Thromboangiitis obliterans

en.wikipedia.org /wiki/Thromboangiitis_obliterans

Thromboangiitis Obliterans

Synonyms Buerger disease, Buerger's disease, Winiwarter-Buerger disease, presenile gangrene [1]

Complete occlusion of the right and stenosis of the left femoral artery as seen in a case of thromboangiitis
obliterans

Classification and external resources

Specialty cardiology

ICD-10 I73.1

ICD-9-CM 443.1

OMIM 211480

DiseasesDB 1762

MedlinePlus 000172
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 eMedicine med/253

Patient UK Thromboangiitis obliterans

MeSH C14.907.137.870

Not to be confused with Berger's disease ( IgA nephropathy )

Thromboangiitis obliterans, also known as Buerger disease (English , German /byrgr/), is a recurring
progressive inflammation and thrombosis (clotting) of small and medium arteries and veins of the hands and feet. It
is strongly associated with use of tobacco products, [2] primarily from smoking, but is also associated with smokeless
tobacco.[3][4]

Signs and symptoms

There is a recurrent acute and chronic inflammation and thrombosis of arteries and veins of the hands and feet. The
main symptom is pain in the affected areas, at rest and while walking ( claudication). [1] The impaired circulation
increases sensitivity to cold. Peripheral pulses are diminished or absent. There are color changes in the extremities.
The colour may range from cyanotic blue to reddish blue. Skin becomes thin and shiny. Hair growth is reduced.
Ulcerations and gangrene in the extremities are common complications, often resulting in the need for amputation of
the involved extremity.[5]

Pathophysiology
There are characteristic pathologic findings of acute inflammation and thrombosis (clotting) of arteries and veins of
the hands and feet (the lower limbs being more common). The mechanisms underlying Buerger's disease are still
largely unknown, but smoking and tobacco consumption are major factors associated with it. It has been suggested
that the tobacco may trigger an immune response in susceptible persons or it may unmask a clotting defect, either
of which could incite an inflammatory reaction of the vessel wall.[6] This eventually leads to vasculitis and ischemic
changes in distal parts of limbs.

A possible role for Rickettsia in this disease has been proposed. [7]

Diagnosis
A concrete diagnosis of thromboangiitis obliterans is often difficult as it relies heavily on exclusion of other
conditions. The commonly followed diagnostic criteria are outlined below although the criteria tend to differ slightly
from author to author. Olin (2000) proposes the following criteria:[8]

1. Typically between 2040 years old and male, although recently females have been diagnosed.[9]
2. Current (or recent) history of tobacco use.
3. Presence of distal extremity ischemia (indicated by claudication, pain at rest, ischemic ulcers or gangrene)
documented by noninvasive vascular testing such as ultrasound.
4. Exclusion of other autoimmune diseases, hypercoagulable states, and diabetes mellitus by laboratory tests.
5. Exclusion of a proximal source of emboli by echocardiography and arteriography.
6. Consistent arteriographic findings in the clinically involved and noninvolved limbs.

Buergers disease can be mimicked by a wide variety of other diseases that cause diminished blood flow to the
extremities. These other disorders must be ruled out with an aggressive evaluation, because their treatments differ
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substantially from that of Buergers disease, for which there is no treatment known to be effective.

Diseases with which Buergers disease may be confused include atherosclerosis (build-up of cholesterol plaques in
the arteries), endocarditis (an infection of the lining of the heart), other types of vasculitis, severe Raynaud's
phenomenon associated with connective tissue disorders (e.g., lupus or scleroderma), clotting disorders of the
blood, and others.

