Polyarteritis nodosa: Difference between revisions

Latest revision as of 17:35, 2 August 2023

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, 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 polyarteritis nodosa. (A) The patient presented with livedo reticularis on the lower legs. (B) Vasculitis involves the muscular artery of the deep dermis.

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.

Authors:

@@ Line 1: / Line 1: @@
 ==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, [[thromboembolism|thrombosis, emboli]], organ ischemia, and infarction
-* Etiology: Idiopathic, HBV, HCV, hairy cell leukemia
+*Etiology: Idiopathic, [[HBV]], [[hepatitis C|HCV]], hairy cell [[leukemia]]
+{{Primary Vasculitis DDX}}
 ==Clinical Features==
-* Cutaneous lesion + adult onset HTN
+[[File:PMC3505767 ad-24-383-g005.png|thumb|Cutaneous polyarteritis nodosa. (A) The patient presented with livedo reticularis on the lower legs. (B) Vasculitis involves the muscular artery of the deep dermis.]]
-* Men > women (2:1)
+*Cutaneous [[rash|lesion]] + adult onset [[hypertension]]
-* Peak age 40-60s
+*Men > women (2:1)
-* Systemic: Fatigue, weight loss, weakness, fever, arthralgia
+*Peak age 40-60s
-* Cutaneous lesions (1/3 patients)
+*Systemic: Fatigue, weight loss, weakness, [[fever]], [[arthralgia]]
-** Tender erythematous nodules
+*Cutaneous lesions (1/3 patients)
-** Palpable pupura (fingers, ankles, malleoli, pretibial)
+**Tender erythematous nodules
-** Digital cyanosis
+**Palpable [[purpura]] (fingers, ankles, malleoli, pretibial)
-** Splinter hemorrhages
+**Digital cyanosis
-** Livedo reticularis
+**Splinter hemorrhages
-* Renovascular arteritis → HTN
+**Livedo reticularis
-* Peripheral neuropathies (mononeuritis multiplex, polyneuropathy)
+*Renovascular arteritis → [[hypertension]]
-* Mesenteric vasculitis (abdominal angina, ischemia, infarction, perforation)
+*Peripheral neuropathies (mononeuritis multiplex, polyneuropathy)
-* Myocardial ischemia and heart failure
+*Mesenteric vasculitis (abdominal angina, [[mesenteric ischemia|ischemia]], infarction, perforation)
-* Myalgia (elevated CK)
+*[[Myocardial ischemia]] and [[heart failure]]
+*[[Myalgia]] (elevated CK)
 ==Differential Diagnosis==
-* Embolism, thrombosis, atherosclerosis
+*Embolism, thrombosis, atherosclerosis
-* HIV, Hepatitis, IE, mycotic aneurysm
+*[[HIV]], [[Hepatitis]], IE, [[mycotic aneurysm]]
-* Fibromuscular dysplagia
+*Fibromuscular dysplasia
-* Microscopic polyangiitis, Wegener's, Chug-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
-==Classification ==
-* American College of Rheumatology 10 criteria (at least 3, has 82% sensitivity and 87% specificity)
+==Evaluation==
-** Unexplained weight loss greater than 4kg
+===Workup===
-** Livedo reticularis
+*Definitive: Tissue biopsy
-** Testicular pain or tenderness
+*Labs:
-** Myalgia (excluding shoulder and hip girdle), weakness of muscles, tenderness of leg muscles, or polyneuropathy
+**Cr, CK, [[LFTs]] (elevated)
-** Mononeuropathy or polyneuropathy
+**CBC ([[Leukocytosis]], normochromic [[anemia]], [[thrombocytosis]])
-** New-onset diastolic blood pressure > 90mmHg
+**[[viral hepatitis|Hepatitis serology]]
-** Elevated serum BUN (>40mg/dL or 14.3mmol/L) or Cr (>1.5mg/dL or 132 mmol/L)
+**[[Urinalysis]] ([[proteinuria]])
-** Evidence of HBV infection (serology)
+**ESR/CRP
-** Characteristic arteriographic abnormalities not resulting fro noninflammatory disease processes
+*Imaging
-** Biopsy of small- or medium-sized artery containing polymorphonuclear cells
+**Angiography preferred: aneurysm or stenosis of medium-sized vessels
-==Workup==
+**Arteriograms
-* Definitive: Tissue biopsy
+**CT/MRI
-* Labs:
+*Consider: [[CXR]], blood cultures, autoimmune serologic testing (ANCA, ANA, RF) to rule out other diseases
-** Cr, CK, LFT (elevated)
-** CBC (Leukocytosis, normochromic anemia, thrombocytosis)
+===Diagnosis===
-** Hepatitis serology
+====American College of Rheumatology Criteria====
-** UA (proteinuria)
+''Need at least 3/10 (82% sensitivity and 87% specificity)''
-** ESR/CRP
+*Unexplained weight loss greater than 4kg
-* Imaging
+*Livedo reticularis
-** Angiography preferred: aneurysm or stenosis of medium-sized vessels
+*[[Testicular Pain]] or tenderness
-** Arteriograms
+*Myalgia (excluding shoulder and hip girdle), weakness of muscles, tenderness of leg muscles, or polyneuropathy
-** CT/MRI
+*Mononeuropathy or polyneuropathy
-*To consider: CXR, blood cx, autoimmune serologic testing (ANCA, ANA, RF) to r/o other diseases
+*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
+*Rheumatology consult
-* May warrant surgical intervention if abdominal involvement
+*May warrant surgical intervention if abdominal involvement
-* Corticosteroid:
+*[[Corticosteroids]]:
-** 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 hypertension
-==Sources==
+==References==
 <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. 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.
+[[Category:Rheumatology]]
+[[Category:Vascular]]