Picture Quiz
A 78 year old has swinging pyrexia. He
has had a laparoscopic cholecystectomy
complicated by gastrointestinal perforation and postoperative multiorgan failure
two weeks previously. On examination his
temperature was 39°C, pulse 100/min and
irregularly irregular, blood pressure
120/80 mm Hg. He has an impaired percussion note and bronchial breathing at
the right base.
Questions
(1) What does the photograph show ?
(2) Explain the patient's physical signs.
(3) List three important investigations in this case.
(4) What is the most likely cause of the sign shown?
(5) List three other causes of this sign.
Answers
(1) Vasculitic rash affecting both legs.
(2) His respiratory signs are due to consolidation secondary to right lower lobe
pneumonia. He is in atrial fibrillation,
which commonly occurs secondary to
pneumonia in this age group.
(3) Arterial blood gases, urea and electrolytes, and blood cultures. Hypoxia and
renal impairment are poor prognostic features of pneumonia. He will also require a
chest x ray examination, a full blood count, and sputum culture.
(4) His vasculitis is most likely secondary to septicaemia from his pneumonia, the
most likely organism being Streptococcus pneumoniae.
(5) Rheumatoid disease, polyarteritis nodosa, infective endocarditis.
Discussion
Vasculitis is defined as inflammation of the
vessel wall, which is usually immunologically mediated. Several classifications exist,
some based on the size of the affected
vessels. We have divided causes into infective and non-infective.
Infective causes
- Subacute bacterial endocarditis is well
known to cause vasculitis. Small petechial
haemorrhages appear at the mucosa of the
pharynx and conjunctiva. They can sometimes be seen in the retinas (Roth spots)
or at the thenar and hypothenar eminences (Janeway lesions). Embolic lesions,
such as hard, tender swellings, occur in fingers, toes, palms, and soles (Osler's nodes).
The rash shown can also be a manifestation of subacute bacterial endocarditis.
- Other infection Almost any severe infection/septicaemic illness can cause a vasculitic rash.
Non-infective causes
- Rheumatoid arthritis is a common
cause of systemic vasculitis. If affecting small
vessels it can cause a widespread cutaneous
vasculitis with necrosis of the skin or nail fold
infarcts. Involvement of medium sized vessels can result in mononeuritis multiplex
(vasculitis of the vasa nervorum), bowel
infarction due to necrotising vasculitis of the
mesenteric vessels, endocarditis, myocardial,
or pericardial involvement.
- Systemic lupus erythematosus is characterised by widespread vasculitis of the capillaries, arterioles, and venules. Arthralgia and rashes are the most common features, but
cerebral and renal disease are the most serious problems. B-cell activation results in
increased autoantibody production to a variety of antigens. Circulating immune complexes are deposited in the tissues causing
vasculitis. Serum antinuclear antibodies are
positive in almost all cases but double
stranded DNA binding is specific, although
only present in half the cases. Treatment is
with non-steroidal anti-inflammatory drugs,
antimalarial drugs (chloroquine), corticosteroids, and immunosuppression in more
severe disease.
- Drugs such as many antibiotics, hydralazine, methyldopa, isoniazid, and penicillamine are common causes of vasculitis.
- Polymyalgia rheumatica (PMR) and Giant cell (temporal) arteritis (GCA) are both
inflammatory systemic illnesses of the elderly affecting large vessels. PMR causes shoulder and neck stiffness and pain typically worse in the morning. GCA is characterised
by unilateral headaches, scalp tenderness, jaw
claudication, and the danger of permanent
visual loss due to involvement of the retinal
artery. Investigations show a raised erythrocyte sedimentation rate (50-100 mm/hr) and
C-reactive protein, abnormal liver biochemistry, and a mild normochromic normocytic
anaemia. Diagnosis can be confirmed by
temporal artery biopsy, which shows giant
cells in the internal elastic lamina, but the
biopsy may be normal. Treatment is with
high dose corticosteroids.
- Behcet's disease is a systemic vasculitis
of unknown cause with a typical
geographical distribution in Turkey, Iran,
and Japan. Oral and genital ulcers as well as
uveitis and cutaneous lesions are characteristic. Other manifestations include a self limiting arthritis, gastrointestinal symptoms, pulmonary, and renal and cerebral lesions.
Treatment is with immunosuppression.
- Other causes mainly affecting small
vessels can be divided into those positive or
negative for antineutrophilic cytoplasmic
antibodies (ANCA). There is correlation
between ANCA titres and disease activity.
ANCA-positive vasculitis:
Wegener's granulomatosis involves mainly the upper respiratory tract, lungs, and
kidneys. It responds well to treatment with
cyclophosphamide and corticosteroids.
Churg-Strauss syndrome is characterised by eosinophilic infiltration of the
lungs, peripheral nerves, and skin. It
responds well to corticosteroids.
Microscopic vasculitis (polyangiitis)
results in haemoptysis and involves usually the lungs and kidneys.
Non-ANCA-positive small vessel vasculitis:
Henoch-Schoenlein-purpura is a common
systemic vasculitis in children. It occurs after
upper respiratory tract infection. There is
IgA immune complex deposition in the
small vessels and it usually manifests as purpura, a transient polyarthritis, abdominal
pain, and glomerulonephritis. It is usually
self limiting but can recur.
Sylvia Feyler, senior house officer, Debra King consultant physician in medicine for the elderly, Wirral Hospital, Merseyside
Email: sylviafeyler@hotmail.com
Debra King, consultant physician in medicine for the elderly, Wirral Hospital, Merseyside
studentBMJ 2001;09:1-42 February ISSN 0966-6494
