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
Polyarteritis nodosa was first described macroscopically by the pathologist K. Rokitansky in 1842.He described the presence of aneurysms macroscopically and therefore missed the inflammatory nature of this disease.Polyarteritis nodosa was better described in 1866 by A. Kussmaul and R. Maier who provided a clinical description of a patient.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.
Classification
There is no established system for the classification of polyarteritis nodosa.
Pathophysiology
The exact pathogenesis of PAN is not fully understood.It is a disease of unknown cause that affects arteries, the blood vessels that carry oxygenated blood to organs and tissues.PAN affects the medium sized arterial vessels.PAN does not affect large arterial vessels, capillaries, small arterioles and venous system. It occurs when certain immune cells attack the affected arteries.Inflammation starts in the vessel intima and results in fibrinoid necrosis by destroying the internal and external elastic lamina.Aneurysms and thrombi may develop at the site of lesions.One hypothesis is that this condition is caused by antibodies against HBV, via atype IIII hypersensitivity reaction.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).Mutation in CECR1 can lead to vascular and inflammatory disorders which also include PAN.
Causes
The common causes of PAN are idiopathic, Hepatitis B infection, Hepatitis C infection, hairy cell leukemia and drug induced. Less common causes include varicella-zoster virus, parvovirus B-19, cytomegalovirus, human T-cell leukemia virus etc.
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 [3] and the annual incidence in three regions of Europe was estimated to be 4.4 to 9.7 per million [4].
PAN is commoner in men than women at a predominance rate of 1.5: 1 [5]. 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.
- ↑ 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.