Thrombotic thrombocytopenic purpura associated with polyarteritis nodosa

K. Fujisaki, K. Masutani, T. Yoshimitsu, K. Nakanishi, M. Matsumoto, H. Yagi, H. Ishizashi, Y. Fujimura, K. Takeda, H. Hirakata, M. Iida

Research output: Contribution to journalArticle

12 Citations (Scopus)

Abstract

We present a case of classical polyarteritis nodosa (PN) overlapping thrombotic thrombocytopenic purpura (TTP). A 70-year-old woman was transferred to our hospital because of general fatigue and fever. On admission, laboratory findings revealed leukocytosis, normochromic normocytic anemia and renal dysfunction. About one week later, she developed disturbance of consciousness, and laboratory findings revealed rapidly progressive thrombocytopenia and renal dysfunction. We suspected the presence of microscopic polyangiitis (MPA), based on mild elevation of myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibody (ANCA). On post-admission Day 11, renal biopsy was performed but the diagnosis of MPA could not be confirmed because of the absence of glomerular crescent formation or vasculitis. However, the biopsy specimen showed many collapsed glomeruli and interstitial inflammation, indicating the presence of occlusive lesions, such as vasculitis in larger arteries. We instituted methylprednisolone pulse therapy, cyclophosphamide and plasma exchange, because the clinical symptoms also satisfied the criteria of TTP. Despite the intensive treatment, the patient died on 43rd day of hospitalization due to thalamic hemorrhage. Autopsy showed typical findings of classical PN including disruption of arterial walls and fibrinoid necrosis in the medium-sized arteries of the kidneys and colon. We detected reduced activity of von Willebrand factor-cleaving protease (VWF-CP) and the presence of plasma inhibitory IgG against VWF-CP. A better understanding of the mechanisms would be useful.

Original languageEnglish
Pages (from-to)305-310
Number of pages6
JournalClinical Nephrology
Volume64
Issue number4
Publication statusPublished - Oct 1 2005

Fingerprint

Thrombotic Thrombocytopenic Purpura
Polyarteritis Nodosa
Microscopic Polyangiitis
Kidney
Vasculitis
Arteries
Biopsy
Antineutrophil Cytoplasmic Antibodies
Plasma Exchange
Leukocytosis
Methylprednisolone
Consciousness
General Hospitals
Thrombocytopenia
Cyclophosphamide
Peroxidase
Fatigue
Anemia
Autopsy
Colon

All Science Journal Classification (ASJC) codes

  • Nephrology

Cite this

Fujisaki, K., Masutani, K., Yoshimitsu, T., Nakanishi, K., Matsumoto, M., Yagi, H., ... Iida, M. (2005). Thrombotic thrombocytopenic purpura associated with polyarteritis nodosa. Clinical Nephrology, 64(4), 305-310.

Thrombotic thrombocytopenic purpura associated with polyarteritis nodosa. / Fujisaki, K.; Masutani, K.; Yoshimitsu, T.; Nakanishi, K.; Matsumoto, M.; Yagi, H.; Ishizashi, H.; Fujimura, Y.; Takeda, K.; Hirakata, H.; Iida, M.

In: Clinical Nephrology, Vol. 64, No. 4, 01.10.2005, p. 305-310.

Research output: Contribution to journalArticle

Fujisaki, K, Masutani, K, Yoshimitsu, T, Nakanishi, K, Matsumoto, M, Yagi, H, Ishizashi, H, Fujimura, Y, Takeda, K, Hirakata, H & Iida, M 2005, 'Thrombotic thrombocytopenic purpura associated with polyarteritis nodosa', Clinical Nephrology, vol. 64, no. 4, pp. 305-310.
Fujisaki K, Masutani K, Yoshimitsu T, Nakanishi K, Matsumoto M, Yagi H et al. Thrombotic thrombocytopenic purpura associated with polyarteritis nodosa. Clinical Nephrology. 2005 Oct 1;64(4):305-310.
Fujisaki, K. ; Masutani, K. ; Yoshimitsu, T. ; Nakanishi, K. ; Matsumoto, M. ; Yagi, H. ; Ishizashi, H. ; Fujimura, Y. ; Takeda, K. ; Hirakata, H. ; Iida, M. / Thrombotic thrombocytopenic purpura associated with polyarteritis nodosa. In: Clinical Nephrology. 2005 ; Vol. 64, No. 4. pp. 305-310.
@article{5e24044e9a7543d3bbc86d1b38daaf8f,
title = "Thrombotic thrombocytopenic purpura associated with polyarteritis nodosa",
abstract = "We present a case of classical polyarteritis nodosa (PN) overlapping thrombotic thrombocytopenic purpura (TTP). A 70-year-old woman was transferred to our hospital because of general fatigue and fever. On admission, laboratory findings revealed leukocytosis, normochromic normocytic anemia and renal dysfunction. About one week later, she developed disturbance of consciousness, and laboratory findings revealed rapidly progressive thrombocytopenia and renal dysfunction. We suspected the presence of microscopic polyangiitis (MPA), based on mild elevation of myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibody (ANCA). On post-admission Day 11, renal biopsy was performed but the diagnosis of MPA could not be confirmed because of the absence of glomerular crescent formation or vasculitis. However, the biopsy specimen showed many collapsed glomeruli and interstitial inflammation, indicating the presence of occlusive lesions, such as vasculitis in larger arteries. We instituted methylprednisolone pulse therapy, cyclophosphamide and plasma exchange, because the clinical symptoms also satisfied the criteria of TTP. Despite the intensive treatment, the patient died on 43rd day of hospitalization due to thalamic hemorrhage. Autopsy showed typical findings of classical PN including disruption of arterial walls and fibrinoid necrosis in the medium-sized arteries of the kidneys and colon. We detected reduced activity of von Willebrand factor-cleaving protease (VWF-CP) and the presence of plasma inhibitory IgG against VWF-CP. A better understanding of the mechanisms would be useful.",
author = "K. Fujisaki and K. Masutani and T. Yoshimitsu and K. Nakanishi and M. Matsumoto and H. Yagi and H. Ishizashi and Y. Fujimura and K. Takeda and H. Hirakata and M. Iida",
year = "2005",
month = "10",
day = "1",
language = "English",
volume = "64",
pages = "305--310",
journal = "Clinical Nephrology",
issn = "0301-0430",
publisher = "Dustri-Verlag Dr. Karl Feistle",
number = "4",

