Vasculitis — Comprehensive Nursing Guide

Giant Cell Arteritis (GCA)

Temporal/cranial arteries, aorta. Age >50. Risk of visual loss.

Takayasu Arteritis

Aorta & major branches. Young Asian women. Pulseless disease.

Polyarteritis Nodosa (PAN)

Necrotising arteritis. Hepatitis B association. No glomerulonephritis.

Kawasaki Disease

Children <5. Coronary artery aneurysm risk. IVIG treatment.

GPA (Granulomatosis with Polyangiitis)

c-ANCA / PR3-ANCA. Upper & lower respiratory + renal.

MPA (Microscopic Polyangiitis)

p-ANCA / MPO-ANCA. Pulmonary-renal syndrome.

EGPA (Eosinophilic GPA)

p-ANCA 40%. Asthma + eosinophilia. Mononeuritis multiplex.

IgA Vasculitis (IgAV / HSP)

IgA immune deposits. Palpable purpura, arthritis, GI, renal.

Cryoglobulinaemic Vasculitis

Hepatitis C association. Purpura, neuropathy, membranoproliferative GN.

c-ANCA (cytoplasmic)

= PR3-ANCA (Proteinase 3)

Strongly associated with GPA (Wegener's)

Sensitivity ~90% in active generalised GPA

p-ANCA (perinuclear)

= MPO-ANCA (Myeloperoxidase)

Associated with MPA and EGPA (40%)

Also drug-induced ANCA vasculitis

Autoantibody-Mediated

ANCA activates neutrophils → degranulation → endothelial injury and microthrombus formation. Key in GPA/MPA/EGPA.

Immune Complex Deposition

Immune complexes deposit in vessel walls → complement activation → inflammation. Key in IgAV, cryoglobulinaemia.

T-Cell Mediated (Granulomatous)

CD4+ T-cells and macrophages form granulomas in vessel walls. Key in GCA and Takayasu arteritis.

Constitutional (systemic)

• Fever — often low-grade, persistent
• Fatigue and malaise
• Weight loss (unintentional)
• Night sweats, myalgia, arthralgia

Organ-Specific Clues

• Skin: palpable purpura, livedo, ulcers
• Renal: haematuria, proteinuria, AKI
• Lung: haemoptysis, infiltrates, cavities
• Neuro: mononeuritis multiplex, CNS involvement

VISUAL LOSS — OPHTHALMOLOGICAL EMERGENCY

Anterior ischaemic optic neuropathy (AION) — occlusion of the posterior ciliary artery → irreversible blindness in the affected eye. Visual loss in GCA is permanent and irreversible once established.

INDUCTION

Prednisolone 60 mg/day (or IV methylprednisolone if visual symptoms)

TAPERING

Slow taper over 12–18+ months guided by symptoms and inflammatory markers

RELAPSING DISEASE

Tocilizumab (IL-6 inhibitor) — approved for GCA; steroid-sparing

Monitor PMR patients for GCA symptoms — headache or visual symptoms require urgent escalation.

GPA

Upper Airway

Sinusitis, epistaxis, nasal crusting, saddle-nose deformity, subglottic stenosis

Pulmonary

Infiltrates, nodules, cavities, haemoptysis, pulmonary haemorrhage

Renal

Crescentic GN — haematuria, proteinuria, rapid AKI

MPA

Hallmark: Pulmonary-Renal Syndrome

Diffuse alveolar haemorrhage + rapidly progressive GN — life-threatening combination

No Upper Airway Involvement

Distinguishes MPA from GPA — no granulomas, no saddle-nose

Other

Skin purpura, peripheral neuropathy, constitutional features

EGPA

Three Phases

1. Allergic: asthma, allergic rhinitis
2. Eosinophilic: peripheral eosinophilia, lung infiltrates
3. Vasculitic: neuropathy, skin, cardiac

Mononeuritis Multiplex

Wrist drop, foot drop — focal nerve ischaemia from vasculitis

Warning Signs

• Rapidly rising creatinine
• Haematuria (RBC casts on urine microscopy)
• Heavy proteinuria
• Oliguria/anuria

Urgent Interventions

• Renal biopsy (confirms crescentic GN)
• Plasma exchange if severe (Cr >500 or dialysis-dependent)
• Dialysis if AKI requires it
• Pulse IV methylprednisolone

ANCA Levels

Rising ANCA may predict relapse — not universal, always correlate clinically

Urinalysis

Dipstick at every visit — blood/protein = nephritis surveillance

Creatinine / eGFR

Trend matters — rising Cr needs urgent review

FBC / CRP

Neutropenia from treatment; CRP for disease activity

CXR / CT Chest

Pulmonary infiltrates, cavities, haemorrhage monitoring

1. Conjunctival Injection

Bilateral, non-purulent. Bulbar conjunctivae. Without exudate.

