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Thrombotic thrombocytopenic purpura associated with anti-glomerular basement membrane disease. 2010

Nezam Torok, and Muhammed Niazi, and Yousef Al Ahwel, and Mohammad Taleb, and Jamil Taji, and Ragheb Assaly
University of Toledo Medical Center, Toledo, OH, USA. Nezam.Torok@utoledo.edu

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.

UI MeSH Term Description Entries
D008297 Male Males
D011697 Purpura, Thrombotic Thrombocytopenic An acquired, congenital, or familial disorder caused by PLATELET AGGREGATION with THROMBOSIS in terminal arterioles and capillaries. Clinical features include THROMBOCYTOPENIA; HEMOLYTIC ANEMIA; AZOTEMIA; FEVER; and thrombotic microangiopathy. The classical form also includes neurological symptoms and end-organ damage, such as RENAL FAILURE. Mutations in the ADAMTS13 PROTEIN gene have been identified in familial cases. Moschkowitz Disease,Purpura, Thrombotic Thrombopenic,Thrombotic Thrombocytopenic Purpura, Congenital,Thrombotic Thrombocytopenic Purpura, Familial,Congenital Thrombotic Thrombocytopenic Purpura,Familial Thrombotic Thrombocytopenia Purpura,Familial Thrombotic Thrombocytopenic Purpura,Microangiopathic Hemolytic Anemia, Congenital,Moschcowitz Disease,Schulman-Upshaw Syndrome,Thrombotic Microangiopathy, Familial,Thrombotic Thrombocytopenic Purpura,Upshaw Factor, Deficiency of,Upshaw-Schulman Syndrome,Familial Thrombotic Microangiopathy,Microangiopathy, Familial Thrombotic,Schulman Upshaw Syndrome,Thrombocytopenic Purpura, Thrombotic,Thrombopenic Purpura, Thrombotic,Thrombotic Thrombopenic Purpura,Upshaw Schulman Syndrome
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000071120 ADAMTS13 Protein An ADAMTS protease that contains eight thrombospondin (TS) motifs. It cleaves VON WILLEBRAND FACTOR to control vWF-mediated THROMBOSIS. Mutations in the ADAMTS13 gene have been identified in familial cases of PURPURA, THROMBOTIC THROMBOCYTOPENIC and defects in ADAMTS13 activity are associated with MYOCARDIAL INFARCTION; BRAIN ISCHEMIA; PRE-ECLAMPSIA; and MALARIA. A Disintegrin and Metalloproteinase with Thrombospondin Motifs 13 Protein,ADAMTS-13 Protein,ADAMTS13 Protease,vWF-Cleaving Protease,von Willebrand Factor-Cleaving Protease,ADAMTS 13 Protein,vWF Cleaving Protease,von Willebrand Factor Cleaving Protease
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D051722 ADAM Proteins A family of membrane-anchored glycoproteins that contain a disintegrin and metalloprotease domain. They are responsible for the proteolytic cleavage of many transmembrane proteins and the release of their extracellular domain. A Disintegrin and Metalloprotease Protein,A Disintegrin and Metalloprotease Proteins,ADAM (A Disintegrin and Metalloprotease) Proteins
D019867 Anti-Glomerular Basement Membrane Disease An autoimmune disease of the KIDNEY and the LUNG. It is characterized by the presence of circulating autoantibodies targeting the epitopes in the non-collagenous domains of COLLAGEN TYPE IV in the basement membranes of kidney glomeruli (KIDNEY GLOMERULUS) and lung alveoli (PULMONARY ALVEOLI), and the subsequent destruction of these basement membranes. Clinical features include pulmonary alveolar hemorrhage and glomerulonephritis. Anti-GBM Disease,Goodpasture Syndrome,Goodpasture's Syndrome,Lung Purpura with Nephritis,Anti GBM Disease,Anti Glomerular Basement Membrane Disease,Goodpastures Syndrome,Syndrome, Goodpasture,Syndrome, Goodpasture's

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