What is Vasculitis?
 

Vasculitis is a general medical term for a group of conditions characterized by inflammation of the blood vessels.

• What Happens to the Vessels: When a blood vessel becomes inflamed, its walls swell, thicken, and weaken.

  • Narrowing/Occlusion: Thickened vessel walls restrict blood flow, starving tissues and organs of oxygen and nutrients (ischemia).

  • Aneurysm/Rupture: Weakened vessel walls can bulge outward (forming an aneurysm) or burst, leading to internal bleeding.

• Primary vs. Secondary Vasculitis:

  • Primary Vasculitis: Occurs as an isolated autoimmune disease with no known external trigger.

  • Secondary Vasculitis: Triggered by an underlying condition, such as a severe infection (e.g., Hepatitis B or C), an autoimmune disease (e.g., Lupus or Rheumatoid Arthritis), a reaction to certain medications, or specific cancers.

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Large Vessel Vasculitis - Affects the aorta (the body's main arterial trunk) and its major primary branches.

 

Giant Cell Arteritis (GCA / Temporal Arteritis)

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• Who It Affects: Almost exclusively adults over 50 years of age.

• Key Features: Severe, new temporal headaches, scalp tenderness, jaw pain while chewing (jaw claudication), and sudden visual changes or vision loss (a medical emergency). Frequently associated with Polymyalgia Rheumatica (shoulder and hip stiffness).

• Specific Treatments:

  • Acute Treatment: High-dose oral systemic corticosteroids. Possibly IV steroids for threatened vision loss. 

  • Steroid-Sparing / Maintenance: Tocilizumab (Actemra), an IL-6 receptor antagonist biologic, is FDA-approved to maintain remission and allow faster corticosteroid tapering. Methotrexate can also be used as an alternative secondary agent.

 

Takayasu Arteritis

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• Who It Affects: Typically young women under 40 years of age.

• Key Features: Often called "pulseless disease" because inflammation narrows major arterial branches, leading to weak or absent limb pulses, arm claudication, and significant blood pressure discrepancies between arms.

• Specific Treatments:

  • Acute Treatment: High-dose corticosteroids to halt active vascular inflammation.

  • Maintenance / Steroid-Sparing: Biologic agents such as TNF inhibitors (Infliximab, Adalimumab) or Tocilizumab, or conventional immunosuppressants (Methotrexate, Azathioprine, Mycophenolate Mofetil).

  • Surgical Intervention: Vascular bypass surgery, angioplasty, or stenting may be needed once active inflammation is controlled to restore blood flow through narrowed or aneurysmal vessels.

 

Medium Vessel Vasculitis - Affects the main visceral arteries and their branches supplying internal organs and extremities.

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Polyarteritis Nodosa (PAN)

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• Key Features: Systemic inflammation affecting skin, peripheral nerves, kidneys, and gastrointestinal tract. Symptoms include painful skin nodules, nerve damage (mononeuritis multiplex, causing wrist or foot drop), severe abdominal pain after eating, and hypertension. Spares the lungs. Strongly linked with Hepatitis B infection.

• Specific Treatments:

  • Idiopathic (Non-Viral) PAN: High-dose oral corticosteroids combined with oral or IV Cyclophosphamide for organ-threatening disease. Once in remission, patients transition to Azathioprine or Methotrexate for maintenance.

  • Hepatitis B-Associated PAN: Antiviral therapy (e.g., Entecavir or Tenofovir) combined with plasma exchange (plasmapheresis) and short-term corticosteroids. Cyclophosphamide is avoided in viral cases to prevent worsening viral replication.

 

Kawasaki Disease

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• Who It Affects: Primarily infants and young children under 5 years of age.

• Key Features: High fever lasting more than 5 days, red "strawberry tongue," cracked lips, bloodshot eyes (conjunctivitis), swollen hands/feet with peeling skin, and swollen cervical lymph nodes.

