Summary

Vasculitis is inflammation of blood vessels, which can affect vessels of any size (small, medium, or large) and any organ system. Think of vasculitis as your immune system attacking your own blood vessels—this inflammation damages the vessel walls, causing them to narrow or block, leading to reduced blood flow to organs and tissues. Vasculitis can be primary (disease itself) or secondary (caused by another condition like infection, drugs, or other autoimmune diseases). There are many types of vasculitis, classified by the size of vessels affected and the organs involved (e.g., giant cell arteritis affects large vessels, granulomatosis with polyangiitis affects small vessels, polyarteritis nodosa affects medium vessels). The presentation varies widely depending on the type and organs affected, but common features include fever, weight loss, fatigue, and organ-specific symptoms (kidney: blood/protein in urine, lung: cough/breathlessness, skin: rash, nervous system: numbness/weakness). The key to management is recognizing the pattern (symptoms, signs, organ involvement), confirming the diagnosis (blood tests, imaging, biopsy), classifying the type (essential for treatment), and providing appropriate treatment (usually immunosuppression—steroids, sometimes other immunosuppressants). Early recognition and treatment are essential to prevent permanent organ damage.

Key Facts

• Definition: Inflammation of blood vessels
• Incidence: Rare to uncommon (varies by type, 1-50 per 100,000)
• Mortality: Varies by type (5-30% if untreated, lower with treatment)
• Peak age: Varies by type (some in young adults, some in older adults)
• Critical feature: Multi-system involvement, inflammation of vessels
• Key investigation: Clinical assessment, blood tests (ANCA, etc.), imaging, biopsy
• First-line treatment: Immunosuppression (steroids, sometimes other agents)

Clinical Pearls

"Think multi-system" — Vasculitis usually affects multiple organ systems. If you see involvement of multiple systems (kidney + lung + skin, etc.), think vasculitis.

"ANCA is helpful but not diagnostic" — ANCA (anti-neutrophil cytoplasmic antibodies) is helpful for some types (granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis), but not all vasculitis is ANCA-positive. Don't rely on ANCA alone.

"Biopsy is often needed" — Biopsy of affected tissue (skin, kidney, lung, etc.) is often needed to confirm the diagnosis and classify the type. Don't skip this if diagnosis is uncertain.

"Type matters for treatment" — Different types of vasculitis need different treatments. Classifying the type is essential for appropriate treatment.

Why This Matters Clinically

Vasculitis is a serious condition that can cause permanent organ damage if not recognized and treated early. Early recognition (especially multi-system involvement), proper classification, and appropriate immunosuppressive treatment are essential. This is a condition that rheumatologists, nephrologists, and other specialists manage, and prompt treatment prevents organ damage.

Structured Approach: ABCDE

A - Airway

• Assessment: Usually patent
• Action: Secure if compromised

B - Breathing

• Look: May have respiratory distress (if lung affected)
• Listen: May have crackles (if lung affected)
• Measure: SpO2 (may be low if lung affected)
• Action: Support if needed

C - Circulation

• Look: May have signs of organ damage
• Feel: Pulse (usually normal), BP (may be high if kidney affected)
• Listen: Heart sounds (usually normal)
• Measure: BP (may be high), HR
• Action: Monitor if organ damage

D - Disability

• Assessment: Neurological status (may have neuropathy, stroke)
• Action: Assess if neurological involvement

E - Exposure

• Look: Full examination, look for multi-system involvement
• Feel: Skin, joints, assess organs
• Action: Complete examination

Specific Examination Findings

Multi-System Examination:

• Kidney: Check BP, edema, signs of renal failure
• Lung: Auscultate, check for respiratory distress
• Skin: Look for rash, ulcers, nodules, purpura
• Nervous system: Check for neuropathy, weakness, stroke signs
• Joints: Check for swelling, tenderness
• Eyes: Check for redness, vision
• GI tract: Check for tenderness, signs of ischemia

Special Tests

Natural History (Without Treatment)

Untreated Vasculitis:

• High mortality: 5-30% mortality (varies by type)
• Organ damage: Almost certain
• Poor outcomes: If not treated

Outcomes with Treatment

Factors Affecting Outcomes:

Good Prognosis:

• Early treatment: Better outcomes
• No organ damage: Better outcomes
• Type: Some types better prognosis
• Good response: Better outcomes

Poor Prognosis:

• Delayed treatment: Higher mortality, more organ damage
• Organ damage: Worse outcomes
• Type: Some types worse prognosis
• Poor response: Worse outcomes

Prognostic Factors

Key Guidelines

1. EULAR Guidelines (2016) — EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. European League Against Rheumatism

Key Recommendations:

• Early recognition
• Appropriate immunosuppression
• Evidence Level: 1A

2. ACR Guidelines (2021) — American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody–associated vasculitis. American College of Rheumatology

Key Recommendations:

• Similar to EULAR
• Evidence Level: 1A

Landmark Trials

Multiple studies on immunosuppressive treatment, outcomes.

Evidence Strength

What is Vasculitis?

Vasculitis is inflammation of blood vessels, which can affect vessels of any size and any organ system. Think of vasculitis as your immune system attacking your own blood vessels—this inflammation damages the vessel walls, causing them to narrow or block, leading to reduced blood flow to organs and tissues.

In simple terms: Your blood vessels have become inflamed, which can affect blood flow to different parts of your body. This is serious and needs treatment, but with proper treatment, most people do well.

Why does it matter?

Vasculitis is a serious condition that can cause permanent organ damage if not recognized and treated early. Early recognition and appropriate immunosuppressive treatment are essential. The good news? With proper treatment, most people achieve remission and do well.

