د. حسين محمد جمعه
اختصاصي الامراض الباطنةالبورد العربي
كلية طب الموصل
2011
Ischaemia of the extremities in a smoker
BMJ December 2011)A 51 year old man presented with a six week history of pain in his right hand, which was associated with blue discoloration and a discharge of pus from the tip of his index finger. He was a lifelong smoker but had no other cardiovascular risk factors.
He had a history of Raynaud’s phenomenon affecting both hands and claudication of both feet.
His blood pressure was 120/80 mm Hg and his cardiovascular examination was normal. No carotid, subclavian, or femoral bruits were heard. Capillary refill was prolonged at six seconds.
The right hand digits were cyanosed, with wet gangrene in the distal right index finger. Brachial pulses were palpable but radial and ulnar pulses were absent bilaterally. Femoral and popliteal
pulses were palpable but all foot pulses were absent. Allen’s test was positive.
Laboratory investigations showed sodium 141 mmol/L
(reference range 135-145), potassium 5.6 mmol/L (3.5-5.0),urea 6.5 mmol/L (2.5-6.7), creatinine 80 mmol/L (70-120),glucose 6 mmol/L (3-7.8), C reactive protein 16 mg/L (0-5),white blood cell count 12.4×10
/L (4-10), and neutrophils9.1×10
/L (1.8-7.5). His autoimmune profile was negative and
his hypercoagulability screen was normal.
Electrocardiography showed a normal sinus rhythm. On angiographic assessment,he had normal proximal vessels but distal small vessel disease and medium vessel disease bilaterally; he also had segmental and distal occlusion of the radial and ulnar arteries and the posterior tibial arteries bilaterally.
On ultrasound examination the vessels were dilated, with a halo of inflammatory tissue around the thrombosed artery centre.
Questions
1. What disease best describes this patient’s presentation?2 .How is the diagnosis made?
3 .What single management provides the best outcome and
reduces the need for amputation?
Answers
1 . Short answer
Buerger’s disease (thromboangiitis obliterans).
Long answer
Buerger’s disease (thromboangiitis obliterans) is aninflammatory, non-atherosclerotic, occlusive vasculitis of small sized and medium sized arteries and veins. The disease affects distal vessels of the extremities and patients are typically young, predominately male, tobacco smokers. The causes are unknown,
but it is thought to be triggered by hypersensitivity to constituents of tobacco.
A positive Allen’stest, which indicates arterial disease, is often present; this, in combination with the
clinical picture of a young male smoker, is strongly suggestive of Buerger’s disease. However, the test is non-specific, and an abnormal result can also be seen in other types of small vessel occlusive disease such as scleroderma, CREST syndrome (calcinosis, Raynaud’s syndrome, oesophageal dysmotility,
sclerodactyly, and telangiectasia), repetitive trauma,
hypercoagulable states, and vasculitis.
2 .How is the diagnosis made?
Short answerBuerger’s disease is a clinical diagnosis of exclusion. After excluding autoimmune disease, thrombophilia, diabetes, and aproximal embolic source, several criteria can help make adiagnosis. These include onset before the age of 50 years, history of tobacco use, ischaemia of the distal extremities, and arteriographic findings typical of Buerger’s disease.
Long answer
The provisional diagnosis of Buerger’s disease is based on five clinical criteria :• Onset before the age of 50 years: Despite the index case describing a presentation of Buerger’s disease in a 51 year old patient, the disease typically presents in patients under 50 years
• History of tobacco use: Tobacco is an important cause of Buerger’s disease and a factor in its progression.
• Ischaemia of the distal extremities:
Patients most often present with symptoms related to ischaemia of the digits.
Patients usually note pain and subsequent discoloration of the digits, which can progress to ischaemic ulceration in the upper or lower extremities with accompanying pain while resting or gangrene. The anterior (41%) or posterior (40%) tibial arteries in the legs and the ulnar artery (11.5%) in the arms are most commonly affected.
