Classification of Common Vasculitides

Vasculitis can be classified according to the size of the vessels it primarily affects

  • Large vessel vasculitis: Temporal arteritis, Takayasu’s arteritis 
  • Medium vessel vasculitis: PAN, Kawasaki, Beurger's 
  • Small vessel vasculitis: 
    • ANCA  associated vasculitis - GPAA, EGPA
    • Immune complex vasculitis - Goodpasture’s, SLE, HSP, cryoglobulinemia

 

Large vessel vasculitis 


Temporal Arteritis

Vasculitis of the superficial temporal artery (branch of the external carotid)

Clinical Features

  • Patients normally > 50 years of age
  • 50% of patients have a history of polymyalgia rheumatica (suggested by pain and stiffness in the proximal muscles esp. in the mornings)
  • Temporal headache 
  • Jaw claudication (chewing)
  • Superficial tenderness (e.g. while showering/combing hair)
  • Visual disturbance - due to anterior ischaemic optic neuropathy
    • Diplopia
    • Loss of vision
    • Changes to colour vision
  • O/E: Tender temporal artery which may be non pulsatile 

Investigations

  • Bloods: ESR raised, CRP raised
  • Temporal artery biopsy - diagnostic investigation of choice
    • Disadvantages: possibility of SKIP lesions
    • Histology: Granulomatous arteritis with mononuclear cell infiltrates and GIANT CELLS 
    • Complications: CN7 palsy, auriculotemporal nerve injury.

Management

  • High dose steroids - e.g. prednisolone 60mg OD
  • Urgent ophthalmology review - same day review if any visual symptoms

  

Polymyalgia Rheumatica

Clinical features

  • Age > 60yrs
  • Pain and stiffness in the shoulders, hips, and neck which develops acutely over a few days to weeks. 
  • Stiffness is worse in the mornings 
  • Low mood/ depression
  • Night sweats
  • Polyarthralgia

Investigations

  • ESR > 40 (CK, EMG normal)

Management

  •  Prednisolone 15 mg OD until symptoms are controlled (average 3-4 weeks), followed by a tapering dose for the following 3-6 months. 

 

Takayasu's Arteritis

Clinical features

  • Classically occurs in young Asian females
  • Systemic upset
  • Intermittent claudication - exertional leg pain
  • Angina

Examination findings

  • Unequal blood pressures in upper limbs of > 10mmHg
  • Early diastolic murmur - aortic regurgitation
  • Carotid bruit and carotidynia (neck tenderness on palpation of carotids)

Management

  • Steroids

 

Medium vessel vasculitis 

 

Polyarteritis Nodosa (PAN)

Pathophysiology

  • Necrotising inflammation of the 'medium' sized blood vessels, resulting in the formation of aneurysms 

Clinical Features

  • Classically a middle aged man with a history of Hepatitis B
  • Mononeuritis multiplex - painful, peripheral neuropathy affecting 2 or more peripheral nerves
  • Hypertension
  • Livedo reticularis
  • Haematuria
  • Acute renal failure

Investigations

  • pANCA (20% positive), Hepatitis B serology (30% positive)
  • Angiogram: beading, microaneurysms 

  

Kawasaki's Disease

Clinical features

Kawasaki’s affects children, usually less than 5 years of age.

  • Prolonged, unexplained fever (lasts at least 5 days), plus
  • 1 or more of the following:
    • Conjunctival injection
    • Red/cracked lips
    • Strawberry tongue
    • Cervical lymphadenopathy 
    • Redness and desquamation of the skin of palms and soles
    • Rash - varies between patients from morbilliform to maculopapular. 

