Page 384 - Medicine and Surgery

P1: KTX
BLUK007-08 BLUK007-Kendall May 12, 2005 19:48 Char Count= 0

380 Chapter 8: Musculoskeletal system

Age Churg–Strauss syndrome
Middle age (allergic granulomatosis)
Definition
Sex Multisystem disorder consisting of asthma and rhinitis,
4M:1F eosinophilia, eosinophilic infiltration, small vessel vas-
culitis and extravascular granulomas.
Aetiology/pathophysiology
Associated with hepatitis B infection in 10–20% be-
Age
cause of hepatitis B surface antigen immune complexes.
Peak incidence 30–40 years.
Also associated with hairy cell leukaemia. Transmural
neutrophil infiltration of medium-sized arteries occurs,
causing degeneration, weakness and microaneurysm
Sex
formation. Veins are also affected and the condition may
M = F
result in thrombosis and tissue infarction.

Clinical features Aetiology/pathophysiology
Polyarteritis nodosa is usually an acute illness charac- It is thought to be an autoimmune disorder.
terised by calf pain, general malaise, myalgia and weight
loss for a few days prior to visceral manifestations:
Musculocutaneous: Migratory arthralgia or arthritis,
Clinical features
Prodromal disorder of allergic rhinitis and asthma.
purpura and subcutaneous haemorrhage.
Peripheral blood eosinophilia and eosinophilic infil-
Cardiovascular system: Coronary arteritis leading to

tration of the lung and gastrointestinal tract.
cardiac arrhythmias, failure and myocardial infarc-
Systemic vasculitis of the medium and small ves-
tion.
sels with vascular and extravascular granulomatosis.
Gastrointestinal system: Mesenteric arteritis causing

Affected organs include the skin with subcutaneous
pain, haemorrhage and mucosal ulceration.
nodules, purpura and haemorrhages; heart with peri-
Genitourinary system: Haematuria, proteinuria, hy-

carditis, cardiac failure and myocardial infarction;
pertension, renal infarction and renal failure.
Nervous system: Polyneuropathy (mononeuritis mul-
neurological system with a mononeuritis multiplex;
tiplex) with motor and sensory deficits. kidney with a focal segmental glomerulonephritis;
gastrointestinal system with an eosinophilic gastroen-
teritis and musculoskeletal system causing myalgia
Investigations and migratory polyarthritis.
Raised ESR and white blood count. Patients are usually
p-ANCApositive.Angiographyrevealsmultiplemicroa-
neurysmsoftherenalorintestinalvessels.Diagnosismay Investigations
be made on biopsy of affected organs. Chest X-ray may show patchy pneumonia-like shadow-
ing,whichcanbefleeting.TheESRisraisedandthereare
raisedlevelsofimmunoglobulins.Anti-myeloperoxidase
Management
anti-neutrophilcytoplasmicantibodies(pANCA)ispos-
Corticosteroids and immunosuppressive agents (e.g. cy-
itive in 70%. The diagnosis can be confirmed on biopsy
clophosphamide) in severe acute cases.
of affected organs.
Wegener’s granulomatosis
Management
See page 124 in Chapter 3 (Respiratory System). Treatment involves steroids and azathiporine.