Angiograms of the upper and lower extremities can be helpful in making

the diagnosis of Buergers disease. In the proper clinical setting, certain
angiographic findings are diagnostic of Buergers. These findings include
a corkscrew appearance of arteries that result from vascular damage,
particularly the arteries in the region of the wrists and ankles. Collateral
circulation gives "tree root" or "spider leg" appearance.[1] Angiograms
may also show occlusions (blockages) or stenosis (narrowings) in
multiple areas of both the arms and legs. Distal plethysmography also
yields useful information about circulatory status in digits. To rule out
other forms of vasculitis (by excluding involvement of vascular regions
atypical for Buergers), it is sometimes necessary to perform angiograms
of other body regions (e.g., a mesenteric angiogram).

Skin biopsies of affected extremities are rarely performed because of the

frequent concern that a biopsy site near an area poorly perfused with
CT angiogram showing segmental stenosis of
blood will not heal well. arteries of the lower leg (indicated by arrows). The
changes are particularly apparent in the blood
vessels in the lower right hand portion of the
Treatment picture (the femoral artery distribution).

Smoking cessation has been shown to slow the progression of the

disease and decrease the severity of amputation in most patients, but does not halt the progression.

In acute cases, drugs and procedures which cause vasodilation are

effective in reducing pain experienced by patient. For example,
prostaglandins like Limaprost[10] are vasodilators and give relief of pain,
but do not help in changing the course of disease. Epidural anesthesia
and hyperbaric oxygen therapy also have vasodilator effect. [1]

In chronic cases, lumbar sympathectomy may be occasionally helpful.[11]

It reduces vasoconstriction and increases blood flow to limb. It aids in
healing and giving relief from pain of ischemic ulcers.[1] Bypass can
sometimes be helpful in treating limbs with poor perfusion secondary to
this disease. Use of vascular growth factor and stem cell injections have Treatment by 100% hyperbaric oxygen.

been showing promise in clinical studies. Debridement is done in

necrotic ulcers. In gangrenous digits, amputation is frequently required.
Above-knee and below-knee amputation is rarely required.[1]

Streptokinase has been proposed as adjuvant therapy in some cases. [12]

Despite the clear presence of inflammation in this disorder, anti-inflammatory agents such as corticosteroids have
not been shown to be beneficial in healing, but do have significant anti-inflammatory and pain relief qualities in low
dosage intermittent form. Similarly, strategies of anticoagulation have not proven effective. physical therapy:
interferential current therapy to decrease inflammation

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Prognosis
Buerger's is not immediately fatal. Amputation is common and major amputations (of limbs rather than fingers/toes)
are almost twice as common in patients who continue to smoke. Prognosis markedly improves if a person quits
smoking. Female patients tend to show much higher longevity rates than men. The only known way to slow the
progression of the disease is to abstain from all tobacco products.

Prevention
Further information: Thrombosis prophylaxis

The cause of the disease is thought to be autoimmune in nature and heavily linked to tobacco use in patients with
Buerger's as primary disease.

Epidemiology
Buerger's is more common among men than women. Although present worldwide, it is more prevalent in the Middle
East and Far East[13] Incidence of thromboangiitis obliterans is 8 to 12 per 100,000 adults in the United States
(0.75% of all patients with peripheral vascular disease). [13]

History

Buerger's disease was first reported by Felix von Winiwarter in 1879 in Austria.[14] It was not until 1908, however,
that the disease was given its first accurate pathological description, by Leo Buerger at Mount Sinai Hospital in New
York City.[15] Buerger called it "presenile spontaneous gangrene" after studying amputations in 11 patients.

Notable sufferers
As reported by Alan Michie in God Save The Queen, published in 1952 (see pages 194 and following), King George
VI was diagnosed with the disease on 12 November 1948. Both legs were affected, the right more seriously than the
left. The King's doctors prescribed complete rest and electric treatment to stimulate circulation, but as they were
either unaware of the connection between the disease and smoking (the King was a heavy smoker) or unable to
persuade the King to stop smoking, the disease failed to respond to their treatment. On 12 March 1949, the King
underwent a lumbar sympathectomy, performed at Buckingham Palace by Dr. James R. Learmonth. The operation,
as such, was successful, but the King was warned that it was a palliative, not a cure, and that there could be no
assurance that the disease would not grow worse. From all accounts, the King continued to smoke.

