Systemic Lupus Erythematosus (SLE) with Acute Nephritis, Antineutrophil Cytoplasmic Antibody- (ANCA-) Associated Vasculitis, and Thrombotic Thrombocytopenic Purpura (TTP): A Rare Case Report with Literature Review
Joint Authors
Halalau, Alexandra
Karmo, Dillon
Farshad, Sohail
Kanaan, Christopher
Savedchuk, Solomiia
Swami, Abhishek
Source
Issue
Vol. 2019, Issue 2019 (31 Dec. 2019), pp.1-5, 5 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2019-12-16
Country of Publication
Egypt
No. of Pages
5
Main Subjects
Abstract EN
Thrombotic thrombocytopenic purpura (TTP) is a potentially fatal disorder that requires urgent identification and treatment.
The association of TTP with systemic lupus erythematosus (SLE) and vasculitis has been reported, however, never simultaneously.
A 33-year-old woman with a history of SLE presented with acute abdominal pain, fever, arthralgias, and skin rash.
She had acute severe hypertension, diffuse abdominal tenderness, and petechial rash.
Diagnostic work-up revealed active urine sediment with proteinuria and hematuria and elevated creatinine, anemia, and thrombocytopenia.
She was diagnosed with acute lupus nephritis and early microangiopathic hemolytic anemia in the setting of hypertensive urgency and started on intravenous methylprednisolone 500 mg once a day.
Within 48 hours, she developed shock with multiorgan dysfunction and succumbed to her illness.
Laboratory tests later showed ADAMTS13 activity less than 10% consistent with TTP and p-antineutrophil cytoplasmic antibody (ANCA) positivity.
Autopsy revealed small-vessel vasculitis of the visceral organs.
Kidney biopsy demonstrated diffuse proliferative glomerulonephritis.
This case illustrates the occurrence of SLE nephritis, p-ANCA vasculitis, and severe TTP with rapidly fatal course, and the importance of having a low threshold for initiating plasma exchange therapy.
Here, we discuss the case and provide a literature review on cases of TTP with SLE and vasculitis.
American Psychological Association (APA)
Farshad, Sohail& Kanaan, Christopher& Savedchuk, Solomiia& Karmo, Dillon& Halalau, Alexandra& Swami, Abhishek. 2019. Systemic Lupus Erythematosus (SLE) with Acute Nephritis, Antineutrophil Cytoplasmic Antibody- (ANCA-) Associated Vasculitis, and Thrombotic Thrombocytopenic Purpura (TTP): A Rare Case Report with Literature Review. Case Reports in Rheumatology،Vol. 2019, no. 2019, pp.1-5.
https://search.emarefa.net/detail/BIM-1144320
Modern Language Association (MLA)
Farshad, Sohail…[et al.]. Systemic Lupus Erythematosus (SLE) with Acute Nephritis, Antineutrophil Cytoplasmic Antibody- (ANCA-) Associated Vasculitis, and Thrombotic Thrombocytopenic Purpura (TTP): A Rare Case Report with Literature Review. Case Reports in Rheumatology No. 2019 (2019), pp.1-5.
https://search.emarefa.net/detail/BIM-1144320
American Medical Association (AMA)
Farshad, Sohail& Kanaan, Christopher& Savedchuk, Solomiia& Karmo, Dillon& Halalau, Alexandra& Swami, Abhishek. Systemic Lupus Erythematosus (SLE) with Acute Nephritis, Antineutrophil Cytoplasmic Antibody- (ANCA-) Associated Vasculitis, and Thrombotic Thrombocytopenic Purpura (TTP): A Rare Case Report with Literature Review. Case Reports in Rheumatology. 2019. Vol. 2019, no. 2019, pp.1-5.
https://search.emarefa.net/detail/BIM-1144320
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1144320