Vasculitis

Aka: Vasculitis

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II. Definition

  1. Primary disorder of vascular inflammation

III. Epidemiology

  1. Cutaneous Small Vessel Vasculitis is most common

IV. Types

  1. Large-Vessel Vasculitis
    1. Giant Cell Arteritis (Carotid Artery)
    2. Takayasu's Disease (Aorta)
  2. Medium-Vessel Vasculitis
    1. Polyarteritis Nodosa
    2. Kawasaki's Disease (coronary arteries)
  3. Small-Vessel Vasculitis (ANCA or Non-ANCA associated)
    1. Granulomatosis with Polyangiitis (respiratory tract, Glomerulonephritis)
      1. Previously known as Wegener's Granulomatosis
    2. Churg-Strauss Syndrome (respiratory tract)
    3. Microscopic Polyangiitis (respiratory tract, Glomerulonephritis)
    4. Henoch-Schonlein Purpura (IgA deposits in skin, GI Tract, joints, Glomerulonephritis)
    5. Essential Cryoglobulinemia (skin, glomeruli)
    6. Cutaneous leuokocytoclastic Vasculitis (skin only)

V. Associated Conditions

  1. Headache
  2. Sinusitis
  3. Mononeuritis multiplex
  4. Cerebrovascular Accident
  5. Hematuria

VI. Signs: General

  1. Fever
  2. Hypertension
  3. Weight loss
  4. Rales on pulmonary exam
  5. Hepatomegaly or Splenomegaly
  6. Palpable Peripheral Nerves
  7. Skin findings (see specific findings based on type below)
    1. Superficial Thrombophlebitis
    2. Skin Ulcerations

VII. Signs: Specific findings based on type

  1. Large Vessel Vasculitis
    1. Arterial Bruit
    2. Pulsation loss
  2. Medium Vessel Vasculitis
    1. Nodules
    2. Necrotizing Livedo Reticularis
  3. Small Vessel Vasculitis
    1. Palpable Purpura
    2. Urticaria

VIII. Labs

  1. Cutaneous lesional Punch Biopsy is single most important study
    1. Biopsy the most characteristic, early active lesions
    2. Send for histopathology and direct immunofluorescence
  2. Inflammatory markers (acute phase reactants) and Infectious disease testing
    1. Complete Blood Count (CBC)
    2. Erythrocyte Sedimentation Rate (ESR)
    3. C-Reactive Protein (C-RP)
    4. Blood Culture
    5. Site-specific culture
  3. Renal tests
    1. Urinalysis
    2. Serum Creatinine
    3. Blood Urea Nitrogen (BUN)
    4. Creatinine phosphokinase (CPK)
  4. Hepatic tests
    1. Liver Function Tests (hepatic panel)
    2. Hepatitis B Surface Antigen
    3. Hepatitis C Antibody
    4. Cryoglobulin
  5. Rheumatologic tests
    1. Antinuclear Antibody
    2. Anticardiolipin Antibody
    3. Rheumatoid Factor
    4. Serum complement
    5. Anti-dsDNA
    6. Extractable nuclear Antigen
    7. Serum Protein Electrophoresis
    8. Antineutrophil cytoplasmic autoantibodies (ANCA)
      1. Reflex if positive to Proteinase 3 or myeloperoxidase

IX. Imaging

  1. Chest XRay or Chest CT
  2. Angiography (CTA or MRA)

X. Diagnostics: If Indicated

  1. Electrocardiogram
  2. Echocardiogram
  3. Electromyogram (EMG)

XI. Differential Diagnosis

  1. Antiphosphospholipid Antibody syndrome
  2. Atheroembolic Disease
  3. Cocaine Abuse
  4. Amphetamine Abuse
  5. Hypersensitivity Reaction
  6. Endocarditis
  7. Multiple Myeloma
  8. Sick Cell Anemia
  9. Secondary Vasculitis
    1. Rheumatoid Arthritis
    2. Systemic Lupus Erythematosus
    3. Scleroderma
    4. Hepatitis B Infection
    5. Hepatitis C Infection
    6. Lymphioma
    7. Solid organ tumor

XII. References

  1. Bohjanen (2009) UMN CME Internal Medicine Review, Minneapolis
  2. Jennette (1994) Arthritis Rheum 37:187 [PubMed]
  3. Sharma (2011) Am Fam Physician 83(5): 556-65 [PubMed]

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Related Studies

Ontology: Vasculitis (C0042384)