The author and journalist John McBeth describes his experiences of the disease, and treatment for it, in a chapter
called 'Year of the Leg' in his book entitled Reporter. Forty Years Covering Asia .

Philippine president Rodrigo Duterte has personally disclosed that he suffers from Buergers disease. [17]

References

1. ^ Jump up to: a b c d e f Ferri, Fred F. (2003). Ferri's Clinical Advisor 2004: Instant Diagnosis and Treatment.
6th edition. p. 840. ISBN 0323026680.
2. Jump up ^ Joyce JW (1990). "Buerger's disease (thromboangiitis obliterans)". Rheum Dis Clin North Am. 16
(2): 46370. PMID 2189162.
3. Jump up ^ Overview of Buerger's disease, Mayo Clinic, by Mayo Clinic Staff, retrieved February 13, 2016

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 4. Jump up ^ Thromboangiitis obliterans, Medline Plus, U.S. National Library of Medicine, retrieved February
13, 2016
5. Jump up ^ Porth, Carol (2007). Essentials of Pathophysiology: Concepts of Altered Health States, 2nd ed .
Lippincott Williams&Wilkins. p. 264. ISBN 9780781770873.
6. Jump up ^ Tanaka K (1998). "Pathology and pathogenesis of Buerger's disease.". Int J Cardiol. 66: S237
42. PMID 9951825. doi:10.1016/s0167-5273(98)00174-0.
7. Jump up ^ Fazeli B, Ravari H, Farzadnia M (July 2011). "Does a species of Rickettsia play a role in the
pathophysiology of Buerger's disease?". Vascular. 20: 334336. PMID 21803838.
doi:10.1258/vasc.2011.cr0271.
8. Jump up ^ Olin JW (September 2000). "Thromboangiitis obliterans (Buerger's disease)". N Engl J Med. 343
(12): 8649. PMID 10995867. doi:10.1056/NEJM200009213431207.
9. Jump up ^ Atlas of Clinical Diagnosis 2e. Elsevier Health Sciences. 2003. p. 238. ISBN 9780702026683.
10. Jump up ^ Matsudaira K, Seichi A, Kunogi J, et al. (January 2009). "The efficacy of prostaglandin E1
derivative in patients with lumbar spinal stenosis". Spine. 34 (2): 11520. PMID 19112336.
doi:10.1097/BRS.0b013e31818f924d.
11. Jump up ^ Clinical Surgery, 2e. John Wiley & Sons. 2012. ISBN 9781118343951.
12. Jump up ^ Hussein EA, el Dorri A (1993). "Intra-arterial streptokinase as adjuvant therapy for complicated
Buerger's disease: early trials". International surgery. 78 (1): 548. PMID 8473086.

13. ^ Jump up to: a b Piazza, Gregory; Creager, Mark A. (2010-04-27). "Thromboangiitis Obliterans". Circulation.
121 (16): 18581861. ISSN 0009-7322. PMC 2880529 . PMID 20421527.
doi:10.1161/CIRCULATIONAHA.110.942383.
14. Jump up ^ von Winiwarter F (1879). "Ueber eine eigenthumliche Form von Endarteriitis und Endophlebitis
mit Gangran des Fusses". Arch Klin Chir. 23: 20226.
15. Jump up ^ Buerger L (1908). "Thrombo-angiitis obliterans: a study of the vascular lesions leading to
presenile spontaneous gangrene" (PDF). Am J Med Sci. 136: 56780. doi:10.1097/00000441-190810000-
00011.
16. Jump up ^ Frialde, Mike (December 10, 2015). "Duterte: I may not last 6 years in office". The Philippine
Star. Retrieved December 17, 2015.

Notes
McBeth, J. (2011). Reporter. Forty Years Covering Asia . Singapore: Talisman Publishing.
ISBN 9789810873646.

External links
00394 at CHORUS

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