II. Epidemiology

  1. Prevalence: May approach 33 Million cases worldwide
  2. Gender: Slight male predominance
  3. Age: 30-40 years old

III. Classification

  1. ANCA negative Vasculitis
  2. Now differentiated from microscopic polyangitis

IV. Pathophysiology

  1. Medium vessel Vasculitis involving muscular arteries
  2. Can affect any organ (esp. Kidney, skin, joint, Muscle, nerve, Intestine), but typically does not involve the lung
  3. May be limited to the skin (Cutaneous Polyarteritis Nodosa) with or without mild systemic symptoms
    1. More than half of cutaneous cases progress to extracutaneous involvement
  4. Effects are related to vessel wall thickening, vessel narrowing and ultimately arterial Occlusion with secondary tissue infarction
    1. Medium sized arteries with immune complex deposition
    2. Immune deposits contain C3 and IgM

V. Associated Conditions

VI. Symptoms

  1. Fever
  2. Weight loss
  3. Hypertension
  4. Abdominal Pain
  5. Melena
  6. Peripheral neuritis

VII. Signs: Skin

  1. Tender erythematous Nodules similar to Erythema Nodosum
  2. Palpable Purpura
  3. Livedo Reticularis
  4. Skin Ulceration
  5. Vesicobullous lesions

VIII. Signs: Systemic

IX. Labs

  1. Vasculitis labs (evaluate differential diagnosis)
  2. ANCA negative
  3. Serum Creatinine
  4. Creatinine Phosphokinase (CPK)
  5. Liver Function Tests
  6. Hepatitis B Surface Antigen
  7. Anti-Hepatitis C Antibody
  8. Urinalysis
  9. C-Reactive Protein (negative result would suggest alternative diagnosis)

X. Imaging

  1. Chest XRay (evaluate differential diagnosis)

XI. Diagnosis

  1. Tissue Biopsy (Punch Biopsy, Incisional Biopsy)
    1. Perivascular inflammation with White Blood Cell infiltrate
  2. Imaging (CT Angiogram, MR Angiogram, Echocardiogram)

XII. Differential Diagnosis

  1. See Vasculitis
  2. Infection
    1. Endocarditis
    2. Mycotic aneurysm
    3. Hepatitis B Infection
    4. Hepatitis C Infection
    5. HIV Infection
  3. Non-infectious causes
    1. Atypical thromboembolic conditions (e.g. atrial myoma, antiphospholipid Antibody syndrome)
    2. Fibromuscular dysplasia

XIII. Management

  1. Antiinflammatory Agents
    1. Colchicine
    2. Dapsone
    3. Cyclophosphamide
  2. Biologic agents
    1. Tumor Necrosis Factor Inhibitors
    2. Interleukin 6 inhibitors
  3. Corticosteroids
    1. Indicated in acute flares on slow taper (e.g. 60 mg to 5-6 mg)
    2. May need to be continued longterm at low dose
  4. Hepatitis B Related Polyarteritis Nodosa
    1. Chronic Hepatitis B Antiviral Therapy
    2. Plasma exchange

XIV. References

  1. Merkel (2011) in UpToDate, Clinical features and diagnosis of Takayasu Arteritis, Accessed 11/30/11
  2. Hocevar (2021) Curr Rheumatol Rep 23(3): 14 +PMID:33569653 [PubMed]

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Ontology: Polyarteritis Nodosa (C0031036)