Think of it like this: It's like your blood vessels becoming inflamed—this can affect blood flow to your organs, but with the right treatment, the inflammation can be controlled.

How is it treated?

1. Diagnosis:

• Assessment: Your doctor will assess you to see which organs are affected
• Tests: You'll have blood tests, urine tests, and sometimes a biopsy (small sample of tissue)
• Why: To confirm the diagnosis and see which type of vasculitis you have

2. Treatment:

• Immunosuppression: You'll get medicines to suppress your immune system (usually steroids, sometimes other medicines)
• Why: To stop your immune system from attacking your blood vessels
• Duration: Usually starts with high doses, then gradually reduced

3. Monitoring:

• Regular check-ups: You'll have regular check-ups to monitor your disease and organ function
• Adjust treatment: Your doctor will adjust your treatment as needed
• Why: To keep the disease under control and prevent organ damage

The goal: Control the inflammation, prevent organ damage, and help you achieve remission.

What to expect

Recovery:

• Treatment: Usually starts with high doses of medicines, then gradually reduced
• Remission: Most people achieve remission (disease under control) within months
• Relapse: Some people may have relapses (disease returns), which can be treated

After Treatment:

• Medicines: You'll need to take medicines long-term (usually)
• Monitoring: Regular monitoring of your disease and organ function
• Lifestyle: Usually can live normally, but need to take medicines and have regular check-ups

Recovery Time:

• Remission: Usually within months
• Long-term: Ongoing management

When to seek help

See your doctor if:

• You have symptoms that affect multiple systems (kidney, lung, skin, etc.)
• You have unexplained fever, weight loss, or fatigue
• You have symptoms that concern you
• You have a known diagnosis of vasculitis and develop new symptoms

Call 999 (or your emergency number) immediately if:

• You have severe symptoms (difficulty breathing, severe pain, etc.)
• You feel very unwell
• You have symptoms that concern you

Remember: If you have symptoms that affect multiple systems, especially if you have unexplained fever, weight loss, or fatigue, see your doctor. Vasculitis is serious, but with proper treatment, most people do well. Also, if you have a known diagnosis of vasculitis and develop new symptoms, see your doctor—this may indicate a relapse or complication.

"The Trap that Backfires."

• ANCA (Anti-Neutrophil Cytoplasmic Antibody) is not just a marker; it drives the disease.
• Mechanism:
  1. Priming: Infection causes cytokines (TNF) to prime neutrophils, moving antigens (MPO/PR3) to the cell surface.
  2. Binding: ANCA antibodies bind to these antigens.
  3. Activation: This activates the neutrophils inappropriately.
  4. NETosis: Neutrophils release Neutrophil Extracellular Traps (NETs) - webs of DNA and enzymes meant to catch bacteria. In vasculitis, these NETs damage the delicate endothelial lining of capillaries (especially in the kidney).
• Result: Necrotizing inflammation of small vessels (Pauci-immune).

"Tissue is the Issue."

1. Renal Biopsy (Gold Standard for Renal Vasculitis)

• Indication: Active urinary sediment (blood/protein) + rising creatinine.
• Technique: Percutaneous ultrasound-guided core biopsy (Left kidney lower pole).
• Findings:
  • Crescentic Glomerulonephritis: Rupture of Bowman's capsule with proliferation of parietal cells (Crescents).
  • Pauci-immune: Little staining for antibody/complement (distinguishes from Lupus).

2. Lung Biopsy

• Indication: Pulmonary nodules/infiltrates without renal signs.
• Technique:
  • Trans-bronchial: Low yield.
  • VATS (Video Assisted Thoracoscopic Surgery): Gold standard. Wedge resection.
• Findings (GPA): Granulomatous inflammation + Necrosis + Vasculitis.

3. Skin Biopsy

• Indication: Purpura.
• Technique: Punch biopsy (4-5mm). Feature: Leukocytoclastic Vasculitis (broken white cells).

"It looks like Vasculitis, but isn't." Treating these with steroids can be fatal.

1. Infective Endocarditis:
   • Features: Fever, Murmur, Splinter haemorrhages, Glomerulonephritis (Immune complex).
   • Testing: Multiple sets of blood cultures + Echo. Rule #1: Exclude IE before diagnosing Vasculitis.
2. Cholesterol Emboli:
   • Features: "Blue Toe Syndrome", Renal failure after angiogram.
   • Clue: Livedo reticularis + Hx of vascular procedure.
3. Antiphospholipid Syndrome:
   • Features: Clots + Livedo. (Thrombosis, not inflammation).
4. Cocaine/Levamisole:
   • Features: "Cocaine nose" (septal perforation) mimics GPA. Can cause positive ANCA.

Q1: Which vasculitis is associated with Hepatitis B? A: Polyarteritis Nodosa (PAN). (Classic association).

Q2: What is the specific ANCA pattern for GPA vs MPA? A:

• GPA (Wegener's) = c-ANCA (PR3).
• MPA = p-ANCA (MPO).
• (Note: Overlap exists).

Q3: What is "Pauci-immune"? A: "Few antibodies". On renal biopsy immunofluorescence, there is NO glowing green IgG/C3. This proves it is ANCA vasculitis, not Lupus (Full House) or Goodpasture's (Linear).

Q4: A patient on Cyclophosphamide develops blood in urine 5 years later. Diagnosis? A: Bladder Cancer (Transitional Cell Carcinoma). A major long-term risk of Cyclophosphamide.

Q5: Treatment for Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)? A: Steroids are often sufficient for mild disease. Mepolizumab (Anti-IL5) is the new game-changer for the asthmatic component.

(End of Comprehensive Expansion)