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 | ||
* 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 | ||
* 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 | ||
==Diagnosis== | ==Diagnosis== | ||
===Clinical Features=== | ===Clinical Features=== | ||
* Cutaneous lesion + adult onset HTN | *Cutaneous lesion + adult onset HTN | ||
* Men > women (2:1) | *Men > women (2:1) | ||
* Peak age 40-60s | *Peak age 40-60s | ||
* Systemic: Fatigue, weight loss, weakness, fever, arthralgia | *Systemic: Fatigue, weight loss, weakness, fever, arthralgia | ||
* Cutaneous lesions (1/3 patients) | *Cutaneous lesions (1/3 patients) | ||
** Tender erythematous nodules | **Tender erythematous nodules | ||
** Palpable pupura (fingers, ankles, malleoli, pretibial) | **Palpable pupura (fingers, ankles, malleoli, pretibial) | ||
** Digital cyanosis | **Digital cyanosis | ||
** Splinter hemorrhages | **Splinter hemorrhages | ||
** Livedo reticularis | **Livedo reticularis | ||
* Renovascular arteritis → HTN | *Renovascular arteritis → HTN | ||
* Peripheral neuropathies (mononeuritis multiplex, polyneuropathy) | *Peripheral neuropathies (mononeuritis multiplex, polyneuropathy) | ||
* Mesenteric vasculitis (abdominal angina, ischemia, infarction, perforation) | *Mesenteric vasculitis (abdominal angina, ischemia, infarction, perforation) | ||
* Myocardial ischemia and heart failure | *Myocardial ischemia and heart failure | ||
* Myalgia (elevated CK) | *Myalgia (elevated CK) | ||
===Classification === | ===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 | ||
** Livedo reticularis | **Livedo reticularis | ||
** Testicular pain or tenderness | **Testicular pain or tenderness | ||
** Myalgia (excluding shoulder and hip girdle), weakness of muscles, tenderness of leg muscles, or polyneuropathy | **Myalgia (excluding shoulder and hip girdle), weakness of muscles, tenderness of leg muscles, or polyneuropathy | ||
** Mononeuropathy or polyneuropathy | **Mononeuropathy or polyneuropathy | ||
** New-onset diastolic blood pressure > 90mmHg | **New-onset diastolic blood pressure > 90mmHg | ||
** Elevated serum BUN (>40mg/dL or 14.3mmol/L) or Cr (>1.5mg/dL or 132 mmol/L) | **Elevated serum BUN (>40mg/dL or 14.3mmol/L) or Cr (>1.5mg/dL or 132 mmol/L) | ||
** Evidence of HBV infection (serology) | **Evidence of HBV infection (serology) | ||
** 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 | ||
* Labs: | *Labs: | ||
** Cr, CK, LFT (elevated) | **Cr, CK, LFT (elevated) | ||
** CBC (Leukocytosis, normochromic anemia, thrombocytosis) | **CBC (Leukocytosis, normochromic anemia, thrombocytosis) | ||
** Hepatitis serology | **Hepatitis serology | ||
** UA (proteinuria) | **UA (proteinuria) | ||
** ESR/CRP | **ESR/CRP | ||
* Imaging | *Imaging | ||
** Angiography preferred: aneurysm or stenosis of medium-sized vessels | **Angiography preferred: aneurysm or stenosis of medium-sized vessels | ||
** Arteriograms | **Arteriograms | ||
** 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== | ==Differential Diagnosis== | ||
* Embolism, thrombosis, atherosclerosis | *Embolism, thrombosis, atherosclerosis | ||
* [[HIV]], [[Hepatitis]], IE, mycotic aneurysm | *[[HIV]], [[Hepatitis]], IE, mycotic aneurysm | ||
* Fibromuscular dysplagia | *Fibromuscular dysplagia | ||
* Microscopic polyangiitis, Wegener's, Chug-Strauss, IgA vasculitis, drug-induced vasculitis, connective tissue disease, SLE, cryoglobulinemic vasculitis | *Microscopic polyangiitis, Wegener's, Chug-Strauss, IgA vasculitis, drug-induced vasculitis, connective tissue disease, SLE, cryoglobulinemic vasculitis | ||
==Management== | ==Management== | ||
* Rheumatology consult | *Rheumatology consult | ||
* May warrant surgical intervention if abdominal involvement | *May warrant surgical intervention if abdominal involvement | ||
* Corticosteroid: | *Corticosteroid: | ||
** [[Prednisone]] 1mg/kg | **[[Prednisone]] 1mg/kg | ||
** [[Methylprednisolone]] (7-15mg/kg, max 1000mg IV) for severe, organ threatening | **[[Methylprednisolone]] (7-15mg/kg, max 1000mg IV) for severe, organ threatening | ||
* Immunosuppressive agent for moderate to severe | *Immunosuppressive agent for moderate to severe | ||
**Cyclophosphamide (600mg/m2<sup>) q2weeks x 3 doses | **Cyclophosphamide (600mg/m2<sup>) q2weeks x 3 doses | ||
* ACEI or ARB for HTN | *ACEI or ARB for HTN | ||
==References== | ==References== | ||
<references/> | <references/> | ||
*Reference: Rosen's Emergency Medicine 8th edition. 2013. Chapter: Erythematosus and the Vasculitides. p1539-1540. | *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. 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. | *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. | *Jacobs-Kosmin, D. (2014, Dec 12). Polyarteritis Nodosa. eMedicine. Retrieved 12/22/2014 from http://emedicine.medscape.com/article/330717-overview. | ||
[[Category:Rheumatology]] | [[Category:Rheumatology]] | ||
Revision as of 12:44, 4 July 2016
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
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.