Page 386 - Medicine and Surgery

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382 Chapter 8: Musculoskeletal system

Rarely there may be CNS involvement with convul- Age

sions, paresis and coma. Most common in 15–30 years.
Microscopy Sex
There is an inflammatory infiltrate in the upper dermis, F > M
joint lining and GI mucosa and glomerular mesangium
with neutrophils and extravasated red blood cells.
Clinical features
Symptoms are symmetrical with fingers affected more
Complications
than toes, it usually begins in a single digit and then
Gastrointestinal complications include infarction and
becomes more generalised. There is initial skin pallor
intussusception as a result of oedema. Renal failure may
due to vasoconstriction progressing through cyanosis to
occur in the acute phase, or may progress over many
hyperaemia (white to blue to red). There may be tingling
years.
or pain in the affected digits especially with restoration
of blood supply during rewarming.
Investigations
The diagnosis is clinical; erythrocyte sedimentation rate,
Investigations
white cell count and eosinophils may be raised. Urine
Primary Raynaud’s phenomenon must be distinguished
microscopy should be performed looking for red and
fromRaynaud’ssyndromeoccurringwithconnectivetis-
white blood cells, casts and protein. Faecal occult blood
sue disorders such as systemic sclerosis.
may be positive without overt bleeding.
Management Management
No specific treatment is used. Symptomatic relief of joint Often avoidance of cold is all that is required. In more
pain and rash may be achieved with nonsteroidal anti- severe cases calcium channel blockers such as nifedipine
inflammatory drugs. Gastrointestinal bleeding and CNS are used. In severe cases prostacyclin infusions may be
manifestations may be improved with the prompt use of required.
steroids. Complications such as acute renal failure and
intussusception should be managed promptly.
Behc¸et’s syndrome
Prognosis Definition
Inmostcasestheoverallprognosisisexcellent,thecourse Achronic,relapsingmultisystemvasculitischaracterised
is variable with cases lasting between a few days and a by oral ulceration.
few weeks. Rarely it may continue for up to a year and
there may be a course of relapse and remission. Severe Incidence
renal involvement and CNS involvement may be life- Rare.
threatening.
Age
Raynaud’s disease and phenomenon Commonest in 20–40 years.
Definition
An exaggerated vascular response to cold, causing a Sex
spasm of the arteries supplying the fingers and toes. M > F

Prevalence Geography
Five to ten per cent of young women in temperate cli- Much more common in Turkey, Iran, China, Korea and
mates. Japan.