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Multi-Disciplinary Approach to Thrombotic Microang ...
Multi-Disciplinary Approach to Thrombotic Microangiopathy in Lupus and Antiphospholipid Syndrome
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Pdf Summary
This case describes thrombotic microangiopathy (TMA), a syndrome marked by thrombocytopenia, microangiopathic hemolytic anemia, and kidney injury, in a 27-year-old man with systemic lupus erythematosus (SLE), class II lupus nephritis, triple-positive antiphospholipid syndrome (APLS), and immune thrombocytopenia.<br /><br />He underwent laparoscopic cholecystectomy for chronic cholecystitis and then developed worsening oliguric acute kidney injury despite IV fluids and immunosuppression adjustments. Imaging did not reveal a structural cause. Laboratory evaluation showed low complement, elevated anti-dsDNA, and positive antiphospholipid antibodies; testing for ADAMTS13 and complement factor H/I antibodies was negative. Peripheral smear did not show schistocytes.<br /><br />Kidney biopsy demonstrated mild mesangial hypercellularity consistent with class II lupus nephritis, but also significant TMA-like vascular and glomerular injury, including arteriolar thrombus and ischemic glomerular changes. The patient improved with eculizumab and temporary hemodialysis, and renal function eventually normalized without escalation of lupus-directed immunosuppression. This supported a diagnosis of secondary autoimmune TMA rather than progression of lupus nephritis.<br /><br />The report highlights that TMA can occur secondary to SLE, APLS, surgery, or other stressors and may mimic lupus nephritis flare or thrombotic thrombocytopenic purpura. Because diagnosis is challenging, a multidisciplinary approach and repeat assessment of complement, ADAMTS13, antiphospholipid, and other related studies are emphasized. Treatment options include corticosteroids, anticoagulation, dialysis, plasma exchange, mTOR inhibition, B-cell therapy, and complement blockade with eculizumab. The case underscores that TMA in lupus patients is associated with worse renal outcomes and requires early recognition and targeted management.
Asset Subtitle
Sabiha Armin
Meta Tag
Author List
Andrew Zarker, Jeffrey Chen, Sabiha Armin
Category
Clinical Vignettes
Concept
Thrombotic Microangiopathy
Concept
Thrombocytopenia
Concept
Microangiopathic Hemolytic Anemia
Concept
Renal Dysfunction
Concept
Thrombotic Thrombocytopenic Purpura
Distinguished
Non-Finalist
Presenter Organization
University of Texas Health Science Center at Houston (UTHealth) Internal Medicine Program
Presenting Author
Sabiha Armin
Track
Adult
Keywords
thrombotic microangiopathy
systemic lupus erythematosus
antiphospholipid syndrome
lupus nephritis
acute kidney injury
eculizumab
complement blockade
hemolytic anemia
thrombocytopenia
kidney biopsy
Thrombotic Microangiopathy
Thrombocytopenia
Microangiopathic Hemolytic Anemia
Renal Dysfunction
Thrombotic Thrombocytopenic Purpura
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