Diagnosis
Polyarteritis nodosa
A systemic vasculitis characterized by necrotizing inflammatory lesions that affect medium-sized arteries resulting in microaneurysm formation, aneurysmal rupture with hemorrhage, thrombosis, and organ ischemia or infarction.
Also known as: Kussmaul disease, Kussmaul-Maier disease, Panarteritis nodosa, Periarteritis nodosa
Etiology
Cause [ouz]
Primary PAN is idiopathic
Secondary PAN is strongly linked with:
Virus: Hepatitis B virus, hepatitis C virus, parvovirus B-19
Bacteria: Klebsiella, Toxoplasma, Pseudomonas, trichinosis, and Yersinia species
Autoimmun: Sjogren syndrome and rheumatoid arthritis
Malignancy: Hairy cell leukemia
Pathophysiology [ouz]
Arise secondary to immune complexes
Inflammatory reaction with vascular lesions in medium-sized muscular arteries occur mainly at bifurcations
Inflammation may start in the vessel intima and progress to include the entire arterial wall, destroying the internal and external elastic lamina, resulting in fibrinoid necrosis
This result in Impairment in endothelial function
Aneurysms develop in the weakened vessel --> risk for rupture, hemorrhage, thrombi, obstruction
Subsequent tissue ischemia or infarction
Clinical manifestations:
Kidney: Aneurysm, rupture and ischemia
Skin lesions: erythema nodosum, palpable purpura, livedo reticularis and ulceration
Neurologic
Cardiac: Heart failure, myocardial infarction
Gastrointestinal: Intestinal angina, nausea, vomiting, melena, or diarrhea
Muscular
Systemic signs: Fatigue, weight loss, fever, arthralgia
Complications [ouz]
Cutaneous ulcerations
Extremity gangrene
Organ infarction
Aneurysm rupture in the kidneys, liver, heart, pancreas, and axillary artery
Stroke
Encephalopathy
Myelopathy
Heart failure
Myocardial infarction
Pericarditis
Renal failure
Gastrointestinal bleeding
Bowel infarction
Peripheral neuropathy
Epidemiology
Incidence per 100.000 [efb][fw1][auq][dh8][ofa][amm][ccz][eio][usg]
Symptoms & findings
Symptoms
Anorexia, Areflexia, Arrhythmia, Arthralgia, Ataxia, Blindness, Blurred vision, Depression, Fatigue, Fever, Headache, Hematochezia, Hypertension, Hypoesthesia, Hyporeflexia, Malaise, Muscle atrophy, Muscle weakness, Myalgia, Nausea, Paralysis, Paresis, Paresthesia, Psychosis, Raynaud phenomenon, Seizure, Tachycardia, Visual disturbances, Vomiting, Weight loss
Clinical findings
Cholecystitis, Elevated Creatinine, Friction rub, Hepatitis, Pneumoperitoneum
Anamneses
None listed.
Localized findings
Approach
Blood test: ANCA, ESR and CRP [fw1]
Serelogy: hepatitis B, hepatitis C, and HIV
Angiography (aneurysms or occlusions of the visceral arteries)
FDG-PET/CT
Biopsy
Treatment
Corticosteroids [u5j][ouz]
1g IV daily repeated over 3 days, prior to oral prednisone (1 mg/kg/d)
Steroid refractory: Corticosteroids + Cyclophosphamide
Hepatitis B–relatedN: Corticosteroids + antiviral agents + plasmapheresis
Steroid-refractory and recurrent: biologic agents (infliximab, etanercept, tocilizumab, tofacitinib, rituximab)
Severe PAN: Plasma exchange
PAN due to deficiency of ADA2 (DADA2): TNF-alpha inhibitors
Hypertension --> ACE-inhibitors
Surgery:
Bowel perforation
Cholecystitis
Aneurysms of blood vessels
Differential diagnoses
Acute extremity ischemia, Acute mesenteric ischemia, Antiphospholipid syndrome, Chronic limb ischemia, Chronic mesenteric ischemia, Churg-Strauss syndrome, Crohn disease, Cryoglobulinemia, Ehlers-Danlos syndrome, Endocarditis, Goodpasture syndrome, Henoch-Schönlein purpura, HIV, Kawasaki disease, Leukocytoclastic small vessel vasculitis, Microscopic polyangiitis, Myxoma, Necrotizing vasculitis, Polyneuropathy, Pulmonary embolism, Pyoderma gangrenosum, Rheumatoid arthritis, Segmental arterial mediolysis, Stroke, Systemic lupus erythematosus, Wegener's granulomatosis
References
[1] Stanton M, Tiwari V. Polyarteritis Nodosa. [Updated 2023 Feb 22]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK482157/
[2] Watts RA, Hatemi G, Burns JC, Mohammad AJ. Global epidemiology of vasculitis. Nat Rev Rheumatol. 2022 Jan;18(1):22-34.
[3] Ecclestone T, Watts RA. Classification and epidemiology of vasculitis: Emerging concepts. Best Pract Res Clin Rheumatol. 2023 Mar;37(1):101845.
[4] Watts RA, Lane SE, Bentham G, Scott DG. Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom. Arthritis Rheum. 2000 Feb;43(2):414-9.
[5] Mohammad AJ, Jacobsson LT, Westman KW, Sturfelt G, Segelmark M. Incidence and survival rates in Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and polyarteritis nodosa. Rheumatology (Oxford). 2009 Dec;48(12):1560-5.
[6] Ormerod AS, Cook MC. Epidemiology of primary systemic vasculitis in the Australian Capital Territory and south-eastern New South Wales. Intern Med J. 2008 Nov;38(11):816-23.
[7] Watts RA, Scott DGI, Ball GV, Bridges SL. (2008). Vasculitis, 2nd. 7-21. Oxford University Press, Oxford.
[8] Kanecki K, Nitsch-Osuch A, Gorynski P, Wierzba W, Tarka P, Tyszko P. Polyarteritis nodosa: decreasing incidence in Poland. Arch Med Sci. 2019 Sep;15(5):1308-1312.
[9] Mohammad AJ, Jacobsson LT, Mahr AD, Sturfelt G, Segelmark M. Prevalence of Wegener's granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome within a defined population in southern Sweden. Rheumatology (Oxford). 2007 Aug;46(8):1329-37.
[10] Mahr A, Guillevin L, Poissonnet M, Aymé S. 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. 2004 Feb 15;51(1):92-9.
[11] http://emedicine.medscape.com/article/330717; [Medscape]
[12] [Clinical Dermatology 4.th Edition by Weller, Hunter, Savin and Dahl]