Muscular polyarteritis nodosa as a cause of fever of undetermined origin: a case report and review of the literature

Kamimura, Takeshi; Hatakeyama, Makio; Torigoe, Kimiaki; Nara, Hiroyuki; Kaneko, Naoko; Satou, Hidetomo; Yoshio, Taku; Okazaki, Hitoaki; Minota, Seiji

doi:10.1007/s00296-004-0531-4

Cited by 39 publications

(14 citation statements)

References 12 publications

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“…The vascular pathology in chronically T. cruzi-infected mice has some features that resemble human polyarteritis nodosa (PAN) with regard to multiorgan involvement of medium-sized to small arteries (21), fibrinoid necrosis, mixed cellular infiltrates including pathogen-specific CD8 ϩ T cells and macrophages (22), lack of immune complex deposition in lesions, and involvement of muscles, skin, and nerves (23)(24)(25)(26). The most notable and interesting differences between the mouse vasculitis we analyzed and PAN include an absence in the mouse of the eponymous skin vasculitic nodules found in human PAN, a lack of neutrophilic inflammation in the mouse, and the relative prominence of the extreme involvement of skeletal muscle in the mouse, which is associated with muscle weakness and paralysis.…”

Section: Discussionmentioning

confidence: 99%

Trypanosoma cruzi Causes Paralyzing Systemic Necrotizing Vasculitis Driven by Pathogen-Specific Type I Immunity in Mice

et al. 2016

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dInfectious agents are often considered potential triggers of chronic inflammatory disease, including autoimmunity; however, direct evidence is usually lacking. Here we show that following control of acute infection of mice with the myotropic Colombiana strain of Trypanosoma cruzi, parasites persisted in tissue at low levels associated with development of systemic necrotizing vasculitis. Lesions occurred in many but not all organs and tissues, with skeletal muscle arteries being the most severely affected, and were associated with myositis, atrophy, paresis/paralysis, and death. Histopathology showed fibrinoid vascular necrosis, rare amastigote nests within skeletal muscle myocytes, and massive leukocyte infiltrates composed mainly of inflammatory monocytes, F4/80 ؉ macrophages, and T. cruzi tetramer-specific CD8 ؉ T lymphocytes capable of producing gamma interferon (IFN-␥) and tumor necrosis factor alpha (TNF-␣) but not interleukin-17 (IL-17). T. cruzi-specific IgG was detected in sera from infected mice, but antibody deposits and neutrophilic inflammation were not features of the lesions. Thus, T. cruzi infection of mice may be a specific infectious trigger of paralyzing systemic necrotizing vasculitis most severely affecting skeletal muscle, driven by pathogen-specific type I immune responses. Vasculitis is a general term for a spectrum of diseases involving leukocyte infiltration of the blood vessel wall. Many subtypes of vasculitis are recognized based on the types of blood vessels involved and the nature of the underlying immunopathology. In some cases, an association of vasculitis with specific infectious agents has been reported; for example, polyarteritis nodosa (PAN) has been associated with hepatitis B virus (HBV), hepatitis C virus (HCV), and HIV infections (1). However, it has been difficult to establish either causality or pathogenetic mechanisms, in part because of the low prevalence of vasculitis and the lack of good animal models (2). In this regard, while studying the chronic phase of C57BL/6J mice infected with the myotropic Colombiana strain of Trypanosoma cruzi, the infectious cause of Chagas' disease in humans, we noticed that the majority of animals developed hind limb paralysis associated with severe systemic necrotizing vasculitis affecting arteries and arterioles in skeletal muscle. Lesions were also present in many other tissues. A review of the literature identified several studies from 1970 to 2000 that described perivascular inflammation and frank arteritis in chronically T. cruzi-infected mouse skeletal muscle, aorta, nerves, and heart (3-9); however, the underlying immunologic mechanisms of disease were not defined. Interestingly, Okumura et al. reported necrotizing arteritis in small arteries beneath the peritoneal lining of the bowel and, occasionally, of the aorta and coronary arteries of T. cruzi-infected mice, which was at that time interpreted as "allergic necrotizing angiitis" (10). Dias et al. reported obliterative changes of the small and medium-sized branches of coronary...

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Section: Discussionmentioning

confidence: 99%

Trypanosoma cruzi Causes Paralyzing Systemic Necrotizing Vasculitis Driven by Pathogen-Specific Type I Immunity in Mice

et al. 2016

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“…We report the first series of 11 cases of vasculitis restricted to the lower limbs and compare their characteristics and outcome with the few cases reported previously. [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] …”

mentioning

confidence: 99%

Vasculitis restricted to the lower limbs: a clinical and histopathological study

Khellaf

Hamidou

Pagnoux

et al. 2006

Annals of the Rheumatic Diseases

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“…As in our cases, extremities, especially lower limb, can be sole or first affected body parts. In the literature, similar cases have been described under different names such as calf muscle vasculitis, limb restricted vasculitis and muscular PAN [5][6][7]. The most striking feature of muscular PAN is a noticeable paucity of systemic organ manifestations except for cutaneous or testicular involvement [5].…”

Section: Discussionmentioning

confidence: 90%

Four Cases of Polyarteritis Nodosa Presenting Initially as Pain and Pitting Edema in Both Lower Extremities

Lee

Lim

et al. 2017

J Rheum Dis

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Polyarteritis nodosa (PAN) has a broad spectrum of clinical presentation, since it affects small and medium-sized muscular arteries with microaneurysm formation, aneurysmal rupture with hemorrhage, thrombosis, and, consequently, organ ischemia or infarction. Although skeletal muscle involvement is well documented in patients with PAN, it can mimic more common diseases, and cause confusion and delays in diagnosis. PAN muscular involvement may have limited or early systemic forms with a benign course and excellent clinical response to corticosteroid therapy. Herein, we describe the clinical course and outcome of four unusual cases of PAN manifested by acute onset of pain and pitting edema in both lower extremities; in addition, we reviewed the relevant literature. (J Rheum Dis 2017;24:48-54)

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Muscular polyarteritis nodosa as a cause of fever of undetermined origin: a case report and review of the literature

Cited by 39 publications

References 12 publications

Trypanosoma cruzi Causes Paralyzing Systemic Necrotizing Vasculitis Driven by Pathogen-Specific Type I Immunity in Mice

Trypanosoma cruzi Causes Paralyzing Systemic Necrotizing Vasculitis Driven by Pathogen-Specific Type I Immunity in Mice

Vasculitis restricted to the lower limbs: a clinical and histopathological study

Four Cases of Polyarteritis Nodosa Presenting Initially as Pain and Pitting Edema in Both Lower Extremities

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