Complications

  • Coronary artery aneurysm  

Investigations

  • Echocardiogram for ?coronary artery aneurysm

Management

  • High dose aspirin (risk of Reye's syndrome) and IV immunoglobulins

 

Buerger's (thromboangiitis obliterans)

Clinical Features

  • Classically, a smoker develops symptoms of limb ischaemia (ulcers, claudication)
  • Raynaud's 
  • Thrombophlebitis 

 

 

Small vessel vasculitis – ANCA associated Vasculitis

 

Granulomatosis with Polyangiitis 

Pathophysiology

  • An autoimmune disease resulting in necrotising, granulomatous inflammation of the vessels of the upper respiratory tract, lower respiratory tract and kidneys. 

Clinical Features

GPA affects the kidneys, upper and lower respiratory tracts. 

  • URT - sinusitis, epistaxis, nasal crusting, saddle nose
  • LRT: haemoptysis, dyspnoea
  • Renal: Rapidly progressive (crescentic) glomerulonephritis - Nephritic syndrome (HTN, haematuria, acute renal failure)
  • Vasculitis rash, proptosis, cranial nerve lesions 

Investigations

  • cANCA (PR3-ANCA) - 90% 
  • CXR: cavitations
  • Renal biopsy: Epithelial crescents affecting Bowman's capsule 

Management

  • Steroids
  • Cyclophosphamide 
  • Plasma exchange
  • Prognosis: survival 8-10 yrs 

   

Eosinophilic Granulomatosis with Polyangiitis 

Clinical Features

  • Sinusitis 
    • Headache, runny nose, nasal congestion, facial pain
  • Asthma like
    • Dyspnoea, wheeze
  • Mononeuritis multiplex - peripheral neuropathy affecting at least 2 different nerves of the peripheral nervous system
    • Burning pain, weakness, paraesthesia etc. 
  • Note: Symptoms can be worsened by leukotriene receptor antagonists (e.g. montelukast).  Questions may describe a patient with presumed asthma, who's condition deteriorates after GP introduces montelukast. 

Investigations

  • Bloods - eosinophilia
  • Antibodies: pANCA (MPO-ANCA) - 60%

 

Microscopic Polyangiitis 

Clinical Features

  • Fever
  • Systemic upset
  • Mononeuritis multiplex 
  • Renal impairment - raised creatinine, haematuria
  • Rash - palpable, purpuric rash

Investigations

  • pANCA (70%), cANCA (40%) 

  

 

Small vessel vasculitis – Immune complex vasculitis

 

Henoch-Schonlein Purpura

Pathophysiology

  • IgA mediated small vessel vasculitis

Clinical Features

  • The most common vasculitis of childhood
  • Age: Typically 2-10 years.
  • Often triggered by infection: 50% of patients have a recent URTI
  • Signs & symptoms:
    • Palpable purpuric rash (with oedema), affecting the extensors and buttocks 
    • Abdominal pain
    • Polyarthritis 
    • Nephritis due to IgA nephropathy  - AKI, haematuria, proteinuria

Management

  • Supportive, analgesia
  • Prognosis: Self-limiting. 1/3rd of patients will have relapse. 

 

Goodpasture’s Syndrome

Clinical Features

  •  Renal - Rapidly progressive (crescentic) glomerulonephritis - nephritic syndrome..  
    • Haematuria
    • Proteinuria - frothy urine, peripheral oedema
    • Acute renal failure
    • Hypertension
  • Pulmonary haemorrhage - haemoptysis, shortness of breath 
  • Systemic - fever, fatigue 

Investigations

  • Antibodies - anti-GBM antibodies (IgG antibodies vs type 4 collagen (alveolar, GBM) 
  • Renal biopsy - Linear deposits of IgG along the basement membrane

Management

  • Plasma exchange 
  • Steroids
  • Cyclophosphamide

 

Bechet’s disease – ulcers and uveitis 

Pathophysiology

  • Autoimmune mediated inflammation of the arteries and veins

Clinical Features

Commonly affects patients from middle-east (Turkey especially) with a positive family history, classic triad:

1. Oral ulcers

2. Genital ulcers (not usually on the penis)

3. anterior uveitis - pain, redness, photophobia, impaired vision

Other features include thrombophlebitis, deep vein thrombosis, arthritis