• Critical Risk: High risk of developing coronary artery aneurysms if untreated.

• Specific Treatments:

  • Acute Phase: Intravenous Immunoglobulin (IVIG) given as a single high-dose infusion along with high-dose aspirin to suppress acute systemic inflammation and protect the coronary arteries.

  • Convalescent Phase: Once fever resolves, low-dose aspirin is continued for 6 to 8 weeks (or indefinitely if coronary artery anomalies develop) to prevent blood clots. Corticosteroids or infliximab are reserved for IVIG-resistant cases.

 

Small Vessel Vasculitis - Affects small arteries, arterioles, capillaries, and venules. It frequently involves the lungs and kidneys.

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ANCA-Associated Vasculitis

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Characterized by the presence of Anti-Neutrophil Cytoplasmic Antibodies (ANCA) in blood testing.

• Granulomatosis with Polyangiitis (GPA / formerly Wegener's):

  • Key Features: Affects upper respiratory tract (chronic sinus infections, nosebleeds, saddle nose deformity), lungs (coughing up blood, nodules), and kidneys (nephritic syndrome / glomerulonephritis).

• Microscopic Polyangiitis (MPA):

  • Key Features: Similar to GPA with lung and kidney involvement, but lacks granulomatous sinus or upper airway lesions.

• Eosinophilic Granulomatosis with Polyangiitis (EGPA / formerly Churg-Strauss):

  • Key Features: Characterized by severe adult-onset asthma, elevated blood eosinophil levels, allergic rhinitis, and peripheral nerve damage.

• Specific Treatments for ANCA Vasculitides:

  • Induction Therapy (Severe/Organ-Threatening GPA/MPA): High-dose pulse IV corticosteroids followed by either Rituximab (anti-CD20 monoclonal antibody) or Cyclophosphamide. Avacopan (Tavneos), a C5a receptor antagonist, can be added to reduce overall steroid exposure. 

  • Maintenance Therapy (GPA/MPA): Rituximab infusions given every 6 months, or oral Azathioprine / Methotrexate for 2 years or longer to prevent relapses.

  • EGPA-Specific Treatment: High-dose corticosteroids. For mild-to-moderate relapsing cases, Mepolizumab (Nucala), an anti-IL-5 antibody, is FDA-approved and highly effective. Cyclophosphamide or Rituximab is added for severe organ involvement.

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Immune Complex Small Vessel Vasculitis

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• IgA Vasculitis (Henoch-Schönlein Purpura / HSP):

  • Key Features: Most common vasculitis in children. Classic tetrad: raised purple skin spots (purpura) on legs/buttocks, joint pain, abdominal pain, and kidney inflammation (hematuria).

  • Specific Treatments: Mostly supportive care (hydration, rest, and pain management) as it is self-limiting in over 85% of pediatric cases. NSAIDs are used for joint pain (unless severe kidney involvement is present). Systemic corticosteroids (Prednisone) are used for severe abdominal pain or severe kidney involvement (IgA nephritis).

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• Cryoglobulinemic Vasculitis:

  • Key Features: Associated with Hepatitis C infection. Abnormal proteins clump in cold temperatures, causing skin purpura, joint pain, and weakness.

  • Specific Treatments: Direct-acting antiviral (DAA) therapy to cure the underlying Hepatitis C infection. For severe organ-threatening flare-ups, short-term corticosteroids, Rituximab, and plasmapheresis are used to clear circulating cryoglobulins.

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Common Symptoms & Warning Signs

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Systemic (General) Symptoms

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Most forms of vasculitis begin with non-specific, flu-like inflammation signs:

• Unexplained, recurring fevers

• Unintentional weight loss and loss of appetite

• Profound fatigue and muscle aches

• Night sweats

 

Organ-Specific Symptoms

• Skin: Palpable purpura (raised, non-blanching purple or red spots), painful open sores/ulcers, or livedo reticularis (a purplish, net-like discoloration).

