Concomitant thrombotic thrombocytopenic purpura and ANCA-associated vasculitis in an adolescent

Abstract

Thrombotic thrombocytopenic purpura (TTP) rarely occurs with systemic vasculitis. A 17-year-old girl presented with non-bloody diarrhea, menorrhagia, and syncope. She had severe anemia (hemoglobin = 3.8 g/dl), thrombocytopenia (platelet = 7,000/mm(3)), and acute kidney injury (serum creatinine, Cr = 2.3 mg%). Peripheral smear examination confirmed the presence of microangiopathic hemolytic anemia. Additionally, she had a positive anti-nuclear antibody (1:1600) and normal complement levels. We considered the diagnosis of TTP, possibly associated with systemic lupus erythematosus, and promptly initiated pulse methylprednisolone and daily 3-4 l of plasma exchange therapy. Following resolution of her thrombocytopenia in 48 h, we performed a kidney biopsy that revealed diffuse proliferative, focal crescentic, and necrotizing glomerulonephritis with mild IgG immunofluorescence staining. Concomitantly, autoimmune work-up was significant for positive perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA = 1:640) and decreased von Willebrand factor cleaving protease activity (<5%). A final diagnosis of TTP with microscopic polyangiitis (p-ANCA-mediated) was made and treatment with daily oral cyclophosphamide and prednisone resolved her renal injury over 2 months (follow-up Cr = 1.0 mg%). Our case highlights the importance of identifying systemic disorders such as ANCA-associated vasculitis with TTP.

Clinicopathologic Characteristics and Outcomes of Renal Thrombotic Microangiopathy in Anti-Neutrophil Cytoplasmic Autoantibody-Associated Glomerulonephritis

“…However, the prevalence in other countries is unclear, especially for renal TMA in ANCA-associated GN. As reported in previous case reports, patients with concomitant renal TMA in ANCA-associated GN presented with more severe renal injury (3)(4)(5)(6)(7)(8)(9). Patients with renal TMA in our cohort also had a higher level of initial serum creatinine, higher percentage of cellular crescents and fibrinoid necrosis, and more severe interstitial infiltration compared with patients without TMA, which, by a large cohort study, further extends previous findings.…”

“…intensive immunosuppressive therapy, such as plasma exchange, as described in previous case reports (3)(4)(5)(6)(7)(8).…”

“…To our knowledge, TMA in ANCA-associated GN has only been reported in isolated case reports (3)(4)(5)(6)(7)(8)(9), with two patients showing pathologic features of renal TMA. The clinicopathologic characteristics, especially the prognostic value of pathologic findings of renal TMA, in ANCA-associated GN are far from clear.…”

Renal thrombotic microangiopathy in an ANCA-associated vasculitis

“…Given the high percentage of parenchymal fibrosis and no evidence of active extra-renal manifestations by the time of biopsy, no specific treatment was initiated. Otherwise, several authors suggest that plasma exchange, treatment with corticosteroids (IV pulses followed by oral therapy), cyclophosphamide or rituximab may be indicated (3,(5)(6)(7).…”

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

“…TTP has been reported to be associated with a variety of autoimmune diseases [4][5][6]. High level of suspicion and rapid initiation of plasmapheresis, also known as plasma exchange (PEX) are critical as the mortality in TTP before the era of plasmapheresis reached 90% [7].…”