy, which confirmed the diagnosis of anti-GBM disease. This case report confirms previously reported findings which were noted in a few sporadic case reports about the possible association between Goodpasture's disease and TTP. In addition, it adds to our current understanding of the pathophysiology of autoimmune diseases in general and supports the theory of an autoimmune mosaic, which has also been noted in various other autoimmune diseases.
Torok
Nezam
N
University of Toledo Medical Center, Toledo, OH, USA. Nezam.Torok@utoledo.edu
Niazi
Muhammed
M
Al Ahwel
Yousef
Y
Taleb
Mohammad
M
Taji
Jamil
J
Assaly
Ragheb
R
D051722:ADAM Proteins / Q000172:deficiency; D000071120:ADAMTS13 Protein; D000328:Adult; D019867:Anti-Glomerular Basement Membrane Disease / Q000150:complications*; D006801:Humans; D008297:Male; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications*
D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human