Successful treatment for thrombotic thrombocytopenic purpura complicated with myeloperoxidase anti-neutrophil cytoplasmic autoantibody-associated vasculitis

Yamauchi, Yoko; Nagatoya, Katsuyuki; Okuno, Ayako; Fujii, Naohiko; Inoue, Toru

doi:10.1093/ndtplus/sfq013

Cited by 9 publications

(12 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…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.…”

Section: Discussionsupporting

confidence: 90%

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

Section: Discussionmentioning

confidence: 89%

“…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.…”

Section: Introductionmentioning

confidence: 98%

See 2 more Smart Citations

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

Chen

Wang

Huang

et al. 2015

Clinical Journal of the American Society of Nephrology

View full text Add to dashboard Cite

Background and objectives Thrombotic microangiopathy (TMA) in ANCA-associated vasculitis (AAV) has been mainly reported in isolated case reports. The aim of this study was to analyze clinical and pathologic characteristics and prognosis of patients with renal TMA in ANCA-associated GN in a large cohort of Chinese patients.Design, setting, participants, & measurements Clinical and renal histopathologic data of 220 patients with biopsy-proven ANCA-associated GN from 1996 to 2013 were retrospectively analyzed. Patients were followed up for a median period of 32 (interquartile range [IQR], 12-65) months, and outcomes of patients were analyzed.Results Among the 220 patients with ANCA-associated GN, 30 were identified having concomitant renal TMA by pathologic evaluation. Compared with the non-TMA group, patients with renal TMA presented with more severe renal injury, as evidenced clinically by a higher level of serum creatinine at diagnosis (5.0 [IQR, 3.5-9.0] versus 3.2 [IQR, 1.7-6.8] mg/dl; P=0.02) and pathologically by a higher percentage of cellular crescents (15.0% [IQR, 6.9%-34.9%] versus 6.9% [IQR, 0%-21.1%]; P=0.04) and more severe interstitial infiltration (2 [IQR, 2-2] versus 2 [IQR, 1-2]; P=0.03) in renal biopsies. Furthermore, multivariate analysis showed that renal TMA was independently associated with mortality of patients with AAV after adjusting for age, sex, initial serum creatinine, tubular atrophy, and interstitial fibrosis (hazard ratio, 1.92; 95% confidence interval, 1.08 to 3.41; P=0.03) or for age, sex, the histopathologic classification scheme proposed by Berden et al. (J Am Soc Nephrol 21: 1628-1636, tubular atrophy, and interstitial fibrosis (hazard ratio, 1.95; 95% confidence interval, 1.07 to 3.55; P=0.03).Conclusions Renal TMA in ANCA-associated GN is not rare and presents with more severe renal injury. Renal TMA is independently associated with all-cause mortality in patients with AAV.

show abstract

Section: Discussionsupporting

confidence: 90%

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

Section: Discussionmentioning

confidence: 89%

See 1 more Smart Citation

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

Chen

Wang

Huang

et al. 2015

Clinical Journal of the American Society of Nephrology

View full text Add to dashboard Cite

show abstract

“…Rituximab (150 mg/m 2 ) was considered because of its CD20(+)B-cell-depleting action and possible suppression of auto-antibody production, and a single administration helped to achieve clinical remission, although the patient remained on dialysis support. Another case report described a 59-yearold woman who presented with MPO-antibody vasculitis and rapidly progressive glomerulonephritis and developed TTP 8 days after pulse methylprednisolone therapy [15]. The ADAMTS13 level was mildly decreased (41.4%, no inhibitory antibodies detected) and initiation of TPE corrected her microangiopathic hemolytic anemia and AKI.…”

Section: Discussionmentioning

confidence: 99%

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

Agrawal

Vaidya

et al. 2011

Pediatr Nephrol

View full text Add to dashboard Cite

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.

show abstract

“…A literature search using the databases Pubmed, Web of Science, Cochrane, and Google Scholar identified 4 case reports [6,[12][13][14] on patients presenting with TTP and vasculitis, an uncommon association.…”

Section: Ttp and Vasculitismentioning

confidence: 99%

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

Farshad

Kanaan

Savedchuk

et al. 2019

Case Reports in Rheumatology

View full text Add to dashboard Cite

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.

show abstract

Successful treatment for thrombotic thrombocytopenic purpura complicated with myeloperoxidase anti-neutrophil cytoplasmic autoantibody-associated vasculitis

Cited by 9 publications

References 9 publications

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

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

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

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

Contact Info

Product

Resources

About