Definition (NCI) An autoimmune necrotizing inflammatory vasculitis that affects small and medium size arteries. It manifests with fever, fatigue, loss of appetite, weakness, skin rash, joint pain, numbness, burning sensation and renal failure.
Definition (CSP) form of necrotizing vasculitis involving small- and medium-sized arteries; signs and symptoms result from infarction and scarring of the affected organ system.
Definition (MSH) A form of necrotizing non-granulomatous inflammation occurring primarily in medium-sized ARTERIES, often with microaneurysms. It is characterized by muscle, joint, and abdominal pain resulting from arterial infarction and scarring in affected organs. Polyarteritis nodosa with lung involvement is called CHURG-STRAUSS SYNDROME.
Concepts Disease or Syndrome (T047)
MSH D010488
ICD9 446.0
ICD10 M30.0
SnomedCT 66121003, 286960005, 195347004, 266323005, 155445002, 155441006
English Periarteritis Nodosa, PANARTERITIS NODOSA, PERIARTERITIS NODOSA, Panarteritis nodosa, Classical polyarteritis nodosa, POLYARTERITIS NODOSA SYSTEMIC, Polyarteritis nodosa NOS, panarteritis nodosa (diagnosis), periarteritis nodosa (diagnosis), polyarteritis nodosa, panarteritis nodosa, polyarteritis nodosa (diagnosis), periarteritis nodosa, Polyarteritis Nodosa, Kussmaul's disease, Polyarteritis nodosum, Polyarteritis Nodosa [Disease/Finding], polyarteritis nodosum, kussmaul disease, nodosa polyarteritis, Panarteritis Nodosa, Classical Polyarteritis Nodosa, Systemic periarteritis nodosa, Polyarteritis nodosa NOS (disorder), PAN, ADA2 DEFICIENCY, POLYARTERITIS NODOSA, Periarteritis nodosa, Polyarteritis nodosa, Polyarteritis nodosa (disorder), Kussmaul; disease, disease; Kussmaul, PAN - Polyarteritis nodosa
French PERIARTERITE NOUEUSE, PANARTERITE NOUEUSE, POLYARTERITE NOUEUSE, Péri-artérite noueuse, Périartérite noueuse, Maladie de Kussmaul-Maier, PAN (Polyartérite noueuse), Panartérite noueuse, Polyartérite noueuse
Portuguese PERIARTERITE NODOSA, POLIARTERITE NODOSA, Doença de Kussmaul, Periarterite nodosa, Pan-arterite nodosa, PANARTERITE NODOSA, Poliarterite nodosa, Periarterite Nodosa, Poliarterite Nodosa
Spanish PERIARTERITIS NUDOSA, POLIARTERITIS NUDOSA, Panarteritis nodosa, Periarteritis nodosa, Enfermedad de Kussmaul, PANARTERITIS NUDOSA, periarteritis nodosa sistémica, panarteritis nodosa, poliarteritis nodosa, SAI, periarteritis nodosa, poliarteritis nodosa, SAI (trastorno), poliarteritis nudosa (trastorno), poliarteritis nudosa, Poliarteritis nudosa, poliarteritis nodosa, Periarteritis Nudosa, Poliarteritis Nudosa
Dutch ziekte van Kussmaul, periarteriitis nodosa, panarteriitis nodosa, polyarteriitis nodosum, Kussmaul; ziekte, ziekte; Kussmaul, polyarteriitis nodosa, Periarteriitis nodosa, Polyarteriitis nodosa
German Panarteritis nodosa, Kussmaul-Krankheit, Polyarteritis nodosum, Periarteritis nodosa, PANARTERIITIS NODOSA, PERIARTERIITIS NODOSA, POLYARTERIITIS NODOSA, Panarteriitis nodosa, Periarteriitis nodosa, Polyarteritis nodosa
Italian Malattia di Kussmaul, Panarterite nodosa, Periarterite nodosa, Poliarterite nodosa
Japanese 結節性汎動脈炎, クスマール病, クスマールビョウ, ケッセツセイタハツドウミャクエン, ケッセツセイハンドウミャクエン, ケッセツセイドウミャクシュウイエン, 結節性多発動脈炎, 動脈周囲炎-結節性, 動脈周囲炎-多発性, 本態性多発動脈炎, 多発性動脈周囲炎, 結節性多発性動脈炎, 動脈炎-壊死性, 多発性結節性動脈炎, 結節性動脈周囲炎, 壊死性動脈炎, 多発動脈炎-結節性
Swedish Polyarteritis nodosa
Czech polyarteritis nodosa, periarteritis nodosa, Periarteriitis nodosa, Polyarteriitis nodosa, Panarteriitis nodosa, Kussmaulova nemoc, nodózní polyarteritida
Finnish Valtimoiden kyhmytulehdus
Korean 결절성 다발동맥염
Polish Zapalenie wielotętnicze guzkowe, Zapalenie okołotętnicze guzkowate
Hungarian Polyarteritis nodosum, Panarteritis nodosa, Kussmaul-betegség, Periarteritis nodosa, Polyarteritis nodosa
Norwegian Polyarteritis nodosa

Ontology: Cutaneous polyarteritis nodosa (C0343190)

Concepts Disease or Syndrome (T047)
SnomedCT 239926000
English cutaneous polyarteritis nodosa, Cutaneous livedo with nodules, Cutaneous polyarteritis nodosa, Nodular livedo, Cutaneous polyarteritis nodosa (disorder)
Spanish livedo cutánea con nódulos, livedo nodular, poliarteritis nodosa cutánea (trastorno), poliarteritis nodosa cutánea