Page 385 - Medicine and Surgery

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Chapter 8: Vasculitis 381

Buerger’s disease Aetiology
The aetiology is unknown but several factors have been
Definition
implicated including upper respiratory tract infection
Buerger’s disease or thrombangitis obliterans is an in-
especially by Streptococcus.Serum concentrations of IgA
flammatory occlusion of small and medium-sized pe-
are raised in approximately half of patients and IgA-
ripheral arteries and veins of the upper and lower limbs.
containing immune complexes have been identified.
Clusters of cases have been noted with no obvious pre-
Aetiology/pathophysiology cipitant.
It occurs almost exclusively in heavy cigarette smokers
and is therefore seen more in countries with high levels
Pathophysiology
of smoking. There is segmental chronic inflammatory
The condition results from inflammation within the
infiltration of the vessel walls with resultant obliteration
walls of small blood vessels, predominantly capillaries
of the lumen and secondary thrombosis.
but small arterioles and venules are also affected. The
inflammation is thought to be the result of a hypersen-
Clinical features sitivity reaction (type III immune complex mediated).
The condition starts with digital ischaemia, ulceration The inflammation of the vessels increases permeability
preceded by claudication in the feet, or rest pain in the resulting in a leaking of fluid and cells from the circula-
fingers or toes. The condition is progressive. Wrist and tion into the surrounding tissue. IgA deposition within
ankle pulses are usually absent but brachial and popliteal the glomeruli of the kidney causes a focal segmental pat-
pulses are present. There may be a previous history of tern of glomerulonephritis with a resultant proliferation
superficial thrombophlebitis. of mesangial cells and a nephritic syndrome.

Investigations Clinical features
Arteriography shows narrowing or occlusion of small This multisystem disorder may occur with simultaneous
peripheral arteries with healthy main vessels. or sequential manifestations:
The characteristic presentation is with the skin lesion.

It is unclear whether other lesions can occur without
Management
the rash. The rash characteristically affects the lower
The condition remits with quitting smoking; nicotine
limbs and buttocks, but is not always confined to these
replacement therapy cannot be used but bupropion
areas. The rash is initially blanching but becomes pur-
(Zyban) is safe. Prostaglandin infusions, thrombolytic
puricandthengoesthroughtheclassiccolourchanges
therapy, surgical sympathectomy and revascularistion
of a bruise, lesions of varying ages are present at one
procedures have been tried.
time. Oedema of the face, dorsum of the hands and
feet, perineum or foreskin may occur especially in
young children
Henoch–Sch ¨ onlein Purpura
Aself-limiting acute arthritis of large distal joints oc-
Definition curs without articular damage with the patient com-
A syndrome resulting from a vasculitis of small blood plaining of swollen, tender painful joints exacerbated
vessels. with movement.
The gastrointestinal manifestations present with col-

icky pain, which may be severe, and associated with
Age
vomiting.Bleedingfromtheaffectedvesselsmaycause
Peak 2–8 years, young adults.
melaena or haematemesis.
Renal involvement is common with haematuria and

Sex proteinuria detectable but is generally not severe in
2M : 1F children.