Polyarteritis nodosa: Difference between revisions
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==Background== | ==Background== | ||
*Necrotizing [[vasculitis]] of small- and medium-sized blood vessels | *Necrotizing [[vasculitis]] of small- and medium-sized blood vessels<ref>Hernández-Rodríguez J, et al. Diagnosis and classification of polyarteritis nodosa. J Autoimmun. 2014 Feb-Mar;48-49:84-9. PMID 24485157</ref> | ||
*Skin, musculoskeletal, CNS, and GI tract (spares lung) | *Skin, musculoskeletal, CNS, and GI tract (spares lung) | ||
*Predilection to arterial bifurcations and branch sites | *Predilection to arterial bifurcations and branch sites<ref>Wolff L, et al. Polyarteritis Nodosa: Old Disease, New Etiologies. Int J Mol Sci. 2023 Nov 23;24(23). PMID 38068989</ref> | ||
*Microaneurysm, [[thromboembolism|thrombosis, emboli]], organ ischemia, and infarction | *Microaneurysm, [[thromboembolism|thrombosis, emboli]], organ ischemia, and infarction | ||
*Etiology: Idiopathic, [[HBV]], [[hepatitis C|HCV]], hairy cell [[leukemia]] | *Etiology: Idiopathic, [[HBV]], [[hepatitis C|HCV]], hairy cell [[leukemia]] | ||
{{Primary Vasculitis DDX}} | |||
==Clinical Features== | ==Clinical Features== | ||
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==Differential Diagnosis== | ==Differential Diagnosis== | ||
*Embolism, thrombosis, atherosclerosis | *Embolism, thrombosis, atherosclerosis | ||
*[[HIV]], [[Hepatitis]], IE, mycotic aneurysm | *[[HIV]], [[Hepatitis]], IE, [[mycotic aneurysm]] | ||
*Fibromuscular dysplasia | *Fibromuscular dysplasia | ||
*Microscopic polyangiitis, [[Granulomatosis with polyangiitis]] (Wegener's), [[Eosinophilic granulomatosis with polyangiitis]] (Churg-Strauss), IgA vasculitis, drug-induced vasculitis, [[connective tissue disease]], [[SLE]], cryoglobulinemic vasculitis | *Microscopic polyangiitis, [[Granulomatosis with polyangiitis]] (Wegener's), [[Eosinophilic granulomatosis with polyangiitis]] (Churg-Strauss), IgA vasculitis, drug-induced vasculitis, [[connective tissue disease]], [[SLE]], cryoglobulinemic vasculitis | ||
Latest revision as of 10:55, 22 March 2026
Background
- Necrotizing vasculitis of small- and medium-sized blood vessels[1]
- Skin, musculoskeletal, CNS, and GI tract (spares lung)
- Predilection to arterial bifurcations and branch sites[2]
- Microaneurysm, thrombosis, emboli, organ ischemia, and infarction
- Etiology: Idiopathic, HBV, HCV, hairy cell leukemia
Vasculitis Syndrome Types
- Large vessel
- Takayasu arteritis
- Giant cell arteritis (temporal arteritis)
- Medium-vessel
- Kawasaki disease
- Polyarteritis nodosa
- Thromboangiitis obliterans (Buerger's disease)
- Primary angiitis of the central nervous system
- Small-vessel
- Henoch-Schönlein purpura
- ANCA-associated vasculitides
- Granulomatosis with polyangiitis (Wegner's)
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
- Microscopic polyangiitis
- Cutaneous leukocytoclastic angiitis (“hypersensitivity vasculitis”)
- Essential cryoglobulinemia, cryoglobulinemic vasculitis due to hepatitis C
- Behçet's disease]
- Secondary vasculitides and other/miscellaneous
- Drug-induced vasculitis
- Serum sickness
- Vasculitis associated with other rheumatic diseases (e.g. SLE)
Clinical Features
- Cutaneous lesion + adult onset hypertension
- Men > women (2:1)
- Peak age 40-60s
- Systemic: Fatigue, weight loss, weakness, fever, arthralgia
- Cutaneous lesions (1/3 patients)
- Tender erythematous nodules
- Palpable purpura (fingers, ankles, malleoli, pretibial)
- Digital cyanosis
- Splinter hemorrhages
- Livedo reticularis
- Renovascular arteritis → hypertension
- Peripheral neuropathies (mononeuritis multiplex, polyneuropathy)
- Mesenteric vasculitis (abdominal angina, ischemia, infarction, perforation)
- Myocardial ischemia and heart failure
- Myalgia (elevated CK)
Differential Diagnosis
- Embolism, thrombosis, atherosclerosis
- HIV, Hepatitis, IE, mycotic aneurysm
- Fibromuscular dysplasia
- Microscopic polyangiitis, Granulomatosis with polyangiitis (Wegener's), Eosinophilic granulomatosis with polyangiitis (Churg-Strauss), IgA vasculitis, drug-induced vasculitis, connective tissue disease, SLE, cryoglobulinemic vasculitis
Evaluation
Workup
- Definitive: Tissue biopsy
- Labs:
- Cr, CK, LFTs (elevated)
- CBC (Leukocytosis, normochromic anemia, thrombocytosis)
- Hepatitis serology
- Urinalysis (proteinuria)
- ESR/CRP
- Imaging
- Angiography preferred: aneurysm or stenosis of medium-sized vessels
- Arteriograms
- CT/MRI
- Consider: CXR, blood cultures, autoimmune serologic testing (ANCA, ANA, RF) to rule out other diseases
Diagnosis
American College of Rheumatology Criteria
Need at least 3/10 (82% sensitivity and 87% specificity)
- Unexplained weight loss greater than 4kg
- Livedo reticularis
- Testicular Pain or tenderness
- Myalgia (excluding shoulder and hip girdle), weakness of muscles, tenderness of leg muscles, or polyneuropathy
- Mononeuropathy or polyneuropathy
- New-onset diastolic blood pressure > 90mmHg
- Elevated serum BUN (>40mg/dL or 14.3mmol/L) or creatinine (>1.5mg/dL or 132 mmol/L)
- Evidence of HBV infection (serology)
- Characteristic arteriographic abnormalities not resulting fro noninflammatory disease processes
- Biopsy of small- or medium-sized artery containing polymorphonuclear cells
Management
- Rheumatology consult
- May warrant surgical intervention if abdominal involvement
- Corticosteroids:
- Prednisone 1mg/kg
- Methylprednisolone (7-15mg/kg, max 1000mg IV) for severe, organ threatening
- Immunosuppressive agent for moderate to severe
- Cyclophosphamide (600mg/m2) q2weeks x 3 doses
- ACEI or ARB for hypertension
References
- Reference: Rosen's Emergency Medicine 8th edition. 2013. Chapter: Erythematosus and the Vasculitides. p1539-1540.
- Merkel PA, et al. Clinical manifestations and diagnosis of polyarteritis nodosa in adults. In: Post T, ed. UpToDate. Waltham, Mass.: UpToDate; 2014. www.uptodate.com. Accessed December 22, 2014.
- Merkel PA, et al. Treatment and prognosis of polyarteritis nodosa. In: Post T, ed. UpToDate. Waltham, Mass.: UpToDate; 2014. www.uptodate.com. Accessed December 22, 2014.
- Jacobs-Kosmin, D. (2014, Dec 12). Polyarteritis Nodosa. eMedicine. Retrieved 12/22/2014 from http://emedicine.medscape.com/article/330717-overview.