2. Lip/Oral Changes

Cracked/red lips, strawberry tongue, erythema of oropharynx

3. Polymorphous Rash

Maculopapular, erythroderma, or multiforme-like. Trunk and extremities.

4. Cervical Lymphadenopathy

At least one node >1.5 cm. Typically unilateral, non-purulent.

5. Extremity Changes

Erythema/oedema of hands and feet (acute). Desquamation of fingertips (subacute — week 2–3)

Risk Factors for CAA

• Fever duration >10 days
• Age <12 months or >8 years
• Male sex
• IVIG resistance / delayed treatment
• High CRP, low albumin, high neutrophil count

Echocardiography Protocol

• At diagnosis (baseline)
• 2 weeks after treatment initiation
• 6–8 weeks after diagnosis
• Ongoing monitoring if CAA present

Dose: IVIG 2 g/kg single infusion

Over 10–12 hours. Start within 10 days of fever onset.

ACUTE PHASE (anti-inflammatory dose)

High-dose aspirin 80–100 mg/kg/day in 4 divided doses until afebrile for 48–72 hrs

SUBACUTE/MAINTENANCE (antiplatelet dose)

Low-dose aspirin 3–5 mg/kg/day once daily. Continue 6–8 weeks (or indefinitely if CAA)

Avoid aspirin during chickenpox/influenza — Reye syndrome risk. Coordinate with vaccinations.

Incomplete (Atypical) Kawasaki

Fever ≥5 days + only 2–3 features. More common in infants <6 months. Higher CAA risk. Requires echo + inflammatory markers (CRP, albumin, echo).

Kawasaki Shock Syndrome (KSS)

Haemodynamic instability requiring vasoactive drugs. Higher rates of IVIG resistance and CAA. Admit to PICU. May require IV methylprednisolone.

HAEMORRHAGIC CYSTITIS PREVENTION

• Mesna — uroprotective agent. Give with IV CYC.
• Adequate hydration: 2–3L/day oral fluid
• Encourage frequent voiding — do not hold urine
• Monitor urine for haematuria

NEUTROPENIA MANAGEMENT

• FBC at nadir (10–14 days post IV CYC)
• G-CSF (filgrastim) if neutrophils <1.0 ×10⁹/L
• Neutropenic precautions if severe
• Avoid live vaccines during treatment

PRE-MEDICATION (30 min before)

• IV methylprednisolone 100 mg
• Paracetamol 1 g oral
• Chlorphenamine (antihistamine)

INFUSION MONITORING

• Start at 50 mg/hr — escalate if tolerated
• VS every 30 min during infusion
• Infusion reaction: stop, antihistamine, adrenaline if anaphylaxis

PML Risk — JC Virus

Progressive multifocal leukoencephalopathy. Rare. Educate: new neurological symptoms (confusion, weakness, speech changes) = seek urgent review.

Bone Loss

Bisphosphonate + Ca2+/VitD. DEXA scan at baseline. Fall prevention.

Hyperglycaemia

BSL monitoring. Diabetic diet advice. Adjust insulin/oral agents as needed.

GI Ulceration

PPI prophylaxis. Take steroids with food. Monitor for GI bleeding.

Hypertension

Regular BP monitoring. Antihypertensives as needed. Low-salt diet advice.

Mood / Psych

Insomnia, mood swings, depression, euphoria. Psychological screening. Support services.

Cushing Features

Weight gain, moon face, buffalo hump. Reassure — usually reversible on taper.

Disease Understanding

• Nature of vasculitis and chronic/relapsing course
• Why long-term medication is essential
• Importance of not stopping steroids abruptly (adrenal crisis)
• Steroid emergency card / medical alert

Warning Signs of Relapse

• Return of headache (GCA), visual changes — URGENT
• Blood or frothy urine (renal relapse)
• Haemoptysis, worsening breathlessness
• New skin rash, joint swelling, neuropathy

Anxiety & Depression

Screening with PHQ-9/GAD-7. Refer to liaison psychiatry or clinical psychology if significant.

Support Groups

Vasculitis UK, Vasculitis Foundation (US). Online communities for isolated patients in GCC regions.

Body Image

Steroid-related changes (moon face, weight gain). Normalise and support. Taper plans reduce over time.

Adherence

Explore barriers — cost, side effects, beliefs. Simplify regimens. Written plans & follow-up support.