Polyarteritis nodosa overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Ali Poyan Mehr, M.D. [2]; Associate Editor(s)-in-Chief: Olufunmilola Olubukola M.D.[3] Cafer Zorkun, M.D., Ph.D. [4]; Haritha Machavarapu, M.B.B.S.
Overview
Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that typically affects the medium (and occasionally, small-sized) muscular arteries. The Chapel Hill International Consensus Conference (CHCC) has differentiated PAN from microscopic polyangiitis which primarily affects small vessels [1]. The diagnosis of PAN is challenging due to of lack of a serological marker, and lack of specific histological characteristics. Polyarteritis nodosa unlike most other vasculidities is not Antineutrophil Cytoplasmic Antibodies (ANCA) positive [2]. PAN is a rare disease and often affects multiple organs but strikingly, PAN does not affect the lungs. The organs commonly affected by PAN include the kidneys, skin, joints, muscles, nerves, and gastrointestinal tract.
While it often has a multisystem presentation at diagnosis, variants like single-organ disease and cutaneous-only PAN do occur as well. PAN has been associated with hepatitis B and C virus infection.
Historical Perspective
PAN was first described macroscopically by the pathologist K. Rokitansky in 1842. This pathologist described the presence of aneurysms macroscopically and therefore missed the inflammatory nature of this disease [3].
PAN was better described in 1866 by A. Kussmaul and R. Maier who provided a clinical description of a patient, including a post-mortem histological examination of blood vessels, arriving at a diagnosis of vasculitis. Kussmaul and Maier introduced the term “periarteritis nodosa” to describe the nodules observed in intermediate-sized vascular arteries but this term was later changed to “Polyarteritis nodosa” when these nodules showed the involvement of all layers of the artery [4].
In 1931, Dr. Lindberg became the first person to recognize PAN limited to skin [5]. In 1970, Trepo and Thivolet reported the association of PAN with hepatitis B virus (HBV) infection (C. Trepo, J. Thivolet), later it became obvious that most PAN cases were associated with HBV.
The etiology of Polyarteritis nodosa remains unknown till date. There are no clear cut causative factors of the disease, no definitive serological diagnostic testing for it and no specified pathognomonic features for polyarteritis nodosa. Therefore, the term “Polyarteritis Nodosa” is often used for any immunologically mediated systemic vasculitis.
Classification
Pathophysiology
The pathogenesis of PAN remains unknown. However, the clinical responses to immunosuppressive therapy suggest that immunological mechanisms play an active pathogenic role. Due to inflammation of the medium to small sized vessels in PAN and the presence of impaired endothelial function, there could be direct endothelial cell activation and damage resulting from proinflammatory cytokines or antibodies (anti-endothelial cells antibodies). These anti-endothelial cells antibodies in turn stimulate greater production of cytokines and adhesion molecules potentiating the inflammation causing more damage to the vessels [6].
Causes
Differentiating Polyarteritis Nodosa from other Diseases
Epidemiology and Demographics
PAN is a very uncommon disease and a rare form of vasculitis. Population prevalence estimates for polyarteritis nodosa (PAN) range from 2 to 33 per million across the European Countries [7] and the annual incidence in three regions of Europe was estimated to be 4.4 to 9.7 per million [8].
PAN is commoner in men than women at a predominance rate of 1.5: 1 [9]. It occurs in all ethnic groups. Most cases of PAN occur in the 5th to 6th decade of life. It can also occur in children although this form of presentation is very rare. PAN has been associated with hepatitis B virus infection.
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
Chest X Ray
CT
MRI
Echocardiography or Ultrasound
Other Imaging Findings
Other Diagnostic Studies
Treatment
Surgery
Medical Therapy
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
References
- ↑ Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL; et al. (1994). "Nomenclature of systemic vasculitides. Proposal of an international consensus conference". Arthritis Rheum. 37 (2): 187–92. PMID 8129773.
- ↑ Kallenberg CG, Brouwer E, Weening JJ, Tervaert JW (1994). "Anti-neutrophil cytoplasmic antibodies: current diagnostic and pathophysiological potential". Kidney Int. 46 (1): 1–15. PMID 7933826.
- ↑ Tesar V, Kazderová M, Hlavácková L (2004). "Rokitansky and his first description of polyarteritis nodosa". J Nephrol. 17 (1): 172–4. PMID 15151275.
- ↑ Kluge FJ, Matteson EL (2003). "[Think clearly, be sincere, act calmly: Adolf Kussmaul (1822-1902) un his significance for medicine in the 21st century]". Z Rheumatol. 62 (5): 484–90. doi:10.1007/s00393-003-0536-5. PMID 14579038.
- ↑ BOSS J (1945). "[Not Available]". Schweiz Z Tuberk. 2 (2): 89–108. PMID 21008159.
- ↑ Filer AD, Gardner-Medwin JM, Thambyrajah J, Raza K, Carruthers DM, Stevens RJ; et al. (2003). "Diffuse endothelial dysfunction is common to ANCA associated systemic vasculitis and polyarteritis nodosa". Ann Rheum Dis. 62 (2): 162–7. PMC 1754444. PMID 12525387.
- ↑ Mohammad AJ, Jacobsson LT, Mahr AD, Sturfelt G, Segelmark M (2007). "Prevalence of Wegener's granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome within a defined population in southern Sweden". Rheumatology (Oxford). 46 (8): 1329–37. doi:10.1093/rheumatology/kem107. PMID 17553910.
- ↑ Watts RA, Lane SE, Bentham G, Scott DG (2000). "Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom". Arthritis Rheum. 43 (2): 414–9. doi:10.1002/1529-0131(200002)43:2<414::AID-ANR23>3.0.CO;2-0. PMID 10693883.
- ↑ Mahr A, Guillevin L, Poissonnet M, Aymé S (2004). "Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate". Arthritis Rheum. 51 (1): 92–9. doi:10.1002/art.20077. PMID 14872461.