Thrombotic thrombocytopenic purpura associated with anti-glomerular basement membrane disease.

Abstract

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.

• Authors: Nezam Torok, Muhammed Niazi, Yousef Al Ahwel, Mohammad Taleb, Jamil Taji, Ragheb Assaly
• Journal: Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association (Nephrol Dial Transplant) Vol. 25 Issue 10 Pg. 3446-9 (Oct 2010) ISSN: 1460-2385 [Electronic] England
• PMID: 20650907 (Publication Type: Case Reports, Journal Article)

Chemical References

• ADAM Proteins
• ADAMTS13 Protein
• ADAMTS13 protein, human

Topics

• ADAM Proteins (deficiency)
• ADAMTS13 Protein
• Adult
• Anti-Glomerular Basement Membrane Disease (complications)
• Humans
• Male
• Purpura, Thrombotic Thrombocytopenic (complications)