Thromboangiitis obliterans

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"Buerger disease" redirects here. Not to be confused with
Berger's disease
.
Thromboangiitis obliterans
Other namesBuerger 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
SpecialtyCardiology, rheumatology Edit this on Wikidata

Thromboangiitis obliterans, also known as Buerger disease (English /ˈbɜːrɡər/; German: [ˈbʏʁɡɐ]) or Winiwarter-Buerger disease, 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

distal parts of limbs.[citation needed
]

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 20 and 40 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.

Buerger's 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 substantially from that of Buerger's disease, for which there is no treatment known to be effective.[citation needed]

Some diseases with which Buerger's disease may be confused include

Raynaud's phenomenon associated with connective tissue disorders (e.g., lupus or scleroderma), clotting disorders or the production of clots in the blood.[citation needed
]

Angiograms of the upper and lower extremities can be helpful in making the diagnosis of Buerger's disease. In the proper clinical setting, certain angiographic findings are diagnostic of Buerger's. 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 Buerger's), it is sometimes necessary to perform angiograms of other body regions (e.g., a mesenteric angiogram).[citation needed]

Skin biopsies of affected extremities are rarely performed because of the frequent concern that a biopsy site near an area poorly perfused with blood will not heal well.[citation needed]

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.[clarification needed]

Treatment

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.[citation needed]

alprostadil (prostaglandin analogue) for relieving pain and healing ulcers.[11]

In

above-knee amputation is rarely required.[1]

Streptokinase has been proposed as adjuvant therapy in some cases.[14]

Despite the clear presence of inflammation in this disorder,

anticoagulation
have not proven effective. physical therapy: interferential current therapy to decrease inflammation.[citation needed]

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.[citation needed]

Epidemiology

Buerger's is more common among men than women. Although present worldwide, it is more prevalent in the Middle East and Far East.

peripheral vascular disease).[15]

History

Buerger's disease was first described by

Mount Sinai Hospital in New York City, who referred to the condition as "presenile spontaneous gangrene".[17]

Notable people affected

As reported by Alan Michie in God Save the Queen, published in 1952 (see pages 194 and following), King

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.[citation needed
]

The author and journalist John McBeth describes his experiences of the disease, and treatment for it, in the chapter "Year of the Leg" in his book Reporter: Forty Years Covering Asia.[18]

Philippine president Rodrigo Duterte disclosed in 2015 that he has Buerger's disease.[19]

References

  1. ^
    ISBN 978-0323026680
    .
  2. PMID 2189162
    .
  3. ^ Mayo Clinic Staff. "Overview of Buerger's disease". Mayo Clinic. Retrieved 13 February 2016.
  4. ^ "Thromboangiitis obliterans". Medline Plus. U.S. National Library of Medicine. Retrieved 13 February 2016.
  5. ISBN 9780781770873
    .
  6. PMID 9951825
    .
  7. S2CID 22660338
    .
  8. PMID 10995867
    .
  9. ISBN 9780702026683
    .
  10. S2CID 22190177
    .
  11. ^
    PMID 32364620
    .
  12. ISBN 978111834395-1
    .
  13. PMID 30378681
    .
  14. PMID 8473086
    .
  15. ^
    PMID 20421527
    .
  16. ^ v. Winiwarter F (1879). "Ueber eine eigenthümliche Form von Endarteriitis und Endophlebitis mit Gangrän des Fusses". Archiv für Klinische Chirurgie. 23: 202–226.
  17. S2CID 31731903
    .
  18. ISBN 9789810873646
    .
  19. ^ Frialde M (December 10, 2015). "Duterte: I may not last 6 years in office". The Philippine Star. Retrieved December 17, 2015.

Further reading

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