School of Medicine and Health Sciences Poster Presentations

Title

Thrombotic Thrombocytopenia Purpura Complicated with ANCA Induced Vasculitis

Poster Number

198

Document Type

Poster

Publication Date

3-2016

Abstract

Case Presentation:

A 68-year-old woman with a past medical history of hypertension and relapsing thrombotic thrombocytopenic purpura (TTP) presented with acute kidney injury. The patient had been diagnosed with TTP 9 years previously and had three subsequent relapses. The last episode was seven years prior to the biopsy. During this period, she had received treatment with steroids, Rituximab, and plasmapheresis. Non-steroidal anti-inflammatory drugs had been taken for hip pain during the previous six months but had been discontinued approximately one month before presentation due to a slight increase in serum creatinine (Cr) from a baseline of 1.0 to 1.3. Despite this measure, renal function continued to deteriorate with Cr reaching 2.3. The patient reported no fevers, chills, sinus, or respiratory symptoms. There was no skin rash. A renal ultrasound showed no evidence of hydronephrosis. Urinalysis revealed nephrotic-range proteinuria and microscopic hematuria with no dysmorphic forms. Hematologic testing revealed normocytic anemia with a hemoglobin level of 9.2. Serology revealed positive perinuclear anti-neutrophil cytoplasmic antibody (P-ANCA) at 1:320, as well as positive antinuclear antigen (ANA), anti-Smith, Sjögren syndrome-related antigen A (SSA), ribonucleoprotein (RNP), and anti-chromatin B antibodies. Anti-ds DNA antibody, cytoplasmic ANCA, and anti-glomerular basement membrane antibodies were all negative. A kidney biopsy demonstrated necrotizing glomerulonephritis with scant immune complex formation, consistent with ANCA-associated glomerulonephritis. No evidence of TTP was found on the biopsy. The patient was admitted for emergent immunosuppression, including intravenous steroids and Rituximab, resulting in improved kidney function.

Discussion:

TTP is characterized by microangiopathy that leads to thrombocytopenia and hemolytic anemia. Clinical manifestations include fever, severe renal disease, and neurological symptoms such as headaches, confusion, and even transient ischemic attacks. Autoantibodies against disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS 13) proteases are thought to be the most common cause of TTP. Several studies have shown an association between TTP and vasculitic disorders, including thromboangiitis obliterans, Behçet's disease, and ANCA-associated vasculitis. ANCA-associated vasculitis divided into three main disorders. These disorders are granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis. Renal-Limited Vasculitis (RLV) is pauci-immune necrotizing glomerulonephritis that is considered as part of GPA and MPA. Patients with RLV may subsequently develop extrarenal manifestations of GPA or MPA. We hypothesize that patients with TTP due to autoimmune antibodies are at risk for autoimmune vasculitis. These disorders are treated successfully with rituximab and steroids.

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Presented at: GW Research Days 2016

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Thrombotic Thrombocytopenia Purpura Complicated with ANCA Induced Vasculitis

Case Presentation:

A 68-year-old woman with a past medical history of hypertension and relapsing thrombotic thrombocytopenic purpura (TTP) presented with acute kidney injury. The patient had been diagnosed with TTP 9 years previously and had three subsequent relapses. The last episode was seven years prior to the biopsy. During this period, she had received treatment with steroids, Rituximab, and plasmapheresis. Non-steroidal anti-inflammatory drugs had been taken for hip pain during the previous six months but had been discontinued approximately one month before presentation due to a slight increase in serum creatinine (Cr) from a baseline of 1.0 to 1.3. Despite this measure, renal function continued to deteriorate with Cr reaching 2.3. The patient reported no fevers, chills, sinus, or respiratory symptoms. There was no skin rash. A renal ultrasound showed no evidence of hydronephrosis. Urinalysis revealed nephrotic-range proteinuria and microscopic hematuria with no dysmorphic forms. Hematologic testing revealed normocytic anemia with a hemoglobin level of 9.2. Serology revealed positive perinuclear anti-neutrophil cytoplasmic antibody (P-ANCA) at 1:320, as well as positive antinuclear antigen (ANA), anti-Smith, Sjögren syndrome-related antigen A (SSA), ribonucleoprotein (RNP), and anti-chromatin B antibodies. Anti-ds DNA antibody, cytoplasmic ANCA, and anti-glomerular basement membrane antibodies were all negative. A kidney biopsy demonstrated necrotizing glomerulonephritis with scant immune complex formation, consistent with ANCA-associated glomerulonephritis. No evidence of TTP was found on the biopsy. The patient was admitted for emergent immunosuppression, including intravenous steroids and Rituximab, resulting in improved kidney function.

Discussion:

TTP is characterized by microangiopathy that leads to thrombocytopenia and hemolytic anemia. Clinical manifestations include fever, severe renal disease, and neurological symptoms such as headaches, confusion, and even transient ischemic attacks. Autoantibodies against disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS 13) proteases are thought to be the most common cause of TTP. Several studies have shown an association between TTP and vasculitic disorders, including thromboangiitis obliterans, Behçet's disease, and ANCA-associated vasculitis. ANCA-associated vasculitis divided into three main disorders. These disorders are granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis. Renal-Limited Vasculitis (RLV) is pauci-immune necrotizing glomerulonephritis that is considered as part of GPA and MPA. Patients with RLV may subsequently develop extrarenal manifestations of GPA or MPA. We hypothesize that patients with TTP due to autoimmune antibodies are at risk for autoimmune vasculitis. These disorders are treated successfully with rituximab and steroids.