Polyarteritis nodosa overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Haritha Machavarapu, M.B.B.S.
Overview
Polyarteritis nodosa is a systemic necrotizing vasculitis that typically affects the medium (and occasionally, small-sized) muscular arteries. Chapel Hill International Consensus Conference (CHCC) has differentiated PAN from microscopic polyangiitis which primarily affects small vessels [1].
Diagnosis of PAN is very difficult probably because of lack of a serological marker for PAN, and also due to the unavailability of suitable tissue for histological confirmation of diagnosis. Polyarteritis nodosa unlike most other vasculidities is not Antineutrophil Cytoplasmic Antibodies (ANCA) positive [2].
It is very rare and often present as a systemic disease affecting multiple organs but strikingly, polyarteritis nodosa (PAN) does not affect the lungs. The organs commonly affected by polyarteritis nodosa (PAN) include the kidneys, skin, joints, muscles, nerves, and gastrointestinal tract. PAN often has a multisystem presentation at diagnosis with the exception of variants like single-organ disease and cutaneous-only PAN. PAN has been associated with hepatitis B virus infection.
Polyarteritis nodosa is a vasculitis of medium-sized arteries, which become swollen and damaged from attack by rogue immune cells.
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
Causes
Differentiating Polyarteritis Nodosa from other Diseases
Epidemiology and Demographics
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.