• Nerves: Numbness, tingling, burning pain, or sudden weakness in a hand or foot ("wrist drop" or "foot drop").

• Eyes: Eye redness, severe pain, double vision, or sudden vision loss.

• Lungs: Shortness of breath, persistent cough, or coughing up blood (hemoptysis).

• Kidneys: Dark or tea-colored urine, leg swelling, or elevated blood pressure.

• Joints: Aching, swollen, or painful joints (arthralgia/arthritis).

 

Diagnostics

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Because vasculitis mimics many other conditions, diagnosis requires a comprehensive combination of testing:

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• Blood Tests:

  • Inflammatory Markers: Erythrocyte Sedimentation Rate (ESR) and C-Reactive Protein (CRP) to measure systemic inflammation.

  • Autoimmune Serologies: ANCA panel (c-ANCA/PR3 and p-ANCA/MPO), ANA, Complement levels (C3/C4), and Cryoglobulins.

  • Organ Function: Serum Creatinine and BUN (kidney clearance), Liver Function Tests.

  • Infection Screening: Hepatitis B, Hepatitis C, and HIV.

• Urinalysis: Checks for protein, microscopic blood, and cellular casts indicating kidney involvement.

• Imaging Studies:

  • Angiography (CT or MR Angiography): Visualizes blood vessel narrowing, blockages, or aneurysms in medium and large vessels.

  • Chest CT: Evaluates lung nodules, infiltrates, or bleeding.

• Tissue Biopsy: Taking a small sample of an affected organ (skin, temporal artery, kidney, lung, or nerve) to examine under a microscope. 

 

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Because long-term corticosteroids and strong immunosuppressants weaken the immune system and affect multiple body systems, supportive care is vital:

• Infection Prophylaxis: Patients taking high-dose steroids and cyclophosphamide or rituximab often take prophylactic antibiotics (e.g., trimethoprim-sulfamethoxazole / Bactrim) to prevent opportunistic fungal pneumonia (Pneumocystis jirovecii).

• Bone Health Protection: Daily calcium and Vitamin D supplementation, baseline bone-density DEXA scans, and bisphosphonate medications (e.g., alendronate) to protect against steroid-induced osteoporosis.

• Cardiovascular & Metabolic Protection: Routine monitoring of blood pressure, blood glucose (to manage steroid-induced diabetes), and cholesterol.

• Vaccination Guidance: Non-live vaccines (such as flu, pneumococcal, and COVID-19) should be kept up to date, ideally administered prior to initiating B-cell depleting therapies like Rituximab. Live vaccines are strictly avoided during immunosuppressive treatment.

 

When to Seek Emergency Evaluation

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• Sudden changes in vision, double vision, or loss of vision in one eye.

• New, severe headache accompanied by jaw pain or scalp tenderness (especially if over age 50).

• Coughing up blood or sudden, severe shortness of breath.

• Dark brown, tea-colored urine or a sudden drop in urine output.

• Sudden weakness, numbness, or loss of movement in a limb (e.g., foot drop).

• Severe, localized abdominal pain, especially after eating.

• High, persistent fever in a child accompanied by a red tongue, bloodshot eyes, or peeling skin.

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                                                            References & Guidelines

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• American College of Rheumatology/Vasculitis Foundation (ACR/VF). 2021 Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis.

• American College of Rheumatology/Vasculitis Foundation (ACR/VF). 2021 Guideline for the Management of ANCA-Associated Vasculitis.

• American College of Rheumatology/Vasculitis Foundation (ACR/VF). 2021 Guideline for the Management of Polyarteritis Nodosa.

• American College of Rheumatology/Vasculitis Foundation (ACR/VF). 2021 Guideline for the Management of Kawasaki Disease.

• European Alliance of Associations for Rheumatology (EULAR). Recommendations for the Use of Imaging in Large Vessel Vasculitis: 2023 Update.