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)
 *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]]
-==Diagnosis==
+{{Primary Vasculitis DDX}}
-===Clinical Features===
-*Cutaneous lesion + adult onset HTN
+==Clinical Features==
+[[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.]]
+*Cutaneous [[rash|lesion]] + adult onset [[hypertension]]
 *Men > women (2:1)
 *Peak age 40-60s
-*Systemic: Fatigue, weight loss, weakness, fever, arthralgia
+*Systemic: Fatigue, weight loss, weakness, [[fever]], [[arthralgia]]
 *Cutaneous lesions (1/3 patients)
 **Tender erythematous nodules
-**Palpable pupura (fingers, ankles, malleoli, pretibial)
+**Palpable [[purpura]] (fingers, ankles, malleoli, pretibial)
 **Digital cyanosis
 **Splinter hemorrhages
 **Livedo reticularis
-*Renovascular arteritis → HTN
+*Renovascular arteritis → [[hypertension]]
 *Peripheral neuropathies (mononeuritis multiplex, polyneuropathy)
-*Mesenteric vasculitis (abdominal angina, ischemia, infarction, perforation)
+*Mesenteric vasculitis (abdominal angina, [[mesenteric ischemia|ischemia]], infarction, perforation)
-*Myocardial ischemia and heart failure
+*[[Myocardial ischemia]] and [[heart failure]]
-*Myalgia (elevated CK)
+*[[Myalgia]] (elevated CK)
-===Classification===
+==Differential Diagnosis==
-*American College of Rheumatology 10 criteria (at least 3, has 82% sensitivity and 87% specificity)
+*Embolism, thrombosis, atherosclerosis
-**Unexplained weight loss greater than 4kg
+*[[HIV]], [[Hepatitis]], IE, [[mycotic aneurysm]]
-**Livedo reticularis
+*Fibromuscular dysplasia
-**Testicular pain or tenderness
+*Microscopic polyangiitis, [[Granulomatosis with polyangiitis]] (Wegener's), [[Eosinophilic granulomatosis with polyangiitis]] (Churg-Strauss), IgA vasculitis, drug-induced vasculitis, [[connective tissue disease]], [[SLE]], cryoglobulinemic vasculitis
-**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==
+==Evaluation==
+===Workup===
 *Definitive: Tissue biopsy
 *Labs:
-**Cr, CK, LFT (elevated)
+**Cr, CK, [[LFTs]] (elevated)
-**CBC (Leukocytosis, normochromic anemia, thrombocytosis)
+**CBC ([[Leukocytosis]], normochromic [[anemia]], [[thrombocytosis]])
-**Hepatitis serology
+**[[viral hepatitis|Hepatitis serology]]
-**UA (proteinuria)
+**[[Urinalysis]] ([[proteinuria]])
 **ESR/CRP
 *Imaging
@@ Line 49: / Line 45: @@
 **Arteriograms
 **CT/MRI
-*To consider: CXR, blood cx, autoimmune serologic testing (ANCA, ANA, RF) to r/o other diseases
+*Consider: [[CXR]], blood cultures, autoimmune serologic testing (ANCA, ANA, RF) to rule out other diseases
-==Differential Diagnosis==
+===Diagnosis===
-*Embolism, thrombosis, atherosclerosis
+====American College of Rheumatology Criteria====
-*[[HIV]], [[Hepatitis]], IE, mycotic aneurysm
+''Need at least 3/10 (82% sensitivity and 87% specificity)''
-*Fibromuscular dysplagia
+*Unexplained weight loss greater than 4kg
-*Microscopic polyangiitis, Wegener's, Chug-Strauss, IgA vasculitis, drug-induced vasculitis, connective tissue disease, SLE, cryoglobulinemic vasculitis
+*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
-*Corticosteroid:
+*[[Corticosteroids]]:
 **[[Prednisone]] 1mg/kg
 **[[Methylprednisolone]] (7-15mg/kg, max 1000mg IV) for severe, organ threatening
 *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
 ==References==
@@ Line 75: / Line 79: @@
 [[Category:Rheumatology]]
+[[Category:Vascular]]