}

TY - JOUR

T1 - Thrombotic thrombocytopenic purpura associated with polyarteritis nodosa

AU - Fujisaki, K.

AU - Masutani, K.

AU - Yoshimitsu, T.

AU - Nakanishi, K.

AU - Matsumoto, M.

AU - Yagi, H.

AU - Ishizashi, H.

AU - Fujimura, Y.

AU - Takeda, K.

AU - Hirakata, H.

AU - Iida, M.

PY - 2005/10/1

Y1 - 2005/10/1

N2 - We present a case of classical polyarteritis nodosa (PN) overlapping thrombotic thrombocytopenic purpura (TTP). A 70-year-old woman was transferred to our hospital because of general fatigue and fever. On admission, laboratory findings revealed leukocytosis, normochromic normocytic anemia and renal dysfunction. About one week later, she developed disturbance of consciousness, and laboratory findings revealed rapidly progressive thrombocytopenia and renal dysfunction. We suspected the presence of microscopic polyangiitis (MPA), based on mild elevation of myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibody (ANCA). On post-admission Day 11, renal biopsy was performed but the diagnosis of MPA could not be confirmed because of the absence of glomerular crescent formation or vasculitis. However, the biopsy specimen showed many collapsed glomeruli and interstitial inflammation, indicating the presence of occlusive lesions, such as vasculitis in larger arteries. We instituted methylprednisolone pulse therapy, cyclophosphamide and plasma exchange, because the clinical symptoms also satisfied the criteria of TTP. Despite the intensive treatment, the patient died on 43rd day of hospitalization due to thalamic hemorrhage. Autopsy showed typical findings of classical PN including disruption of arterial walls and fibrinoid necrosis in the medium-sized arteries of the kidneys and colon. We detected reduced activity of von Willebrand factor-cleaving protease (VWF-CP) and the presence of plasma inhibitory IgG against VWF-CP. A better understanding of the mechanisms would be useful.

AB - We present a case of classical polyarteritis nodosa (PN) overlapping thrombotic thrombocytopenic purpura (TTP). A 70-year-old woman was transferred to our hospital because of general fatigue and fever. On admission, laboratory findings revealed leukocytosis, normochromic normocytic anemia and renal dysfunction. About one week later, she developed disturbance of consciousness, and laboratory findings revealed rapidly progressive thrombocytopenia and renal dysfunction. We suspected the presence of microscopic polyangiitis (MPA), based on mild elevation of myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibody (ANCA). On post-admission Day 11, renal biopsy was performed but the diagnosis of MPA could not be confirmed because of the absence of glomerular crescent formation or vasculitis. However, the biopsy specimen showed many collapsed glomeruli and interstitial inflammation, indicating the presence of occlusive lesions, such as vasculitis in larger arteries. We instituted methylprednisolone pulse therapy, cyclophosphamide and plasma exchange, because the clinical symptoms also satisfied the criteria of TTP. Despite the intensive treatment, the patient died on 43rd day of hospitalization due to thalamic hemorrhage. Autopsy showed typical findings of classical PN including disruption of arterial walls and fibrinoid necrosis in the medium-sized arteries of the kidneys and colon. We detected reduced activity of von Willebrand factor-cleaving protease (VWF-CP) and the presence of plasma inhibitory IgG against VWF-CP. A better understanding of the mechanisms would be useful.

UR - http://www.scopus.com/inward/record.url?scp=26844531389&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=26844531389&partnerID=8YFLogxK

M3 - Article

C2 - 16240903

AN - SCOPUS:26844531389

VL - 64

SP - 305

EP - 310

JO - Clinical Nephrology

JF - Clinical Nephrology

SN - 0301-0430

IS - 4

ER -