vasculitides (systemic vasculitis)

Question 1

definition

Answer 1

In its strictest sense, the term vasculitis denotes inflammation of a blood vessel, which is characterised by the presence of an inflammatory infiltrate and destruction of the vessel wall. Commonly, however, vasculitis refers to the systemic vasculitides, which are autoimmune disorders characterised by inflammation of blood vessels. The systemic vasculitides are a diverse group of disorders that demonstrate a wide range of organ involvement and clinical severity.

Question 2

investigations

Answer 2

1st investigations:

• ESR, CRP
• anti-neutrophil cytoplasmic auto-antibodies (ANCA)
• serum urea and creatinine (check for glumerulonephritis)
• urinalysis (check for glumerulonephritis)
• biopsy of affected tissue

Question 3

symptoms

Answer 3

The large vessel vasculitides have classical clinical patterns resulting from the vessels affected.
Medium and small vessel vasculitides are characterized by multiorgan involvement with less specific clinical features.

Possible features of all diseases:
General: Fever, night sweats, malaise, weight loss.
Skin: Rash (vasculitic, purpuric, maculopapular, livedo reticularis).
Joint: Arthralgia or arthritis.
GI: Abdominal pain, haemorrhage from mucosal ulceration, diarrhoea.
Kidney: Glomerulonephritis, renal failure.
Lung: Dyspnoea, cough, chest pain, haemoptysis, lung haemorrhage.
CVS: Pericarditis, coronary arteritis, myocarditis, heart failure, arrhythmias.
CNS: Mononeuritis multiplex, infarctions, meningeal involvement.
Eyes: Retinal haemorrhage, cotton wool spots

Features characteristic of specific subtypes:
GCA: (see giant cell arteritis).
TA: Constitutional upset, head or neck pain, tenderness over affected arteries (aorta and the
major branches), dizziness, fainting, reduced peripheral pulses, hypertension.
PAN: Microaneurysms, thrombosis, infarctions (e.g. causing GI perforations), hypertension,
testicular pain.
KD: Age <5 years, fever of >5 days, fissured lips, red swollen palms and soles followed by
desquamation, skin rash, inflamed oral cavity, conjunctival congestion, lymphadenop-
athy, coronary artery aneurysm.
CSS: Asthma, eosinophilia.
HSP: Purpura (leg and buttocks), arthritis, gut symptoms, glomerulonephritis with IgA deposition.
MP: Non-specific with multiple organs affected. Glomerulonephritis with no glomerular Ig
deposits.
WG: Granulomatous vasculitis of upper and lower repiratory tract, nasal discharge, ulceration
and deformity, haemoptysis, sinusitis, corneal thinning, glomerulonephritis.
RP: Affecting cartilage (e.g. ear pinna, nose, larynx) causing swelling, hoarse voice, tenderness, cartilage destruction and deformity (e.g. saddle nose).
MEC: Arthritis, splenomegaly, skin vasculitis, renal disease, cryoglobulins (IgG and IgM mix).

Question 4

types of primary vasculitis

Answer 4

Primary vasculitides are classified according to the main vessel size affected.
Large: Giant cell arteritis (GCA), Takayasu’s aortitis (TA).
Medium: Polyarteritis nodosa (PAN), Kawasaki’s disease (KD).
Small: Churg–Strauss syndrome (CSS), microscopic polyangiitis (MP), Henoch–Schonlein
purpura (HSP), Wegener’s granulomatosis (WG), mixed essential cryoglobulinaemia (MEC), relapsing polychondritis (RP).