Definition (MSHCZE) Zánětlivé onemocnění cév, zejm. tepen, nejč. v rámci autoimunního, systémového či revmatického onemocnění. Stěna cév je poškozena zánětem, infiltrována leukocyty (neutrofily při akutním, lymfocyty při chronickém zánětu), v některých případech obrovskými buňkami (velkobuněčná v). Může dojít k fibrinoidní nekróze, vzniku aneurysmat. Postižení je obv. segmentární, postižené úseky se střídají s normálními. Poškození cévy může vést ke krvácení či ischemizaci oblasti (části orgánu). Klinické projevy dále obv. zahrnují zánětlivé příznaky a orgánová poškození. V. tvoří i podstatu mnoha projevů systémových chorob např. SLE. V léčbě v. se často uplatňují kortikoidy a imunosuprese. (cit. Velký lékařský slovník online, 2012 http://lekarske.slovniky.cz/ )
Definition (MEDLINEPLUS)

Vasculitis is an inflammation of the blood vessels. It happens when the body's immune system attacks the blood vessel by mistake. It can happen because of an infection, a medicine, or another disease. The cause is often unknown.

Vasculitis can affect arteries, veins and capillaries. Arteries are vessels that carry blood from the heart to the body's organs. Veins are the vessels that carry blood back to the heart. Capillaries are tiny blood vessels that connect the small arteries and veins.

When a blood vessel becomes inflamed, it can

  • Narrow, making it more difficult for blood to get through
  • Close off completely so that blood can't get through
  • Stretch and weaken so much that it bulges. The bulge is called an aneurysm. If it bursts, it can cause dangerous bleeding inside the body.

Symptoms of vasculitis can vary, but usually include fever, swelling and a general sense of feeling ill. The main goal of treatment is to stop the inflammation. Steroids and other medicines to stop inflammation are often helpful.

NIH: National Heart, Lung, and Blood Institute
Definition (NCI) An inflammatory process involving the wall of the vessels (large, medium, or small-sized vessels). The inflammatory process may cause necrosis or formation of granulomas in the vascular wall. It may be the result of an autoimmune disorder, infection, or malignancy. Representative examples include polyarteritis nodosa, temporal arteritis, Wegener's granulomatosis, Kawasaki disease, Takayasu arteritis, and Henoch-Schonlein purpura.
Definition (NCI_CTCAE) A disorder characterized by inflammation involving the wall of a vessel.
Definition (CSP) inflammation of a blood vessel.
Definition (MSH) Inflammation of any one of the blood vessels, including the ARTERIES; VEINS; and rest of the vasculature system in the body.
Concepts Disease or Syndrome (T047)
MSH D014657
SnomedCT 195375002, 266325003, 393589007, 31996006
LNC LA17783-4
English Angiitides, Angiitis, Vasculitides, VASCULITIS, Vasculitis, vasculitides (diagnosis), vasculitides, vascular inflammation or infection, Vasculitis NOS, Vasculitis [Disease/Finding], infection of the blood or lymph vessel, infection of the blood vessel, Inflammation of blood vessel, Vasculitis (disorder), vasculitis, angiitis, syndrome; vasculitis, vasculitis; syndrome, Vasculitis, NOS, Angiitis, NOS
Portuguese VASCULITE, Vasculite NE, Angeíte, Vasculites, Angiite, Vasculite
Spanish VASCULITIS, Vasculitis NEOM, angeítis, angitis, Síndromes vasculíticos, Angeítis, vasculitis (trastorno), vasculitis, Vasculitides, Vasculitis, Angiitis
German VASKULITIS, Vaskulitis NNB, Vaskulitiden, Angiitis, Vaskulitis
Italian Vasculiti, Angioite, Vasculite NAS, Angite, Vasculite
Dutch angiïtis, vasculitis NAO, syndroom; vasculitis, vasculitis; syndroom, vasculitiden, vasculitis, Angiitis, Vasculitis
French Vascularite SAI, ANGEITE, Vascularites, Vascularite, Vasculite, Angéite
Japanese 血管炎ＮＯＳ, ｹｯｶﾝｴﾝNOS, ｹｯｶﾝｴﾝ, 血管炎, 脈管炎(ミャクカンエン), 脈管炎
Swedish Kärlinflammation
Czech vaskulitida, Vaskulitida NOS, Vaskulitidy, Angiitida, Vaskulitida, angiitida
Finnish Verisuonitulehdus
Russian VASKULITY, ANGIIT, АНГИИТ, ВАСКУЛИТЫ
Croatian VASKULITIS
Polish Zapalenie naczyń
Hungarian Vasculitis, Vasculitidek, Vasculitis k.m.n., Angiitis
Norwegian Vaskulitt, Angiitt, Blodkarbetennelse
Derived from the NIH UMLS (Unified Medical Language System)

Related Topics in Cardiovascular Medicine

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