Vasculitides

Question 1

Define vasculitides

Answer 1

Vasculitis is the autoimmune inflammation and necrosis of blood vessels. Primary vasculitides are classified according to the main vessel size affected

Question 2

What are the causes/risk factors of vasculatides?

Answer 2

Unknown. Postulated to be of autoimmune origin. Immune complex deposition in vessel walls triggers classical complement activation and inflammation

Risk factors:
• Systemic vasculitis is more common in those above 50 and in Caucasians.
- Polyarteritis Nodosa is associated with HBV (Hepatitis B).
- Mixed Essential Cryoglobulinaemic Vasculitis is associated with HCV (Hepatitis C).
- GCA is associated with PMR (Polymyalgia Rheumatica).
- Microscopic Polyangiitis is associated with presence of pANCA.
- Wegener’s Granulomatosis (Granulomatosis with polyangiitis) is associated with presence of c-ANCA.

Question 3

What are the signs and symptoms of vasculatides?

Answer 3

Possible features of all diseases:
• General: FLAWS
• 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:
• Takayasu’s Aortitis: Constitutional upset, head or neck pain, tenderness over affected arteries (aorta and the major branches), dizziness, fainting, reduced peripheral pulses, hypertension. There may be arm claudication, especially on repetitive motions.
• 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, lymphadenopathy, coronary artery aneurysm.
• Churg-Strauss Syndrome (eosinophilic granulomatosis): Refractory asthma, eosinophilia.
• Henoch-Schönlein Purpura : 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 respiratory tract, nasal discharge, ulceration and deformity, haemoptysis, sinusitis, corneal thinning, glomerulonephritis, saddle nose
• Relapsing Polychondritis: 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

What investigations are carried out for vasculatiedes?

Answer 4

• FBC - normocytic anaemia (ACD), thrombocytosis and Neutrophilia.
• ESR/CRP - elevated
• Autoantibodies -
c-ANCA: Anti-proteinase 3, associated with WG.
- p-ANCA: Anti-myeloperoxidase seen in MP and CSS but also IBD, PBC and chronic active hepatitis.
- ANA, anti-dsDNA may suggest SLE.
- Rheumatoid factor: Positive in 50%.
- Cryoglobulins (immunoglobulins and complement components that precipitate at temperatures <37 0C).
• Urinalysis - haematuria, proteinuria. Red cell casts
• CXR - diffuse, nodular or flitting shadows. Atelectasia, cavitating lesions (wegener’s)
• Biopsy - renal, lung (transbronchial), temporal artery (in GCA).
• Angiography - to identify aneurysms (in PAN).