Abstract
Polyarteritis nodosa is an uncommon but potentially lethal multisystem nongranulomatous necrotizing vasculitis of small- or medium-sized arteries, principally at branching and bifurcation points. It is typically ANCA negative. It is associated with certain viral infections (hepatitis B most common), drug abuse, hyposensitization/desensitization treatment, B cell neoplasms, and acute otitis media. Ocular manifestations occur in 10–20% of patients and include necrotizing scleritis, peripheral ulcerative keratitis, uveitis, and choroidal and retinal vasculitis.
Access provided by Autonomous University of Puebla. Download chapter PDF
Similar content being viewed by others
Keywords
Overview
-
Definition
-
An uncommon but potentially lethal multisystem nongranulomatous necrotizing vasculitis of small- or medium-sized arteries, principally at branching and bifurcation points.
-
Typically ANCA negative.
-
Associated with certain viral infections (hepatitis B most common), drug abuse, hyposensitization/desensitization treatment, B cell neoplasms, and acute otitis media.
-
Ocular manifestations occur in 10–20% of patients and include necrotizing scleritis, peripheral ulcerative keratitis, uveitis, and choroidal and retinal vasculitis.
-
-
Symptoms
-
Redness
-
Exquisite pain – periorbital or with extraocular movements
-
Photophobia
-
Blurry vision
-
-
Laterality
-
Unilateral or bilateral
-
-
Course
-
Progressive and fatal without treatment due to renal and cardiac complications (5-year mortality rate is 80–95% if untreated)
-
-
Age of onset
-
40–60 years
-
-
Gender/race
-
M:F = 2:1.
-
No racial predilection per se, but polyarteritis nodosa (PAN) has the highest prevalence in Alaskan Eskimo because the population has a very high rate of hepatitis B infection
-
-
Systemic association
Multisystem involvement tends to occur early
-
Renal (75%)
-
Glomerulonephritis, hematuria, hypertension
-
Primary cause of death
-
-
Cardiac (75%)
-
Coronary thrombosis, pericarditis, pericardial hemorrhage, acute aortitis.
-
Myocardial involvement leads to dysrhythmias and infarction.
-
Second leading cause of death.
-
-
Cutaneous (20–50%)
-
Cutaneous or subcutaneous nodules along superficial arteries around the knee, anterior lower leg, and dorsum of foot
-
Nodules can rupture resulting in cutaneous hematomas or ecchymosis.
-
-
Infarction or gangrene involving the fingers or toes
-
-
Gastrointestinal
-
Abdominal pain caused by intestinal or mesenteric ischemia
-
Infarcts in the liver and spleen
-
Other findings: peritonitis, bowel gangrene and perforation, and intra-abdominal hemorrhage
-
-
Neurologic
-
Ischemia to peripheral nerves, causing mononeuritis multiplex
-
-
Genitourinary
-
Epididymitis: virtually pathognomonic in appropriate clinical context
-
-
Musculoskeletal
-
Non-deforming arthritis
-
Myalgia
-
-
Exam: Ocular
External
-
Orbital inflammation, sometimes causing exophthalmos/proptosis
Anterior Segment
-
Conjunctival hyperemia, hemorrhage, or infarction
-
Episcleritis
-
Necrotizing scleritis with peripheral ulcerative keratitis (most common type of scleritis in PAN)
-
Acute nongranulomatous anterior uveitis
Posterior Segment
-
Choroidal and retinal vasculitis (most common)
-
Exudative retinal detachment
-
Vascular occlusion
-
Hypertensive retinopathy
Neuro-Ophthalmic Findings
-
Optic nerve vasculitis causing optic disc edema
-
Vasculitis involving the central and peripheral nervous systems leading to third, fifth, sixth, or seventh cranial nerve palsies, hemianopia, nystagmus, amaurosis fugax, and/or Horner’s syndrome
Exam: Systemic
-
Cutaneous or subcutaneous nodules along superficial arteries of lower extremities
-
Peripheral nerve paresis or paresthesia
-
Abdominal tenderness
-
Testicular/epididymal tenderness
Imaging
-
FA
-
Delayed choroidal filling
-
Retinal arteritis
-
-
ICG
-
Choroidal infarction
-
Laboratory and Radiographic Testing
-
Diagnosis is made on clinical and histological grounds.
-
Biopsy of involved tissue may show hemorrhagic vasculitis and fibrinoid necrosis
-
Testicular and skin biopsy confirm diagnosis in 50–80% of patients
-
-
Hepatitis B panel: 33% have positive surface antigen (HBsAg).
-
Other labs are nonspecific and simply reflect the systemic nature of PAN.
-
Elevated ESR/CRP and neutrophil count
-
Hypocomplementemia
-
Elevated BUN and serum creatinine
-
Urinalysis: RBC, red cell casts, or protein
-
p-ANCA is NOT typically associated with PAN, but a subset of PAN called microscopic polyangiitis (involving even smaller vessels) IS associated with p-ANCA
-
-
Abdominal and renal angiography.
-
Saccular arterial aneurysms in renal, hepatic, and gastrointestinal vasculature
-
Differential Diagnosis
-
Systemic vasculitides
-
Granulomatosis with polyangiitis
-
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
-
Microscopic polyangiitis
-
-
Rheumatoid arthritis
-
Adamantiades-Behcet’s disease
-
Systemic lupus erythematosus
-
Dermatomyositis
-
Progressive systemic sclerosis
-
Idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN)
-
Syphilis
-
Mooren’s ulcer (no scleritis)
Treatment
-
Prednisone 1 mg/kg/day + cyclophosphamide 1–2 mg/kg/day.
-
Biologics in refractory cases, including adalimumab, infliximab, rituximab, tocilizumab, and tofacitinib.
-
Treatment of HBV may produce remission.
Referral/Co-management
-
Rheumatology
-
Nephrology
-
Urology
-
Cardiology
-
Neurology
-
Gastroenterology
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2021 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Ueberroth, J.A. (2021). Polyarteritis Nodosa. In: Foster, C.S., Anesi, S.D., Chang, P.Y. (eds) Uveitis. Springer, Cham. https://doi.org/10.1007/978-3-030-52974-1_8
Download citation
DOI: https://doi.org/10.1007/978-3-030-52974-1_8
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-52973-4
Online ISBN: 978-3-030-52974-1
eBook Packages: MedicineMedicine (R0)