Infect Chemother.  2011 Feb;43(1):82-85. 10.3947/ic.2011.43.1.82.

ANCA-associated Vasculitis after Scrub Typhus

• Kang Y
• Jang HW
• Han SH
• Jeong SJ
• Ku NS
• Baek JH
• Kim CO
• Choi JY
• Song YG
• Lee S
• Park YB
• Lee SK
• Kim SM
• Kim JM

• KMID: 2170340
• DOI: http://doi.org/10.3947/ic.2011.43.1.82

Abstract

Anti-neutrophilic cytoplasmic antibody (ANCA)-associated vasculitis is a primary systemic vasculitis that affects the small vessels, and ANCA is involved as the common pathogenesis. Environmental factors such as infectious agents have been considered to play a role in triggering the autoimmunity. We report here on a case of ANCA-associated vasculitis that developed after scrub typhus. A 64-year-old male was admitted because of fever, chills, pain, weakness and hypoesthesia of his calves. He was diagnosed as having scrub typhus based on the findings of an eschar and the positive serum anti-orientia antibody. The fever continued despite the antibiotic treatment. Neurologic symptoms such as numbness, hypoesthesia and weakness began to develop in the hands, feet and calves with a persisting fever. The nerve conduction velocity study revealed mononeuritis multiplex of the superficial peroneal nerve and the median nerve. Microscopic hematuria then additionally developed, and the serology showed a positive myeloperoxidase (MPO) test. A nerve biopsy was conducted on the left superficial peroneal nerve and the result showed non-infectious systemic vasculitis of the medium-small arteries. He was diagnosed as having microscopic polyangiitis along with ANCA associated vasculitis. The fever resolved and the neurologic symptoms began to improve after steroid pulse treatment (methylprednisolone 1 g/day). The neuropathy gradually improved after discharge. We presume that the ANCA-associated vasculitis was triggered by scrub typhus.

Keyword

ANCA-associated vasculitis; Scrub typhus; Tsutsugamushi disease

MeSH Terms

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
Antibodies, Antineutrophil Cytoplasmic
Arteries
Autoimmunity
Biopsy
Chills
Cytoplasm
Fever
Foot
Hand
Hematuria
Humans
Hypesthesia
Male
Median Nerve
Microscopic Polyangiitis
Middle Aged
Mononeuropathies
Neural Conduction
Neurologic Manifestations
Peroneal Nerve
Peroxidase
Scrub Typhus
Systemic Vasculitis
Vasculitis
Antibodies, Antineutrophil Cytoplasmic
Peroxidase

Figure

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  Figure 1 The nerve conduction velocity findings show mononeuritis multiplex; Conduction block and low sensory nerve action potentials are observed on the left median nerve and left superficial peroneal nerve.

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  Figure 2 The biopsy of the left superficial peroneal nerve shows non-infectious systemic vasculitis of the medium-small arteries with fibrinoid necrosis (H & E, ×430).

Reference

1. Seong SY, Choi MS, Kim IS. Orientia tsutsugamushi infection: overview and immune responses. Microbes Infect. 2001. 3:11–21.

2. Ozaki S. ANCA-associated vasculitis: diagnostic and therapeutic strategy. Allergol Int. 2007. 56:87–96.

3. Seo P, Stone JH. The antineutrophil cytoplasmic antibody-associated vasculitides. Am J Med. 2004. 117:39–50.

4. Gorson KC. Vasculitic neuropathies: an update. Neurologist. 2007. 13:12–19.

5. Jung SJ, Cheon SH, Lee CH, Park YB, Lee SK. A case of dermatomyositis associated with scrub typhus. Korean J Med. 2007. 73:Suppl 3. S1081–S1084.

6. Woo YS, Jang CR, Jeong SS, Keum DJ, Jang KY, Son CH, Lee SW, Chung WT. A case of polymyositis associated with scrub typhus. Korean J Med. 2001. 61:298–301.

7. Chang WH. Current status of tsutsugamushi disease in Korea. J Korean Med Sci. 1995. 10:227–238.

8. Molina V, Shoenfeld Y. Infection, vaccines and other environmental triggers of autoimmunity. Autoimmunity. 2005. 38:235–245.

9. Ercolini AM, Miller SD. The role of infections in autoimmune disease. Clin Exp Immunol. 2009. 155:1–15.

10. Pendergraft WF 3rd, Preston GA, Shah RR, Tropsha A, Carter CW Jr, Jennette JC, Falk RJ. Autoimmunity is triggered by cPR-3(105-201), a protein complementary to human autoantigen proteinase-3. Nat Med. 2004. 10:72–79.

11. Kain R, Exner M, Brandes R, Ziebermayr R, Cunningham D, Alderson CA, Davidovits A, Raab I, Jahn R, Ashour O, Spitzauer S, Sunder-Plassmann G, Fukuda M, Klemm P, Rees AJ, Kerjaschki D. Molecular mimicry in pauci-immune focal necrotizing glomerulonephritis. Nat Med. 2008. 14:1088–1096.

12. Gomez-Puerta JA, Bosch X. Anti-neutrophil cytoplasmic antibody pathogenesis in small-vessel vasculitis: an update. Am J Pathol. 2009. 175:1790–1798.