Polyarteritis nodosa: Difference between revisions
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* Microaneurysm, thrombosis, emboli, organic ischemia, and infarction | * Microaneurysm, thrombosis, emboli, organic ischemia, and infarction | ||
* Etiology: Idiopathic, HBV, HCV, hairy cell leukemia | * Etiology: Idiopathic, HBV, HCV, hairy cell leukemia | ||
==Clinical Features== | |||
==Diagnosis== | |||
===Clinical Features=== | |||
* Cutaneous lesion + adult onset HTN | * Cutaneous lesion + adult onset HTN | ||
* Men > women (2:1) | * Men > women (2:1) | ||
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* Myocardial ischemia and heart failure | * Myocardial ischemia and heart failure | ||
* Myalgia (elevated CK) | * Myalgia (elevated CK) | ||
== | |||
===Classification === | |||
* American College of Rheumatology 10 criteria (at least 3, has 82% sensitivity and 87% specificity) | * American College of Rheumatology 10 criteria (at least 3, has 82% sensitivity and 87% specificity) | ||
** Unexplained weight loss greater than 4kg | ** Unexplained weight loss greater than 4kg | ||
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** Characteristic arteriographic abnormalities not resulting fro noninflammatory disease processes | ** Characteristic arteriographic abnormalities not resulting fro noninflammatory disease processes | ||
** Biopsy of small- or medium-sized artery containing polymorphonuclear cells | ** Biopsy of small- or medium-sized artery containing polymorphonuclear cells | ||
==Workup== | ==Workup== | ||
* Definitive: Tissue biopsy | * Definitive: Tissue biopsy | ||
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** CT/MRI | ** CT/MRI | ||
*To consider: CXR, blood cx, autoimmune serologic testing (ANCA, ANA, RF) to r/o other diseases | *To consider: CXR, blood cx, autoimmune serologic testing (ANCA, ANA, RF) to r/o other diseases | ||
==Differential Diagnosis== | |||
* Embolism, thrombosis, atherosclerosis | |||
* HIV, Hepatitis, IE, mycotic aneurysm | |||
* Fibromuscular dysplagia | |||
* Microscopic polyangiitis, Wegener's, Chug-Strauss, IgA vasculitis, drug-induced vasculitis, connective tissue disease, SLE, cryoglobulinemic vasculitis | |||
==Management== | ==Management== | ||
Revision as of 07:43, 30 December 2014
Background
- Necrotizing vasculitis of small- and medium-sized blood vessels
- Skin, musculoskeletal, CNS, and GI tract (spares lung)
- Predilection to arterial bifurcations and branch sites
- Microaneurysm, thrombosis, emboli, organic ischemia, and infarction
- Etiology: Idiopathic, HBV, HCV, hairy cell leukemia
Diagnosis
Clinical Features
- Cutaneous lesion + adult onset HTN
- Men > women (2:1)
- Peak age 40-60s
- Systemic: Fatigue, weight loss, weakness, fever, arthralgia
- Cutaneous lesions (1/3 patients)
- Tender erythematous nodules
- Palpable pupura (fingers, ankles, malleoli, pretibial)
- Digital cyanosis
- Splinter hemorrhages
- Livedo reticularis
- Renovascular arteritis → HTN
- Peripheral neuropathies (mononeuritis multiplex, polyneuropathy)
- Mesenteric vasculitis (abdominal angina, ischemia, infarction, perforation)
- Myocardial ischemia and heart failure
- Myalgia (elevated CK)
Classification
- American College of Rheumatology 10 criteria (at least 3, has 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 Cr (>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
Workup
- Definitive: Tissue biopsy
- Labs:
- Cr, CK, LFT (elevated)
- CBC (Leukocytosis, normochromic anemia, thrombocytosis)
- Hepatitis serology
- UA (proteinuria)
- ESR/CRP
- Imaging
- Angiography preferred: aneurysm or stenosis of medium-sized vessels
- Arteriograms
- CT/MRI
- To consider: CXR, blood cx, autoimmune serologic testing (ANCA, ANA, RF) to r/o other diseases
Differential Diagnosis
- Embolism, thrombosis, atherosclerosis
- HIV, Hepatitis, IE, mycotic aneurysm
- Fibromuscular dysplagia
- Microscopic polyangiitis, Wegener's, Chug-Strauss, IgA vasculitis, drug-induced vasculitis, connective tissue disease, SLE, cryoglobulinemic vasculitis
Management
- Rheumatology consult
- May warrant surgical intervention if abdominal involvement
- Corticosteroid:
- 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 HTN
Sources
- 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.